Publisher: Hindawi   (Total: 343 journals)

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Showing 1 - 200 of 343 Journals sorted alphabetically
Abstract and Applied Analysis     Open Access   (Followers: 3, SJR: 0.343, CiteScore: 1)
Active and Passive Electronic Components     Open Access   (Followers: 8, SJR: 0.136, CiteScore: 0)
Advances in Acoustics and Vibration     Open Access   (Followers: 51, SJR: 0.147, CiteScore: 0)
Advances in Aerospace Engineering     Open Access   (Followers: 67)
Advances in Agriculture     Open Access   (Followers: 12)
Advances in Artificial Intelligence     Open Access   (Followers: 22)
Advances in Astronomy     Open Access   (Followers: 51, SJR: 0.257, CiteScore: 1)
Advances in Bioinformatics     Open Access   (Followers: 20, SJR: 0.565, CiteScore: 2)
Advances in Biology     Open Access   (Followers: 11)
Advances in Chemistry     Open Access   (Followers: 35)
Advances in Civil Engineering     Open Access   (Followers: 51, SJR: 0.539, CiteScore: 1)
Advances in Computer Engineering     Open Access   (Followers: 8)
Advances in Condensed Matter Physics     Open Access   (Followers: 11, SJR: 0.315, CiteScore: 1)
Advances in Decision Sciences     Open Access   (Followers: 4, SJR: 0.303, CiteScore: 1)
Advances in Electrical Engineering     Open Access   (Followers: 52)
Advances in Electronics     Open Access   (Followers: 101)
Advances in Emergency Medicine     Open Access   (Followers: 16)
Advances in Endocrinology     Open Access   (Followers: 6)
Advances in Environmental Chemistry     Open Access   (Followers: 10)
Advances in Epidemiology     Open Access   (Followers: 9)
Advances in Fuzzy Systems     Open Access   (Followers: 5, SJR: 0.161, CiteScore: 1)
Advances in Geology     Open Access   (Followers: 19)
Advances in Geriatrics     Open Access   (Followers: 6)
Advances in Hematology     Open Access   (Followers: 13, SJR: 0.661, CiteScore: 2)
Advances in Hepatology     Open Access   (Followers: 3)
Advances in High Energy Physics     Open Access   (Followers: 26, SJR: 0.866, CiteScore: 2)
Advances in Human-Computer Interaction     Open Access   (Followers: 21, SJR: 0.186, CiteScore: 1)
Advances in Materials Science and Engineering     Open Access   (Followers: 31, SJR: 0.315, CiteScore: 1)
Advances in Mathematical Physics     Open Access   (Followers: 9, SJR: 0.218, CiteScore: 1)
Advances in Medicine     Open Access   (Followers: 3)
Advances in Meteorology     Open Access   (Followers: 24, SJR: 0.48, CiteScore: 1)
Advances in Multimedia     Open Access   (Followers: 1, SJR: 0.173, CiteScore: 1)
Advances in Nonlinear Optics     Open Access   (Followers: 7)
Advances in Numerical Analysis     Open Access   (Followers: 9)
Advances in Nursing     Open Access   (Followers: 37)
Advances in Operations Research     Open Access   (Followers: 13, SJR: 0.205, CiteScore: 1)
Advances in Optical Technologies     Open Access   (Followers: 4, SJR: 0.214, CiteScore: 1)
Advances in Optics     Open Access   (Followers: 9)
Advances in OptoElectronics     Open Access   (Followers: 6, SJR: 0.141, CiteScore: 0)
Advances in Orthopedics     Open Access   (Followers: 11, SJR: 0.922, CiteScore: 2)
Advances in Pharmacological and Pharmaceutical Sciences     Open Access   (Followers: 9, SJR: 0.591, CiteScore: 2)
Advances in Physical Chemistry     Open Access   (Followers: 13, SJR: 0.179, CiteScore: 1)
Advances in Polymer Technology     Open Access   (Followers: 14, SJR: 0.299, CiteScore: 1)
Advances in Power Electronics     Open Access   (Followers: 44, SJR: 0.184, CiteScore: 0)
Advances in Preventive Medicine     Open Access   (Followers: 6)
Advances in Public Health     Open Access   (Followers: 28)
Advances in Regenerative Medicine     Open Access   (Followers: 4)
Advances in Software Engineering     Open Access   (Followers: 11)
Advances in Statistics     Open Access   (Followers: 10)
Advances in Toxicology     Open Access   (Followers: 4)
Advances in Tribology     Open Access   (Followers: 15, SJR: 0.265, CiteScore: 1)
Advances in Urology     Open Access   (Followers: 13, SJR: 0.51, CiteScore: 1)
Advances in Virology     Open Access   (Followers: 8, SJR: 0.838, CiteScore: 2)
AIDS Research and Treatment     Open Access   (Followers: 2, SJR: 0.758, CiteScore: 2)
Analytical Cellular Pathology     Open Access   (Followers: 3, SJR: 0.886, CiteScore: 2)
Anatomy Research Intl.     Open Access   (Followers: 4)
Anemia     Open Access   (Followers: 6, SJR: 0.669, CiteScore: 2)
Anesthesiology Research and Practice     Open Access   (Followers: 15, SJR: 0.501, CiteScore: 1)
Applied and Environmental Soil Science     Open Access   (Followers: 20, SJR: 0.451, CiteScore: 1)
Applied Bionics and Biomechanics     Open Access   (Followers: 7, SJR: 0.288, CiteScore: 1)
Applied Computational Intelligence and Soft Computing     Open Access   (Followers: 15)
Archaea     Open Access   (Followers: 4, SJR: 0.852, CiteScore: 2)
Autism Research and Treatment     Open Access   (Followers: 36)
Autoimmune Diseases     Open Access   (Followers: 3, SJR: 0.805, CiteScore: 2)
Behavioural Neurology     Open Access   (Followers: 9, SJR: 0.786, CiteScore: 2)
Biochemistry Research Intl.     Open Access   (Followers: 6, SJR: 0.437, CiteScore: 2)
Bioinorganic Chemistry and Applications     Open Access   (Followers: 11, SJR: 0.419, CiteScore: 2)
BioMed Research Intl.     Open Access   (Followers: 5, SJR: 0.935, CiteScore: 3)
Biotechnology Research Intl.     Open Access   (Followers: 1)
Bone Marrow Research     Open Access   (Followers: 2, SJR: 0.531, CiteScore: 1)
Canadian J. of Gastroenterology & Hepatology     Open Access   (Followers: 4, SJR: 0.867, CiteScore: 1)
Canadian J. of Infectious Diseases and Medical Microbiology     Open Access   (Followers: 8, SJR: 0.548, CiteScore: 1)
Canadian Respiratory J.     Open Access   (Followers: 3, SJR: 0.474, CiteScore: 1)
Cardiology Research and Practice     Open Access   (Followers: 11, SJR: 1.237, CiteScore: 4)
Cardiovascular Therapeutics     Open Access   (Followers: 2, SJR: 1.075, CiteScore: 2)
Case Reports in Anesthesiology     Open Access   (Followers: 11)
Case Reports in Cardiology     Open Access   (Followers: 8, SJR: 0.219, CiteScore: 0)
Case Reports in Critical Care     Open Access   (Followers: 12)
Case Reports in Dentistry     Open Access   (Followers: 8, SJR: 0.229, CiteScore: 0)
Case Reports in Dermatological Medicine     Open Access   (Followers: 2)
Case Reports in Emergency Medicine     Open Access   (Followers: 19)
Case Reports in Endocrinology     Open Access   (Followers: 2, SJR: 0.209, CiteScore: 1)
Case Reports in Gastrointestinal Medicine     Open Access   (Followers: 3)
Case Reports in Genetics     Open Access   (Followers: 2)
Case Reports in Hematology     Open Access   (Followers: 9)
Case Reports in Hepatology     Open Access   (Followers: 2)
Case Reports in Immunology     Open Access   (Followers: 6)
Case Reports in Infectious Diseases     Open Access   (Followers: 6)
Case Reports in Medicine     Open Access   (Followers: 3)
Case Reports in Nephrology     Open Access   (Followers: 5)
Case Reports in Neurological Medicine     Open Access   (Followers: 1)
Case Reports in Obstetrics and Gynecology     Open Access   (Followers: 11)
Case Reports in Oncological Medicine     Open Access   (Followers: 2, SJR: 0.204, CiteScore: 1)
Case Reports in Ophthalmological Medicine     Open Access   (Followers: 3)
Case Reports in Orthopedics     Open Access   (Followers: 6)
Case Reports in Otolaryngology     Open Access   (Followers: 7)
Case Reports in Pathology     Open Access   (Followers: 7)
Case Reports in Pediatrics     Open Access   (Followers: 8)
Case Reports in Psychiatry     Open Access   (Followers: 18)
Case Reports in Pulmonology     Open Access   (Followers: 3)
Case Reports in Radiology     Open Access   (Followers: 12)
Case Reports in Rheumatology     Open Access   (Followers: 10)
