1. Autoimmune cytopenias associated with autoantibodies to nuclear envelope polypeptides
- Author
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Coppo, Paul, Clauvel, Jean-Pierre, Bengoufa, Djaouida, Fuentes, Vincent, Gouilleux-Gruart, Valérie, Courvalin, Jean-Claude, Lassoued, Kaiss, Gouilleux-Gruart, Val�rie, Université Pierre et Marie Curie - Paris 6 (UPMC), Laboratoire Jean Dausset, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Développement normal et pathologique des lymphocytes et signalisation, Université de Picardie Jules Verne (UPJV)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Institut Jacques Monod (IJM (UMR_7592)), Université Paris Diderot - Paris 7 (UPD7)-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Université de Picardie Jules Verne (UPJV)-Institut National de la Santé et de la Recherche Médicale (INSERM), and Université de Picardie Jules Verne (UPJV)-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)
- Subjects
Adult ,Male ,Vasculitis ,Hemolytic anemia ,Pathology ,medicine.medical_specialty ,Neutropenia ,Adolescent ,Anti-nuclear antibody ,Nuclear Envelope ,Pancytopenia ,[SDV]Life Sciences [q-bio] ,Immunoblotting ,medicine.disease_cause ,Autoimmune Diseases ,Liver disorder ,Autoimmunity ,03 medical and health sciences ,0302 clinical medicine ,Coombs test ,Autoimmune Process ,hemic and lymphatic diseases ,medicine ,Humans ,Aged ,Autoantibodies ,Retrospective Studies ,030304 developmental biology ,0303 health sciences ,Systemic lupus erythematosus ,medicine.diagnostic_test ,business.industry ,Liver Diseases ,Autoantibody ,Nuclear Proteins ,Hematology ,Middle Aged ,medicine.disease ,3. Good health ,Coombs Test ,Treatment Outcome ,Purpura, Thrombocytopenic ,030220 oncology & carcinogenesis ,Immunology ,Female ,Anemia, Hemolytic, Autoimmune ,Peptides ,business ,Follow-Up Studies - Abstract
International audience; 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.
- Published
- 2004