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

Salam Abbara; Jean-Benoit Monfort; Léa Savey; Philippe Moguelet; David Saadoun; Claude Bachmeyer; Olivier Fain; Benjamin Terrier; Zahir Amoura; Alexis Mathian; Laurent Gilardin; David Buob; Chantal Job-Deslandre; Jean-François Dufour; Rebecca Sberro-Soussan; Gilles Grateau; Sophie Georgin-Lavialle

doi:10.3389/fmed.2022.1000167

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

Front Med (Lausanne). 2022 Oct 28:9:1000167. doi: 10.3389/fmed.2022.1000167. eCollection 2022.

Authors

Salam Abbara¹, Jean-Benoit Monfort², Léa Savey¹, Philippe Moguelet³, David Saadoun⁴, Claude Bachmeyer¹, Olivier Fain⁵, Benjamin Terrier⁶, Zahir Amoura⁷, Alexis Mathian⁷, Laurent Gilardin⁸, David Buob³, Chantal Job-Deslandre⁹, Jean-François Dufour¹⁰, Rebecca Sberro-Soussan¹¹, Gilles Grateau¹, Sophie Georgin-Lavialle^{1

12}

Affiliations

¹ Département de Médecine Interne, Centre de Référence des Maladies Auto-Inflammatoires et des Amyloses d'Origine Inflammatoire (CEREMAIA), Hôpital Tenon, Sorbonne Université, AP-HP, Paris, France.
² Département de Dermatologie, Hôpital Tenon, Sorbonne Université, AP-HP, Paris, France.
³ Département d'Anatomo-Pathologie, Hôpital Tenon, Sorbonne Université, AP-HP, Paris, France.
⁴ Département de Médecine Interne et Immunologie Clinique, Centre National de Référence Maladies Autoimmunes Systémiques Rares, Centre National de Référence Maladies Autoinflammatoires et Amylose Inflammatoire, INSERM UMR_S 959, Immunologie-Immunopathologie-Immunotherapie, i3 and Département Hospitalo-Universitaire Inflammation-Immunopathologie-Biothérapie i2B, Groupe Hospitalier Pitié-Salpêtrière, Sorbonne Université, AP-HP, Paris, France.
⁵ Service de Médecine Interne, Hôpital Saint-Antoine, Sorbonne Université, APHP, Paris, France.
⁶ Service de Médecine Interne, Centre de Référence Maladies Systémiques et Autoimmunes Rares d'Ile de France, Hôpital Cochin, Université Paris Cité, AP-HP, Paris, France.
⁷ Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Groupement Hospitalier Pitié-Salpêtrière, Sorbonne Université, AP-HP, Paris, France.
⁸ Département de Médecine Interne et Immunologie Clinique, Groupe Hospitalier Pitié-Salpêtrière, Sorbonne Université, AP-HP, Paris, France.
⁹ Service de Pédiatrie, Immunologie, Hématologie et Rhumatologie, Centre de Référence pour les Rhumatismes Inflammatoires et les Maladies Auto-Immunes Systémique Rare de l'Enfant (RAISE), Hôpital Necker-Enfants Malades, AP-HP, Université Paris Cité, Paris, France.
¹⁰ Service Médecine Interne, Hôpital Nord-Ouest, Centre Hospitalier Villefranche sur Saône, Gleize, France.
¹¹ Service de Transplantation Rénale Adulte, Hôpital Necker-Enfants Malades, AP-HP, Université Paris Cité, Paris, France.
¹² INSERM U938, Centre de Recherche Saint-Antoine (CRSA), Paris, France.

Abstract

Objective: The 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.

Methods: Patients with vasculitis were selected from patients followed for FMF in the French JIR-cohort.

Results: Twenty-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.

Conclusion: This 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.

Keywords: Behçet syndrome; IgA vasculitis; anti-neutrophil cytoplasmic antibody-associated vasculitis; familial Mediterranean fever; polyarteritis nodosa; pyrin; vasculitis.