Vasculitis and familial Mediterranean fever: Description of 22 French adults from the juvenile inflammatory rheumatism cohort

Abstract

ObjectiveThe frequency of vasculitis may be increased in patients with Familial Mediterranean Fever (FMF), according to several studies. Our aim was to assess the characteristics of French adult patients with both diseases.MethodsPatients with vasculitis were selected from patients followed for FMF in the French JIR-cohort.ResultsTwenty-two patients were included [polyarteritis nodosa (PAN) n = 10, IgA vasculitis n = 8, unclassified vasculitis n = 2, granulomatosis with polyangiitis n = 1, and microscopic polyangiitis n = 1]. Pathogenic mutations in exon 10 were found in all 21 patients (96%) for which MEFV testing results were available, and 18 (82%) had two pathogenic mutations. Histology showed vasculitis in 59% of patients. Most patients with FMF-associated PAN were HBV-negative and had an inactive FMF before PAN onset, and 40% had a peri-renal or central nervous system bleeding. Most patients with FMF-associated IgA vasculitis had an active FMF before vasculitis onset, and 25% had digestive bleeding. Both patients with unclassified vasculitis had ischemic and/or hemorrhagic complications.ConclusionThis study confirms the predominance of PAN and IgA vasculitis in patients with FMF and the high frequency of bleeding in FMF-associated PAN. FMF should be considered in case of persistent symptoms and/or inflammatory syndrome despite vasculitis treatment in Mediterranean patients.

Cutaneous vasculitis in autoinflammatory diseases

“…29,30 Clinical manifestations similar to those in idiopathic IgA vasculitis without FMA showed purpura (100%) [25][26][27][28][29][30][31][32] on the legs in all patients, followed by renal involvement (52% to 80%), abdominal pain (50% to 71.7%), and arthralgia/arthritis (33% to 71.7%); however, these patients revealed a higher complication of intussusception (8.7%) and possibly less IgA vascular deposit than patients with idiopathic IgA vasculitis. 29,30…”

“…[25][26][27]29,30 A recent French study of FMF vasculitis in 22 patients also revealed that both PAN (10 of 22 [46%]) and IgA vasculitis (8 of 22 [36%]) are the most frequent forms of vasculitis in FMF, with a higher frequency for FMF-associated PAN than FMF-associated IgA vasculitis. 30…”

“…Numerous reports suggest a higher frequency of vasculitis in patients with FMF compared with the general population 25–30 . Cutaneous vasculitis in FMF, presenting as: (1) FMF‐associated IgA vasculitis, a dermal small‐vessel vasculitis; and (2) FMF‐associated polyarteritis nodosa (PAN), a dermosubcutaneous to subcutaneous medium‐sized arteritis, are the most frequent forms of vasculitis in FMF 25–27,29,30 .…”

“…Renal involvement (49%) in FMF-associated PAN characterized by suspected glomerular involvement (34%), perirenal hematoma (49%), and higher central nervous system involvement (31%) present as the striking differences with idiopathic PAN sparing glomerular and perirenal involvement. 29,30 Systemic corticosteroids were the most often used agents for treating vasculitis in patients with FMF.…”

“…16 Numerous reports suggest a higher frequency of vasculitis in patients with FMF compared with the general population. [25][26][27][28][29][30] Cutaneous vasculitis in FMF, presenting as: (1) FMF-associated IgA vasculitis, a dermal small-vessel vasculitis; and (2) FMF-associated polyarteritis nodosa (PAN), a dermosubcutaneous to subcutaneous medium-sized arteritis, are the most frequent forms of vasculitis in FMF. [25][26][27]29,30 A recent French study of FMF vasculitis in 22 patients also revealed that both PAN (10 of 22 [46%]) and IgA vasculitis (8 of 22 [36%]) are the most frequent forms of vasculitis in FMF, with a higher frequency for FMF-associated PAN than FMF-associated IgA vasculitis.…”