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. Am.

Vitiligo Autoantigen VIT75 Is Identified as Lamin A in Vitiligo by Serological Proteome Analysis Based on Mass Spectrometry

“…Anti-lamin A, B1, A/C, and ABC autoantibodies have been frequently reported in most sera from patients with autoimmune diseases, including SLE, chronic autoimmune hepatitis, rheumatoid arthritis, linear scleroderma, and autoimmune cytopenias (McKeon et al, 1983;WesierskaGadek et al, 1988;Senecal et al, 1999;Coppo et al, 2004). Although these Abs to lamins have no disease specificity, high titers of IgG anti-lamin B1 autoantibodies are highly Includes only the first-degree relatives.…”

Clinical relevance of HEp-2 indirect immunofluorescent patterns: the International Consensus on ANA patterns (ICAP) perspective

“…To date, a total of 15 nuclear HEp-2 IIFA patterns have been described, that is, AC-1–AC-14 and AC-29. Table 1 summarises the clinical relevance of these patterns 8 9 14–79. Since AC-29 was only recently described,14 the advice for follow-up testing for autoantibodies to topoisomerase I (Scl-70) in case of clinical suspicion of systemic sclerosis is also added as a note to the clinical relevance of AC-1.…”

Detection of serum anti-melanocyte antibodies and identification of related antigens in patients with vitiligo

“…Nuclear laminar proteins play an important role in maintaining the morphological structures of cells. These proteins were sub-grouped into lamin A, B, and C. Anti-lamin antibodies, which are anti-nuclear antibodies, have been previously observed in the serum of patients with autoimmune diseases (Wesierska-Gadek et al, 1988;Senecal et al, 1999;Nesher et al, 2001;Coppo et al, 2004), and have been used to screen for diseases such as autoimmune hepatitis (lamin A and C) and systemic lupus erythematosus (SLE; lamin B). In this study, the identification of the Mr 75 x 10 3 membrane antigen was consistent with the result obtained by Li et al (2011).…”