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RHEUMATOLOGY (76 journals)

Showing 1 - 76 of 76 Journals sorted alphabetically
ACR Open Rheumatology     Open Access   (Followers: 5)
Advances in Rheumatology     Open Access   (Followers: 3)
African Journal of Rheumatology     Full-text available via subscription  
Aktuelle Rheumatologie     Hybrid Journal   (Followers: 2)
Annals of Rheumatology and Autoimmunity     Open Access   (Followers: 3)
Annals of the Rheumatic Diseases     Hybrid Journal   (Followers: 34)
Archives of Osteoporosis     Hybrid Journal   (Followers: 1)
Arthritis & Rheumatology     Hybrid Journal   (Followers: 64)
Arthritis Care & Research     Hybrid Journal   (Followers: 37)
Arthritis Research & Therapy     Open Access   (Followers: 14)
Australasian Musculoskeletal Medicine     Full-text available via subscription   (Followers: 5)
Best Practice & Research Clinical Rheumatology     Hybrid Journal   (Followers: 17)
BMC Musculoskeletal Disorders     Open Access   (Followers: 29)
BMC Rheumatology     Open Access   (Followers: 4)
Case Reports in Rheumatology     Open Access   (Followers: 10)
Clinical and Experimental Rheumatology     Full-text available via subscription   (Followers: 3)
Clinical Medicine Insights : Arthritis and Musculoskeletal Disorders     Open Access   (Followers: 3)
Clinical Rheumatology     Hybrid Journal   (Followers: 21)
Current Opinion in Rheumatology     Hybrid Journal   (Followers: 13)
Current Reviews in Musculoskeletal Medicine     Open Access   (Followers: 13)
Current Rheumatology Reports     Hybrid Journal   (Followers: 3)
Current Rheumatology Reviews     Hybrid Journal   (Followers: 4)
Current Treatment Options in Rheumatology     Hybrid Journal  
Egyptian Rheumatologist     Open Access   (Followers: 1)
Egyptian Rheumatology and Rehabilitation     Open Access   (Followers: 2)
Forum Reumatologiczne     Hybrid Journal  
Future Rheumatology     Full-text available via subscription   (Followers: 1)
Gait & Posture     Hybrid Journal   (Followers: 17)
Indian Journal of Rheumatology     Open Access   (Followers: 1)
Indonesian Journal of Rheumatology     Open Access  
International Journal of Clinical Rheumatology     Open Access   (Followers: 4)
International Journal of Rheumatic Diseases     Hybrid Journal   (Followers: 2)
International Journal of Rheumatology     Open Access   (Followers: 6)
International Musculoskeletal Medicine     Hybrid Journal   (Followers: 7)
Internet Journal of Rheumatology and Clinical Immunology     Open Access   (Followers: 4)
JCR Journal of Clinical Rheumatology     Hybrid Journal   (Followers: 7)
Journal of Musculoskeletal Research     Hybrid Journal   (Followers: 9)
Journal of Orthopedics & Rheumatology     Open Access  
Journal of Rheumatology     Open Access   (Followers: 32)
Modern Rheumatology     Hybrid Journal   (Followers: 4)
Modern Rheumatology Case Reports     Hybrid Journal  
Multiple Sclerosis and Related Disorders     Hybrid Journal   (Followers: 8)
Musculoskeletal Care     Hybrid Journal   (Followers: 19)
MYOPAIN. A journal of myofascial pain and fibromyalgia     Hybrid Journal   (Followers: 16)
Nature Reviews Rheumatology     Full-text available via subscription   (Followers: 25)
OA Arthritis     Open Access   (Followers: 1)
OA Inflammation     Open Access  
Open Access Rheumatology: Research and Reviews     Open Access   (Followers: 3)
Open Journal of Orthopedics and Rheumatology     Open Access  
Open Journal of Rheumatology and Autoimmune Diseases     Open Access   (Followers: 4)
Open Rheumatology Journal     Open Access  
Orthopädie & Rheuma     Full-text available via subscription  
Osteoarthritis and Cartilage     Full-text available via subscription   (Followers: 20)
Osteoarthritis and Cartilage Open     Open Access  
Osteologie     Hybrid Journal  
Osteoporosis and Sarcopenia     Open Access  
Pain. Joints. Spine     Open Access   (Followers: 1)
Reumatismo     Open Access  
Reumatología Clínica (English Edition)     Full-text available via subscription  
Revista Argentina de Reumatología     Open Access  
Revista Colombiana de Reumatologia     Open Access  
Revista Colombiana de Reumatología (English Edition)     Hybrid Journal  
rheuma plus     Hybrid Journal  
Rheumatic Disease Clinics of North America     Full-text available via subscription   (Followers: 4)
Rheumatica Acta: Open Access     Open Access  
Rheumatology     Hybrid Journal   (Followers: 33)
Rheumatology & Autoimmunity     Open Access   (Followers: 3)
Rheumatology Advances in Practice     Open Access   (Followers: 1)
Rheumatology and Therapy     Open Access   (Followers: 3)
Rheumatology International     Hybrid Journal   (Followers: 3)
Rheumatology Practice and Research     Open Access  
RMD Open     Open Access   (Followers: 1)
Scandinavian Journal of Rheumatology     Hybrid Journal   (Followers: 5)
Seminars in Arthritis and Rheumatism     Hybrid Journal   (Followers: 8)
The Lancet Rheumatology     Hybrid Journal  
Zeitschrift fur Rheumatologie     Hybrid Journal   (Followers: 6)
Similar Journals
Journal Cover
Rheumatology International
Journal Prestige (SJR): 0.906
Citation Impact (citeScore): 2
Number of Followers: 3  
 
