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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.

Author information

1
General Clinical Training Center Niigata University Medical and Dental Hospital Niigata Japan.
2
Division of Clinical Nephrology and Rheumatology Niigata University Graduate School of Medical and Dental Sciences Niigata Japan.
3
Health Administration Center Niigata University Niigata Japan.
4
School of Health Sciences, Medical Laboratory Science, Faculty of Medicine Niigata University Niigata Japan.

Abstract

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.

KEYWORDS:

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

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