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What Triggers Scleroderma?

Reviewed on 5/30/2020

What Is Scleroderma?

Skin hardening first affects the fingers in people with scleroderma.
Skin hardening first affects the fingers in people with scleroderma.

Scleroderma is a rare autoimmune disease characterized by progressive skin and connective tissue tightening and hardening. Scleroderma is a type of systemic connective tissue disease (systemic sclerosis) that may also affect subcutaneous tissue, muscles, and internal organs.

What Are Signs and Symptoms of Scleroderma?

Generalized symptoms and signs of scleroderma include

Symptoms and signs of systemic sclerosis may affect many parts of the body:

What Causes Scleroderma?

The exact cause of scleroderma is unknown.

Risk factors for development of the disease include the following:

  • Genetic predisposition
  • Environmental factors (for example, triggers) such as
    • Silica exposure
    • Solvent exposure (vinyl chloride, trichloroethylene, epoxy resins, benzene, carbon tetrachloride)
    • Radiation exposure or radiotherapy
  • Past treatment with the immune checkpoint inhibitor pembrolizumab for metastatic melanoma

How Do Doctors Diagnose Scleroderma?

The diagnosis of scleroderma is based on a physical examination. Your doctor may order tests to help with the initial diagnosis or to rule out other conditions.

Laboratory testing may include the following:

  • Complete blood cell count (CBC)
  • Serum muscle enzyme levels
  • Erythrocyte sedimentation rate
  • Serum CXCL4 level
  • N-terminal pro-brain natriuretic peptide
  • Autoantibody assays

Other testing may include the following:

  • For gastrointestinal symptoms:
    • X-rays of the gastrointestinal tract
    • Esophagogastroduodenoscopy with appropriate biopsies
    • Esophageal manometry assessment
    • Gastric emptying study
    • Colonoscopy
  • For cardiopulmonary symptoms:
  • Other studies:
    • X-rays of extremities
    • Nail-fold capillary microscopy

What Is the Treatment for Scleroderma?

Treatment of scleroderma aims to relieve symptoms and signs and manage complications. Doctors should reevaluate patients should be every 3-6 months, depending on the disease activity and progression.

Treatment of skin hardening and scarring may include the following:

Treatment for itching may include

Treatment for Raynaud phenomenon may include the following:

  • Calcium channel blockers
  • Prazosin
  • Prostaglandin derivatives (for example, prostaglandin E1)
  • Dipyridamole
  • Aspirin
  • Topical nitrates
  • In serious cases, a tissue plasminogen activator, heparin, and urokinase may be necessary. In very severe cases, intravenous iloprost or related prostanoids may be indicated.

Treatment for digital ulcers (ulcers on the fingers or toes) may include

  • bosentan,
  • combination therapy with iloprost and bosentan, and
  • ambrisentan and other endothelin receptor antagonists.

Treatment for gastrointestinal symptoms may include

  • antacids, histamine 2 (H2) blockers, proton pump inhibitors;
  • reflux and aspiration precautions;
  • prokinetic agents;
  • octreotide;
  • stool softeners; and
  • laxatives.

Treatment for pulmonary fibrosis (lung scarring) may include

Treatment for pulmonary arterial hypertension (PAH) in scleroderma may include the following:

  • Prostaglandin derivatives such as epoprostenol, treprostinil, beraprost, and iloprost
  • Phosphodiesterase type 5 (PDE-5) inhibitors such as sildenafil and tadalafil
  • Endothelin receptor antagonists such as bosentan, ambrisentan, and macitentan

Other treatments...

  • Carpal tunnel entrapment symptoms may be treated with local corticosteroid injections.
  • Muscle inflammation (myositis) may be treated with steroids (first choice), methotrexate, or azathioprine.
  • Joint pain may be treated with acetaminophen and nonsteroidal anti-inflammatory drugs (NSAIDs).

Surgery for scleroderma symptoms may include the following:

  • Digital (involves fingers and/or toes) sympathectomy and botulinum toxin injections for patients with severe Raynaud's phenomenon
  • Digital debridement or amputation for severe ischemic or infected digital lesions
  • Hand surgery to correct severe flexion contractures
  • Removal of severely painful, draining, or infected calcinotic deposits
  • Fundoplication to treat severe esophageal reflux with complicating aspiration pneumonitis


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What Are Complications of Scleroderma?

Renal crisis occurs in about 10% of all patients with systemic sclerosis. If this condition is not treated promptly and aggressively, it leads to leads to renal failure requiring dialysis, kidney transplantation, or death.

Other complications of systemic sclerosis include

What Is the Life Expectancy for Scleroderma?

Systemic sclerosis has the highest mortality rate among systemic autoimmune diseases due to pulmonary hypertension, pulmonary fibrosis (interstitial lung disease), and scleroderma renal crisis.

The 5-year survival rate (patients are still alive 5 years following diagnosis) among patients with diffuse cutaneous systemic sclerosis is about 84%. The 5-year survival among the patients with limited skin (cutaneous) systemic sclerosis is high, over 90%.

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Reviewed on 5/30/2020
Jimenez, Sergio A. "Scleroderma." Sept. 9, 2019. Medscape.com. <http://emedicine.medscape.com/article/331864-overview>.

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