Case Reports in Surgery     Open Access   (Followers: 12)
Case Reports in Transplantation     Open Access  
Case Reports in Urology     Open Access   (Followers: 12)
Case Reports in Vascular Medicine     Open Access  
Case Reports in Veterinary Medicine     Open Access   (Followers: 5)
Child Development Research     Open Access   (Followers: 21, SJR: 0.144, CiteScore: 0)
Chinese J. of Engineering     Open Access   (Followers: 2, SJR: 0.114, CiteScore: 0)
Chinese J. of Mathematics     Open Access  
Chromatography Research Intl.     Open Access   (Followers: 5)
Complexity     Hybrid Journal   (Followers: 8, SJR: 0.531, CiteScore: 2)
Computational and Mathematical Methods in Medicine     Open Access   (Followers: 2, SJR: 0.403, CiteScore: 1)
Computational Biology J.     Open Access   (Followers: 7)
Computational Intelligence and Neuroscience     Open Access   (Followers: 15, SJR: 0.326, CiteScore: 1)
Concepts in Magnetic Resonance Part A     Open Access   (Followers: 1, SJR: 0.354, CiteScore: 1)
Concepts in Magnetic Resonance Part B, Magnetic Resonance Engineering     Open Access   (Followers: 1, SJR: 0.26, CiteScore: 1)
Conference Papers in Science     Open Access   (Followers: 2)
Contrast Media & Molecular Imaging     Open Access   (Followers: 2, SJR: 0.842, CiteScore: 3)
Critical Care Research and Practice     Open Access   (Followers: 13, SJR: 0.499, CiteScore: 1)
Current Gerontology and Geriatrics Research     Open Access   (Followers: 10, SJR: 0.512, CiteScore: 2)
Depression Research and Treatment     Open Access   (Followers: 19, SJR: 0.816, CiteScore: 2)
Dermatology Research and Practice     Open Access   (Followers: 4, SJR: 0.806, CiteScore: 2)
Diagnostic and Therapeutic Endoscopy     Open Access   (SJR: 0.201, CiteScore: 1)
Discrete Dynamics in Nature and Society     Open Access   (Followers: 6, SJR: 0.279, CiteScore: 1)
Disease Markers     Open Access   (Followers: 1, SJR: 0.9, CiteScore: 2)
Economics Research Intl.     Open Access   (Followers: 1)
Education Research Intl.     Open Access   (Followers: 19)
Emergency Medicine Intl.     Open Access   (Followers: 9, SJR: 0.298, CiteScore: 1)
Enzyme Research     Open Access   (Followers: 5, SJR: 0.653, CiteScore: 3)
Evidence-based Complementary and Alternative Medicine     Open Access   (Followers: 30, SJR: 0.683, CiteScore: 2)
Game Theory     Open Access   (Followers: 1)
Gastroenterology Research and Practice     Open Access   (Followers: 1, SJR: 0.768, CiteScore: 2)
Genetics Research Intl.     Open Access   (Followers: 1, SJR: 0.61, CiteScore: 2)
Geofluids     Open Access   (Followers: 5, SJR: 0.952, CiteScore: 2)
Hepatitis Research and Treatment     Open Access   (Followers: 6, SJR: 0.389, CiteScore: 2)
Heteroatom Chemistry     Open Access   (Followers: 3, SJR: 0.333, CiteScore: 1)
HPB Surgery     Open Access   (Followers: 9, SJR: 0.824, CiteScore: 2)
Infectious Diseases in Obstetrics and Gynecology     Open Access   (Followers: 5, SJR: 1.27, CiteScore: 2)
Interdisciplinary Perspectives on Infectious Diseases     Open Access   (Followers: 1, SJR: 0.627, CiteScore: 2)
Intl. J. of Aerospace Engineering     Open Access   (Followers: 81, SJR: 0.232, CiteScore: 1)
Intl. J. of Agronomy     Open Access   (Followers: 6, SJR: 0.311, CiteScore: 1)
Intl. J. of Alzheimer's Disease     Open Access   (Followers: 12, SJR: 0.787, CiteScore: 3)
Intl. J. of Analytical Chemistry     Open Access   (Followers: 22, SJR: 0.285, CiteScore: 1)
Intl. J. of Antennas and Propagation     Open Access   (Followers: 13, SJR: 0.233, CiteScore: 1)
Intl. J. of Atmospheric Sciences     Open Access   (Followers: 21)
Intl. J. of Biodiversity     Open Access   (Followers: 3)
Intl. J. of Biomaterials     Open Access   (Followers: 5, SJR: 0.511, CiteScore: 2)
Intl. J. of Biomedical Imaging     Open Access   (Followers: 3, SJR: 0.501, CiteScore: 2)
Intl. J. of Breast Cancer     Open Access   (Followers: 14, SJR: 1.025, CiteScore: 2)
Intl. J. of Cell Biology     Open Access   (Followers: 4, SJR: 1.887, CiteScore: 4)
Intl. J. of Chemical Engineering     Open Access   (Followers: 8, SJR: 0.327, CiteScore: 1)
Intl. J. of Chronic Diseases     Open Access   (Followers: 1)
Intl. J. of Combinatorics     Open Access   (Followers: 1)
Intl. J. of Computer Games Technology     Open Access   (Followers: 10, SJR: 0.287, CiteScore: 2)
Intl. J. of Corrosion     Open Access   (Followers: 11, SJR: 0.194, CiteScore: 1)
Intl. J. of Dentistry     Open Access   (Followers: 8, SJR: 0.649, CiteScore: 2)
Intl. J. of Differential Equations     Open Access   (Followers: 8, SJR: 0.191, CiteScore: 0)
Intl. J. of Digital Multimedia Broadcasting     Open Access   (Followers: 5, SJR: 0.296, CiteScore: 2)
Intl. J. of Electrochemistry     Open Access   (Followers: 10)
Intl. J. of Endocrinology     Open Access   (Followers: 4, SJR: 1.012, CiteScore: 3)
Intl. J. of Engineering Mathematics     Open Access   (Followers: 7)
Intl. J. of Food Science     Open Access   (Followers: 5, SJR: 0.44, CiteScore: 2)
Intl. J. of Forestry Research     Open Access   (Followers: 3, SJR: 0.373, CiteScore: 1)
Intl. J. of Genomics     Open Access   (Followers: 2, SJR: 0.868, CiteScore: 3)
Intl. J. of Geophysics     Open Access   (Followers: 5, SJR: 0.182, CiteScore: 1)
Intl. J. of Hepatology     Open Access   (Followers: 4, SJR: 0.874, CiteScore: 2)
Intl. J. of Hypertension     Open Access   (Followers: 8, SJR: 0.578, CiteScore: 1)
Intl. J. of Inflammation     Open Access   (SJR: 1.264, CiteScore: 3)
Intl. J. of Inorganic Chemistry     Open Access   (Followers: 4)
Intl. J. of Manufacturing Engineering     Open Access   (Followers: 2)
Intl. J. of Mathematics and Mathematical Sciences     Open Access   (Followers: 3, SJR: 0.177, CiteScore: 0)
Intl. J. of Medicinal Chemistry     Open Access   (Followers: 6, SJR: 0.31, CiteScore: 1)
Intl. J. of Metals     Open Access   (Followers: 7)
Intl. J. of Microbiology     Open Access   (Followers: 8, SJR: 0.662, CiteScore: 2)
Intl. J. of Microwave Science and Technology     Open Access   (Followers: 6, SJR: 0.136, CiteScore: 1)
Intl. J. of Navigation and Observation     Open Access   (Followers: 20, SJR: 0.267, CiteScore: 2)
Intl. J. of Nephrology     Open Access   (Followers: 2, SJR: 0.697, CiteScore: 1)
Intl. J. of Oceanography     Open Access   (Followers: 8)
Intl. J. of Optics     Open Access   (Followers: 10, SJR: 0.231, CiteScore: 1)
Intl. J. of Otolaryngology     Open Access   (Followers: 3)
Intl. J. of Partial Differential Equations     Open Access   (Followers: 2)
Intl. J. of Pediatrics     Open Access   (Followers: 6)
Intl. J. of Peptides     Open Access   (Followers: 2, SJR: 0.46, CiteScore: 1)
Intl. J. of Photoenergy     Open Access   (Followers: 3, SJR: 0.341, CiteScore: 1)
Intl. J. of Plant Genomics     Open Access   (Followers: 4, SJR: 0.583, CiteScore: 1)
Intl. J. of Polymer Science     Open Access   (Followers: 28, SJR: 0.298, CiteScore: 1)
Intl. J. of Population Research     Open Access   (Followers: 4)
Intl. J. of Quality, Statistics, and Reliability     Open Access   (Followers: 17)
Intl. J. of Reconfigurable Computing     Open Access   (SJR: 0.123, CiteScore: 1)
Intl. J. of Reproductive Medicine     Open Access   (Followers: 6)
Intl. J. of Rheumatology     Open Access   (Followers: 4, SJR: 0.645, CiteScore: 2)
Intl. J. of Rotating Machinery     Open Access   (Followers: 2, SJR: 0.193, CiteScore: 1)
Intl. J. of Spectroscopy     Open Access   (Followers: 8)
Intl. J. of Stochastic Analysis     Open Access   (Followers: 3, SJR: 0.279, CiteScore: 1)
Intl. J. of Surgical Oncology     Open Access   (Followers: 1, SJR: 0.573, CiteScore: 2)
Intl. J. of Telemedicine and Applications     Open Access   (Followers: 7, SJR: 0.403, CiteScore: 2)
Intl. J. of Vascular Medicine     Open Access   (SJR: 0.782, CiteScore: 2)
Intl. J. of Zoology     Open Access   (Followers: 2, SJR: 0.209, CiteScore: 1)
Intl. Scholarly Research Notices     Open Access   (Followers: 230)

