title: Systemic vasculitis during the course of systemic sclerosis: report of 12 cases and review of the literature.
abstract: Although the presence of antineutrophil cytoplasmic antibodies (ANCA) has been reported in patients with systemic sclerosis (SSc), the association of SSc and systemic vasculitis has rarely been described. We obtained information on cases of systemic vasculitis associated with SSc in France from the French Vasculitis Study Group and all members of the French Research Group on Systemic Sclerosis. We identified 12 patients with systemic vasculitis associated with SSc: 9 with ANCA-associated systemic vasculitis (AASV) and 3 with mixed cryoglobulinemia vasculitis (MCV). In all AASV patients, SSc was of the limited type. The main complication of SSc was pulmonary fibrosis. Only 2 patients underwent a D-penicillamine regimen before the occurrence of AASV. The characteristics of AASV were microscopic polyangiitis (n = 7) and renal limited vasculitis (n = 2). Anti-myeloperoxidase antibodies were found in 8 of the 9 patients. The Five Factor Score was above 1 in 3 of the 9 patients. Of the 3 patients with MCV, Sjögren syndrome was confirmed in 2. We compared our findings with the results of a literature review (42 previously reported cases of AASV with SSc). Although rare, vasculitis is a complication of SSc. AASV is the most frequent type, and its diagnosis can be challenging when the kidney is injured. Better awareness of this rare association could facilitate earlier diagnosis and appropriate management to reduce damage.
authors.affiliation: From the Department of Nephrology and Internal Medicine (TQ, PV), Hospital of Valenciennes, Valenciennes; Université Paris Descartes, National Reference Centre for Scleroderma and Systemic Vasculitis (LM, LG), Cochin Hospital, Paris; Department of Internal Medicine (PC), AP-HP, Hôpital Pitié-Salpêtrière, Paris; Department of Rheumatology (OM), Bichat Hospital, Paris; Department of Vascular Medicine (UMP), Saint-Joseph Hospital, Paris; and Department of Internal Medicine, National Reference Centre for Scleroderma (PYH, EH), Claude Huriez Hospital, University of Lille, Lille, France.
authors.forename: Thomas
authors.initials: T
authors.lastname: Quéméneur
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
authors.forename: Olivier
authors.initials: O
authors.lastname: Meyer
authors.forename: Ulrique
authors.initials: U
authors.lastname: Michon-Pasturel
authors.forename: Philippe
authors.initials: P
authors.lastname: Vanhille
authors.forename: Pierre-Yves
authors.initials: PY
authors.lastname: Hatron
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D018501:Antirheumatic Agents / Q000627:therapeutic use; D015415:Biomarkers / Q000032:analysis; D001706:Biopsy; D003449:Cryoglobulinemia / Q000188:drug therapy / Q000276:immunology; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005602:France; D006801:Humans; D008875:Middle Aged; D010396:Penicillamine / Q000627:therapeutic use; D012595:Scleroderma, Systemic / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D014657:Vasculitis / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology
chemical_list: D000305:Adrenal Cortex Hormones; D019268:Antibodies, Antineutrophil Cytoplasmic; D018501:Antirheumatic Agents; D015415:Biomarkers; D003520:Cyclophosphamide; D010396:Penicillamine