  Hybrid Journal Hybrid journal (It can contain Open Access articles)
ISSN (Print) 1437-160X - ISSN (Online) 0172-8172
Published by Springer-Verlag Homepage  [2469 journals]
  • Correction to: Assessment of systemic and ocular inflammation in juvenile
           idiopathic arthritis via choroidal vascularity index

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      PubDate: 2022-07-01
       
  • Polymyositis and dermatomyositis: ocular manifestations and potential
           sight-threatening complications

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      Abstract: Abstract Dermatomyositis (DM) and polymyositis (PM) are idiopathic inflammatory myopathies characterized by progressive, symmetric, mainly proximal muscle weakness. DM is also characterized by cutaneous involvement. However, other clinical features, systemic involvement, histopathological findings, response to treatment, and prognosis, differ significantly. Although uncommon, ocular manifestations in DM and PM may potentially affect any structure within the eye. Notwithstanding being generally mild, ocular involvement in DM and PM may result in significant morbidity. Left untreated, significant retinal inflammation associated with hemorrhage and detachment may occur, leading to significant vision loss. This review aims to present an up-to-date overview for rheumatologists about the ocular involvement and potential complications of DM and PM and when to refer to the ophthalmologist to avoid sight-threatening complications.
      PubDate: 2022-07-01
       
  • The spectrum of hemophagocytic lymphohistiocytosis: a retrospective study
           comparing adult macrophage activation syndrome to malignancy-associated
           hemophagocytic lymphohistiocytosis

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      Abstract: Abstract Secondary hemophagocytic lymphohistiocytosis (sHLH) is a life-threatening inflammatory syndrome that can be triggered by autoimmune diseases, malignancy, or infection. In rheumatologic patients, sHLH is referred to as macrophage activation syndrome (MAS). Differentiating between triggers is important for prompt treatment and prognosis. Data comparing subsets of sHLH are limited due to the rarity of this disease. We aim to explore differences in clinical features that may differentiate MAS from malignancy-associated HLH (mHLH) patients. We conducted a single-center retrospective study assessing clinical characteristics, laboratory parameters, treatment regimens and outcomes in 34 patients with sHLH over a 16 year period. We compared patients with MAS to those with mHLH. Hepatomegaly was not present in the MAS group but was present in the mHLH group (0 vs. 25%, p = 0.024). MAS patients had on average nearly double the concentration of platelets at 50.0 (IQR: 31.0–78.0 Kµ/L) vs. 29.0 Kµ/L (IQR: 14.0–37.5 Kµ/L), p = 0.003. Soluble IL-2R concentrations were four times lower in the MAS group with a median soluble IL-2R concentration of 6814.5 kU/L (IQR: 2101–2610 kU/L) vs. 27972.0 kU/L (IQR: 12,820–151,650 kU/L), p = 0.010. The MAS group fared better overall than the mHLH group but was not statistically significant (mortality 22 vs. 44%, p = 0.18). MAS and mHLH patients exhibited different laboratory parameters and clinical features, most notably differences in platelet counts, soluble IL-2R concentration and hepatomegaly, which may help differentiate these conditions early in their course.
      PubDate: 2022-07-01
       
  • Association of various myositis-specific autoantibodies with
           dermatomyositis and polymyositis triggered by pregnancy

