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

doi:10.12998/wjcc.v9.i8.2008

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World Journal of Clinical Cases

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Case Report

World J Clin Cases. Mar 16, 2021; 9(8): 2008-2014
Published online Mar 16, 2021. doi: 10.12998/wjcc.v9.i8.2008

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

Yi-Xuan Liao, Yan-Fei Guo, Yu-Xia Wang, Ai-Hua Liu, Chun-Li Zhang

Yi-Xuan Liao, Yan-Fei Guo, Yu-Xia Wang, Department of Pulmonary and Critical Care Medicine, Beijing Hospital, National Center of Gerontology; Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing 100730, China

Ai-Hua Liu, Department of Rheumatology and Immunology, Beijing Hospital, National Center of Gerontology; Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing 100730, China

Chun-Li Zhang, Department of Hematology, Beijing Hospital, National Center of Gerontology; Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing 100730, China

Author contributions: Guo YF designed the outline of the paper, reviewed and revised the manuscript; Liao YX reviewed the literature and contributed to manuscript drafting; Wang YX reviewed the literature and revised the manuscript; Liu AH performed the autoimmune diseases consultation, reviewed the literature and followed up the patient; Zhang CL performed the hematological diseases consultation and reviewed the literature; all authors issued final approval for the version to be submitted.

Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.

Conflict-of-interest statement: The authors declare that they have no conflicts of interest.

CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).

Corresponding author: Yan-Fei Guo, MD, Chief Doctor, Department of Pulmonary and Critical Care Medicine, Beijing Hospital, No. 1 Dahua Road, Dongdan, Dongcheng District, Beijing 100730, China. yanfeiguo2003@126.com

Received: December 4, 2020
Peer-review started: December 4, 2020
First decision: December 30, 2020
Revised: January 12, 2021
Accepted: January 27, 2021
Article in press: January 27, 2021
Published online: March 16, 2021
Processing time: 84 Days and 3.7 Hours

Abstract

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.

Keywords: Systemic lupus erythematosus; Primary hyperfibrinolysis; Antiphospholipid antibody; Protein C deficiency; Protein S deficiency; Case report

Core Tip: Systemic lupus erythematosus (SLE) with both protein C (PC) and protein S (PS) deficiency, and primary hyperfibrinolysis has not been reported in previous literature. We report a patient with SLE presenting with pleural effusion who was found to have both primary hyperfibrinolysis and PC and PS deficiency. The balance between the prevention of thrombosis and hemorrhage should be considered.