ASN's Mission

ASN leads the fight to prevent, treat, and cure kidney diseases throughout the world by educating health professionals and scientists, advancing research and innovation, communicating new knowledge, and advocating for the highest quality care for patients.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on Twitter

Kidney Week

Abstract: FR-PO538

A Case of Scleroderma Renal Crisis in a Patient with Systemic Sclerosis Sine Scleroderma

Session Information

Category: Trainee Case Report

  • 101 AKI: Epidemiology, Risk Factors, and Prevention

Authors

  • Cho, Janis, Northwestern University- Feinberg School of Medicine, Chicago, Illinois, United States
  • Kanwar, Yashpal S., Northwestern University Medical School, Chicago, Illinois, United States
  • Ghossein, Cybele, Northwestern University- Feinberg School of Medicine, Chicago, Illinois, United States
Introduction

Scleroderma renal crisis (SRC) typically presents with abrupt onset of accelerated hypertension (HTN) and acute kidney injury (AKI) usually in patients with diffuse cutaneous Systemic Sclerosis (dcSSc). We report a case of SRC in a patient with systemic sclerosis sine scleroderma (ssSSc), a rare form that presents with visceral involvement in the absence of skin manifestation.

Case Description

A 64 year old female with a history of Raynaud’s phenomenon, seronegative rheumatoid arthritis and HTN was admitted to Northwestern Memorial Hospital with shortness of breath, uncontrolled HTN and AKI. Her blood pressure (BP) ranged from 165/94 to 194/100 mmHg. She had bilateral crackles, no lower extremity edema and no skin manifestations of scleroderma. Anti nuclear antibody was positive >1:1280 with a negative serologic work up including anti-SCL-70, anti-centromere and RNA polymerase III antibodies. Urinalysis was without protein or blood. HTN was uncontrolled and captopril was started with good BP response. AKI worsened and a kidney biopsy was performed (Fig). Pathology showed ischemic glomerulopathy, marked arteriosclerosis with onion skinning and vascular lesions suspicious for thrombotic microangiopathy concerning for SRC. Her clinical course deteriorated rapidly with the development of severe pulmonary HTN, heart and renal failure.

Discussion

ssSSc is a rare form of systemic sclerosis. Two types of ssSSc patients have been described and the course can follow either limited scleroderma or dcSSc. The latter can have delayed sclerodactyly and early vital organ involvement similar to our case. Clinicians need high degree of suspicion to diagnose ssSSc given the lack of skin findings. While serologic testing can help with diagnosis, SRC is a clinical diagnosis that can be enhanced by renal biopsy. This case highlights the difficulty in diagnosing patients with SRC in the setting of ssSSc.

Arteriosclerosis with onion skinning