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      Abstract: Abstract Although pregnancy is an important risk factor for autoimmune rheumatic diseases, little is known regarding the association between pregnancy and dermatomyositis (DM) or polymyositis (PM). Herein, we present two patients with DM that developed during the perinatal period. The first patient was positive for anti-aminoacyl synthetase (ARS) antibody and developed DM in the 14th week of pregnancy. Despite treatment, her foetus died of intrauterine growth restriction in the 27th week. The second patient was positive for anti-melanoma differentiation-associated gene 5 (MDA-5) antibody and developed DM 1 week after miscarriage at 9 weeks of gestation. The patient developed severe interstitial pneumonia, and intensive therapy including tofacitinib and rituximab administration was required. Our cases and a literature review revealed that various myositis-specific autoantibodies, including anti-ARS, anti-Mi-2, anti-TIF-1γ, and anti-MDA-5, are associated with DM and PM triggered by pregnancy. We also found that delay in commencing treatment in case of active disease including myositis and interstitial pneumonia, and poor response to corticosteroids were related to poor foetal outcomes in DM and PM. Although rare in pregnant women, it is critical to consider the possibility of DM and PM in patients presenting with rash, fever, weakness, and cough, and testing for myositis-specific autoantibodies is recommended.
      PubDate: 2022-07-01
       
  • Sjögren’s syndrome with adult-onset Still’s disease: an
           overlap syndrome'

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      Abstract: Abstract Adult-onset Still’s disease (AOSD) is a rare systemic auto-inflammatory disease that is an exclusive diagnosis that needs to previously exclude infections, tumors, and rheumatic diseases. There are few reports on AOSD overlapping with other rheumatic diseases. We reported a 55-year-old male who presented with a high, daily spiking fever associated with an evanescent salmon-pink rash, arthralgia, and sore throat. He had a history of dryness of the mouth and eyes for decades with no medical treatment. On admission, tests for antinuclear antibody (ANA) and anti-SSA/Ro-52 antibody were positive, and salivary gland biopsy showed focal lymphocytic sialadenitis with a focus score of ≥ 1 foci/4 mm2, which was consistent with a diagnosis of primary Sjögren’s syndrome (SS). However, the disease activity of SS was low at the time of the report. Combined with significantly elevated acute phase reactants, the patient also met the classification criteria of both Yamakuchi and Futel for AOSD. His clinical symptoms were relieved quickly with glucocorticoid therapy. We also reviewed the literature on SS with AOSD and AOSD with other rheumatic diseases, and scattered case reports were retrieved. So we think that AOSD is not an absolutely exclusive diagnosis and can occur in patients with other rheumatic diseases. To our knowledge, this is the only literature review of a reported AOSD case in a SS patient.
      PubDate: 2022-07-01
       
  • Associations between cardiac and pulmonary involvement in patients with
           juvenile dermatomyositis—a cross-sectional study

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      Abstract: Abstract This study aimed at exploring the association between detectable cardiac and pulmonary involvement in long-term juvenile dermatomyositis (JDM) and to assess if patients with cardiac and pulmonary involvement differ with regard to clinical characteristics. 57 JDM patients were examined mean 17.3 (10.5) years after disease onset; this included clinical examination, myositis specific/associated autoantibodies (immunoblot), echocardiography, pulmonary function tests and high-resolution computed tomography. Cardiac involvement was defined as diastolic and/or systolic left ventricular dysfunction and pulmonary involvement as low diffusing capacity for carbon monoxide, low total lung capacity and/or high-resolution computed tomography abnormalities. Patients were stratified into the following four groups: (i) no organ involvement, (ii) pulmonary only, (iii) cardiac only, and (iv) co-existing pulmonary and cardiac involvement. Mean age was 25.7 (12.4) years and 37% were males. One patient had coronary artery disease, seven had a history of pericarditis, seven had hypertension and three had known interstitial lung disease prior to follow-up. There was no association between cardiac (10/57;18%) and pulmonary (41/57;72%) involvement (p = 0.83). After stratifying by organ involvement, 21% of patients had no organ involvement; 61% had pulmonary involvement only; 7% had cardiac involvement only and 11% had co-existing pulmonary or cardiac involvement. Patients with co-existing pulmonary or cardiac involvement had higher disease burden than the remaining patients. Patients with either cardiac or pulmonary involvement only, differed in clinical and autoantibody characteristics. We found no increased risk of developing concomitant cardiac/pulmonary involvement in JDM. Our results shed light upon possible different underlying mechanisms behind pulmonary and cardiac involvement in JDM.
      PubDate: 2022-07-01
       
  • Hypercalcemia in IgG4-related disease: coincidental or associated'
           Case based review

