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Clin Case Rep. 2019 Mar 12;7(4):801-805. doi: 10.1002/ccr3.2102. eCollection 2019 Apr.

An adult case of atypical familial Mediterranean fever (pyrin-associated autoinflammatory disease) similar to adult-onset Still's disease.

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General Clinical Training Center Niigata University Medical and Dental Hospital Niigata Japan.
Division of Clinical Nephrology and Rheumatology Niigata University Graduate School of Medical and Dental Sciences Niigata Japan.
Health Administration Center Niigata University Niigata Japan.
School of Health Sciences, Medical Laboratory Science, Faculty of Medicine Niigata University Niigata Japan.


We present a 55-year-old woman with periodic fever and symptoms similar to adult-onset Still's disease (AOSD). She had a heterogeneous mutation of the MEFV gene and colchicine was effective. Atypical familial Mediterranean fever (pyrin-associated autoinflammatory disease) should be considered in patients with periodic fever accompanied by symptoms similar to AOSD.


Familial Mediterranean fever; adult‐onset Still’s disease; elevated liver enzymes; pyrin‐associated autoinflammatory diseases; skin rash

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