Systemic Lupus Erythematosus with Hemophagocytic Lymphohistiocytosis Mimicking Tuberculosis in a Young Male: A Diagnostic Challenge

Systemic lupus erythematosus (SLE) can rarely present with hemophagocytic lymphohistiocytosis (HLH), a severe hyperinflammatory state with high mortality if unrecognized. We report a 21-year-old male who presented with fever, ascites, pleural effusion, anemia, and acute kidney injury. Infectious causes were ruled out, and autoimmune markers confirmed SLE. Bone marrow biopsy showed hemophagocytosis, fulfilling HLH-2004 criteria for HLH. The patient responded well to corticosteroids and hydroxychloroquine, with plans for cyclophosphamide and renal biopsy. This case emphasizes the importance of early recognition of macrophage activation syndrome in SLE patients with systemic inflammation and cytopenias.

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Publication Details

Journal
Annals of African Medicine
Published
2025-08-20
DOI
https://doi.org/10.4103/aam.aam_172_25
Primary Topic
Autoimmune and Inflammatory Disorders Research
Type
article
Field-Weighted Citation Impact
0.00
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article

Systemic Lupus Erythematosus with Hemophagocytic Lymphohistiocytosis Mimicking Tuberculosis in a Young Male: A Diagnostic Challenge

Prakash Shende, Tejas Kore, Vivek H Lapsiwala, Pranavi Kalakota
1 links
Annals of African Medicine
Autoimmune and Inflammatory Disorders Research
article

Systemic Lupus Erythematosus with Hemophagocytic Lymphohistiocytosis Mimicking Tuberculosis in a Young Male: A Diagnostic Challenge

Prakash Shende, Tejas Kore, Vivek H Lapsiwala, Pranavi Kalakota
article en

Abstract

Systemic lupus erythematosus (SLE) can rarely present with hemophagocytic lymphohistiocytosis (HLH), a severe hyperinflammatory state with high mortality if unrecognized. We report a 21-year-old male who presented with fever, ascites, pleural effusion, anemia, and acute kidney injury. Infectious causes were ruled out, and autoimmune markers confirmed SLE. Bone marrow biopsy showed hemophagocytosis, fulfilling HLH-2004 criteria for HLH. The patient responded well to corticosteroids and hydroxychloroquine, with plans for cyclophosphamide and renal biopsy. This case emphasizes the importance of early recognition of macrophage activation syndrome in SLE patients with systemic inflammation and cytopenias.

Annals of African Medicine
Dr. D. Y. Patil Medical College, Hospital and Research Centre (IN)
Openalex Percentile: Top 74%
Autoimmune and Inflammatory Disorders Research
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