title: The Clinical Relevance of Antifibrillarin (anti-U3-RNP) Autoantibodies in Systemic Sclerosis.
abstract: Systemic sclerosis (SSc) is a heterogeneous autoimmune disease associated with several antinuclear autoantibodies useful to diagnosis and prognosis. The aim of the present multicentric study was to determine the clinical relevance of antifibrillarin autoantibodies (AFA) in patients with SSc. The clinical features of 37 patients with SSc positive for AFA (AFA+) and 139 SSc patients without AFA (AFA-) were collected retrospectively from medical records to enable a comparison between AFA- and AFA+ patients. Antifibrillarin autoantibodies were screened by an indirect immunofluorescence technique using HEp2 cells and identified by an in-house Western blot technique and/or an EliA test. Comparing AFA+ and AFA- patients, AFA+ patients were significantly younger at disease onset (36.9 versus 42.9; P = 0.02), more frequently male (P = 0.02) and of Afro-Caribbean descent (65% versus 7.7%; P < 0.001). At diagnosis, the Rodnan skin score evaluating the cutaneous manifestations was higher (13.3 versus 8.7; P = 0.01) and myositis was also more common in the AFA+ group (31.4% versus 12.2%; P < 0.01). Patients with AFA+ were not associated with diffuse cutaneous SSc or with lung involvement and no difference in survival was observed. Antifibrillarin autoantibodies are associated with patients of Afro-Caribbean origin and can identify patients with SSc who are younger at disease onset and display a higher prevalence of myositis.
authors.affiliation: Immunology Department, AP-HP Saint-Antoine Hospital, Paris, France.
authors.forename: F
authors.initials: F
authors.lastname: Tall
authors.affiliation: Immunology Department, Lyon-Sud Hospital, Hospices Civils de Lyon; Claude Bernard, Pierre-Benite, France.
authors.forename: M
authors.initials: M
authors.lastname: Dechomet
authors.affiliation: Internal Medicine Department, AP-HP Saint-Antoine Hospital, Paris, France.
authors.forename: S
authors.initials: S
authors.lastname: Riviere
authors.affiliation: Respiratory Diseases Department, Louis Pradel Hospital, Bron, France.
authors.forename: V
authors.initials: V
authors.lastname: Cottin
authors.affiliation: Immunology Department, AP-HP Saint-Antoine Hospital, Paris, France.
authors.forename: E
authors.initials: E
authors.lastname: Ballot
authors.affiliation: Internal Medicine Department, AP-HP Saint-Antoine Hospital, Paris, France.
authors.forename: K P
authors.initials: KP
authors.lastname: Tiev
authors.affiliation: Immunology Department, AP-HP Saint-Antoine Hospital, Paris, France.
authors.forename: R
authors.initials: R
authors.lastname: Montin
authors.affiliation: Internal Medicine Department, AP-HP Saint-Antoine Hospital, Paris, France.
authors.forename: C
authors.initials: C
authors.lastname: Morin
authors.affiliation: Immunology Department, AP-HP Saint-Antoine Hospital, Paris, France.
authors.forename: Y
authors.initials: Y
authors.lastname: Chantran
authors.affiliation: Internal Medicine Department, Lyon-Sud Hospital, Pierre-Bénite, France.
authors.forename: C
authors.initials: C
authors.lastname: Grange
authors.affiliation: Dermatology Department, Edouard Herriot Hospital, Lyon, France.
authors.forename: D
authors.initials: D
authors.lastname: Jullien
authors.affiliation: Internal Medicine Department, Edouard Herriot Hospital, Lyon, France.
authors.forename: J
authors.initials: J
authors.lastname: Ninet
authors.affiliation: Immunology Department, AP-HP Bicêtre Hospital, Kremlin Bicêtre, France.
authors.forename: P
authors.initials: P
authors.lastname: Chretien
authors.affiliation: Internal Medicine Department, AP-HP Saint-Antoine Hospital, Paris, France.
authors.forename: J
authors.initials: J
authors.lastname: Cabane
authors.affiliation: Immunology Department, Lyon-Sud Hospital, Hospices Civils de Lyon; Claude Bernard, Pierre-Benite, France.|University Lyon I, University of Lyon, Pierre-Bénite, France.
authors.forename: N
authors.initials: N
authors.lastname: Fabien
authors.affiliation: Immunology Department, AP-HP Saint-Antoine Hospital, Paris, France.|UFR 967, Faculte de medecine, University Pierre et Marie Curie, Paris6, France.
authors.forename: C
authors.initials: C
authors.lastname: Johanet
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D002460:Cell Line; D002868:Chromosomal Proteins, Non-Histone / Q000276:immunology*; D005006:Ethnicity; D005260:Female; D005602:France; D006801:Humans; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000175:diagnosis / Q000276:immunology; D015995:Prevalence; D012189:Retrospective Studies; D020718:Ribonucleoproteins, Small Nucleolar / Q000276:immunology; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000276:immunology*; D016019:Survival Analysis
chemical_list: D001323:Autoantibodies; D002868:Chromosomal Proteins, Non-Histone; D020718:Ribonucleoproteins, Small Nucleolar; C047458:fibrillarin; C117377:ribonucleoprotein, U3 small nucleolar