Introduction
Systemic Lupus Erythematosus (SLE) is an autoimmune disease that can affect multiple organ systems. The estimated incidence of SLE is 23.2 cases per 100,000 persons in North America, commonly presenting in African American, Hispanic, and Asian women.

Case Presentation
A 16yo previously healthy Hispanic female presented multiple times to the hospital for generalized myalgias, fever, diffuse rash over a three-week period during which she was diagnosed with group A strep treated with amoxicillin and cytomegalovirus (CMV). During her last emergency department visit, patient had new onset difficulty breathing and hypotension that was refractory to 3 Liters isotonic fluid resuscitation. Her physical exam was notable for decreased basilar breath sounds. Labs were significant for white blood cell 2.5 thou/mcL, hemoglobin 9.6 g/dL, hematocrit 30.6%, platelets 185 thou/mcL , 77% segmented neutrophils, aspartate aminotransferase 105, alanine aminotransferase 83, negative influenza, COVID, and group A strep. Pertinent imaging included chest x-ray and transthoracic echocardiogram demonstrating bilateral pleural effusion and trace pericardial effusion without tamponade, respectively.

Patient was admitted for septic shock with respiratory failure; vancomycin, cefepime, levophed and high flow nasal cannula were initiated. Patient failed to improve despite antibiotics. Her illness severity was not consistent with an acute CMV infection. A peripheral smear to evaluate hematologic abnormalities revealed rouleaux formation consistent with warm antibody-mediated hemolytic anemia. This prompted additional evaluation for an autoimmune disorder that revealed positive ANA with a titer of 1:320, double-stranded DNA >300, C3 28 mg/dL, anti-Smith antibody 2.8 AI and ribonucleoprotein antibody 1.8 AI.

Final Diagnosis
Patient’s significantly elevated ANA titer and double stranded DNA, coupled with low complement levels were consistent with acute SLE. Based on the 2019 American College of Rheumatology and European League Against Rheumatism classification criteria, patient received 33 points.

Management
Patient demonstrated clinical response to steroids, so she was placed on high dose pulse steroids for three days followed by a steroid taper. Patient was weaned to room air; vasopressor support discontinued. Patient was started on Hydroxychloroquine and mycophenolate mofetil, which continued at discharge. Repeat transthoracic echocardiogram showed trivial pericardial effusion. Renal involvement was unable to be assessed as patient was menstruating.

References and Resources

1. Rees F, Doherty M, Grainge MJ et al. The worldwide incidence and prevalence of systemic lupus erythematosus: a systematic lupus erythematosus: a systematic review of epidemiological studies. Rheumatology. 2017;56: 1945-61.
2. Stojan G, Petri M. Epidemiology of systemic lupus erythematosus: an update. Current Opinion in Rheumatology. 2018; 30:144-50.