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Protracted arthritis in familial Mediterranean fever
Abstract:
A review of the files of familial Mediterranean fever (FMF) confirmed the rarity of patients suffering protracted arthritic attacks and the propensity of the joints, in general, to recover. While 70% of those afflicted suffered bouts of synovitis, only 57 patients (5% of the FMF-population) experienced protracted attacks involving a total of 84 joints, 36 of them knees and 25 hips. Functional and, usually, anatomical integrity was regained in all but 27 joints. Of the 27 joints producing residual incapacity, 21 were hips. Seven hips showed roentgenologically typical aseptic necrosis of the femoral head and 14 only sclerosis and narrowing of the joint space. Eight hips eventually required total prosthetic replacement. We suggest that the poor prognosis of the hip, in contrast to other joints affected by protracted FMF-arthritis, is related not directly to the metabolic aberrration underlying the disease but to attenuation of the arterial blood supply of the femoral head by synovial exudation. Early aspiration of exudate could alter the prognosis by preventing the complication of aseptic necrosis.
Insights
Familial Mediterranean Fever (FMF) rarely causes prolonged arthritis, with hips being most vulnerable. Early fluid removal may prevent hip damage and preserve joint function in FMF patients.
Area of Science:
- Rheumatology
- Genetics
- Orthopedics
Background:
- Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder.
- Arthritic attacks are common in FMF, but protracted joint involvement is rare.
- Most affected joints in FMF recover fully, but some may experience residual incapacity.
Purpose of the Study:
- To investigate the incidence and outcomes of protracted arthritic attacks in Familial Mediterranean Fever (FMF).
- To identify joints most susceptible to long-term damage in FMF patients.
- To explore potential interventions for improving joint prognoses in FMF.
Main Methods:
- Retrospective review of patient files diagnosed with Familial Mediterranean Fever (FMF).
- Analysis of joint involvement, duration of attacks, and functional recovery.
- Radiographic assessment and documentation of joint damage, including aseptic necrosis and joint space narrowing.
Main Results:
- Protracted arthritic attacks occurred in only 5% of FMF patients, affecting 84 joints.
- Hips were disproportionately affected, with 21 of 27 joints with residual incapacity being hips.
- Seven hips showed aseptic necrosis, and 14 had joint space narrowing; 8 required prosthetic replacement.
Conclusions:
- The hip joint has a poorer prognosis in protracted FMF arthritis compared to other joints.
- Synovial exudation potentially compromises blood supply to the femoral head, leading to aseptic necrosis.
- Early aspiration of synovial fluid may prevent hip complications and improve outcomes in FMF.