How is systemic sclerosis associated with end-stage renal disease (ESRD)?

Updated: Mar 05, 2020
  • Author: Julia R Nunley, MD; Chief Editor: Dirk M Elston, MD  more...
  • Print


Systemic sclerosis (SSc) is a multisystem disorder that may affect the skin and several internal organs, specifically the gastrointestinal (GI) tract, lungs, heart, and kidneys. [25] The pathogenesis of SSc is unknown, but the end result is excessive fibrosis. SSc may be divided into 2 discrete types according to the extent of cutaneous involvement and includes limited cutaneous SSc and diffuse cutaneous SSc.

In individuals with limited cutaneous SSc, the onset of sclerosis is gradual and confined to areas distal to the elbows and knees, as well as to the face and neck. Digital ulcers are common and typically follow a long history of Raynaud phenomenon. Esophageal dysmotility may develop in some patients as a part of CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias).

The prognosis of limited cutaneous SSc is generally good, although pulmonary fibrosis or pulmonary hypertension may develop. Diffuse cutaneous SSc, unlike limited cutaneous SSc, develops acutely and is associated with constitutional symptoms and arthralgias. Cutaneous SSc involves the trunk and areas distal to and proximal to the elbows and knees. Diffuse cutaneous SSc has a worse prognosis than limited cutaneous SSc because of a higher incidence of internal organ involvement, including the kidneys. Other skin changes observed in both forms of SSc include hypopigmentation and hyperpigmentation, xerosis, alopecia, telangiectasias, and pruritus.

Renal involvement occurs in fewer than 20% of patients with SSc and tends to occur early, usually within the first 2 years of the disease and almost always within the first 5 years. Renal disease develops precipitously and without warning, although in some patients, rapidly progressive skin thickening may precede renal disease. Fulminant renal failure is usually associated with malignant hypertension.

Renal involvement is a poor prognostic indicator and, until recently, resulted in either ESRD or death. Immediate hospitalization and use of an angiotensin-converting enzyme inhibitor (ACEI) has improved overall renal outcome greatly.

Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!