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Similar Journals
Journal Cover
Case Reports in Rheumatology
Number of Followers: 10  

  This is an Open Access Journal Open Access journal
ISSN (Print) 2090-6889 - ISSN (Online) 2090-6897
Published by Hindawi Homepage  [343 journals]
  • Hypertrophic Osteoarthropathy: A Secondary Manifestation of Malignant
           Melanoma

    • Abstract: Background. Hypertrophic osteoarthropathy (HOA) is a rare finding in the setting of metastatic melanoma. A majority of cases of secondary HOA involve lung malignancies. Evaluation of presenting symptoms such as polyarthralgia and clubbing followed by review of imaging studies are diagnostic steps for HOA. Case Presentation. We present a 60-year-old female with a history of metastatic melanoma who presented with bilateral and symmetric polyarthralgia and clubbing. A plain film radiograph demonstrated periosteal thickening involving the metacarpals and proximal phalanges as well as the distal radius and ulna, consistent with HOA. The patient was treated with nonsteroidal anti-inflammatory agents for supported care. Conclusion. HOA may be a secondary manifestation of metastatic melanoma. Recognition and supportive care of this condition may lead to improved quality of life for patients.
      PubDate: Mon, 18 Jan 2021 17:50:01 +000
       
  • A Case of Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic
           Antibodies-Associated Vasculitis Overlap Syndrome

