The clinical patterns of myalgia in children with familial Mediterranean fever.

OBJECTIVES: To study the frequency and clinical patterns of myalgia in a defined group of children with familial Mediterranean fever (FMF). METHODS: A prospective 4-year (September 1995-September 1999) study of children with FMF seen in the pediatric FMF clinic of Jordan University teaching hospital. Diagnosis of FMF was made according to published criteria. Once the diagnosis of FMF and myalgia was made, details about myalgia were collected by interview with the child and his/her parents and entered into a special study form. RESULTS: Of 264 children with FMF seen over the study period, 65 (25%) developed myalgia. Three clinical patterns of myalgia were identified: the spontaneous pattern, the exercise-induced pattern, and the protracted febrile myalgia syndrome (PFMS), seen in 8%, 81%, and 11% of patients, respectively. The three patterns differed in the severity of pain, height of fever, and duration of the episode. In 33 children with the exercise-induced myalgia, in which response to colchicine could be reliably assessed, a favorable response was achieved in 97%. Three children with the PFMS had a dramatic response to corticosteroids. CONCLUSIONS: Myalgia in children with FMF is common and can follow three different clinical patterns.

The acute scrotum in Arab children with familial Mediterranean fever.

Over a period of 7 years, among 175 boys under the age of 16 years with familial Mediterranean fever (FMF), 16 (9%) developed 28 episodes of scrotal swelling that was unilateral in 26 (93%) and bilateral in 2 (7%). Fever and pain were present in 15 (94%) children; fever was characterized by a gradual onset and pain was moderate in intensity. The episodes were self-limiting and lasted from 8 h to 5 days. Scrotal swelling was the presenting feature of FMF in 4 (25%) patients. Six (38%) children underwent surgery; the operative findings, available in 3, showed a normal testis and epididymis and inflammation of the tunica vaginalis. The self-limiting nature of the episodes lasting for a few days was similar to the clinical course of serositis seen in FMF. This strongly suggests that inflammation of the tunica vaginalis, resulting in scrotal swelling, is another feature of FMF serositis. The gradual onset of fever, pain, swelling, and recurrence in a boy of Mediterranean origin, especially in the presence of a relevant family history, strongly points toward the diagnosis of FMF and conservative management. Early diagnosis and prophylactic colchicine therapy are expected to avert recurrences, which may result in ischemic testicular necrosis and FMF nephropathy.

Familial Mediterranean fever in children: the expanded clinical profile.

The clinical picture of familial Mediterranean fever (FMF) has been appreciably expanded in the last 10 years. Over 8 years, we studied the expanded clinical profile of FMF in 476 children. Of these, 81% had abdominal pain, 41% chest pain, 42% arthritis, 12% severe myalgia, 12% skin manifestations, 4% scrotal swelling, 3% recurrent episodic fever, and one child (0.2%) developed recurrent hyperbilirubinaemia. Two (0.4%) children developed renal complications which were reversed by colchicine; however of 19 probands, 36 family members suffered from chronic renal failure. Our study indicates a familial predisposition to nephropathy in certain families with FMF. This study is the first to report the expanded clinical profile of FMF in a large group of Arab children, giving an opportunity to compare the findings with those in children with FMF in other ethnic groups, and to help in the study of genotype-phenotype correlation.