SHM Converge Scientific Abstract ePoster Gallery-Unlocked Mysteries: A Prisoner of Unique Sle Presentations

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This case report describes a rare and diagnostically challenging presentation of systemic lupus erythematosus (SLE) in a 41-year-old African American male prisoner. He initially presented with bilateral submandibular, sublingual, and parotid swelling, facial angioedema, and fever, leading clinicians to first suspect Ludwig’s angina. Broad-spectrum antibiotics failed, and his condition evolved with marked CK elevation, acute kidney injury, hematuria, proteinuria, anemia, arthralgias, generalized edema, rash, and subcutaneous nodules.<br /><br />Dermatologic evaluation was pivotal: skin biopsy showed neutrophilic urticarial dermatosis, an uncommon finding associated with autoimmune disease. SLE became the leading diagnosis, while sarcoidosis was considered less likely due to negative ACE levels and no hilar lymphadenopathy. Serologic workup showed strongly positive anti-dsDNA (1975 IU/mL), positive anti-Smith antibodies, low complement levels, and severe anemia. Although the first kidney biopsy suggested post-infectious glomerulonephritis, later immunofluorescence confirmed ISN/RPS Class IV diffuse lupus nephritis, aligning with SLE.<br /><br />The patient met American College of Rheumatology classification criteria with a total score of 31. Treatment with high-dose corticosteroids, hydroxychloroquine (Plaquenil), and mycophenolate mofetil (CellCept) led to significant improvement, and he is now receiving outpatient rituximab with good disease control.<br /><br />The authors emphasize that SLE is rare in African American men and that this patient’s initial presentation closely mimicked infection, making diagnosis especially difficult. They note that angioedema and bilateral submandibular swelling are exceptionally uncommon SLE manifestations, possibly representing a first reported case of this type. The report highlights the need for vigilance and a broad differential diagnosis when evaluating atypical facial swelling, rash, and multisystem involvement, as timely recognition and treatment can prevent severe complications such as kidney failure and other organ damage.

Asset Subtitle

Maruf Sarwar

Meta Tag

Author List Gilbert-Roy B. Kamoga, Janani Baskaran, Maruf Sarwar, Tyeler Barker

Category Clinical Vignettes

Concept Systemic Lupus Erythematosus

Concept ACR/EULAR Classification Criteria

Concept Renal involvement

Concept ANA

Concept Anti-dsDNA Antibody

Distinguished Non-Finalist

Presenter Organization White River Health Systems

Presenting Author Maruf Sarwar

Track Adult

Keywords

systemic lupus erythematosus

SLE

lupus nephritis

angioedema

submandibular swelling

neutrophilic urticarial dermatosis

anti-dsDNA

anti-Smith antibodies

mycophenolate mofetil

rituximab

Systemic Lupus Erythematosus

ACR/EULAR Classification Criteria

Renal involvement

ANA

Anti-dsDNA Antibody