Coexistence of Mixed Connective Tissue Disease and Familial Mediterranean Fever in a Japanese Patient

We herein report a Japanese patient with familial Mediterranean fever (FMF) who developed the clinical manifestations of mixed connective tissue disease (MCTD) and Sjögren's syndrome. The patient was a 36-year-old woman presenting with a periodic short-duration (2-3 days) fever and pleural pain...

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Detalles Bibliográficos
Autores principales: Fujita, Yuya, Asano, Tomoyuki, Sato, Shuzo, Furuya, Makiko Yashiro, Temmoku, Jumpei, Matsuoka, Naoki, Kobayashi, Hiroko, Watanabe, Hiroshi, Suzuki, Eiji, Koga, Tomohiro, Endo, Yushiro, Kawakami, Atsushi, Migita, Kiyoshi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2019
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6709329/
https://www.ncbi.nlm.nih.gov/pubmed/30996171
http://dx.doi.org/10.2169/internalmedicine.2376-18
Descripción
Sumario:We herein report a Japanese patient with familial Mediterranean fever (FMF) who developed the clinical manifestations of mixed connective tissue disease (MCTD) and Sjögren's syndrome. The patient was a 36-year-old woman presenting with a periodic short-duration (2-3 days) fever and pleural pain. An Mediterranean fever (MEFV) gene analysis detected a complex allele mutation (P369S/R408Q) in exon 3 of the MEFV gene. Serological and clinical data showed the coexistence of MCTD and Sjögren's syndrome. Treatment with colchicine (1.0 mg/day) successfully eliminated febrile attack and pleuritis, leading to the diagnosis of FMF. Four months after the initiation of colchicine treatment, she presented with MCTD-related pulmonary artery hypertension. This is the first report of FMF coexisting with MCTD.

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