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Vasculitis in familial Mediterranean fever
H Ozdogan1, N Arisoy, O Kasapçapur
1Department of Rheumatology, Cerrahpaşa Medical Faculty, University of Istanbul, Turkey.
Objective:
To evaluate the frequency of vasculitis, mainly in the forms of Henoch-Schönlein purpura and polyarteritis nodosa (PAN), and to investigate the presence of occult blood in the first stool specimens after an abdominal attack in Turkish patients with familial Mediterranean fever (FMF).
Methods:
Review of the charts of 207 patients with FMF seen between 1983 and 1993 with respect to clinical vasculitis. A prospective study designed to test the presence of occult blood in the first stool specimens obtained after abdominal attack and at least one week later in 36 patients with FMF compared with healthy and diseased controls.
Results:
There were 15 patients with Henoch-Schönlein purpura (7%), 2 with definite and one with probable PAN (1%), one of whom developed perirenal hematoma. The diagnosis of FMF was made after the onset of Henoch-Schönlein purpura in 9 and subsequent to the development of PAN in one patient. Occult blood was positive in the first stool specimens obtained after an attack in 17 of the 36 patients with FMF (47%), a finding not reported previously.
Conclusion:
Vasculitis seems to be an important but not a widely recognized feature of FMF.
Insights
Vasculitis, including Henoch-Schönlein purpura and polyarteritis nodosa, is a significant feature in Familial Mediterranean Fever (FMF). Occult blood was detected in nearly half of FMF patients after abdominal attacks.
Area of Science:
- Rheumatology
- Gastroenterology
- Pediatric Rheumatology
Background:
- Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder.
- Vasculitis is a known complication, but its frequency in FMF is not well-established.
- Henoch-Schönlein purpura (HSP) and polyarteritis nodosa (PAN) are common vasculitic conditions.
Observation:
- This study evaluated 207 Turkish patients with FMF for clinical vasculitis.
- A prospective component assessed occult blood in stool samples post-abdominal attack in 36 FMF patients.
- Controls included healthy individuals and patients with other diseases.
Findings:
- The study identified Henoch-Schönlein purpura in 7% and polyarteritis nodosa in 1% of FMF patients.
- Occult blood in stool was found in 47% of FMF patients after abdominal attacks, a novel finding.
- In some cases, FMF diagnosis followed the onset of vasculitis.
Implications:
- Vasculitis is an important, under-recognized manifestation of FMF.
- Screening for vasculitis and occult blood may be beneficial in FMF patients experiencing abdominal pain.
- Further research is warranted to understand the link between FMF and vasculitis.