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Polyarteritis nodosa in patients with Familial Mediterranean Fever (FMF): a concomitant disease or a feature of FMF?
S Ozen1, E Ben-Chetrit, A Bakkaloglu
1Department of Pediatrics, Hacettepe University, Ankara, Turkey. sozen@gen.hun.edu.tr
Background:
Familial Mediterranean Fever (FMF) is caused by mutations in the gene encoding pyrin and is characterized by self-limited, recurrent attacks of fever and serositis. Vasculitis has been increasingly reported in FMF. A study evaluating the prognosis in FMF and polyarteritis nodosa (PAN) patients has not been reported previously.
Objectives:
To determine the special characteristics and the prognosis of PAN in FMF patients.
Methods:
A questionnaire was used for the present survey. The setting was 7 referral centers from Turkey and Israel. Seventeen patients who were diagnosed with FMF and who developed PAN were included. PAN was diagnosed in those who met the Chapel Hill consensus criteria for microscopic polyarteritis or classic PAN. The clinical features of these 17 patients and the outcomes of their vasculitis were analyzed.
Results:
The age at diagnosis of PAN in these FMF patients ranged from 3.5 to 37 years. All patients had constitutional symptoms, elevated acute phase reactants, and myalgia at the time PAN was diagnosed. The diagnosis of PAN was confirmed by renal angiography in 8 patients, by renal biopsy in 6 patients, and by muscle and/or nodule biopsies in 6 patients. A number of patients had definite features of both classic PAN and microscopic polyarteritis.
Conclusions:
When compared with other PAN patients, those with FMF tended to have a younger age at PAN onset, more frequent perirenal hematomas, and an overall better prognosis. The cases with overlapping features of microscopic and classic PAN pose a problem for the current classification of vasculitis. We suggest that the clinical representation of PAN in FMF patients has certain characteristics and may be a feature of FMF per se.
Insights
Patients with Familial Mediterranean Fever (FMF) who develop polyarteritis nodosa (PAN) show distinct characteristics and a better prognosis compared to other PAN patients. This suggests PAN in FMF may be a unique manifestation of the disease.
Area of Science:
- Rheumatology
- Genetics
- Internal Medicine
Background:
- Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder caused by pyrin gene mutations, presenting with recurrent fever and serositis.
- Vasculitis, specifically polyarteritis nodosa (PAN), is increasingly observed in FMF patients.
- No prior studies have evaluated the prognosis of PAN in FMF patients.
Purpose of the Study:
- To investigate the unique clinical features and prognosis of polyarteritis nodosa (PAN) in patients with Familial Mediterranean Fever (FMF).
Main Methods:
- A survey involving 17 patients diagnosed with both FMF and PAN across 7 referral centers in Turkey and Israel.
- PAN diagnosis was confirmed using Chapel Hill consensus criteria for microscopic polyarteritis or classic PAN.
- Analysis of clinical presentations and vasculitis outcomes, with diagnostic confirmation via angiography, biopsy, or both.
Main Results:
- PAN onset in FMF patients occurred between 3.5 and 37 years of age.
- All patients exhibited constitutional symptoms, elevated acute phase reactants, and myalgia at PAN diagnosis.
- Diagnostic confirmation involved renal angiography (8 patients), renal biopsy (6 patients), and muscle/nodule biopsies (6 patients); some cases showed features of both classic PAN and microscopic polyarteritis.
Conclusions:
- FMF patients with PAN tend to have an earlier onset, more frequent perirenal hematomas, and a better prognosis than other PAN cohorts.
- Overlapping features of classic and microscopic PAN in FMF cases challenge current vasculitis classification.
- The distinct clinical presentation suggests PAN in FMF may represent a specific characteristic of FMF itself.