Diagnostic Challenges in Lupus: Systemic Lupus Erythematosus Initially Presenting as Unicentric Castleman Disease
- Glomerular Diseases: Epidemiology and Case Reports
November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Trials
- Miller, Allison, Oregon Health and Science University, Portland, Oregon, United States
- Kung, Vanderlene Liu, Oregon Health and Science University, Portland, Oregon, United States
- Thanawala, Ruchi, Oregon Health and Science University, Portland, Oregon, United States
- Avasare, Rupali S., Oregon Health and Science University, Portland, Oregon, United States
Multiple conditions mimic Castleman Disease (CD), a group of lymphoproliferative disorders, including infection, neoplams and autoimmune disease. There is well-described clinical overlap between systemic lupus erythematosus (SLE) and CD as both can manifest with lymphadenopathy, proteinuria and positive anti-nuclear antibody (ANA) serology. Distinguishing between these two entities can be a vexing process and requires a multidisciplinary approach to care.
A 23 year-old woman presented with dyspnea. Chest imaging revealed a large mediastinal mass and pleural effusion (Figure 1). The mass was resected and the patient was diagnosed with HHV-8 and EBV-negative CD. A clinician noted persistent hypoalbuminemia and discovered 8.2 grams per day of proteinuria despite surgical management. Work up included a kidney biopsy that showed a “full house” immune complex-mediated glomerulonephritis with focal activity, endocapillary hypercellularity and large subendothelial deposits. Serologic testing revealed positive ANA, anti-double-stranded DNA antibody and hypocomplementemia. The patient's final diagnosis was revised to SLE and she achieved remission with immunosuppressive therapy.
To our knowledge, this is the first reported case of unicentric CD mimicking SLE in a patient with nephrotic syndrome. Because unicentric CD is managed with surgery alone, this report highlights the need for a broad diagnostic workup to rule out similarly presenting diseases with vastly different treatments. In this patient, workup for nephrotic syndrome prompted kidney biopsy and serologic testing that was essential for establishing the correct diagnosis and management.