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Systemic sclerosis

Scleroderma is a rare, chronic disease characterized by excessive deposits of collagen. Progressive systemic scleroderma or systemic sclerosis, the generalised type of the disease, can be fatal. The localised type of the disease tends not to be fatal. The term 'localised, generalised sclerderma' can be used to describe cases where the disease covers a large area of the body - typically more than 40%. more...

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Signs and symptoms

Scleroderma affects the skin, and in more serious cases, it can affect the blood vessels and internal organs. The most evident symptom is the hardening of the skin and associated scarring. Typically the skin appears reddish or scaly in appearance. Blood vessels may also be more visible. Where large areas are affected, fat and muscle wastage will weaken limbs and affect appearance.

The seriousness of the disease varies hugely between cases. The two most important factors to consider are, the level of internal involvement (beneath the skin), and the total area covered by the disease. For example there are cases where the patient has no more than one or two lesions (affected areas), perhaps covering a few inches. These are less serious cases and tend not to involve the internal bodily functions.

Cases with larger coverage are far more likely to affect the internal tissues and organs. Where an entire limb is affected, symptoms will almost certainly have serious consequences on the use of that limb. The heart and lungs will be affected when the disease covers this area of the torso. Some patients also experience gastrointestinal problems, including heartburn and acid reflux. Internal scarring may sometimes spread beyond what can be seen by the naked eye.

There is discoloration of the hands and feet in response to cold. Most patients (>80%) have Raynaud's phenomenon, a vascular symptom that can affect the fingers, and toes.

Systemic scleroderma and Raynaud's can cause painful ulcers on the fingers or toes, which are known as digital ulcers.

Types

There are three major forms of scleroderma: diffuse, limited (CREST syndrome) and morphea/linear. Diffuse and limited scleroderma are both a systemic disease, whereas the linear/morphea form is localized to the skin. (Some physicians consider CREST and limited scleroderma one and the same, others treat them as two separate forms of scleroderma.)

Diffuse scleroderma

Diffuse scleroderma is the most severe form - it has a rapid onset, involves more widespread skin hardening, will generally cause much internal organ damage (specifically the lungs and gastrointestinal tract), and is generally more life threatening.

Limited scleroderma/CREST syndrome

The limited form is much milder: it has a slow onset and progression, skin hardening is usually confined to the hands and face, internal organ involvement is less severe, and a much better prognosis is expected.

The limited form is often referred to as "CREST" syndrome. CREST is an acronym for:

  • Calcinosis
  • Raynaud's syndrome
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasia

These five are the major symptoms of the CREST syndrome.

Read more at Wikipedia.org


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Lung Transplantation In Systemic Sclerosis - Abstract
From CHEST, 10/1/00 by I Rosas

I Rosas(*); S M Studer; J C Conte; S Yang; M Borja; S P Gaine and J B Orens. Divisions of Pulmonary & Critical Care Medicine, The Johns Hopkins University, Baltimore, MD and The National Heart Lung & Blood Institute, Bethesda, MD.

PURPOSE: Scleroderma is a systemic condition that affects multiple visceral organs including kidney, heart and lung. Prior to the use of hemodialysis and ACE inhibitors most patients died of renal insufficiency. Presently lung disease due to pulmonary fibrosis and/or hypertension is the leading cause of death. Because of the systemic nature of this disease, scleroderma is considered a contraindication for lung transplantation at most centers. A significant number of patients will develop progressive lung disease that is unresponsive to conventional medical therapy. We have opted to transplant some of these patients with limited systemic involvement. We report our experience.

METHODS: A total of nine patients were transplanted over a 24-month period. Two patients with the CREST variant had isolated pulmonary hypertension. Three had both pulmonary fibrosis and hypertension. The remaining five with diffuse Ssc had predominantly pulmonary fibrosis.

RESULTS: The incidence of postoperative renal insufficiency and chronic renal failure was comparable to that of the general, transplant population. Only one patient from the Ssc group required ambulatory hemodialysis for a limited period of time. Analysis of other common complications including infection and rejection failed to reveal a difference amongst the two groups, Overall survival by Kaplan-Meir analysis was 60% at 4 years.

CONCLUSION: In our experience post-transplant survival and prognosis for patients with Ssc who have limited extrapulmonary disease compares favorablv to current ISHLT statistics for lung transplantation overall. We conclude that lung transplantation should be considered for selected patients with scleroderma and end-stage pulmonary disease.

COPYRIGHT 2000 American College of Chest Physicians
COPYRIGHT 2001 Gale Group

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