RESUMO
Anti-melanoma differentiation-associated gene 5 (MDA-5) antibody-positive dermatomyositis is a disease with a poor prognosis associated with rapid progressive interstitial pneumonia. Autoimmune diseases have occasionally been reported to occur after hematopoietic stem cell transplantation (HSCT). We experienced a case of anti-MDA-5 antibody-positive dermatomyositis after HSCT. In this case, a sufficient dose of cyclophosphamide could not be administered due to an impaired bone marrow function. We discuss the complications of autoimmune diseases after HSCT.
Assuntos
Doenças Autoimunes , Dermatomiosite , Transplante de Células-Tronco Hematopoéticas , Leucemia Mielogênica Crônica BCR-ABL Positiva , Humanos , Transplante de Medula Óssea/efeitos adversos , Doença Crônica , Dermatomiosite/complicações , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Leucemia Mielogênica Crônica BCR-ABL Positiva/complicações , Leucemia Mielogênica Crônica BCR-ABL Positiva/terapiaRESUMO
BACKGROUND: Familial mediterranean fever (FMF) is a single inherited autoinflammatory disease characterized by periodic fever with relatively short duration of 1 to 3 days and sterile serositis. Although the prevalence rate is highest in the Mediterranean coastal area, a large number of cases have been reported recently by genetic analysis by identification of MEFV (Mediterranean fever) which is responsible gene in Japan too. In outpatient department of rheumatology, diagnosis and treatment of FMF is performed in cases where fever and abdominal pain attack are repeated for a short period of time. PATIENTS AND METHODS: We examined cases in which symptoms considered periodic seizures were repeated, excluding autoimmune diseases, infectious diseases, and malignant tumors. In both cases, genetic analysis is performed as auxiliary diagnosis. RESULTS: Seven cases satisfied the Tel-Hashomer criteria criteria and MEFV gene mutation was detected. Everyone was a female, and half had seizure symptoms at menstruation. Even though there is a difference in the amount of colchicine to be used, either one is effective. CONCLUSION: In cases of periodic symptoms or cases called periodic fever, exclusion diagnosis is carried out, there is a need to suspect FMF, determine the effect of colchicine, and perform genetic analysis.