1. Autoimmune cytopenias associated with autoantibodies to nuclear envelope polypeptides.
- Author
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Coppo P, Clauvel JP, Bengoufa D, Fuentes V, Gouilleux-Gruart V, Courvalin JC, and Lassoued K
- Subjects
- Adolescent, Adult, Aged, Anemia, Hemolytic, Autoimmune complications, Anemia, Hemolytic, Autoimmune drug therapy, Anemia, Hemolytic, Autoimmune immunology, Anemia, Hemolytic, Autoimmune pathology, Autoimmune Diseases complications, Autoimmune Diseases drug therapy, Autoimmune Diseases pathology, Coombs Test, Female, Follow-Up Studies, Humans, Immunoblotting, Liver Diseases complications, Liver Diseases pathology, Male, Middle Aged, Neutropenia complications, Neutropenia drug therapy, Neutropenia immunology, Neutropenia pathology, Pancytopenia complications, Pancytopenia drug therapy, Pancytopenia pathology, Peptides immunology, Purpura, Thrombocytopenic complications, Purpura, Thrombocytopenic drug therapy, Purpura, Thrombocytopenic immunology, Purpura, Thrombocytopenic pathology, Retrospective Studies, Treatment Outcome, Vasculitis complications, Vasculitis pathology, Autoantibodies immunology, Autoimmune Diseases immunology, Nuclear Envelope immunology, Nuclear Proteins immunology, Pancytopenia immunology
- 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.
- Published
- 2004
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