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      Abstract: Abstract Immunoglobulin (Ig) G4-related disease (IgG4RD) is a chronic autoimmune disorder characterized by dense lymphoplasmacytic infiltrations and fibrosis of storiform pattern. The most typical manifestations include major salivary or lacrimal gland involvement, autoimmune pancreatitis, and retroperitoneal fibrosis. While the increase in IgG4 is the typical feature of the disease, hypercalcemia has been rarely reported in IgG4RD so far, only one of these cases has been shown parathyroid gland involvement (isolated involvement). In this study, we present a 43-year-old female patient with weight loss, pancreatic mass, lymphadenopathy, nodular lesion in the lung, hypercalcemia, and also increased level of serum IgG4. Histopathological investigation following parathyroidectomy revealed a dense lymphoplasmacytic infiltrate with an IgG4 to IgG ratio of > 50% in the fat tissue surrounding the parathyroid gland, particularly at the perivascular areas. This is the first systemic IgG4RD case in combination with hypercalcemia in the literature who was detected to have parathyroid adenoma. Our aim in this review is to emphasize that, although rarely, IgG4RD may be accompanied by hypercalcemia and parathyroid gland may be one of its target sites.
      PubDate: 2022-07-01
       
  • Disease characteristics and clinical outcomes of adults and children with
           anti-MDA-5 antibody-associated myositis: a prospective observational
           bicentric study

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      Abstract: Abstract To study the demographic, clinical and serologic characteristics of anti-MDA5-positive DM from two geographically and ethnically disparate inception cohorts in India. To identify the clinical and serological parameters at inception that could predict mortality among these individuals. Individuals with anti-MDA5 antibody-positive DM diagnosed between 2017 and 2020 from two centres in India were prospectively followed up. The clinical and serological characteristics at baseline and the treatment outcome at follow-up were assessed for this study. Anti-MDA5 antibody was positive in 25 (7.5%) out of the 330 individuals with myositis. These 25 (21 adults, 4 juvenile) patients were followed up for a median duration of 14 months. Among adults, a majority had cutaneous manifestations 21 (84%) followed by, arthritis 17 (80%), and interstitial lung disease 12 (ILD, 57.1%). Four (19%) had rapidly progressive ILD (RP-ILD). Eight (38%) presented as clinically amyopathic DM. Among cutaneous manifestations, majority (62%) had classic features (gottron’s papules/sign, heliotrope rash) while 8 (38%) had cutaneous ulceration and 2 each had periorbital edema and tendon rupture. Eight (38%) were positive for anti-Ro-52 antibody. Out of 21 adults, 8 (38%) succumbed to the diseases. RP-ILD (n = 4; 19%), ulcerative gottron’s (n = 5) and anti-Ro-52 (n = 8) were significantly associated with mortality (p < 0.05). Upon binary logistic regression, positive anti-Ro-52 antibody predicted mortality [HR 17.3 (95%CI 1.4–210, p = 0.025)]. All juvenile anti-MDA5-positive DMs had classic cutaneous features with 2 of them having ulcerative gottron’s. None of the juvenile patients had ILD and everyone survived till the last follow-up. Indian adults with anti-MDA5 DM have high mortality. Rarer atypical features like tendon rupture or periorbital edema could assist in diagnosis. Ulcerative gottron’s, positive anti-Ro 52 antibodies, and RP-ILD are valuable clinical-serological markers that portend poor prognosis.
      PubDate: 2022-07-01
       
  • Hepatitis B vaccination response of treatment-naive patients with juvenile
           idiopathic arthritis

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      Abstract: Abstract To evaluate the vaccine response of treatment-naive juvenile idiopathic arthritis (JIA) patients who were fully vaccinated against Hepatitis B Virus (HBV) and then compare their antibody status with healthy controls. In this multicenter study, initial visit hepatitis B surface antigen (HbsAg) and anti-hepatitis B surface antibody (anti-Hbs) titers of 262 treatment-naive JIA patients who were followed up regularly between May 2015 and October 2019 were evaluated retrospectively from patients’ medical records and compared with 276 healthy peers. Both HbsAg and anti-Hbs antibody titers were tested by the ELISA technique. Anti-HBs titers ≥ 10 IU/L were considered as reactive indicating seroprotection against HBV. In the JIA group, seropositivity rate was 59.1% while 72.9% of the control group were immune against HBV (p = 0.002). The median titer for anti-Hbs was 14 (range: 0–1000) IU/L in the patient group and 43.3 (range: 0–1000) IU/L in the control group (p = 0.01). Neither JIA patients nor healthy controls were positive for HbsAg. Patients with JIA vaccinated according to the national vaccination schedule were evaluated at their first visit in pediatric rheumatology outpatient clinics for anti-Hbs presence and it was found that they have lesser seroprotectivity than their age and sex-matched routinely vaccinated, healthy peers. So, to complete missing vaccines and booster vaccine doses, assessing the immune status of the patients at the time of diagnosis against HBV should be in the check-list of physicians dealing with pediatric rheumatic diseases.
      PubDate: 2022-07-01
       
