title: Systemic vasculitis associated with vemurafenib treatment: Case report and literature review.
abstract: BACKGROUND
  Vemurafenib, an inhibitor of mutated B-rapidly accelerated fibrosarcoma, is frequently used in the treatment of melanoma and Erdheim-Chester disease (ECD) patients. Inflammatory adverse effects have been increasingly reported after vemurafenib treatment.
  
  
  METHODS
  We report 6 cases of vemurafenib-associated vasculitis, of whom a personal case of a 75-year-old man with history of ECD who developed purpura and rapidly progressive pauci-immune glomerulonephritis during treatment with vemurafenib.
  
  
  METHODS
  In the 5 others cases from the literature, all patients presented skin vasculitis, and with joint involvement in 60% of them. Vemurafenib treatment was stopped (n = 3), continued at reduced doses (n = 1), or continued at the same dose (n = 2).
  
  
  RESULTS
  Three patients (50%) received corticosteroids combined with cyclophosphamide (n = 1), and all achieved remission of vasculitis. One patient experienced vasculitis relapse after vemurafenib therapy was restarted.
  
  
  CONCLUSIONS
  Systemic vasculitis is a rare vemurafenib-associated adverse event that may be life-threatening.
authors.affiliation: APHP, Service de Médecine Interne et Immunologie clinique, Groupe Hospitalier Pitié-Salpêtrière DHU Inflammation, Immunopathologie, Biothérapie, Université Pierre et Marie Curie APHP, Service de Dermatologie, Groupe Hospitalier Pitié-Salpêtrière APHP, Service de Néphrologie et Dialyse, Hôpital Tenon APHP, Service d'Anatomopathologie, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
authors.forename: Adrien
authors.initials: A
authors.lastname: Mirouse
authors.forename: Léa
authors.initials: L
authors.lastname: Savey
authors.forename: Fanny
authors.initials: F
authors.lastname: Domont
authors.forename: Cloé
authors.initials: C
authors.lastname: Comarmond
authors.forename: Stéphane
authors.initials: S
authors.lastname: Barete
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Plaisier
authors.forename: Philippe
authors.initials: P
authors.lastname: Rouvier
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
authors.forename: David
authors.initials: D
authors.lastname: Saadoun
mesh_terms: D000368:Aged; D000970:Antineoplastic Agents / Q000009:adverse effects* / Q000627:therapeutic use; D031249:Erdheim-Chester Disease / Q000188:drug therapy; D006801:Humans; D007211:Indoles / Q000009:adverse effects* / Q000627:therapeutic use; D008297:Male; D013449:Sulfonamides / Q000009:adverse effects* / Q000627:therapeutic use; D056647:Systemic Vasculitis / Q000139:chemically induced*; D000077484:Vemurafenib
chemical_list: D000970:Antineoplastic Agents; D007211:Indoles; D013449:Sulfonamides; D000077484:Vemurafenib