Modern rheumatology
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Modern rheumatology · Mar 2019
ReviewA benefit and the prospects of IL-6 inhibitors in idiopathic multicentric Castleman's disease.
Castleman's disease (CD), a heterogeneous group of lymphoproliferative disorders, is divided into unicentric CD (UCD) and multicentric CD (MCD) based on the number of regions of enlarged lymph nodes with characteristic histopathologic features. The clinical pictures and treatment differ greatly between these UCD and MCD. In MCD, cases of human herpesvirus 8-negative patients with unknown etiology are defined as idiopathic MCD (iMCD). ⋯ However, an IL-6 inhibitor is not effective for all iMCD cases, and refractory cases occur despite these treatments. In this review, we briefly summarize the role of IL-6 in iMCD, and we discuss the efficacy that has been reported for tocilizumab (TCZ), the anti-IL-6 receptor antibody, for patients with iMCD in Japan. Factors predicting the therapeutic response to IL-6 remain to be identified, and the verification of the long-term safety of IL-6 inhibition is needed.
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Modern rheumatology · Mar 2019
Efficacy and safety of interleukin-1 inhibitors in familial Mediterranean fever patients complicated with amyloidosis.
Colchicine is the mainstay of the treatment of familial Mediterranean fever (FMF). However, 10% of FMF patients do not respond well to colchicine. Efficacy of interleukin (IL)-1 inhibitors in reducing attacks have been demonstrated in colchicine-resistant FMF (crFMF) patients recently. Colchicine is still the only approved drug for the prevention of amyloidosis in FMF and utility of IL-1 inhibitors in crFMF cases who already has amyloidosis remain to be elucidated. Herein, we evaluated efficacy and safety of IL-1 inhibitors in patients with crFMF-associated AA amyloidosis in a relatively large single center study. ⋯ We evaluated the clinical and laboratory responses to IL-1 inhibitors in crFMF-associated amyloidosis patients. We found significant decreases in CRP, ESR and proteinuria after IL-1 inhibitor therapy. This study confirmed that IL-1 inhibitors are effective for controlling attacks and inflammatory activity in FMF patients complicated with AA amyloidosis. Moreover, they reduce or stabilize amount of proteinuria and preserve renal function in short-term follow-up. Prolonged prospective clinical trials are warranted to assess their long-term efficacy in this particular patient group.
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Modern rheumatology · Jan 2019
Practice Guideline2017 Clinical practice guidelines of the Japan Research Committee of the Ministry of Health, Labour, and Welfare for Intractable Vasculitis for the management of ANCA-associated vasculitis.
The Japan Research Committee for Intractable Vasculitis has fully revised the clinical practice guidelines (CPG) for the management of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) to improve and standardize the medical treatment of the disease in Japan. ⋯ The revised CPG has demonstrated evidence-based treatment recommendations for AAV.
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Modern rheumatology · Sep 2018
Autologous haematopoietic stem cell transplantation for Japanese patients with systemic sclerosis: Long-term follow-up on a phase II trial and treatment-related fatal cardiomyopathy.
The objective of this study is to elucidate the efficacy and safety of autologous haematopoietic stem cell transplantation (HSCT) for Japanese patients with systemic sclerosis (SSc). ⋯ HSCT is a feasible treatment bringing favourable results to more than half of our patients with SSc. Careful selection of the patients is essential for whom benefited from HSCT, considering the risk-benefit balance of the treatment.
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Modern rheumatology · Jul 2018
Randomized Controlled Trial Multicenter StudyEfficacy and safety of baricitinib in Japanese patients with rheumatoid arthritis: Subgroup analyses of four multinational phase 3 randomized trials.
To evaluate efficacy/safety of baricitinib for rheumatoid arthritis (RA) in Japanese subpopulations from four phase 3 studies, and assess whether results in these subpopulations are consistent with the overall study populations. ⋯ Data for baricitinib, with/without methotrexate, in Japanese subpopulations across all stages of the RA treatment continuum accord with the efficacy/safety profile in overall study populations. Baricitinib appears to be similarly effective in Japanese patients.