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The arthritis of familial Mediterranean fever
A Garcia-Gonzalez1, M H Weisman
1Department of Medicine, University of California, San Diego 92103.
Abstract:
Familial Mediterranean fever (FMF) is a disease of unknown etiology and pathogenesis. In addition to fever, arthritis is among its most frequent manifestations. The arthritis of FMF is typically an acute, episodic, self-limited process with no sequelae. The radiographic features of FMF arthritis are usually limited to transient, often severe osteoporosis. Synovial fluid analysis many mimic septic arthritis with very high white blood cell counts; cultures are uniformly negative. The course of FMF is almost always benign, with no residual articular incapacity. Some patients, limited to certain ethnic groups, develop renal amyloidosis. Colchicine therapy modifies the natural history of the disease by decreasing the attack frequency and preventing amyloid deposition. At present, a lipocortin deficiency appears to be the likely candidate for a pathogenic mechanism. An unusual case with dramatic periarticular features (periostitis) and a protracted course with an excellent response to synovectomies is reported here. There is no explanation for the exuberant periarticular bone formation noted in this case, but a variety of recently discovered growth factors may be implicated.
Insights
Familial Mediterranean fever (FMF) typically causes episodic arthritis. An unusual FMF case presented with periostitis, responding well to synovectomies, suggesting potential growth factor involvement.
Area of Science:
- Rheumatology
- Genetics
- Internal Medicine
Background:
- Familial Mediterranean fever (FMF) is an autoinflammatory disorder of unknown cause.
- Arthritis is a common manifestation, usually acute, self-limited, and without lasting joint damage.
- Renal amyloidosis is a serious complication in some ethnic groups.
Observation:
- The typical radiographic findings in FMF arthritis include transient osteoporosis.
- Synovial fluid analysis can mimic septic arthritis but cultures are negative.
- This report details an unusual FMF case with significant periarticular ossification (periostitis).
Findings:
- The patient's unusual presentation involved protracted arthritis with dramatic periarticular bone formation.
- The condition responded well to synovectomies, a treatment not typically associated with FMF.
- The exact mechanism for the exuberant bone formation remains unclear, but growth factors are hypothesized.
Implications:
- This case expands the spectrum of FMF manifestations beyond typical arthritis.
- The response to synovectomy suggests novel therapeutic avenues for complex FMF cases.
- Further research into growth factor roles may elucidate FMF pathogenesis and guide future treatments.