Autoimmune/Rheumatic

Scleroderma (Systemic Sclerosis)

Scleroderma, also known as systemic sclerosis, is a group of rare diseases that involve the hardening and tightening of the skin and connective tissues. Some types can also harm the blood vessels, internal organs, and the digestive tract. It is an autoimmune disease in which the immune system triggers excessive collagen production.

6

Symptoms

3

Causes

6

Treatments

1

Prevention

Condition Overview

Understand key symptoms, causes, diagnosis options, and treatment pathways for Scleroderma (Systemic Sclerosis). This overview is intended for patient awareness and should be followed by specialist consultation.

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Symptoms

  • Hardening and tightening of skin
  • Raynaud's phenomenon
  • CREST syndrome features (Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasia)
  • Digestive problems
  • Heart, lung, kidney involvement
  • Joint pain

Causes

  • Autoimmune — overproduction of collagen
  • Genetic and environmental factors
  • Possible immune response to injury

Diagnosis

  • Physical examination
  • Blood tests (ANA, anti-Scl-70, anti-centromere antibodies)
  • Pulmonary function tests
  • Echocardiogram
  • CT scan of lungs
  • GI tests

Treatment

  • No cure; treatment focuses on specific symptoms
  • Immunosuppressants
  • Proton pump inhibitors for GERD
  • Calcium channel blockers for Raynaud's
  • Bosentan or sildenafil for pulmonary hypertension
  • ACE inhibitors for renal crisis

Risk Factors

  • Female sex
  • Age 30–50
  • African American and Choctaw Native American ancestry
  • Certain occupational exposures (silica, solvents)

Prevention

  • No known prevention

Prevalence

About 300,000 Americans have scleroderma; it affects 75,000–100,000 adults.