Association of Vasculitis and Familial Mediterranean Fever.

Salam Abbara,David Saadoun,Gilles Grateau,Stéphanie Ducharme-Bénard,Sophie Georgin-Lavialle

doi:10.3389/fimmu.2019.00763

Abstract

Certain types of vasculitis occur more frequently and present differently in patients with familial Mediterranean fever (FMF). We assessed the characteristics of patients with FMF and systemic vasculitis through a systematic review of the literature. Medline was searched by two independent investigators until December 2017. We screened 310 articles and selected 58 of them (IgA vasculitis n = 12, polyarteritis nodosa (PAN) n = 25, Behçet's disease (BD) n = 7, other vasculitis n = 14). Clinical case reports were available for 167 patients (IgA vasculitis n = 46, PAN n = 61, BD n = 46, other vasculitis n = 14), and unavailable for 45 patients (IgA vasculitis n = 38, PAN n = 7). IgA vasculitis was the most common vasculitis in FMF patients with a prevalence of 2.7–7%, followed by PAN with a prevalence of 0.9–1.4%. Characteristics of FMF did not differ between patients with and without vasculitis. Patients with FMF and IgA vasculitis displayed more intussusception (8.7%) and possibly less IgA deposits on histological analysis than patients with IgA vasculitis alone. Patients with FMF and PAN had a younger age at vasculitis onset (mean age = 17.9 years), as well as more perirenal hematomas (49%) and CNS involvement (31%) than patients with PAN alone. Glomerular involvement was noted in 33% of patients diagnosed with PAN, suggesting an alternative diagnosis. Sequencing of the MEFV gene confirmed the presence of two pathogenic variants in 73% of FMF patients with IgA vasculitis or PAN. The majority of patients with BD were from one case series, and presented more skin, gastrointestinal, and CNS involvement than patients with isolated BD. In conclusion, FMF, particularly when supported by two pathogenic MEFV mutations, could predispose to IgA vasculitis, or a PAN-like vasculitis with more perirenal bleeding and CNS involvement.

Highlights

Familial Mediterranean fever (FMF) is the most common hereditary autoinflammatory disease, usually affecting people of Mediterranean descent [1]
The following data were recorded from the articles when available: diagnostic criteria for vasculitis, study population, number of cases of FMF-associated vasculitis, age, sex, ethnicity, family history, comorbid diseases, age at FMF and vasculitis onset, age at FMF and vasculitis diagnosis, presence or absence of Mediterranean fever (MEFV) mutation, age at colchicine initiation, mean colchicine dose, current colchicine dose, clinical presentation of FMF and vasculitis, history of treatment for FMF and vasculitis, activity of FMF under treatment based on symptoms and C-reactive protein, therapeutic response of vasculitis at last follow-up, presence or absence of amyloidosis, renal function, and variation of C-reactive protein, and serum amyloid A under treatment
We confirmed that IgA vasculitis is the most prevalent vasculitis in FMF patients with a prevalence of 2.7–7%, followed by polyarteritis nodosa (PAN) with a prevalence of 0.9–1.4%

Summary

INTRODUCTION

Familial Mediterranean fever (FMF) is the most common hereditary autoinflammatory disease, usually affecting people of Mediterranean descent [1]. The following data were recorded from the articles when available: diagnostic criteria for vasculitis, study population, number of cases of FMF-associated vasculitis, age, sex, ethnicity, family history, comorbid diseases, age at FMF and vasculitis onset, age at FMF and vasculitis diagnosis, presence or absence of MEFV mutation, age at colchicine initiation, mean colchicine dose, current colchicine dose, clinical presentation of FMF and vasculitis, history of treatment for FMF and vasculitis, activity of FMF under treatment based on symptoms and C-reactive protein, therapeutic response of vasculitis at last follow-up (remission, partial response, or death), presence or absence of amyloidosis, renal function, and variation of C-reactive protein, and serum amyloid A under treatment. A 2-sided p-value

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CONCLUSION