    • Abstract: An overlap of systemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibodies- (ANCA-) associated vasculitis (AAV) is extremely rare: approximately 40 cases have been reported to date. A literature review indicates that they are more common in women in their forties, and simultaneous onset has been reported in 69% of cases. In addition, both lupus nephritis and ANCA-associated glomerulonephritis were observed on renal biopsy. This report presents the case of a 35-year-old woman with an 8-month history of polyarthralgia who was admitted to our hospital. She was diagnosed with SLE due to typical clinical presentation of the disease: polyarthritis, lymphocytopenia, hypocomplementemia, presence of antinuclear and anti-dsDNA antibodies, and proteinuria. However, purpura were scattered, and the titer of antimyeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA) was high. A skin biopsy revealed leukocytoclastic vasculitis that involved poor immune complex deposition. A renal biopsy showed necrotizing glomerulonephritis with cellular and fibrocellular crescent formation that involved deposition of IgM and C3c only in the mesangial area and the peripheral capillaries. Additionally, no electron-dense deposits were observed under electron microscopy. These pathological findings were consistent with AAV rather than with SLE. Therefore, we finally diagnosed the patient with both SLE and microscopic polyangiitis. After treatment with methylprednisolone and intravenous cyclophosphamide pulse therapies, renal function improved and MPO-ANCA levels decreased. In cases of suspected overlap between SLE and AAV, appropriate diagnosis and treatment are important.
      PubDate: Thu, 07 Jan 2021 08:35:00 +000
       
  • Systemic Lupus Erythematosus Presenting as Acute Lupus Pneumonitis during
           Pregnancy

    • Abstract: Introduction. This is a case of new-onset systemic lupus erythematosus (SLE) manifesting as acute pneumonitis during pregnancy. No prior reports have documented pneumonitis as the presenting manifestation of SLE in pregnant women. Case Presentation. A 23-year-old pregnant female presented with high-grade fever, cough, arthralgias, and respiratory failure. Infectious workup was negative. She was positive for ANA, anti-dsDNA, anti-SSA, hypocomplementemia, and pulmonary infiltrates, supporting the diagnosis of SLE and pneumonitis. The patient received methylprednisolone achieving adequate clinical and serological response. Conclusion. When SLE patients present with fever, cough, and respiratory failure, pulmonary infiltrates should raise the suspicion of pneumonitis in the absence of infection and hemorrhage. Even though acute lupus pneumonitis (ALP) is rare and seen only in 2% of SLE patients, a high index of suspicion aids in prompt diagnosis of this life-threatening condition. Also, positive anti-SSA antibodies may be associated with lupus pneumonitis.
      PubDate: Mon, 21 Dec 2020 11:50:00 +000
       
  • Rheumatoid Arthritis and Autoimmune Hemolytic Anemia as First
           Manifestation of Rhupus

    • Abstract: “Rhupus” syndrome is a rare condition that describes the coexistence of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA), which prevalence among patients with SLE varies from 0.01% to 9.7%. There are few reported cases of the association between autoimmune hemolytic anemia and rheumatoid arthritis with systemic lupus erythematosus (rhupus). We report a rare case of rhupus in a 29-year-old woman, associated with autoimmune hemolytic anemia.
      PubDate: Wed, 09 Dec 2020 18:05:00 +000
       
  • Complicated Rheumatoid Nodules in Lung

    • Abstract: A 65-year-old nonsmoker lady carrying a diagnosis of seropositive erosive rheumatoid arthritis for nine years presented with acute shortness of breath, following a spontaneous pneumothorax while on combination therapy with methotrexate, leflunomide, and tocilizumab. Imaging studies revealed multiple cavitory lung nodules, and a transbronchial lung biopsy favoured a diagnosis of rheumatoid lung nodules. Her initial pathological samples were negative for any infectious cause. A follow-up computerized tomography scan (CT scan) confirmed enlargement of lung nodules with a positive antibody test for aspergillosis which needed antifungal therapy, and currently, her arthritis is managed well with rituximab therapy, sulfasalazine, and hydroxychloroquine.
      PubDate: Thu, 03 Dec 2020 09:20:00 +000
       
  • IgA Vasculitis Nephritis: A Case Series and Comparison of Treatment
           Guidelines

    • Abstract: Immunoglobulin A (IgA) vasculitis nephritis (IgAVN) and IgA nephropathy (IgAN) share many pathological parallels and are viewed as related diseases by many groups. Current treatment guidelines remain vague, controversial, and without consensus, especially regarding the role of immunosuppressive medications. We present five cases of IgAVN encountered at our tertiary care center between 2016 and 2020, which were treated with different immunosuppression regimens. Infection was the leading cause of death in this series. These cases provide evidence that IgAVN should be distinguished from IgAN on a spectrum of IgA-associated glomerulonephritis. The outcomes presented herein suggest that the morbidity and systematic involvement IgAVN is greater than previously believed and that these substantial risks should be reflected in contemporary treatment guidelines.
      PubDate: Wed, 02 Dec 2020 08:35:00 +000
       
  • Sudden Cardiac Death due to Coronary Artery Vasculitis in a Patient with
           Relapsing Polychondritis

    • Abstract: Relapsing polychondritis (RP) is a systemic autoimmune disease characterized by relapsing and remitting inflammation of the cartilaginous structures of the ears, nose, tracheobronchial tree, and joints. Diagnosis is challenging due to the heterogeneity of clinical manifestations, the relapsing and remitting nature of the disease, the presence of coexistent diseases in at least one-third of patients, and the lack of a diagnostic blood test. Although RP-associated cardiac disease is the second most common cause of death behind tracheobronchial complications, coronary artery vasculitis is rare. This report describes a case of sudden cardiac death due to vasculitis affecting the coronary arteries in a patient with RP. The pathologic findings included obliterative coronary arteritis with plasma cells and storiform fibrosis, features suggesting that IgG4-related disease (IgG4-RD) may have contributed to the patient’s cardiac disease. The literature on vasculitis and cardiac disease in RP and the possible role of IgG4-RD in this setting is also reviewed. The primary take-home message from this case report is the importance of frequent screening for cardiac disease, regardless of symptoms, in patients with RP. In addition, considering the diagnosis of IgG4-RD in some cases thought to be RP may also be warranted.
      PubDate: Tue, 17 Nov 2020 07:35:01 +000
       
  • Hematologic Chaos in Lupus Flare: A Case of Fulminant and Simultaneous
           Antiphospholipid, Anti-ADAMTS13, and Red Blood Cell Autoantibodies

    • Abstract: Systemic lupus erythematosus may present with several distinct autoimmune phenomena simultaneously. We report a patient presenting with three serious hematologic disorders: thrombotic thrombocytopenic purpura, catastrophic antiphospholipid syndrome, and warm-type IgG red cell autoantibodies. The case is an example of the complex clinical nature of lupus and the importance of accurately identifying individual complications in order to optimize management.
      PubDate: Mon, 16 Nov 2020 06:50:01 +000
       
