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Familial Mediterranean fever--renal involvement by diseases other than amyloid
M Tekin1, F Yalçinkaya, N Tümer
1Ankara University Faculty of Medicine, Turkey.
Background:
In patients with familial Mediterranean fever (FMF) renal involvement is usually in the form of AA amyloidosis. There is increasing evidence that renal involvement may be due to diseases other than amyloid as well.
Methods:
Amongst 302 children with FMF we observed and followed 28 with typical clinical and laboratory features of vasculitis. The diagnosis of FMF was established according to the Tel Hashomer criteria.
Results:
Polyarteritis nodosa, protracted febrile attacks and Henoch-Schönlein purpura were diagnosed in 4, 13, and 11 patients, respectively. The presentation was often difficult to distinguish from FMF attacks, but protracted febrile attacks lasting several weeks, hypertension, thrombocytosis, and dramatic responses to corticosteroid therapy that were observed in many cases were different from what is observed in classical FMF.
Conclusions:
We suggest that FMF, perhaps as a consequence of impaired control of inflammatory responses, predisposes to vasculitis with renal involvement.
Insights
Familial Mediterranean fever (FMF) may predispose children to vasculitis with renal involvement, beyond typical AA amyloidosis. Early recognition and treatment are crucial for managing these complex cases.
Area of Science:
- Pediatric Rheumatology
- Nephrology
- Genetics
Background:
- Familial Mediterranean fever (FMF) commonly causes renal AA amyloidosis.
- Emerging evidence suggests FMF patients may develop other renal diseases.
- Understanding these alternative renal manifestations is critical for comprehensive patient care.
Purpose of the Study:
- To investigate non-amyloid renal involvement in children with FMF.
- To characterize the clinical features and outcomes of vasculitis in FMF patients.
- To explore the potential link between FMF and the development of vasculitis.
Main Methods:
- Retrospective analysis of 302 children diagnosed with FMF.
- Detailed observation and follow-up of 28 FMF patients exhibiting vasculitis features.
- Diagnosis of FMF confirmed using the Tel Hashomer criteria.
Main Results:
- Identified 28 FMF patients with vasculitis, including Polyarteritis nodosa (4), protracted febrile attacks (13), and Henoch-Schönlein purpura (11).
- Observed distinct clinical features such as prolonged febrile episodes, hypertension, and thrombocytosis in these patients.
- Noted significant improvement with corticosteroid therapy, differentiating from typical FMF presentations.
Conclusions:
- Familial Mediterranean fever may increase susceptibility to vasculitis with renal complications.
- Impaired inflammatory response control in FMF could be a contributing factor.
- This association highlights the need for vigilance for vasculitis in FMF patients.