title: Cold agglutinin-induced haemolysis in association with antinuclear antibody-negative SLE.
abstract: Systemic lupus erythematosus (SLE) is a chronic relapsing autoimmune disease associated with several autoantibodies targeted to nuclear and cytoplasmic antigens. Serum antinuclear antibody (ANA) is considered an important diagnostic marker of SLE. However, 2-3% of patients with typical clinical picture of SLE may have persistently negative ANA tests. Autoimmune haemolytic anaemia (AIHA) in SLE is usually mediated by warm IgG anti-erythrocyte antibodies. Our report describes a female patient who presented with clinical manifestations of SLE including photosensitivity, joint pains and AIHA. Further workup revealed high cold IgM agglutinin titres. A comprehensive workup for infectious aetiologies was negative. Autoimmune studies revealed negative ANA, but positive anti-double-stranded DNA and antiphospholipid antibodies. Lymphoproliferative disorder was excluded by imaging studies. Initial treatment with steroids proved of little benefit; however, rituximab resulted in significant clinical improvement. To the best of our knowledge, this is perhaps the first report of ANA-negative SLE presenting with cold AIHA.
authors.affiliation: Department of Internal Medicine, Saint Vincent Hospital, University of Massachusetts Medical School, Worcester, Massachusetts, USA.
authors.forename: Vinod K
authors.initials: VK
authors.lastname: Chaubey
authors.forename: Lovely
authors.initials: L
authors.lastname: Chhabra
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood*; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D003450:Cryoglobulins / Q000009:adverse effects; D017707:Erythrocyte Transfusion; D005260:Female; D006461:Hemolysis; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000097:blood* / Q000276:immunology / Q000628:therapy; D000069283:Rituximab; D013256:Steroids / Q000008:administration & dosage; D014057:Tomography, X-Ray Computed
chemical_list: D000974:Antibodies, Antinuclear; D058846:Antibodies, Monoclonal, Murine-Derived; D003450:Cryoglobulins; D013256:Steroids; C029443:cold agglutinins; D000069283:Rituximab