  • Case Series of Chronic Inflammatory Rheumatic Disease Patients Infected by
           Coronavirus Disease 2019 (COVID-19)

    • Abstract: Coronavirus disease 2019 (COVID-19) is a viral infection that appeared in December 2019. The risk of infection seems to be increased in chronic inflammatory rheumatic diseases due to both immune disturbances related to the disease and treatment. In this case report, we describe the clinical features of 5 rheumatic immune disease patients with the concomitant presence of COVID-19. Among these patients, 3 had rheumatoid arthritis and 2 had systemic lupus erythematosus. Patients’ age ranged between 38 and 63 years. Only one patient (SLE) had a severe subtype of COVID-19. All the patients were cured of COVID-19 and were subsequently discharged.
      PubDate: Mon, 16 Nov 2020 06:20:01 +000
       
  • Amnesia in a Patient with Rheumatoid Arthritis: A Case of Granulomatosis
           with Polyangiitis

    • Abstract: A 56-year-old male with a history of seropositive rheumatoid arthritis complained of polyarthritis and forgetfulness. The initial CT scan of the head without contrast was negative for acute pathologies. However, as he continued to decline despite being on oral prednisone, an MRI of the head was ordered and revealed a subtle small region of acute infarction in the left mammillary body. He was diagnosed with granulomatosis with polyangiitis in light of his positive C-ANCA antibodies, PR3 antibody, and a kidney biopsy, which showed focal necrotizing pauci-immune crescentic glomerulonephritis. Despite undergoing steroid pulse therapy, the patient developed diffuse alveolar damage which finally responded to rituximab infusion.
      PubDate: Thu, 29 Oct 2020 13:35:01 +000
       
  • Complete Recovery from COVID-19 Bilateral Pneumonia in an Immunosuppressed
           Man with Immune-Mediated Necrotizing Myopathy

    • Abstract: Immune-mediated necrotizing myopathy (IMNM) is a rare form of idiopathic immune myopathy (IIM) that requires immunotherapies, including immunosuppressive medications, if severe. There is a paucity of data regarding outcomes of patients with immune-mediated polymyositis who continue immunosuppressive medications during the COVID-19 pandemic. This is the first reported case of COVID-19 in a patient with IMNM. Despite being on two immunotherapies, having risk factors, and having radiographic abnormalities on chest X-ray, the patient had an unremarkable COVID-19 course. He was discharged from the emergency department with a 7-day course of azithromycin and quickly resumed his immunotherapies, but he experienced a flare in his myositis. The 14-week follow-up computed tomography (CT) was negative for residual pneumonitis or fibrosis. More data are needed regarding management and prognosis of patients with connective tissue diseases who become infected with SARS-CoV-2.
      PubDate: Tue, 27 Oct 2020 13:05:01 +000
       
  • Elevated Cardiac Troponin T in Patients with Lupus Myositis Presenting
           with Noncardiac Chest Pain

    • Abstract: Patients with systemic lupus erythematosus (SLE) presenting with chest pain pose a unique diagnostic challenge, with causes ranging from cardiopulmonary disease to esophageal disorders and musculoskeletal chest wall pain. The most common biomarkers for myocardial injury are cardiac troponin T and I (cTnT and cTnI) due to their high sensitivity for the early detection of myocardial infarction. In the idiopathic inflammatory myopathies, cTnT is commonly elevated, and this reflects skeletal muscle breakdown rather than myocardial damage. Similar observations have not been reported in SLE myositis to date. We present two cases of patients with SLE and associated myositis who presented with chest pain and elevated cTnT. Both patients had a normal cTnI, transthoracic echocardiogram, and cardiac magnetic resonance imaging, likely indicating noncardiac chest pain. Clinicians should be aware that the specificity of cTnT might be lower in SLE myositis and that cTnI elevation may be more specific in detecting myocardial insult.
      PubDate: Fri, 23 Oct 2020 14:35:01 +000
       
  • Lupus and NMOSD: The Blending of Humoral Autoimmunity

    • Abstract: Systemic lupus erythematous (SLE) is a chronic autoimmune disease that can target any organ of the body. It may coexist with other autoimmune neurologic conditions such as neuromyelitis optica spectrum disorder (NMOSD). NMOSD, previously known as Devic’s disease, is an autoimmune inflammatory disorder of the central nervous system (CNS) that targets the spinal cord, optic nerves, and certain brain regions. Most current evidence suggests that NMOSD is best described as a CNS astrocytopathy. While these diseases share several immunosuppressive treatment options, timely diagnosis of NMOSD is critical as patients may benefit from treatment tailored specifically to NMOSD as opposed to SLE. Steroids, plasmapheresis, intravenous immunoglobulin, cyclophosphamide, azathioprine, mycophenolate mofetil, and rituximab are used to treat both SLE and NMOSD. However, there are several new therapies (inebilizumab, eculizumab, and satralizumab) recently approved specifically for use in NMOSD. In this case series, we report on three patients with coexisting SLE and NMOSD. We describe a 31-year-old woman who suffered an NMOSD flare after 11 years of clinical remission in the context of receiving an influenza vaccination; her SLE remained quiescent on hydroxychloroquine. Next, we describe a 52-year-old woman with emergence of neurologically devastating seropositive NMOSD in the setting of active treatment for SLE with intravenous cyclophosphamide, oral steroids, and hydroxychloroquine. Last, we describe a 48-year-old woman with emergence of seronegative NMOSD in the setting of SLE that was well-controlled on azathioprine and hydroxychloroquine. These cases illustrate the importance of accurate diagnosis and targeted treatment of NMOSD when coexisting with SLE.
      PubDate: Thu, 15 Oct 2020 12:20:00 +000
       
  • Coexistence of Axial Spondyloarthritis and Idiopathic Inflammatory
           Myopathy

    • Abstract: Both axial spondyloarthritis (axSpA) and idiopathic inflammatory myopathy (IIM) are infrequent, and their coexistence is even rarer; there are a few reported cases in the literature. The aim of this study was to assess their association and clinical and laboratory features in our patients. The clinical data of patients with axSpA and IIM diagnosed in China-Japan Friendship Hospital from July 2015 to February 2019 were retrospectively analyzed. This study included 7 patients with axSpA who met the IIM criteria, including 3 males and 4 females. The age of onset was 16 to 39 years. Four patients were HLA-B27 positive, and three were negative. All patients were first diagnosed as axSpA, and then IIM was detected after 0.5–20 years (mean ± SD, 9.9 ± 5.0 years). After being diagnosed to have axSpA and IIM, those patients were given prednisone and immunosuppressant drugs, and their symptoms gradually improved. Our study provides further evidence of the coexistence of IIM with axSpA. In patients with axSpA who have skin rash, interstitial lung disease (ILD), myalgia, or muscle weakness, we should suspect that they may have IIM.
      PubDate: Wed, 07 Oct 2020 19:05:00 +000
       