  • Pericarditis as a manifestation of IgG4-related disease

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      Abstract: Abstract IgG4-related disease (IgG4-RD) is a systemic, immune-mediated fibro-inflammatory disease that can affect virtually every organ system. It is usually insidious in onset and often mimics malignant or other inflammatory disorders. Diagnosis frequently requires a combination of clinical, serological, radiographic, and histopathological features, including increased serum-IgG4 levels and tissue infiltration of IgG4-positive plasma cells with associated fibrosis. Unlike more frequently affected sites, including the hepatobiliary system, salivary glands and retroperitoneum, pericardial involvement of IgG4-RD has only rarely been described. We report the case of a 76-year-old woman presenting with refractory pericarditis and imminent cardiac tamponade, successfully treated with therapeutic pericardiectomy. A diagnosis of IgG4-RD was made based on elevated serum-IgG4 levels and the presence of typical pericardial histopathological findings, meeting all 3 of the 2011 comprehensive diagnostic criteria for IgG4-RD. Following pericardiectomy, the patient remained in remission without a need for glucocorticoids or additional immunosuppressive therapy. Adding to this case, we reviewed the literature for previously described cases of IgG4-RD presenting with pericarditis and described their characteristics and the available treatment options. Our case-based literature review provides a clear overview of the diagnostic process for IgG4-RD and the need to apply classification criteria with the necessary caution, particularly in the case of rare disease manifestations, including pericarditis.
      PubDate: 2022-07-01
       
  • Toll-like receptor-7 activation in CD8+ T cells modulates inflammatory
           mediators in patients with rheumatoid arthritis

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      Abstract: Abstract Rheumatoid arthritis (RA) is an autoimmune disorder of unknown etiology with aberrant immunological responses leading to inflammation, swelling and pain of the joints. CD8+ T cells have been known to be one of the major immune modulators in the progression of RA and the presence of toll-like receptors (TLRs) on these cells further accentuate their role in RA. Herein, we report an increased expression of TLR7 in the endosomes of CD8+ T cells of RA patients correlating with disease severity. The stimulation of TLR7 with Imiquimod (IMQ) in these CD8+ T cells drives the signalling cascade via NFkB and pERK activation and hence an increase in the mRNA transcripts of signature cytokines and cytolytic enzymes. However, a parallel synthesis of Tristetraprolin (TTP), an mRNA destabilizing protein prevents the translation of the mRNA transcripts, leading to a rapid degeneration of the target mRNA. We thus report that a direct TLR7 ligation by its agonist increases cytokine transcript signature but not an equivalent protein surge.
      PubDate: 2022-07-01
       
  • Differential efficacy of TNF inhibitors with or without the immunoglobulin
           fragment crystallizable (Fc) portion in rheumatoid arthritis: the ANSWER
           cohort study

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      Abstract: Abstract Rheumatoid factor (RF) binds to the fragment crystallizable (Fc) portion of immunoglobulin. It could bind to the Fc portion of anti-TNF inhibitors (TNFi) and attenuate the clinical efficacy. We tried to determine whether the therapeutic efficacy of TNFi with Fc might be lower than that of TNFi without Fc in rheumatoid arthritis (RA) patients with high titres of RF. The Kansai Consortium for Well-being of Rheumatic Disease Patients (ANSWER) cohort is an observational multi-center registry of patients with RA in the Kansai district of Japan. RA patients treated with TNFi were included and divided into two groups based on the structural characteristics between TNFi with Fc (infliximab, adalimumab, golimumab, and etanercept) and TNFi without Fc (certolizumab pegol). Patients were classified into 4 groups according to RF titre quartiles. The sequential disease activity score in 28 joints using erythrocyte sedimentation rate (DAS28-ESR) was compared by Mann–Whitney U test between TNFi with and without Fc in each RF titre group. Multiple linear regression analysis was used to analyze the effect of TNFi without Fc for the change of DAS28-ESR adjusted after potential confounders. A total of 705 RA patients were classified into four groups (RF1; RF 0–15.0 IU/mL, RF2; 15.0–55.0, RF3; 55.0–166, RF4; 166–7555). In RF4, RA patients treated with TNFi without Fc had a significantly lower DAS28-ESR than those treated with TNFi with Fc [3.2 (2.3–4.2) vs. 2.7 (2.0–3.0)] after 12 months. This effect of TNFi without Fc for the change of DAS28-ESR after 12 months treatment retained in multivariate analysis in RF4. TNFi without Fc may be more efficacious than TNFi with Fc in RA patients with high RF titres.
      PubDate: 2022-07-01
       
