# Systemic Sclerosis

**IRI:** https://folio.openlegalstandard.org/R7lCj2dvGu7618ONPPEpJB

## Labels

**Preferred Label:** Scleroderma

**Alternative Labels:**

- Autoimmune Connective Tissue Disorder
- Progressive Systemic Sclerosis

## Definition

Systemic sclerosis (also known as scleroderma) constitutes a spectrum of disease in which thickening of the skin is the clinical hallmark. Raynaud's phenomenon, often medically severe and progressive, is present frequently and may be the peripheral manifestation of a vasospastic abnormality in the heart, lungs, and kidneys. The CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) is a variant that may slowly progress over years to the generalized process, systemic sclerosis.

## Sub Class Of

- https://folio.openlegalstandard.org/RBJ0dgV4Z2Xk7aGfJt2nSj7

**Deprecated:** False