  • Bilateral Retrobulbar Optic Neuritis as the First Manifestation of
           Neuro-Behçet Disease

    • Abstract: Background. Behçet disease (BD) is a polygenic and chronic autoinflammatory multisystem vasculitis. Acute optic neuritis has been rarely reported in patients with BD, especially in children. Case Presentation. We reported an 8-year-old girl with a sudden visual loss and color vision impairment. The patient had a history of recurrent oral aphthous ulcers, genital ulcers, and chronic abdominal pain. On ophthalmic examination, anterior and posterior chambers and funduscopy of both eyes were normal. The results of laboratory tests for infectious and rheumatic diseases were normal. Brain magnetic resonance imaging and the result of cerebrospinal fluid analysis for oligoclonal bands and auto-antibodies were also normal. Pathergy skin test and human leukocyte antigens (HLA) B5 and HLA-B51 were positive. The patient was recognized as a case of BD-related bilateral retrobulbar optic neuritis and was treated by corticosteroids, azathioprine, colchicine, and infliximab. Conclusion. Retrobulbar optic neuritis may be the first manifestation of neuro-BD.
      PubDate: Tue, 06 Oct 2020 12:05:00 +000
       
  • Lupus Never Fails to Deceive US: A Case of Rowell’s Syndrome

    • Abstract: Background. Rowell’s syndrome is comprised of the presentation of erythema multiforme- (EM-) like lesions in association with lupus erythematosus (LE), along with serologies of speckled antinuclear antibodies (ANAs), positive rheumatoid factor (RF), positive anti-La/anti-Ro, and the clinical finding of chilblains. As per the redefined criteria by Zeitouni et al., three major criteria in addition to at least 1 minor criterion are necessary for diagnosis. Case Presentation. A 20-year-old male presented with a one-week history of worsening nonpruritic erythematous maculopapular skin rash (resembling EM) which appeared on the face and subsequently spread to the trunk, arms, legs, palms, and soles. There was no mucosal involvement. At the onset of rash, the patient reported headaches, associated with photosensitivity and intermittent fevers. Workup for viral meningitis yielded negative results. Laboratory investigation revealed mild anemia, elevated inflammatory markers, a positive ANA with speckled pattern, a positive anti-Ro/SSA, anti-La/SSB antibodies, and a positive rheumatoid factor (RF). Lupus anticoagulant antibody was positive along with a low-positive anticardiolipin IgM antibody and a negative beta-2 glycoprotein antibody. Anti-dsDNA, anti-Smith, anti-Jo-1, anti-centromere, and anti-Scl-70 antibodies were negative. Hepatitis serologies, herpes simplex virus 1 and 2, mycoplasma, Epstein–Barr virus, HIV, and parvovirus B19 were negative. Left arm skin biopsy demonstrated vacuolar interface dermatitis and positive colloidal iron stain suggestive of dermal mucin deposition, favoring the diagnosis of cutaneous collagen vascular disease. Cutaneous lesions improved with administration of oral prednisolone. Conclusion. Rowell’s syndrome should be considered in patients who present with cutaneous LE and lesions resembling EM. Further serological markers should be pursued in the absence of obvious EM-precipitating factors.
      PubDate: Wed, 23 Sep 2020 01:20:00 +000
       
  • Primary Sjögren’s Syndrome Accompanied by Clinical Features of
           TAFRO Syndrome

    • Abstract: Sjögren’s syndrome (SS) is associated with not only sicca symptoms but also various symptoms caused by extraglandular manifestation. The pathophysiology and comorbidities of TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly), which is thought to be a variant of multicentric Castleman’s disease, are not fully understood, and there are few data on the effectiveness of treatments. We report a patient of SS with TAFRO syndrome-like clinical features. A 52-year-old woman was admitted to our hospital because of abdominal distension. Laboratory data showed thrombocytopenia, and image findings showed massive ascites without evidence of malignant disease as confirmed by cytology. She was diagnosed with SS based on dysfunction of salivary secretion and positivity for anti-Ro/SS-A and La/SS-B antibodies, accompanied by clinical features of TAFRO syndrome based on the presence of anasarca and thrombocytopenia. High-dose corticosteroid for inflammation, anasarca, and thrombocytopenia was not effective. Cyclosporine was administered next, but anasarca and thrombocytopenia did not immediately improve until tolvaptan and eltrombopag were added. Although tolvaptan and eltrombopag were used for only a few months, the patient maintained a good condition with cyclosporine and low-dose prednisolone. In SS patients, activation of antigen-specific T lymphocytes is thought to be an important trigger that accelerates the immune response and is followed by hypercytokinemia. Therefore, using cyclosporine to suppress the activity of T lymphocytes is a reasonable treatment for SS accompanied with TAFRO syndrome-like pathophysiology. It might also be useful to administer tolvaptan or eltrombopag before the effects of immunosuppressants appear. If refractory inflammation with anasarca, thrombocytopenia, or lymphadenopathy is observed in an SS patient, complications with TAFRO syndrome-like pathophysiology should be considered.
      PubDate: Thu, 17 Sep 2020 14:05:00 +000
       
  • Development of Arthritis as the Initial Involvement in Adult-Onset
           Cutaneous Polyarteritis Nodosa: Two Cases and Literature Review

    • Abstract: Articular symptoms are commonly present in polyarteritis nodosa (PAN). Meanwhile, they may occur as the initial and main involvement of PAN, raising a concern of a delay in a definitive diagnosis of disease unless the histological evidence is obtained. Herein, we report two cases of cutaneous PAN (c-PAN) in which arthritis developed as the initial clinical episode of disease and we argued, through a review of the literature, the clinical feature of patients presenting with arthritis as the initial symptom of PAN. To our knowledge, only six cases have been reported in the English literature, and all six patients were categorized as having c-PAN. Of those patients, four had arthritis with indicating destructive changes. A median of 9 years elapsed prior to the induction of immunosuppressive treatment despite the fact that the other reviewed cases as well as our two patients, who received treatment significantly earlier, showed no evidence of joint destruction. Taken together, this report suggests that the early induction of therapy based on the definitive diagnosis of PAN may be required for preventing joint disruption even though it is not easy to make a diagnosis of PAN unless biopsied tissue can provide the evidence of necrotizing vasculitis.
      PubDate: Wed, 16 Sep 2020 13:20:00 +000
       
  • Rapidly Progressive Pulmonary Apical Fibrosis and Parenchymal Destruction
           in a Patient with Ankylosing Spondylitis

