Patient with Recurrent Polyserositis (Familial Mediterranean Fever).
Familial Mediterranean fever (FMF) is a hereditary autosomal recessive ,systemic, auto-inflammatory disorder characterized by sporadic, unpredictable attacks of fever and serosal inflammation. FMF is caused by mutations in MEFV, a gene located on the short arm of chromosome 16 (16p13) which encodes a protein 'Pyrin'. The disorder has been given various names including familial paroxysmal polyserositis, periodic peritonitis, recurrent polyserositis, benign paroxysmal peritonitis, and periodic disease or periodic fever, As the name indicates, FMF occurs within families and is much more common in individuals of Mediterranean descent than in persons of any other ethnicity. It has been described in several ethnic groups including Sephardic Jews, Armenians, Turks, North Africans, Arabs, Greeks, and Italians. However, the disease is not restricted to these groups and sporadic cases have been reported. Diagnosis is usually clinical and it classically presents with unprovoked, recurrent attacks of fever and painful polyserositis mainly affecting the peritoneum (most common), synovium, and pleura that usually (but not always) begin in childhood. We present a atypical case of FMF with type 1 Diabetes Mellitus and FMF who had no fever, Mediterranean ancestory or family history and discuss his clinical features,diagnosis and management.
Senior consultant and Head, Department of Internal Medicine.
Arun
A
Agarwal
Consultant, Department of Pathology, Narayana Multispecialty Hospital, Jaipur, Rajasthan.
Samiksha
S
Sharma
D000293:Adolescent; D003078:Colchicine / Q000008:administration & dosage*; D003922:Diabetes Mellitus, Type 1 / Q000150:complications / Q000175:diagnosis; D010505:Familial Mediterranean Fever / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D006760:Hospitalization; D006801:Humans; D008297:Male; D010346:Patient Care Management / Q000379:methods*; D010998:Pleurisy / Q000000981:diagnostic imaging*; D013902:Radiography, Thoracic / Q000379:methods; D050257:Tubulin Modulators / Q000008:administration & dosage
D050257:Tubulin Modulators; D003078:Colchicine