title: Autoimmune cytopenias associated with autoantibodies to nuclear envelope polypeptides.
abstract: A subset of anti-nuclear autoantibodies (ANA) are directed against nuclear envelope (NE) polypeptides and display by indirect immunofluorescence (IIF) a ring-like fluorescent pattern. We report herein 19 patients with autoimmune cytopenias associated with antibodies (Abs) to NE polypeptides. Anti-NE specificity was determined by immunoblot, using NE preparations and purified lamina fractions. Eleven sera reacted with lamin B(1), and two reacted with both lamin B(1) and an unidentified 150-kDa protein (p150). One serum reacted with only p150. Four sera reacted with lamins A and C, and one reacted with and an unidentified 52-kDa NE polypeptide (p52). Autoimmune cytopenias included hemolytic anemia (7 cases), thrombocytopenia (13 cases), and neutropenia (6 cases). Five patients had 2 (3 cases) or 3 (2 cases) different cytopenias. Antiphospholipid antibodies (APLA) were detected in 14 patients, 2 of whom experienced thromboembolic events. A liver disorder was present in 7 patients. Systemic lupus erythematosus and lupus-like syndrome were diagnosed in 11 and 2 patients, respectively. Cytopenias responded to steroids alone (13 patients), or together with intravenous immunoglobulins (2 patients), or cyclophosphamide (2 patients). Two patients did not require treatment. Our results suggest that anti-NE Abs need to be sought for in patients with peripheral cytopenias, particularly when they are associated with APLA and/or liver disorders. Their detection strongly suggests an autoimmune process. Such cytopenias are often manifestations of a lupus or lupus-like disease and are responsive to steroids.
authors.affiliation: Service d'Immuno-Hématologie, Hôpital Saint-Louis, Paris, France.
authors.forename: Paul
authors.initials: P
authors.lastname: Coppo
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Clauvel
authors.forename: Djaouida
authors.initials: D
authors.lastname: Bengoufa
authors.forename: Vincent
authors.initials: V
authors.lastname: Fuentes
authors.forename: Valérie
authors.initials: V
authors.lastname: Gouilleux-Gruart
authors.forename: Jean-Claude
authors.initials: JC
authors.lastname: Courvalin
authors.forename: Kaiss
authors.initials: K
authors.lastname: Lassoued
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D001323:Autoantibodies / Q000276:immunology*; D001327:Autoimmune Diseases / Q000150:complications / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D003298:Coombs Test; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015151:Immunoblotting; D008107:Liver Diseases / Q000150:complications / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009503:Neutropenia / Q000150:complications / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D009685:Nuclear Envelope / Q000276:immunology*; D009687:Nuclear Proteins / Q000276:immunology*; D010198:Pancytopenia / Q000150:complications / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D010455:Peptides / Q000276:immunology; D011696:Purpura, Thrombocytopenic / Q000150:complications / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D012189:Retrospective Studies; D016896:Treatment Outcome; D014657:Vasculitis / Q000150:complications / Q000473:pathology
chemical_list: D001323:Autoantibodies; D009687:Nuclear Proteins; D010455:Peptides