    • Abstract: Pulmonary apical fibrosis is a rare complication of ankylosing spondylitis (AS). The essential characteristics of this lesion are its very slow progression and frequently asymptomatic nature. Herein, we are presenting a patient with AS who rapidly developed pulmonary apical fibrosis in a 3-year period despite decreased musculoskeletal pains. The 60-year-old male applied with complaints of progressively increasing cough in the recent two years, dyspnea, and fatigue. He had no chronic disease except AS. He had no continuous medication except nonsteroid anti-inflammatory drugs for 2-3 days monthly since his musculoskeletal pains decreased in the recent years. His physical examination revealed reduced breath sounds in the upper zones of the right lung. Chest X-ray revealed increased diffuse opacity in the upper zones of the right lung. Thoracic high-resolution computed tomography showed a consolidation accompanied with traction bronchiectases compatible with chronic fibrosis in the upper lobe of the right lung. However, thoracic computed tomography of the patient performed 3 years ago did not reveal pulmonary apical fibrosis and parenchymal destruction. Biopsy revealed no finding of malignancy, granulomatous inflammation, or vasculitis. The results of cultures were negative. So, the patient was diagnosed as pulmonary involvement of AS, which developed in a 3-year period. This case has shown that extra-articular complications may continue to develop in patients with AS even if their musculoskeletal complaints have subsided. So, patients with AS should be followed up regularly with systemic examinations.
      PubDate: Tue, 15 Sep 2020 02:50:01 +000
       
  • Acetabula Osteoid Osteoma Mimicking Juvenile Idiopathic Arthritis and
           Chronic Recurrent Multifocal Osteomyelitis

    • Abstract: Osteoid osteoma (OO) is a benign bone tumor that usually presents between 10 and 35 years of age. The metaphysis and diaphysis of the femur and tibia are the typical locations. The diagnosis is usually straightforward when images reveal a radiolucent nidus surrounded by reactive sclerosis. However, the diagnosis is more difficult when it occurs at atypical locations with nonspecific and misleading appearance on images. OO may mimic juvenile idiopathic arthritis (JIA), bone infection, or malignancy. We present a 14-year-old male with a 4-month history of left hip pain. His pain was worse with playing hockey and lacrosse and in the morning and sometimes woke him up at night. His examination was significant for pain with flexion and external rotation of the left hip and for mild limitation of full external rotation. Blood work revealed normal complete blood count, erythrocyte sedimentation rate, and C-reactive protein. Left hip X-ray was unremarkable. Left hip MR arthrogram showed marked edema of the medial and posterior walls of the left acetabulum. CT-guided biopsy of the left acetabulum showed unremarkable flow cytometry and chronic inflammatory component raising concern about chronic recurrent multifocal osteomyelitis (CRMO). Bone scan revealed focal increased uptake in the left acetabulum and no additional abnormality. Repeat MRI with intravenous contrast showed a left hip effusion, focal synovial enhancement in the medial left hip, and acetabula edema. The patient failed treatment for presumed JIA and CRMO with nonsteroidal anti-inflammatory drugs (NSAIDs), steroids, methotrexate, and adalimumab. CT scan of the left hip was performed for further evaluation of the bone and showed 11 × 6 mm low attenuation focus with subtle internal nidus in the posteromedial aspect of the acetabular rim, suggestive of intra-articular OO. Radiofrequency ablation was performed with no complications, and the left hip pain improved. The atypical location resulted in delay of diagnosis for 12 months after presentation. We highlight the diagnostic pitfalls observed in atypical OO locations and the difficulties this creates with making the diagnosis. OO mimicking JIA has previously been described. We submit CRMO as another differential diagnosis which may be mimicked and demonstrate the vital role of CT scan in the diagnosis.
      PubDate: Fri, 28 Aug 2020 08:20:00 +000
       
  • Doxycycline-Induced Antinuclear Antibody and Antineutrophil Cytoplasmic
           Antibody Associated Vasculitis: A Case Report and Literature Review

    • Abstract: Drug-induced antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has been increasingly recognized in the literature with numerous medications listed as causative agents in disease pathology. Doxycycline is a commonly prescribed medication within the United States which is a synthetic, broad-spectrum antibiotic with antimicrobial properties and at low doses exhibits anti-inflammatory effects. In this report, we describe a case of doxycycline-induced ANCA-associated vasculitis with laboratory and biopsy findings supporting the diagnosis, which to the best of our knowledge is the first described case of doxycycline-induced AAV in the literature. The patient was started on doxycycline for treatment of potential Lyme disease. She began to develop progressively worsening myasthenia, erythematous macular rash, anorexia, anemia, and fatigue for several weeks following the course of doxycycline with initial concern of a paraneoplastic process. Ultimately, the patient was discovered to be positive for antinuclear antibody (ANA), perinuclear antineutrophil cytoplasmic antibody (pANCA), and myeloperoxidase (MPO) antibody for which she was treated with a course of prednisone leading to complete remission of disease. A brief review of the pathogenesis of ANCA vasculitides will also be discussed within this article.
      PubDate: Thu, 27 Aug 2020 09:20:00 +000
       
  • Tocilizumab Was Effective in Repairing the Large Geode in a Patient with
           Rheumatoid Arthritis

    • Abstract: Rheumatoid arthritis is characterized by multiple chronic arthritis subsequently inducing joint destruction. Although subchondral geode is a well-known feature of high-disease activity, a large geode is rare. Moreover, the treatment effect of biologic agents in the repair of large geode has not been reported. The present report shows the significant effect of interleukin-6 receptor blocker, tocilizumab, in repairing the large geode in the left humeral lateral epicondyle. This case implies that tocilizumab might be an effective treatment in patients with rheumatoid arthritis even with large geode.
      PubDate: Tue, 25 Aug 2020 12:05:01 +000
       
  • Pneumomediastinum as a Manifestation of Granulomatosis with Polyangiitis

    • Abstract: A 38-year-old hypertensive male with a smoking history presented to the emergency room with significant hemoptysis, arthritis, and a purpuric rash. Other findings included a dropping hemoglobin and acute kidney injury with microscopic hematuria. The pulmonary computed tomography was significant for alveolar hemorrhage and a rarely reported pneumomediastinum. Along with this constellation of findings, a positive antiproteinase 3 helped to confirm this patient’s diagnosis of granulomatosis with polyangiitis. Treatment commenced with sessions of plasma exchange and pulse steroids along with cyclophosphamide infusions. The patient had since improved and made a full recovery. This case report highlights the rarely described pneumomediastinum in association with vasculitides.
      PubDate: Fri, 31 Jul 2020 06:50:01 +000
       
  • Bullous Lupus: An Atypical Case of Refractory Disease in a Patient with
           Sulfa Allergy

