Cold Agglutinin Induced Hemolysis in a Newly Diagnosed Systemic Lupus Erythematosus

Srinivasan, Nandakumar; Oswal, Alok; Garg, Sindhu; Gosmonova, Albina; Nahar, Julie; Nahar, Roopesh

doi:10.1097/maj.0b013e3181ac3bd5

Cited by 19 publications

(15 citation statements)

References 16 publications

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“…Though extremely rare, cold antibody AIHA in SLE has been reported 12 13. Binding of antibodies to RBCs activates the classical pathway of the complement system leading to the formation of membrane attack complex and intravascular haemolysis.…”

Section: Discussionmentioning

confidence: 99%

Cold agglutinin-induced haemolysis in association with antinuclear antibody-negative SLE

Chaubey

Chhabra

2013

BMJ Case Reports

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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.

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Section: Discussionmentioning

confidence: 99%

Cold agglutinin-induced haemolysis in association with antinuclear antibody-negative SLE

Chaubey

Chhabra

2013

BMJ Case Reports

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“…After CAD is established, patients should be evaluated for infections, underlying malignancies, and autoimmune disease because more than 70% of cases may be attributable to these processes. 2,6,53 CAD has been associated with lupus 54,55 and rheumatoid arthritis. 56 The IgM antibodies resulting in classical CAD are monoclonal, and the differential diagnosis includes disorders such as IgM monoclonal gammopathy of undetermined significance (MGUS) and Waldenström macroglobulinemia.…”

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confidence: 99%

Cold agglutinin disease

Swiecicki

Hegerova

Gertz

2013

Blood

266

298

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Cold agglutinin disease is a rare and poorly understood disorder affecting 15% of patients with autoimmune hemolytic anemia. We reviewed the clinical and pathologic features, prognosis, and management in the literature and describe our institutional experience to improve strategies for accurate diagnosis and treatment. Retrospective analysis identified 89 patients from our institution with cold agglutinin disease from 1970 through 2012. Median age at symptom onset was 65 years (range, 41 to 83 years), whereas the median age at diagnosis was 72 years (range, 43 to 91 years). Median survival of all patients was 10.6 years, and 68 patients (76%) were alive 5 years after the diagnosis. The most common symptom was acrocyanosis (n = 39 [44%]), and many had symptoms triggered by cold (n = 35 [39%]) or other factors (n = 20 [22%]). An underlying hematologic disorder was detected in 69 patients (78%). Thirty-six patients (40%) received transfusions during their disease course, and 82% received drug therapy. Rituximab was associated with the longest response duration (median, 24 months) and the lowest proportion of patients needing further treatment (55%). Our institution’s experience and review of the literature confirms that early diagnostic evaluation and treatment improves outcomes in cold agglutinin disease.

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“…[10] and Srinivasan et al . [11] Patients presenting with anemia usually visit family physicians. Therefore, increased awareness among primary physicians about SLE presenting as hemolytic anemia especially in young ladies is important for the early diagnosis and appropriate management.…”

Section: Discussionmentioning

confidence: 99%

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

Mohanty

Ansari

Kumari

et al. 2019

J Family Med Prim Care

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Cold Agglutinin Induced Hemolysis in a Newly Diagnosed Systemic Lupus Erythematosus

Cited by 19 publications

References 16 publications

Cold agglutinin-induced haemolysis in association with antinuclear antibody-negative SLE

Cold agglutinin-induced haemolysis in association with antinuclear antibody-negative SLE

Cold agglutinin disease

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

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