  • IgG4-related disease in pediatric patients: a single-center experience

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      Abstract: Objective Immunoglobulin G4-related disease (IgG4-RD) is a systemic, immune-mediated, and fibroinflammatory disease that can affect almost any organ system. We aimed to present our single-center experience of pediatric patients with IgG4-RD, a rare disease in children. Methods Pediatric patients diagnosed with IgG4-RD at the Hacettepe University between June 2014 and September 2020 were evaluated retrospectively. Patients with definite, probable, or possible diagnosis of IgG4-RD were included. Results A total of eight patients with a median age of 13.4 (IQR 9.5–15.0) years were included in the study. Clinical presentations were IgG4-related ophthalmic disease in six patients, IgG4-related lymphadenopathy in one patient, and IgG4-related sialadenitis and lymphadenopathy of several lymph nodes accompanied by pancreatitis, ulcerative colitis, and pulmonary manifestations in one patient. Elevated serum IgG4 was detected in three of eight patients (37.5%). The main histopathological feature was fibrosis and lymphoplasmacytic infiltrates. Corticosteroids were used as first-line treatment in almost all patients with or without steroid-sparing agents. Azathioprine, methotrexate and rituximab were used as steroid-sparing agents. Relapse occurred in two of seven patients. Radiotherapy was used as the last resort in one patient with severe orbital disease. Conclusion IgG4 RD mainly presents with orbital manifestations in pediatric population but has wide phenotypic clinical variability. Although rare, early recognition and treatment are essential for a better outcome in these patients.
      PubDate: 2022-07-01
       
  • Evaluation of flare rate and reduction strategies for bDMARDs in juvenile
           idiopathic arthritis: real world data from a single-centre cohort

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      Abstract: Abstract This study aimed to determine the flare rate (FR) in a cohort of Juvenile Idiopathic Arthritis (JIA) patients with tapered or abruptly discontinued biologic disease-modifying anti-rheumatic drugs (bDMARDs) and to identify predictors of flare. This retrospective observational study included 191 bDMARD dose-reduction events in patients with JIA followed-up at a referral hospital during the period 2000–2019. FR was analysed according to reduction strategies. To identify predictors of flare, Kaplan–Meier and Cox-regression models were plotted at 6 months (6 m), 12 months (12 m) and 24 months (24 m) following tapering (TP) or withdrawal (WD). 165 episodes of TP and 71 episodes of WD were included; 45 episodes where treatment was withdrawn after TP were included in both strategies. FR after TP was 13.4% at 6 m and increased up to 26.6% at 12 m and 51.4% at 24 m. After WD, FR was higher, 52.1% of events had a flare at 6 m and 67.6% at 12 m. Previous TP did not increase time in remission after WD of bDMARDs in the Kaplan–Meier analysis. Factors associated with flares were identified after TP at 6 m: female sex, higher number of previous bDMARDs and longer time on bDMARD treatment were positively associated with flares. Polyarticular subtype and younger age at diagnosis were associated with flares at 12 and 24 m after TP. No factors were identified in multivariable analysis after WD. TP is a successful strategy to maintain remission with lower bDMARD doses. Previous TP of bDMARDs does not seem to increase time in remission after WD.
      PubDate: 2022-07-01
       
  • Myopathy associated with anti-signal recognition particle antibodies with
           pulmonary involvement and response to rituximab

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      Abstract: Abstract The authors present the case of a 76-year-old female patient with progressive decrease in proximal muscle strength, fatigue, dyspnea, diffuse hand edema and painful triphasic Raynaud’s phenomenon. Anti-SRP and anti-SSA antibodies were detected, muscle biopsy revealed changes consistent with necrotizing myopathy and capillaroscopy had findings compatible with systemic sclerosis. High-resolution chest computed tomography revealed interstitial lung disease with a non-specific interstitial pneumonia pattern. Lung function tests demonstrated a forced vital capacity 93% and a diffusing capacity for carbon monoxide of 65% predicted. After multidisciplinary discussion, she was diagnosed with immune-mediated necrotizing myopathy/systemic sclerosis overlap syndrome with pulmonary involvement. Initially, dual immunomodulation therapy with high-dose steroids and intravenous immunoglobulin was started, but after 4 weeks, the patient had clinical and analytical deterioration. At this time, she was started on rituximab, with an excellent and sustained response at both muscle and lung, sustained after 12 months.
      PubDate: 2022-07-01
       
