Recurrent Sterile Pleuropericarditis After Withdrawal of Renal Transplant Immunosuppression in Anuric ESRD: A Case of Suspected IL-1-Mediated Autoinflammation.

Recurrent pericarditis and serositis pose a significant diagnostic challenge in patients with end-stage renal disease (ESRD) and a history of extensive immunosuppression. A 29-year-old man with anuric ESRD secondary to IgA nephropathy on intermittent hemodialysis, with a history of 2 failed kidney transplantations followed by posttransplant nephrectomies and recent withdrawal of immunosuppression, presented with recurrent fevers, pericarditis, pleural effusions, and pericardial effusions. Fevers were unresponsive to multiple courses of broad-spectrum antibiotics. A broad malignancy workup, including imaging, PET scan, cytology, and microbial cell-free DNA was unremarkable. Multiple rounds of blood, pleural fluid, and pericardial fluid studies were negative for infectious etiologies, and serologic studies were unremarkable. Given the negative workup and clinical response to anti-inflammatory therapy, his presentation was most consistent with an IL-1-mediated autoinflammatory phenotype such as idiopathic recurrent pericarditis or undifferentiated systemic autoinflammatory disorder. Rilonacept was attempted as an IL-1-targeted alternative because of long-term colchicine toxicity concerns in ESRD; however, exposure was limited, and sustained disease control occurred after resumption of colchicine. This case highlights the need for further study of IL-1 inhibitor use in ESRD patients, a population with limited representation in landmark recurrent pericarditis trials such as RHAPSODY and AIRTRIP.