A Sinister Crisis in Scleroderma

Case Report

Austin J Orthopade & Rheumatol. 2016; 3(3): 1038.

A Sinister Crisis in Scleroderma

Elizabeth H Federici¹, Nancy J Olsen¹, Michael G Bayerl² and Eileen F Hennrikus¹*

¹Department of Medicine, Penn State Hershey Medical Center, USA

²Department of Pathology, Penn State Hershey Medical Center, USAzil

*Corresponding author: Eileen F Hennrikus, Department of Medicine, Penn State Hershey Medical Center, Pennsylvania, USA

Received: August 11, 2016; Accepted: September 16, 2016; Published: September 19, 2016


We describe a presentation of Systemic Sclerosis (SSc) with rapid development of Scleroderma Renal Crisis (SRC) and multiorgan failure leading to death. Diagnoses were confirmed on post-mortem examination. SSc usually presents with skin thickening of the fingers and the presence of Anti-Nuclear Antibodies (ANA).Other associated clinical findings include fingertip lesions, telangiectasia, abnormal nailfold capillaries, pulmonary arterial hypertension, interstitial lung disease, and Raynaud’s phenomenon. ANA testing is negative in only a small minority of patients. Scleroderma Renal Crisis is characterized by acute renal failure, hypertension, and microangiopathic hemolytic anemia with thrombocytopenia, but occurs without hypertension in some cases. This case demonstrates an atypical case of SSc and SRC in that ANA testing was negative and Scleroderma Renal Crisis developed without hypertension. This presentation illustrates the challenge in diagnosing and managing SSc and SRC in a critically ill patient.

Keywords: Systemic Sclerosis; Scleroderma renal crisis; Anti-nuclear antibodies


Systemic Sclerosis (SSc) is an autoimmune disease in which autoantibodies against cellular antigens exist alongside diffuse fibrosis and vascular changes of the skin and internal organs. SSc most commonly involves the kidneys, esophagus, heart, and lungs, although nearly all internal organs may be affected [1]. Scleroderma associated renal failure was first reported in 1863 but was not recognized as a complication of SSc until described in a case series by Moore and Sheehan in 1952 [2]. Scleroderma Renal Crisis (SRC) is typically characterized by accelerated hypertension and acute renal failure, but occurs without hypertension in 10% of cases [3]. Microangiopathic hemolytic anemia and thrombocytopenia are recognized in approximately half of cases of SRC [4]. This report describes a man who presented with a suspected diagnosis of Systemic Sclerosis and developed normotensive Scleroderma Renal Crisis during hospitalization.

Case Presentation

A 56-year-old African American man was admitted with recurrent episodes of abdominal distention, nausea and vomiting. On initial examination he had skin thickening of the fingers of both hands extending proximally beyond the metacarpophalangeal joints to the upper arms and skin thickening of the toes, feet, and ankles. A small bowel follow-through demonstrated mild bowel dilation and marked delayed transit of contrast, but no evidence of mechanical obstruction. There was a “hide-bound” or “stacked coin” appearance of the bowel (Figure 1).