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[Subacute and chronic joint involvement occurring during the course of familial Mediterranean fever (author's
Abstract:
Subacute and chronic joint involvement occur in less than 5 p. cent of patients with familial mediterranean fever, whereas acute joint lesions are observed in approximately 3 out of 4 cases. The various aspects of these atypical forms are discussed in relation to 10 such cases. In most of these patients the lesions were merely due to the prolongation of the acute process, presenting as a subacute mono-arthrites lasting for several months before regression. X Rays show only regional demineralisation, sometimes predominating in the subchondral zone. Symptoms totally regress without sequelae. A time destructive arthropathy may however develop, especially in the hip. The presentation associates limitation of joint motion with, on X Rays, joint space narrowing and osteophytes. In 2 of our cases and one case reported in the literature, there was a progressive development of a chronic caxopathy without any previous acute involvement of this joint. At last, in some cases, familial mediterranean fever may be associated with a known arthritidis, usually ankylosing spondylitis.
Insights
Familial Mediterranean Fever (FMF) rarely causes chronic joint issues, but when it does, symptoms can include prolonged arthritis or destructive joint disease, especially in the hip. Most cases resolve without lasting damage.
Area of Science:
- Rheumatology
- Internal Medicine
- Genetics
Background:
- Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder.
- Acute joint involvement is common in FMF, affecting up to 75% of patients.
- Chronic and subacute joint manifestations are less frequent, occurring in under 5% of cases.
Purpose of the Study:
- To describe the atypical subacute and chronic joint manifestations in Familial Mediterranean Fever.
- To analyze the clinical and radiological features of these rare joint involvements.
- To discuss the differential diagnosis and potential long-term outcomes of joint disease in FMF.
Main Methods:
- Retrospective case series analysis of 10 patients with FMF and atypical joint involvement.
- Review of clinical presentations, including duration, affected joints, and symptoms.
- Radiographic evaluation (X-rays) to assess joint changes, including demineralization, joint space narrowing, and osteophytes.
Main Results:
- Subacute arthritis, often monoarticular and prolonged, was the most common atypical presentation.
- Radiographic findings included regional demineralization, particularly in the subchondral zone.
- Destructive arthropathy, especially hip coxopathy, developed in some patients, with joint space narrowing and osteophytes.
- Rare cases presented with chronic coxopathy without preceding acute episodes.
- Association with other arthritides like ankylosing spondylitis was noted in some instances.
Conclusions:
- Subacute and chronic joint involvement in FMF, though rare, can present as prolonged arthritis or destructive arthropathy.
- Radiological findings are typically indicative of inflammation and degenerative changes.
- While most acute lesions resolve without sequelae, chronic forms may lead to significant joint damage, particularly in the hip.
- FMF-associated arthropathy requires careful monitoring and differentiation from other rheumatic conditions.