A LITTLE CHANGE GOES A LONG WAY: A RARE PRESENTATION OF SARCOIDOSIS

Abstract

SESSION TITLE: Tuesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Sarcoidosis is a granulomatous disorder, affecting multiple systems throughout the body. We present a case of sarcoidosis, presenting with nephrotic syndrome. CASE PRESENTATION: The patient is a 51-year-old female, with history of well-controlled type II diabetes and hypertension, who presented initially with bilateral lower extremity edema, first noted one week prior to admission. Her history was notable for a negative smoking and drug history, no exposures, and no relevant family history. Her physical exam was notable for 2+ to 3+ bilateral lower extremity edema, presacral edema, and 1+ upper extremity edema. Labs showed elevated serum creatinine. A 24-hour urine collection demonstrated nephrotic-range proteinuria. Chest imaging showed bilateral hilar lymphadenopathy. She was discharged on high-dose diuretics; however, edema continued to worsen and she gained 18 kg over two months. A renal biopsy was performed two months later, which demonstrated interstitial nephritis with well-formed granulomas. Electron microscopy was performed, which demonstrated podocyte effacement, consistent with minimal change disease. Endobronchial ultrasound was later performed with fine needle aspiration at lymph node stations 4R, 7, and 11R, which again demonstrated granulomatous inflammation. She was discharged home with steroids and aggressive diuresis, with resultant improvement in edema. DISCUSSION: Sarcodosis is a multi-system disease that can affect the kidneys in up to 30% of cases1. The most typical form of renal sarcoidosis is granulomatous interstitial nephritis (GIN). The majority of patients with GIN present with elevated creatinine noted at the time of diagnosis. Urinalysis in GIN is usually bland or demonstrates sterile pyuria or hematuria. Very few patients present with significant proteinuria. Minimal change disease (MCD) has only been reported in singular case reports in patients with sarcoidosis. In a retrospective study of 26 patients with biopsy-proven glomerulonephritis and sarcoidosis, only three patients had MCD, and only one was diagnosed with MCD concurrently with sarcoidosis. In adult patients, MCD is most commonly associated with drugs, particularly NSAIDs. However, in patients with mediastinal lymphadenopathy and MCD, more ominous etiologies, including Hodgkin’s lymphoma, non-Hodgkin’s lymphoma, and leukemia, should be ruled out. Steroids are the mainstay of treatment for MCD in association with sarcoidosis, but these agents may be contraindicated in the setting of malignancy. CONCLUSIONS: Minimal change disease is an exceedingly rare presenting symptom of sarcoidosis. When minimal change disease is diagnosed in the setting of mediastinal lymph adenopathy, malignancy should be ruled out by lymph node sampling. Reference #1: Bergner R, Löffler C. Renal sarcoidosis: approach to diagnosis and management. Curr Opin Pulm Med. 2018;24(5):513-520. Reference #2: Mahévas M, Lescure FX, Boffa JJ, et al. Renal sarcoidosis: clinical, laboratory, and histologic presentation and outcome in 47 patients. Medicine (Baltimore). 2009;88(2):98-106. Reference #3: Stehlé T, Joly D, Vanhille P, et al. Clinicopathological study of glomerular diseases associated with sarcoidosis: a multicenter study. Orphanet J Rare Dis. 2013;8:65. DISCLOSURES: Speaker/Speaker's Bureau relationship with boehringer ingelheim Please note: $5001 - $20000 Added 06/10/2019 by Brad Bemiss, source=Web Response, value=Travel and payment for lecture No relevant relationships by Krishnan Warrior, source=Web Response