Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2024 Dec 28;18(1):643.
doi: 10.1186/s13256-024-05002-3.

Evans syndrome as a presentation in systemic lupus erythematous, coexisting with Hashimoto's thyroiditis and pernicious anemia: a case report

Maryam Mansour  1 , Arwa Shamasnah  2 , Deema Alsaadi  2 , Saja Abu Saif  2 , Akram Krama  2   3
Affiliations
Case Reports

Evans syndrome as a presentation in systemic lupus erythematous, coexisting with Hashimoto's thyroiditis and pernicious anemia: a case report

Maryam Mansour et al. J Med Case Rep. .

Abstract

Background: Evans syndrome is a rare disorder characterized by the simultaneous or sequential combination of autoimmune hemolytic anemia and immunological thrombocytopenia, together with a positive direct antiglobulin test. This syndrome, which can be primary or secondary, is a rare initial manifestation of autoimmune diseases, notably systemic lupus erythematosus, with 1.7-2.7% of patients with systemic lupus erythematosus developing secondary Evans syndrome, sometimes preceding the onset of systemic lupus erythematosus symptoms.

Case presentation: A 47-year-old Middle Eastern female presented with symptoms including shortness of breath, chest pain, and weakness. Physical examination revealed pallor, pale conjunctiva, icteric sclera, tachycardia, and tachypnea. She was diagnosed with Evans syndrome owing to hemolytic anemia, thrombocytopenia, and a positive Coombs test, where initial resistance to treatment prompted intensive therapy with methylprednisolone, intravenous immunoglobulin, and rituximab. Subsequent identification of systemic lupus erythematosus on the basis of positive anti-nuclear antibodies and anti-double-stranded deoxyribonucleic acid antibodies led to treatment with mycophenolate mofetil and hydroxychloroquine. Further evaluations uncovered Hashimoto's thyroiditis and pernicious anemia, necessitating thyroxine supplementation and vitamin B12 injections.

Conclusion: Hematologic abnormalities play a crucial role in the diagnostic framework of systemic lupus erythematosus. This case highlights a patient initially diagnosed with Evans syndrome, revealing an underlying systemic lupus erythematosus. The presentation underscores the significance of hematologic manifestations as integral components of the diagnostic framework for autoimmune diseases, emphasizing the intricate relationship between Evans syndrome and systemic lupus erythematosus.

Keywords: Autoimmune hemolytic anemia; Evans syndrome; Systemic lupus erythematosus.

PubMed Disclaimer

Conflict of interest statement

Declarations. Ethics approval and consent to participate: This article does not contain any studies with human participants or animals performed by any of the authors. Consent for publication: Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Competing interests: Authors declare no competing interests.

Similar articles

See all similar articles

References

    1. Evans RS, Takahashi K, Duane RT, Payne R, Liu C. Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology. AMA Arch Intern Med. 1951;87(1):48–65. - PubMed
    1. Hansen DL, Möller S, Andersen K, Gaist D, Frederiksen H. Evans syndrome in adults—incidence, prevalence, and survival in a nationwide cohort. Am J Hematol. 2019;94(10):1081–90. - PubMed
    1. Audia S, Grienay N, Mounier M, Michel M, Bonnotte B. Evans’ Syndrome: from diagnosis to treatment. J Clin Med. 2020. 10.3390/jcm9123851. - PMC - PubMed
    1. Mendonca S, Srivastava S, Kapoor R, Gupta D, Gupta P, Sharma ML. Evans syndrome and its link with systemic lupus erythematosus. Saudi J Kidney Dis Transpl. 2016;27(1):147–9. - PubMed
    1. Michel M, Chanet V, Dechartres A, Morin AS, Piette JC, Cirasino L, Emilia G, Zaja F, Ruggeri M, Andrès E, et al. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases. Blood. 2009;114(15):3167–72. - PubMed

Publication types

MeSH terms

Supplementary concepts

LinkOut - more resources