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Abstract: TH-PO727

A Rare Case of Paraneoplastic Scleroderma Renal Crisis in a Patient with Squamous Cell Carcinoma of the Tongue

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Abdalla, Marwa, MedStar Georgetown University Hospital, Washington, District of Columbia, United States
  • Durbin, Garrett, MedStar Georgetown University Hospital, Washington, District of Columbia, United States
  • Kwon, Donghyang, MedStar Georgetown University Hospital, Washington, District of Columbia, United States
  • Choi, Michael J., MedStar Georgetown University Hospital, Washington, District of Columbia, United States
  • Pourafshar, Negiin, MedStar Georgetown University Hospital, Washington, District of Columbia, United States
Introduction

Scleroderma Renal Crisis (SRC) is a life-threatening emergency that presents with hypertension, Acute Kidney Injury (AKI) and Thrombotic Microangiopathy (TMA) in patients with Systemic Sclerosis (SSc). It is associated with 20% mortality at 6 months and 19-40% of patients requiring long term dialysis1,2. Paraneoplastic Scleroderma Renal crisis (PSRC) is a rare subset of SRC that has been reported previously3. We report a rare case of PSRC without history of SSc in a patient with squamous cell carcinoma of the tongue.

Case Description

61-year-old male with history of squamous cell carcinoma of the tongue on radiotherapy presented for evaluation of decreased oral intake for few days. He was noted to have tachycardia and hypertension (BP167/109 mmHg). Labs showed anemia, thrombocytopenia and serum creatinine of 1.5mg/dL (baseline 1mg/dL). Urinalysis showed small blood and 30 mg/dL protein with granular casts. Renal ultrasound was unremarkable. The patient was treated initially for a presumed acute tubular injury (ATN) from prolonged pre-renal state. Due to worsening renal function with a creatinine 8.9 mg/dL the patient was initiated on hemodialysis. A renal biopsy was obtained revealing a widespread prominent mucoid intimal hyperplasia with severe occlusion and secondary changes of bloodless glomeruli. Mesangial lysis, acute tubular injury with focal ATN and minimal interstitial inflammation was seen. Repeat physical exam revealed thickening of the skin of the hands. Further testing revealed evidence of schistocytes, elevated LDH and positive RNA polymerase III. A diagnosis of PSRC was made and the patient was started on captopril. The patient continued to be dialysis dependent at the time of discharge.

Discussion

Paraneoplastic SCR has been reported in a few cases of lung, breast and abdominal cancers3. The pathogenesis is postulated to be related to cancer induced profibrotic cytokines and growth factors leading to renal vascular damage3. To our knowledge, there are no reported cases of squamous cell cancer associated PSCR . Our case adds to the literature of the rare PSCR in this patient population. This case highlights the importance of considering PSCR in the differential diagnosis of cancer patients presenting with AKI, TMA and higher than baseline blood pressure measurements.