SHM Converge Scientific Abstract ePoster Gallery-Secondary Autoimmune Neutropenia: Severe Neutropenia in an Acute Lupus Flare

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This case report describes a 39-year-old woman who presented with a diffuse, pruritic morbilliform rash, oral ulcers, tongue swelling, conjunctival injection, dysphonia, lymphadenopathy, and profound neutropenia (ANC 0) shortly after taking amoxicillin for streptococcal pharyngitis. Because of the rash and fever, she was initially isolated for possible measles and treated empirically with broad-spectrum antibiotics during a transient neutropenic fever.<br /><br />Her evaluation revealed widespread lymphadenopathy, anemia, and later evidence of autoimmune disease. Serologic testing showed a strongly positive ANA with speckled pattern, anti-dsDNA, anti-histone, and anti-Smith antibodies, along with low C4 and elevated urine protein:creatinine ratio, supporting systemic lupus erythematosus (SLE) or possibly drug-induced lupus. Biopsies further supported an autoimmune process: skin biopsy showed interface dermatitis, lymph node biopsy showed a decreased CD4:CD8 ratio without monoclonal B cells, and bone marrow biopsy demonstrated hypercellularity with nearly absent granulopoiesis. EBV testing suggested recent infection, and renal findings were consistent with IgA nephropathy, likely post-viral.<br /><br />The authors emphasize that neutropenia in lupus is multifactorial and may result from anti-neutrophil antibodies, impaired granulocyte production, and immune dysregulation. In this patient, the findings were most consistent with secondary autoimmune neutropenia during an acute lupus flare. She improved with high-dose steroids and hydroxychloroquine, and the neutropenia resolved; mycophenolate and losartan were started at discharge.<br /><br />Key takeaways: severe neutropenia should prompt consideration of autoimmune causes; infections and lupus often coexist and may trigger one another; and lupus can present atypically, without classic malar rash, photosensitivity, or arthralgias.

Asset Subtitle

Kara E. Rieth

Meta Tag

Author List Bryan Marquez, Kara E. Rieth, Sahil Patel, Sarah Jawadi, Yusra Azhar

Category Clinical Vignettes

Concept Systemic Lupus Erythematosus

Concept Severe Neutropenia

Concept ANA

Concept Anti-dsDNA Antibody

Concept Autoimmune Neutropenia

Distinguished Non-Finalist

Presenter Organization University of New Mexico School of Medicine, Internal Medicine Department

Presenting Author Kara E. Rieth

Track Adult

Keywords

systemic lupus erythematosus

autoimmune neutropenia

amoxicillin rash

morbilliform eruption

anti-dsDNA

anti-Smith antibodies

neutropenic fever

bone marrow biopsy

drug-induced lupus

IgA nephropathy

Systemic Lupus Erythematosus

Severe Neutropenia

ANA

Anti-dsDNA Antibody

Autoimmune Neutropenia