  • Comparison of contrast-enhanced MRI features of the (teno)synovium in the
           wrist of patients with juvenile idiopathic arthritis and pediatric
           controls

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      Abstract: Abstract To directly compare and describe the differences between juvenile idiopathic arthritis (JIA) patients and pediatric controls regarding features of the synovial and tenosynovial membrane on contrast-enhanced magnetic resonance imaging (MRI) of the wrist. T1-weighted contrast-enhanced MRI scans of 25 JIA patients with clinically active wrist arthritis and 25 children without a history of joint complaints nor any clinical signs of joint inflammation were evaluated by two readers blinded to clinical data. The synovium was scored at five anatomical sites based on thickening of the synovium (0–3 scale) and synovial enhancement (0–2 scale). Thickening and/or enhancement of the tenosynovium was scored at four anatomical sites using a 0–3 scale. Significantly higher scores for synovial thickening (median 4 vs. 1, p < 0.001) and synovial enhancement (median 4 vs. 1, p < 0.001) are found in the wrist of JIA patients as compared to controls. JIA patients experienced the highest synovial scores at the mid-/inter-carpal, 2nd –5th carpometacarpal, and radiocarpal joints. No significant difference in tenosynovial scores is found between both groups (median 0 vs. 0, p = 0.220). This study highlights the higher synovial thickening/enhancement scores on contrast-enhanced MRI of the wrist in JIA patients compared to pediatric controls. Tenosynovial thickening and/or enhancement was rarely present in both groups. In JIA patients, synovial thickening and enhancement were particularly present at three anatomical sites. These results substantially support rheumatologists and radiologists when navigating through MRI of the wrist in search for JIA disease activity.
      PubDate: 2022-07-01
       
  • Juvenile Dermatomyositis Magnetic Resonance Imaging Score (JIS) does not
           correlate with criteria for clinically inactive disease: a single-centre
           retrospective evaluation

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      Abstract: Abstract The Paediatric Rheumatology International Trials Organisation (PRINTO) criteria for clinically inactive disease (CID) and their proposal for glucocorticoid tapering do not consider MRI findings, despite the growing use of MRI and development of reliable MRI scoring tools. We aim to evaluate how CID correlates with MRI scores and physician decision making. We retrospectively used the Juvenile Dermatomyositis Imaging Score (JIS) to score MRIs of all children with JDM over a 10-year period. Demographic, diagnosis, treatment and core set measures data were collected. Correlation between CID and JIS was assessed as well as correlation with the physician treatment decision. There were 25 patients with 59 follow-up episodes to analyse correlation between physician treatment decision and JIS; and 50 episodes for the CID category and JIS correlation. JIS was not significantly associated with the CID category but did correlate with the physician decision. No significant association was found between clinical decision and CID category. The JIS area under the ROC curve (AUC) was 0.80 (95% CI 0.62–0.99) with a score ≥ 8 to predict an escalation. JIS sensitivity and specificity were both 78% with accuracy of 78%, compared to only 67%, 46% and 49%, respectively, for the CID criteria. Clinical criteria alone are not sufficient to assess disease activity status. Clinical decision trends correlated to MRI findings but not PRINTO CID criteria. Multi centre prospective studies are needed to replicate our findings and establish how to best use MRI as a biomarker of disease activity.
      PubDate: 2022-07-01
       
  • Probiotic use in the prophylaxis of periodic fever, aphthous stomatitis,
           pharyngitis, and adenitis (PFAPA) syndrome: a retrospective cohort study

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      Abstract: Abstract Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is an autoinflammatory recurrent fever syndrome that mainly affects children. Probiotics are currently used to prevent upper respiratory tract infections and flares of diseases associated with immune dysregulation. We aimed to evaluate the response to probiotic treatment in PFAPA patients. Patients with PFAPA syndrome who received probiotics and were followed between July 2019 and July 2021 were included in this retrospective study. Demographic and clinical features and response to probiotics were assessed. Twenty out of 111 children with PFAPA syndrome (F/M:1) were included. The median (min–max) ages at symptoms onset and diagnosis were 24 (3–72) and 51.5 (11–120) months, respectively. All 20 patients received probiotics during the disease course. The probiotic preparation they received included a combination of two lactobacilli as Lactobacillus plantarum HEAL9 (Lp HEAL9) and Lactobacillus paracasei 8700:2 (Lpa 8700:2). The median age at probiotic onset was 60 (33–192) months, while the duration of probiotic use was 4.5 (3–19) months. All patients except one experienced a decrease in attack frequency with probiotic use. After probiotic treatment, the median number of episodes during 3 months decreased from 3 to 1 (p < 0.001). Eight (40%) patients had no attacks during the 3 months after probiotic initiation. And, 5 (45%) of 11 patients who had ≥ 1 attacks on probiotics mentioned that the attack severity decreased significantly after probiotic initiation. Our results suggest that probiotic strains Lactobacillus plantarum HEAL9 and Lactobacillus paracasei 8700:2 could be beneficial in PFAPA patients by decreasing the attack frequency.
      PubDate: 2022-07-01
       
