Systemic sclerosis (scleroderma)

Table of Contents

Alternative Names

CREST syndrome; Limited scleroderma; Progressive systemic sclerosis; Systemic sclerosis; Localized scleroderma; Morphea, linear


Treatment

There is still no treatment for the disease process of scleroderma. However, certain drugs and treatments help combat the complications.

Drugs used to treat scleroderma include:

  • Corticosteroids
  • Immune suppressing medications (methotrexate, Cytoxan)
  • Nonsteroidal anti-inflammatory drugs (NSAIDs)

Other treatments for specific symptoms may include:

  • Drugs for heartburn or swallowing problems
  • Blood pressure medications (particularly ACE inhibitors) for high blood pressure or kidney problems
  • Light treatments for skin thickening
  • Medicines to improve breathing (See: Pulmonary hypertension)
  • Medications to treat Raynaud's phenomenon

Treatment usually includes a combination of physical therapy and skin and joint protection techniques (for example, avoiding cold in the case of Raynaud's phenomenon).


Support Groups

See: Scleroderma - resources


Expectations (prognosis)

In most patients, the disease slowly gets worse. People who only have skin symptoms have a better outlook. Death may occur from gastrointestinal, heart, kidney, or lung complications.

One type of scleroderma, called limited scleroderma, involves only problems of the skin of the hands and face. It gets worse very slowly and usually does not affect other parts of the body.

Systemic scleroderma can involve many organs in the body. In some people, it will progress slowly and not involve any organs in the body. In other people, organs such as the lungs, kidneys, intestines, gallbladder, and heart become involved.

For some people, symptoms and problems develop quickly over the first few years, and continue to get worse. Others get worse much more slowly. Lung problems are the most common cause of death in patients with scleroderma.


Complications
  • Heart failure
  • Kidney failure
  • Malabsorption
  • Pulmonary fibrosis (the most common cause of death in people with scleroderma)
  • Pulmonary hypertension

Calling your health care provider

Call for an appointment with your health care provider if:

  • You have symptoms of scleroderma
  • You have scleroderma and symptoms become worse or new symptoms develop


Review Date: 02/07/2010
Reviewed By: Mark James Borigini, MD, Rheumatologist in the Washington, DC Metro area. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)