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.

Cold agglutinin-induced hemolytic anemia as the primary presentation in SLE - A case report

“…A similar case was reported by Chaubey et al . [10] and Srinivasan et al . [11] Patients presenting with anemia usually visit family physicians.…”

Childhood-Onset Systemic Lupus Erythematosus in Southwestern Iran: A Clinical and Serological Study

“…This finding was significantly higher than in previously published reports (2-3%). 19,20 An increased number of ANA negative patients can be explained by the technical inaccuracy in laboratories, too low levels of ANA in patient's serum, and by binding ANA in the form of immune complex. 20 The serum level of complements are found to be decreased in active disease, and is often improved with treatment in SLE.…”

A 79‐Year‐Old Female Patient With Altered Mental Status and Anemia

“…Warm agglutinin AIHA is the predominant type seen in SLE, which is mediated by IgG antibodies reacting at body temperature, whereas cold agglutinin AIHA is caused by IgM complement–fixing antibody that reacts at a temperature of 4°C (5). While cold agglutinin AIHA can be seen in association with SLE, it is rare with only a handful of reported cases in the literature (7–9).…”