  • The prevalence and clinical characteristics of anti-HMGCR
           (anti-3-hydroxy-3-methyl-glutaryl-coenzyme A reductase) antibodies in
           idiopathic inflammatory myopathy: an analysis from the MyoCite registry

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      Abstract: Abstract This study aimed to determine the prevalence and clinical characteristics of anti-HMGCR antibodies in idiopathic inflammatory myositis (IIM) at a tertiary care centre in northern India. Data (adult and children) were retrieved from the MyoCite dataset, identifying patients with polymyositis, dermatomyositis, and antibody-negative IIM whilst fulfilling the ACR/EULAR criteria. SLE, sarcoidosis, and systemic sclerosis were included for comparison as disease controls. The baseline clinical profile, laboratory tests, and muscle biopsies were retrieved and analysed. Descriptive statistics and non-parametric statistics were used for comparison. Among 128 IIM (112 adults, 16 children, M:F 1:2.8) of age 37 (24–47) years and 6 (3–17) months disease duration, 4 (3.6%) young adults tested positive for anti-HMGCR antibodies. All children and disease control tested negative for the antibody. Anti-HMGCR + IIM exhibited higher muscle enzymes [AST (367 vs 104 IU/L, p = 0.045), ALT (502 vs 78 IU/L, p = 0.004), and CPK (12,242 vs 699 IU/L, p = 0.001] except lactate dehydrogenase with less frequent systemic features such as fatigue than antibody-negative IIM. One young girl presented with a Limb–girdle muscular dystrophy (LGMD) with chronic pattern. None of the patients exhibited rashes, statin exposure, or cancer, though one had anti-Ro52 and mild disease. Our observations depict a younger population while affirming previous literature, including NM-like presentation, and chronic LGMD-like pattern of weakness in one case. Although a small number of children were included, ours is one of the few paediatric studies that evaluated HMGCR antibodies thus far. Further investigations in a larger Indian cohort are warranted to substantiate our findings.
      PubDate: 2022-07-01
       
  • Abdominal aortic diameter and cardiovascular status in patients with
           idiopathic retroperitoneal fibrosis

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      Abstract: Abstract Although much debated, an exaggerated inflammatory response to advanced atherosclerosis has been implicated in the pathogenesis of idiopathic retroperitoneal fibrosis (RPF). Clinical presentation, infrarenal abdominal aortic diameter and RPF mass thickness were retrospectively analyzed in 166 patients with idiopathic RPF seen at our referral center between April 1998 and December 2019. Patients were stratified to their infrarenal abdominal aortic diameter at presentation (i.e., non-ectatic [< 25 mm]; ectatic [25–29 mm]; and aneurysmal [≥ 30 mm]) to compare characteristics across groups with an undilated or dilated aorta. Ectatic or aneurysmal aortic dilatation was present in 34% of patients. Most clinical characteristics did not differ across abdominal aortic diameter stratified groups, but RPF mass thickness was greater in patients presenting with aortic aneurysmal dilatation compared to that in patients with an undilated aorta (49.0 mm [IQR 34.0–62.0] vs 32.5 mm [IQR 25.3–47.8]; P < 0.001). A positive linear association was found between aortic diameter on a continuous scale and RPF mass thickness (β 0.32 [95% CI 0.34–0.96]; P < 0.001). This association remained significant after adjusting for age, sex and acute-phase reactant levels (β 0.28 [95% CI 0.15–0.95]; P < 0.01). Treatment success across aortic diameter stratified groups did not differ (P = 0.98). Treatment induced RPF mass regression was not associated with an increase in aortic expansion rate (P = 0.44). Aortic dilatation was prevalent among patients. Infrarenal abdominal aortic diameter was independently associated with RPF mass thickness. Findings support the concept that at least in a subset of patients, RPF may be secondary to advanced atherosclerosis.
      PubDate: 2022-07-01
       
 
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