Abstract: PUB066
Scleroderma Renal Crisis Sans Scleroderma
Session Information
Category: Acute Kidney Injury
- 103 AKI: Mechanisms
Authors
- Drury, Zachary, University of Utah Hospital, Salt Lake City, Utah, United States
- Agarwal, Adhish, University of Utah Hospital, Salt Lake City, Utah, United States
- Gregory, Martin C., University of Utah Hospital, Salt Lake City, Utah, United States
- Yadav, Niraj K., University of Utah Hospital, Salt Lake City, Utah, United States
- Revelo Penafiel, Monica Patricia, University of Utah Hospital, Salt Lake City, Utah, United States
- Lloyd, Isaac, Intermountain Healthcare, Salt Lake City, Utah, United States
- Abraham, Josephine, University of Utah Hospital, Salt Lake City, Utah, United States
Introduction
Scleroderma renal crisis (SRC) is an uncommon autoimmune disease that can present with hypertension, acute kidney injury (AKI), proteinuria, hematuria. Rarely is SRC the initial manifestation of scleroderma (scleroderma renal crisis sans scleroderma). We report a case of a patient presenting with SRC complicated by malignant hypertension, thrombotic microangiopathy, and acute kidney injury (AKI).
Case Description
A 47 year old female with four months of headache, blurry vision, and chest palpitations who presented to an outside hospital in hypertensive crisis. Serum Creatinine (Scr) was 1.1 mg/dl initially, however steadily increased to a peak level of 4.46 mg/dl. Urinalysis showed small blood and protein, and spot urine protein to creatinine ratio was 1,412 mg/g. SSA antibody was positive, while SLC 70 antibody and centromere antibody were negative. Serum aldosterone was 60.3 ng/dl and plasma renin activity 43.4ng/ml/hr with aldosterone/renin ratio 1.4. Evaluation for renal artery stenosis was negative. A kidney biopsy showed thrombotic microangiopathy with scattered subendothelial immune complex deposits. The patient was transferred to our facility where her SCr continued to worsen. She was started on lisinopril 2.5mg when her creatinine level was 3.89 mg/dl. SCr stabilized after three days of ACE inhibition.
Discussion
SRC is a medical emergency requiring prompt diagnosis and treatment. Diagnosis can be challenging when this is the initial presentation of scleroderma. SRC should be considered in the differential diagnosis for patients presenting with AKI, new-onset microscopic hematuria, proteinuria, malignant hypertension and thrombotic microangiopathy. ACE inhibition is crucial for patient survival and can lead to renal recovery, which could take as long as 24 months after a renal crisis.