Case Presentation: Systemic Lupus Erythematosus (SLE) is known for its diverse clinical presentations. We present the case of a 41-year-old African American male prisoner with an initial presentation featuring bilateral submandibular, sublingual, and parotid swelling with concurrent facial angioedema and fever. The patient was presumed to have Ludwig’s Angina, yet initial treatment with broad spectrum antibiotics failed to remedy his clinical disposition. Diffuse arthralgias, generalized edema, and maculopapular rash with subcutaneous nodules shifted our differential to rheumatologic process . He developed significant CK elevation, AKI, hematuria, proteinuria and anemia. SLE and sarcoidosis were our leading differential diagnosis. ACE levels were negative and Chest X-Ray was absent of hilar lymphadenopathy, reducing the likelihood of Sarcoidosis. Immunological exploration revealed highly positive dsDNA titer of 1975 IU/mL and anti-Smith antibodies, in addition to complement deficiencies and severe anemia. Our patient’s presentation fulfilled the American College of Rheumatology (ACR) diagnostic criteria for SLE, with a total ACR score of 18.[1] A subsequent kidney biopsy by the nephrology revealed pathology consistent with diffuse lupus nephritis, specifically ISN/RPS class IV, in perfect accordance with SLE. An enigmatic dermatological discovery emerged as a skin biopsy revealed Neutrophilic Urticarial Dermatosis, a seldom-seen manifestation linked to autoimmune conditions, among them SLE. The complexity of clinical and laboratory evidence ultimately crystallized into a diagnosis of SLE. Treatment featuring high-dose corticosteroids and azathioprine, heralded significant clinical improvement.
Discussion: SLE affects around 200,000 people in the U.S., accounting for approximately 0.06% of the population, and is more prevalent in African Americans than in other racial group.[2, 3] SLE is rare in African American males, with a female-to-male ratio of 7:1 to 15:1 before menopause, dropping to 8:1 in older adults.[4] Thus, cases of black male patients diagnosed with this disease are exceedingly rare, with an incidence of only 2.4 per 100,000.[5] While treated SLE now boasts 5 and 10-year survival rates exceeding 90%, untreated SLE significantly elevates the risk of life-threatening complications, including organ damage like heart attacks, strokes, and kidney failure.[6] Angioedema in lupus is a rarity, with its prevalence expected to be under 1%.[7] Bilateral submandibular swelling in SLE is exceptionally uncommon, with fewer than 10 cases reported since 1972. [8-13] This patient’s presenting constellation of symptoms, reminiscent of Ludwig’s Angina, challenged standard diagnostic paradigms for SLE and could be the first documented case of its kind in medical literature. Rapid identification and commencement of SLE treatment in this intricate clinical scenario averted potential fatal complications, emphasizing the pivotal role of timely intervention in enhancing patient outcomes.
Conclusions: This case underscores the intricate tapestry of SLE, advocating for a comprehensive diagnostic approach when confronted with patients bearing complicated autoimmune profiles, profound hematologic complications, and elusive dermatologic presentations. The rarity of the initial presentation exposes the diagnostic complexities inherent in atypical SLE manifestations, necessitating heightened awareness and vigilance in clinical practice leading to swift treatment.