Scleroderma history and symptoms
|
Scleroderma Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Scleroderma history and symptoms On the Web |
|
American Roentgen Ray Society Images of Scleroderma history and symptoms |
|
Risk calculators and risk factors for Scleroderma history and symptoms |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Symptoms
Skin
- 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. 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 have been cases where the patient has no more than one or two lesions, perhaps covering a few inches. Less serious cases tend not to involve the internal bodily functions.
- There is discoloration of the hands and feet in response to cold. Most patients (over 80%) have Raynaud's phenomenon, a vascular symptom that can affect the fingers and toes.
- Systemic scleroderma and Raynaud's phenomenon can cause painful ulcers on the fingers or toes which are known as digital ulcers.
- Calcinosis is also common in systemic scleroderma, and is often seen near the elbows, knees or other joints.
Lungs
Some impairment in lung function is almost universally seen in patients with diffuse scleroderma on pulmonary function testing;[1] however, it does not necessarily cause symptoms, such as shortness of breath. Some patients can develop pulmonary hypertension, or elevation in the pressures of the pulmonary arteries. This can be progressive, and lead to right sided heart failure. The earliest manifestation of this may be a decreased diffusion capacity on pulmonary function testing.
Other pulmonary complications in more advanced disease include aspiration pneumonia, pulmonary hemorrhage and pneumothorax.[2]
Musculoskeletal
The first joint symptoms that patients with scleroderma have are typically non specific joint pains, which can lead to arthritis, or cause discomfort in tendons or muscles.[2] Joint mobility, especially of the small joints of the hand, may be restricted by calcinosis or skin thickening.[3] Patients who have progressed later in their disease may develop muscle weakness, or myopathy, either from the disease, or its treatments.[4]
Gastrointestinal
Diffuse scleroderma can affect any part of the gastrointestinal tract.[5] The most common manifestation in the esophagus is reflux esophagitis, which may be complicated by peptic stricturing, or benign narrowing of the esophagus.[6] This is best initially treated with proton pump inhibitors for acid suppression,[7] but may require bougie dilatation in the case of stricture.[5]
Scleroderma can decrease motility anywhere in the gastrointestinal tract.[5] The most common source of decreased motility involvement is the esophagus and the lower esophageal sphincter, leading to dysphagia and chest pain. As Scleroderma progresses, esophageal involvement from abnormalities in decreased motility may worsen due to progressive fibrosis (scarring). If this is left untreated, acid from the stomach can back up into the esophagus causing esophagitis, and GERD. Further scarring from acid damage to the lower esophagus many times leads to the development of fibrotic narrowing, also known as strictures which can be treated by dilatation, and Barrett's esophagus. The small intestine can also become involved, leading to bacterial overgrowth and malabsorption, of bile salts, fats, carbohydrates, proteins, and vitamins. The colon can be involved, and can cause pseudo-obstruction or ischemic colitis.[2]
Rarer complications include pneumatosis cystoides intestinalis, or gas pockets in the bowel wall, wide mouthed diverticula in the colon and esophagus, and liver fibrosis. Patients with severe gastrointestinal involvement can become profoundly malnourished.[6]
Scleroderma may also be associated with gastric antral vascular ectasia (GAVE), also known as watermelon stomach. This is a condition where atypical blood vessels proliferate usually in a radially symmetric pattern around the pylorus of the stomach. GAVE can be a cause of upper gastrointestinal bleeding or iron deficiency anemia in patients with scleroderma.[6]
References
- ↑ Steen VD (2005). "The lung in systemic sclerosis". Journal of clinical rheumatology. 11 (1): 40–6. PMID 16357695.
- ↑ 2.0 2.1 2.2 Invalid
<ref>tag; no text was provided for refs namedPrimer - ↑ Valentini G, Black C (2002). "Systemic sclerosis". Best practice & research. Clinical rheumatology. 16 (5): 807–16. PMID 12473275.
- ↑ Olsen NJ, King LE, Park JH (1996). "Muscle abnormalities in scleroderma". Rheum. Dis. Clin. North Am. 22 (4): 783–96. PMID 8923596.
- ↑ 5.0 5.1 5.2 Sallam H, McNearney TA, Chen JD (2006). "Systematic review: pathophysiology and management of gastrointestinal dysmotility in systemic sclerosis (scleroderma)". Aliment. Pharmacol. Ther. 23 (6): 691–712. doi:10.1111/j.1365-2036.2006.02804.x. PMID 16556171.
- ↑ 6.0 6.1 6.2 Rose S, Young MA, Reynolds JC (1998). "Gastrointestinal manifestations of scleroderma". Gastroenterol. Clin. North Am. 27 (3): 563–94. PMID 9891698.
- ↑ Hendel L, Hage E, Hendel J, Stentoft P (1992). "Omeprazole in the long-term treatment of severe gastro-oesophageal reflux disease in patients with systemic sclerosis". Aliment. Pharmacol. Ther. 6 (5): 565–77. PMID 1420748.