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Indonesian Journal of Rheumatology
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This is an Open Access Journal

Open Access journal
ISSN (Print) 2086-1435 - ISSN (Online) 25811142
Published by Universitas Indonesia

[19 journals]

Avascular Osteonecrosis in Systemic Sclerosis Patient: Risk Factors and
Role of Vasculopathy'
- Authors: Safarina Kharima Laitupa, Perdana Aditya Rahman
  Pages: 532 - 545
  Abstract: Avascular Necrosis (AVN) or osteonecrosis refers to the death of osteocytes and osteoblasts. Sites such as the femoral head, the head of the humerus and the mandibular with restricted access to local blood supply are particularly vulnerable to osteonecrosis. Various traumatic and non-traumatic causes of AVN are known, including systemic autoimmune diseases. Among traumatic causes, physical trauma, decompression sickness or radiation may be cited. In the non-trauma cases, two theories are disputed: the first concerns the occurrence of an intravascular coagulation and the second one attributes the ischemia to extravascular compression. AVN has been well described in patients with autoimmune diseases such as systemic lupus erythematosus, but in systemic sclerosis (SSc) patients, there have been limited case reports and case series. We present a case of a 32-year old woman with systemic sclerosis on corticosteroid and avascular osteonecrosis and elaborating possible etiologies or mechanism of avascular osteonecrosis in SSc.
  PubDate: 2022-03-01
  DOI: 10.37275/ijr.v13i2.162
  Issue No: Vol. 13, No. 2 (2022)
Progressive Systemic Sclerosis with Interstitial Lung Disease Post Radio
Ablation in Graves Disease's Patient: A Case Report
- Authors: Maha Chakri Willheljulya, Sumartini Dewi, Andri Reza
  Pages: 557 - 563
  Abstract: Systemic sclerosis (SSc) is a connective tissue disease marked by degenerative microvascular abnormalities and immune system activation, resulting in skin and internal organ fibrosis. The link between thyroid problems and systemic sclerosis is unknown. Although several studies have demonstrated that patients with systemic sclerosis develop Graves' illness as the condition progresses, only a few have reported that Graves' patients develop progressive systemic sclerosis. It is an uncommon instance of multiple autoimmune illnesses in which SSc develops gradually after the birth of the first child and the patient had Graves' disease since the age of 17 and had thyroid ablation 5 years ago. SSc illness has a characteristic clinical appearance, involving the skin to the rest of the body and internal organs, particularly the lungs.
  PubDate: 2022-03-01
  DOI: 10.37275/ijr.v13i2.188
  Issue No: Vol. 13, No. 2 (2022)
Pathogenesis, Clinical Presentations and Diagnosis of IgG4-related
Disease: A Review
- Authors: Perdana Aditya Rahman, Cesarius Singgih Wahono, Fajar Maulana Raharjo, Handono Kalim, Mokhamad Fahmi Rizki Syaban
  Pages: 564 - 575
  Abstract: IgG4-Related Disease (IgG4RD) was identified by the International Classification of Diseases (ICD) in 2012. Numerous diseases, including Mikulicz’s disease, Kuttner’s tumor, Riedel’s thyroiditis, and Ormond’s disease, are pathologically associated with IgG4. Here, we present a review of the clinical presentation and pathogenesis of IgG4-associated disease. IgG4-RD term has been used to refer to a group of diseases involving multiple organs in which there is an abundant IgG4-positive lymphoplasmacytic infiltration, storiform fibrosis, obliterative phlebitis, and mild to moderate tissue eosinophilia, all of which show clinically as a tumefactive lesion, usually in more than one organ. IgG4 exhibits a unique property called an unstable disulfide bond between its heavy chain, as described by Fab-arm exchange which enables the recombination of a single IgG4 heavy chain with other IgG4 heavy chains, resulting in a bispecific antibody incapable of cross-linking and thus of forming an immune complex. IgG4-RD pathomechanism that causes serum IgG4 increase and tissue IgG4-plasma-cell deposition that is pathogenic, rather than the IgG4 itself. Genetic predisposition, autoimmunity, T-cell dysregulation, infection, and dysbiosis are just a few of the underlying pathomechanisms. Clinical symptoms are also frequently complex and may involve many organs. Confirmation of a diagnosis required a comprehensive anamnesis and examination.