    • Abstract: Bullous systemic lupus erythematosus (BSLE) is a rare cutaneous autoimmune disorder characterized by rapid, widespread vesiculobullous lesions in patients with Systemic Lupus Erythematosus (SLE). BSLE can present as the initial manifestation of SLE and may be a marker of severe disease. In this case report, we present a case of a 22-year-old African American woman with BSLE and impaired renal function with subsequent nephrotic range proteinuria concerning for lupus nephritis and autoimmune hemolytic anemia, refractory to systemic corticosteroids, immunoglobulin, and mycophenolate mofetil, requiring dapsone after careful desensitization due to prior history of angioedema with sulfa drugs. This case highlights the importance of the prompt recognition of BSLE as the initial manifestations of SLE and illustrates the association of BSLE with severe disease and the benefit of concomitant use of dapsone with corticosteroids and other immunosuppressant drugs, even in patients with a history of sulfa allergy.
      PubDate: Mon, 27 Jul 2020 05:20:01 +000
       
  • A Case of Sclerosing Mesenteritis during Etanercept Therapy for Psoriatic
           Arthritis

    • Abstract: Sclerosing mesenteritis (SM) is a chronic nonspecific mesenteric inflammation. I report a case of a 72-year-old male treated with etanercept for psoriatic arthritis for 7 years who developed abdominal discomfort, urinary retention, acute kidney injury, and bilateral ureteric obstruction. CT abdomen revealed retroperitoneal mass. Biopsy showed sclerosing mesenteritis. One year later, after discontinuation of etanercept, CT abdomen showed regression of the mass. To my knowledge, this is first case report of reversible sclerosing mesenteritis associated with etanercept therapy.
      PubDate: Mon, 13 Jul 2020 00:20:00 +000
       
  • Superior Mesenteric Artery Syndrome: A Potentially Fatal but Reversible
           Gastrointestinal Manifestation of Systemic Sclerosis

    • Abstract: Superior mesenteric artery syndrome (SMAS) is a rare gastrointestinal disorder characterised by vascular compression of the third part of the duodenum, in the angle between the superior mesenteric artery (SMA) and the abdominal aorta. It presents as an uncommon cause of upper gastrointestinal obstruction. In patients with systemic sclerosis (SSc), gastrointestinal involvement may result in oesophageal dysmotility, gastroesophageal reflux disease (GERD), gastroparesis, small intestinal bacterial overgrowth (SIBO), chronic intestinal pseudoobstruction (CIPO), and fecal incontinence. Malnutrition may thus result in weight loss and reduced mesenteric and retroperitoneal adipose tissue, decreasing the angle between the SMA and aorta causing SMAS. Enteral or parenteral feeding can potentially reverse SMAS in SSc. We report a case of SMAS in an elderly female with SSc and concurrent gastrointestinal involvement, and discuss the important management considerations and potential adverse outcomes when untreated.
      PubDate: Sat, 04 Jul 2020 09:20:00 +000
       
  • An Atypical Presentation of Extrapulmonary Sarcoidosis

    • Abstract: Sarcoidosis is an idiopathic, chronic, multisystem, granulomatous, inflammatory disease involving almost all organs. Sarcoidosis can occur with an atypical presentation of hepatosplenic involvement, like in the case of our patient. In this case report, we present a rare case of extrapulmonary sarcoidosis with isolated involvement of the liver and spleen in a 39-year-old Caucasian female. There is a possibility of this isolated involvement of an organ in the complete absence of pulmonary disease, which makes the diagnosis of sarcoidosis very difficult as it is usually not suspected. Ultrasound and CT are important in ruling out other differential diagnoses, but a definitive diagnosis is possible only on histological examination, differentiating sarcoid lesions from tuberculosis, primary biliary cirrhosis, metastasis, malignancy, and other granulomatous infections or diseases. Hence, the most credible criterion for diagnosis remains histology. After diagnosis, regular follow-up for systemic manifestations is recommended. Asymptomatic patients with hepatosplenic sarcoidosis have a good prognosis without any medical intervention, while patients with abnormal labs or symptoms must commence treatment.
      PubDate: Thu, 25 Jun 2020 10:35:00 +000
       
  • Rituximab in Antimelanoma Differentiation-Associated Protein-5
           Dermatomyositis with Interstitial Lung Disease

    • Abstract: In this paper, we report a challenging case of a middle-age woman who developed antimelanoma differentiation-associated protein-5 dermatomyositis (anti-MDA5 DM) with interstitial lung disease (ILD) and was successfully treated with rituximab (RTX), after failure of a first-line therapy.
      PubDate: Sat, 30 May 2020 08:05:00 +000
       
  • Abatacept in the Treatment of Juvenile Dermatomyositis-Associated
           Calcifications in a 16-Year-Old Girl

    • Abstract: Calcinosis is a feared complication of JDM that may be seen in up to 40% of children with JDM. It is associated with negative impact on the patients’ quality of life due to weakness, functional disability, joint contractures, muscle atrophy, skin ulcers, and secondary infections. Calcinosis can present as superficial nodules or plaques, larger nodular deposits extending into deeper tissue layers, accumulation of calcifications along the fascial planes of muscles or tendons, or an exoskeleton of calcium leading to limitations in mobility and joint contractures. Currently, there are no known effective treatments for calcinosis and current therapy is based on anecdotal retrospective studies and cases series. We report the case of a child with JDM-associated calcinosis with extensive intramuscular calcifications who failed conventional therapies but demonstrated improvement as evident by decrease in calcinosis and improved physical function with use of abatacept. We found that use of abatacept was associated with improvement in functional outcome and recurrence did not occur. This case suggests use of abatacept as a safe and effective treatment option for calcinosis due to JDM. Furthermore, large-scale clinical studies are needed to validate our findings and to evaluate the long-term outcomes.
      PubDate: Thu, 28 May 2020 16:05:00 +000
       
  • Familial Mediterranean Fever Complicated by a Triad of Adrenal Crisis:
           Amyloid Goiter and Cardiac Amyloidosis

    • Abstract: Background. FMF is a common disease in the Mediterranean populations and may be complicated by AA amyloidosis. The coexistence of adrenal and thyroid amyloidosis in AA amyloidosis secondary to familial Mediterranean fever (FMF) is an extremely rare reported condition. We presented a previously unreported triad of adrenal, thyroid, and cardiac amyloidosis secondary to FMF. Presentation of Case. We reported a 23-year-old Palestinian male patient presented with hypotension, vomiting, diarrhea, and abdominal pain. The patient was subsequently diagnosed to have an adrenal crisis with both amyloid goiter and cardiac amyloidosis. Conclusion. It is crucial to recognize the adrenal crisis in patients with AA amyloidosis secondary to FMF who present similarly to acute FMF inflammatory episodes. The adrenal crisis has high morbidity and mortality, especially if not recognized early in the course of the disease.
      PubDate: Wed, 20 May 2020 03:35:00 +000
       
 
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