Perdana Aditya Rahman
Rheumatology And Immunology Division, Department Of Internal Medicine, University Of Brawijaya, Malang, Indonesia

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Journal : Indonesian Journal of Rheumatology

Avascular Osteonecrosis in Systemic Sclerosis Patient: Risk Factors and Role of Vasculopathy? Safarina Kharima Laitupa; Perdana Aditya Rahman
Indonesian Journal of Rheumatology Vol. 13 No. 2 (2021): Indonesian Journal of Rheumatology
Publisher : Indonesian Rheumatology Association

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.37275/ijr.v13i2.162

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.
Pathogenesis, Clinical Presentations and Diagnosis of IgG4-related Disease: A Review Perdana Aditya Rahman; Cesarius Singgih Wahono; Fajar Maulana Raharjo; Handono Kalim; Mokhamad Fahmi Rizki Syaban
Indonesian Journal of Rheumatology Vol. 13 No. 2 (2021): Indonesian Journal of Rheumatology
Publisher : Indonesian Rheumatology Association

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.37275/ijr.v13i2.189

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.
Single Case Experience of Immunosuppressant Administration to Systemic Sclerosis-ILD Patients with Aspergilloma Wilujeng Anggraini; Perdana Aditya Rahman
Indonesian Journal of Rheumatology Vol. 14 No. 1 (2022): Indonesian Journal of Rheumatology
Publisher : Indonesian Rheumatology Association

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.37275/ijr.v14i1.197

Abstract

Background: Systemic sclerosis (SSc) is a chronic autoimmune disease that still poses a great challenge to clinicians. SSc is characterized by immune dysregulation and progressive fibrosis that typically affects variable internal organ involvement such as lungs. Interstitial lung disease (ILD) is a common manifestation of SSc and a leading cause of death. The immunosuppressive drug is the main treatment to suppress the inflammation process in SSc. Case presentation: In this case we report a 40-year-old female to suffer ILD-SSc. According to High-Resolution Computed Tomography (HRCT) thorax, we found that it was interstitial lung disease with aspergilloma. She got methylprednisolone 3x8 mg and azathioprine 2x50 mg. At the end of the treatment, the patient showed improvement in her clinical condition and showed no worsening condition in the HRCT evaluation for her fungal aspergilloma. Conclusion: Systemic sclerosis (SSc) is a rare autoimmune disease involving the skin and internal organs. The immunosuppressive agent is still the drug of choice for most autoimmune diseases. Immunosuppressive may promote fungal growth and have been associated with increased risk in most serious fungal diseases including aspergilloma.