  PubDate: 2022-03-01
  DOI: 10.37275/ijr.v13i2.189
  Issue No: Vol. 13, No. 2 (2022)
Sarcoidosis Manifested as Recurrent Pericardial Effusion with Signs of
Impending Tamponade
- Authors: Christine Elizabeth Suryajaya, Susantina Prodjosoewojo, Arto Y. Soeroto, Nuraini Yasmin Kusumawardhani, Rudi Supriyadi, Laniyati Hamijoyo
  Pages: 576 - 582
  Abstract: Pericardial effusion is a fatal and life-threatening condition. If it is not addressed thoroughly, complications such as constrictive pericarditis may occur. Etiologies of pericardial effusion varied and one of the most common etiologies is tuberculosis. Other etiologies include inflammation, malignancy or other autoimmune disorder such as sarcoidosis. Sarcoidosis is a diagnosis of exclusion and is confirmed by biopsy result that showed non-caseating epitheloid-cell granuloma, with no other organism or particles. Epidemiology of sarcoidosis in Japan is 1-2 case per 100.000 patients, with the peak incidence between the age of 20 to 39 years old. We reported a case of 37 years old woman presented to our emergency department with shortness of breath and signs of pericardial tamponade. She was previously healthy with no other significant past medical. She was first treated as a case of extrapulmonary tuberculosis and shown no improvement with anti-tuberculosis medications. Several work ups were then done in search of other etiologies of her pericardial effusion. A biopsy form one of her abdominal lymph nodes was performed, which pathologically revealed sarcoidosis. She was placed on corticosteroid and methotrexate with improvement of symptoms. One month followed up showed complete resolution of her pericardial effusion.
  PubDate: 2022-03-01
  DOI: 10.37275/ijr.v13i2.200
  Issue No: Vol. 13, No. 2 (2022)
Difference between the Decrease in P1NP Concentration and MRSS Within 3
Months Treatment of Systemic Sclerosis
- Authors: Devy Priyantini Hanafi, Sumartini Dewi, Andri Reza Rahmadi
  Pages: 546 - 556
  Abstract: Introduction: Systemic sclerosis is characterized by extensive and progressive organ fibrosis leads to organ failure and death. Progression of skin thickening is a predictor of morbidity and mortality. Due to the limitation of modified Rodnan skin score (MRSS) sensitivity in detecting small changes in skin fibrosis, thus we proposed Procollagen Type I N-Terminal Propeptide (P1NP) as potential biomarker. This study aimed to analyze the difference between the decrease in P1NP concentration and MRSS within 3 months treatment of systemic sclerosis. Methods: We conducted a retrospective cohort of paired numerical comparative analytic methods, as follow up of a study done by Vincent et al. and Dewi et al. Analyzis of MRSS changes and serum P1NP concentrations were done prior to treatment (baseline), and on week 4th, 8th and 12th treatment. Result: Fifty-nine subjects were enrolled in the study. We analyzed the results of P1NP and MRSS at 4th, 8th and 12th weeks of treatment, there was a significant decrease in mean rank of P1NP and MRSS (p=0.033 and <0.001). The effect of MRSS change was greater than P1NP. The highest decreasing effect of MRSS was obtained at week 8th ('2 = 0.424, 42.4% decrease effect), and the largest decrease effect of P1NP was obtained at week 12th ('2 = 0.120; 12% decrease effect). Conclusion: There was a difference in decreasing P1NP concentrations and MRSS in systemic sclerosis within 3 months of observation. MRSS showed a larger decrease in change than P1NP after treatment.
  PubDate: 2021-09-01
  DOI: 10.37275/ijr.v13i2.184
  Issue No: Vol. 13, No. 2 (2021)

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