P3.M258: ImmunoCrossroads: The Role of Med-Peds and the Intersection of Autoimmune Disorders

Introduction: This case outlines the clinical course of a patient admitted for systemic lupus erythematosus (SLE) exacerbation, newly diagnosed lupus nephritis, severe oro-esophageal ulcerations, and Crohn’s disease. Her pediatric, diagnosis evolving from adolescence into adulthood, highlights the challenges Med-Peds physicians are particularly skilled in navigating.

Case Description: A 21-year-old female with SLE and Crohn's disease presented to the emergency department with new onset mouth sores, diffuse pain, and progressive weakness. The patient was chronically immunosuppressed on azathioprine, hydroxychloroquine, and infliximab. On initial presentation, labs were remarkable for WBC of 18k, CRP 5.0, ESR 53, and significant proteinuria. Physical exam revealed tender submandibular lymphadenopathy with oral and esophageal mucositis. She developed superimposed oral candidiasis and was appropriately treated with micafungin. Mucositis subsequently spread, resulting in vulvar ulcerations. EGD with biopsy revealed HSV-1 esophagitis. IV acyclovir was started, and WBC normalized. The patient could not tolerate oral intake with nutrition shake supplementation, so she was escalated to a nasogastric tube with scheduled feedings. A PEG tube was finally placed to meet her nutritional needs. Patient leukocytosis increased to 25.7 and she developed a fever; a sepsis workup was initiated and Zosyn and ceftriaxone were started for empiric coverage. Urine protein:creatinine increased, and renal biopsy revealed cellular crescents and endocapillary hypercellularity consistent with grade III lupus nephritis (LN). p-ANCA was positive with titers >1:320. The patient improved with prednisone, hydroxychloroquine, and acyclovir. On discharge, we added mycophenolate mofetil for LN therapy and atovaquone for pneumocystis pneumonia prophylaxis to her regimen. She was instructed to follow up with rheumatology and nephrology outpatient.

Discussion: SLE is a pediatric and adult disease, with most diagnoses occurring between the ages of 15 to 45. LN is a common complication of SLE and involves ds-DNA antibody-antigen complex deposition in the glomeruli. LN should be suspected in patients with known SLE who develop proteinuria, elevated serum creatinine, or urine sediment with hematuria. Creatinine and BUN may be normal, which can slow disease recognition. Thus, kidney biopsy is a critical diagnostic tool for patients with SLE. Treatment for LN includes anti-inflammatory and immunosuppressive agents. Mycophenolate mofetil is beneficial for decreasing renal flares and progression to ESRD. However, it is critical to acknowledge the consequences of chronic immunosuppression, as demonstrated here with the development of HSV-1 spread. Complications such as esophagitis, pneumocystis pneumonia, or neutralizing antibodies to biological therapies are other common manifestations of immunosuppression.

Conclusion: Understanding the developing pathophysiology from pediatric presentations into adulthood provides valuable insight into lifelong diseases. Patients with autoimmune disorders benefit from a physician who can monitor the course of their disease, coordinate with specialists, and provide continuity of care from childhood through adulthood.