Membrane autoantibodies in systemic lupus erythematosus: a case of autoimmune hemolytic anemia, antiphospholipid antibodies, and transient acquired activated protein C resistance

Staropoli, John F.; Cott, Elizabeth M. Van; Makar, Robert S.

doi:10.1111/j.1537-2995.2008.01877.x

Cited by 5 publications

(1 citation statement)

References 31 publications

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“…Anticardiolipin antibodies (ACA) occur in approximately 40% of patients with SLE, but ACA can be present in AIHA with or without SLE, suggesting the role of ACA in both primary and secondary AIHA. Data regarding levels of IgG and IgM are not clear, with 1 study reporting elevated IgM and normal IgG levels, similar to our patient [ 9 , 13 , 14 ].…”

Section: Discussionsupporting

confidence: 61%

Warm Autoimmune Hemolytic Anemia as the Initial Presentation of Systemic Lupus Erythematosus (SLE): A Case Report

et al. 2021

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Patient: Female, 32-year-old Final Diagnosis: Anemia • hemolytic anemia • systemic lupus erythematosus Symptoms: Fatigue • weakness Medication: — Clinical Procedure: — Specialty: Hematology • Immunology Objective: Unknown etiology Background: Autoimmune hemolytic anemia is an acquired disorder resulting in the presence of antibodies against red blood cell (RBC) antigens causing hemolysis. Autoimmune hemolytic anemia is of 2 types, Warm antibody mediated and cold agglutinin disease. Warm autoimmune hemolytic anemia (warm agglutinin disease) usually presents with fatigue and other constitutional symptoms and is diagnosed by the presence of IgG antibodies. The disease can occur as idiopathic or secondary to other autoimmune diseases, infections, or even malignancies. The systemic lupus erythematosus (SLE) is an autoimmune disease prevalent in young females. Autoimmune hemolytic anemia can occur as a part of the SLE spectrum however warm autoimmune hemolytic anemia as the initial manifestation of SLE is extremely rare. Case Report: Here, we describe a unique case of a 32-year-old woman who presented with vague clinical presentation found to have warm autoimmune hemolytic anemia and further immunological and inflammatory work-up during and after hospitalization lead to the diagnosis of systemic lupus erythematosus. Conclusions: The systemic lupus erythematosus (SLE) is an autoimmune chronic inflammatory disease with unclear etiology affecting multi organs. Variable presentation in addition to the lack of definite pathognomonic features or tests makes the diagnosis of SLE challenging. On the whole autoimmune hemolytic anemia can not only be part of other disease processes but can be an initial presentation, highlighting the importance of thorough work-up in patients presenting with autoimmune hemolytic anemia to aid in timely diagnosis and management of underlying secondary conditions. It is important for providers to be aware of various disease spectrums that contain autoimmune hemolytic anemia for day-to-day clinical practice.

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Section: Discussionsupporting

confidence: 61%

Warm Autoimmune Hemolytic Anemia as the Initial Presentation of Systemic Lupus Erythematosus (SLE): A Case Report

et al. 2021

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Systemic Lupus Erythematosus

Silverman¹,

Eddy²

2011

Textbook of Pediatric Rheumatology

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Systemic lupus erythematosus combined with primary hyperfibrinolysis and protein C and protein S deficiency: A case report

Liao¹,

Guo²,

Wang³

et al. 2021

WJCC

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BACKGROUND Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by systemic involvement and multiple autoantibodies in the serum. Patients with protein C (PC) and protein S (PS) deficiency are prone to thrombosis. In contrast, patients with primary hyperfibrino-lysis tend to bleed. CASE SUMMARY A 52-year-old female patient with bilateral pleural effusion was diagnosed with "tuberculous pleurisy" and treated with anti-tuberculosis drugs and prednisone. The coagulation-related laboratory results showed decreased fibrinogen, PC activity, PS activity, and antithrombin Ш activity. The immune-related laboratory results showed positive antinuclear antibody, anti-Smith antibody, anticardiolipin antibody (ACL), anti-β2-glycoprotein I antibody (aβ2GPI) and direct Coomb’s test and decreased complement 3 and complement 4. Thoracoscopy was performed and bloody pleural fluid was drained. Pathology of the pleural biopsy showed lymphocytes, plasma cells, and a few eosinophils in adipose and fibrous connective tissue. Results of whole exome sequencing of blood showed no genetic mutations suggesting the presence of hereditary hematological diseases. The patient was finally diagnosed with SLE and primary hyperfibrinolysis, and was treated with prednisolone, hydroxychloroquine, and compound cyclophosphamide. CONCLUSION PC and PS deficiency in SLE might be related to ACL and aβ2GPI. SLE and primary hyperfibrinolysis can coexist in one patient, with both a risk of thrombosis and a risk of bleeding.

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Membrane autoantibodies in systemic lupus erythematosus: a case of autoimmune hemolytic anemia, antiphospholipid antibodies, and transient acquired activated protein C resistance

Cited by 5 publications

References 31 publications

Warm Autoimmune Hemolytic Anemia as the Initial Presentation of Systemic Lupus Erythematosus (SLE): A Case Report

Warm Autoimmune Hemolytic Anemia as the Initial Presentation of Systemic Lupus Erythematosus (SLE): A Case Report

Systemic Lupus Erythematosus

Systemic lupus erythematosus combined with primary hyperfibrinolysis and protein C and protein S deficiency: A case report

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