Scleroderma history and symptoms: Difference between revisions
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In the past scleroderma renal crisis was almost uniformily fatal.<ref name=steen>{{cite journal |author=Steen VD |title=Scleroderma renal crisis |journal=Rheum. Dis. Clin. North Am. |volume=29 |issue=2 |pages=315-33 |year=2003 |pmid=12841297 |doi=}}</ref> While outcomes have improved significantly with the use of [[ACE inhibitors]]<ref>{{cite journal |author=Rhew EY, Barr WG |title=Scleroderma renal crisis: new insights and developments |journal=Current rheumatology reports |volume=6 |issue=2 |pages=129-36 |year=2004 |pmid=15016343 |doi=}}</ref><ref name=steen11033587>{{cite journal |author=Steen VD, Medsger TA |title=Long-term outcomes of scleroderma renal crisis |journal=Ann. Intern. Med. |volume=133 |issue=8 |pages=600-3 |year=2000 |pmid=11033587 |doi=}}</ref> the prognosis is often guarded, as a significant number of patients are refractory to treatment and develop [[renal failure]]. Approximately 10% of all scleroderma patients develop renal crisis at some point in the course of their disease. Patients that have rapid skin involvement have the highest risk of renal complications.<ref name=jimenez/> | In the past scleroderma renal crisis was almost uniformily fatal.<ref name=steen>{{cite journal |author=Steen VD |title=Scleroderma renal crisis |journal=Rheum. Dis. Clin. North Am. |volume=29 |issue=2 |pages=315-33 |year=2003 |pmid=12841297 |doi=}}</ref> While outcomes have improved significantly with the use of [[ACE inhibitors]]<ref>{{cite journal |author=Rhew EY, Barr WG |title=Scleroderma renal crisis: new insights and developments |journal=Current rheumatology reports |volume=6 |issue=2 |pages=129-36 |year=2004 |pmid=15016343 |doi=}}</ref><ref name=steen11033587>{{cite journal |author=Steen VD, Medsger TA |title=Long-term outcomes of scleroderma renal crisis |journal=Ann. Intern. Med. |volume=133 |issue=8 |pages=600-3 |year=2000 |pmid=11033587 |doi=}}</ref> the prognosis is often guarded, as a significant number of patients are refractory to treatment and develop [[renal failure]]. Approximately 10% of all scleroderma patients develop renal crisis at some point in the course of their disease. Patients that have rapid skin involvement have the highest risk of renal complications.<ref name=jimenez/> | ||
==Less Common Symptoms== | ==Less Common Symptoms== | ||
==References== | ==References== | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
History
Common 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]
Renal
Renal involvement, in scleroderma, is considered a poor prognostic factor and not infrequently a cause of death in patients with scleroderma.[8]
The most important clinical complication of scleroderma involving the kidney is scleroderma renal crisis. Symptoms of scleroderma renal crisis are malignant hypertension (high blood pressure with evidence of acute organ damage), hyperreninemia (high renin levels), azotemia (kidney failure with accumulation of waste products in the blood) and microangiopathic hemolytic anemia (destruction of red blood cells).[9] Apart from the high blood pressure, hematuria (blood in the urine) and proteinuria (protein loss in the urine) may be indicative.[10]
In the past scleroderma renal crisis was almost uniformily fatal.[11] While outcomes have improved significantly with the use of ACE inhibitors[12][13] the prognosis is often guarded, as a significant number of patients are refractory to treatment and develop renal failure. Approximately 10% of all scleroderma patients develop renal crisis at some point in the course of their disease. Patients that have rapid skin involvement have the highest risk of renal complications.[14]
Less Common Symptoms
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 Klippel J (ed). Systemic sclerosis and related syndromes. Primer on the rheumatic diseases, 11th edition. The Arthritis Society. 1997;269. ISBN 1-91242-316-2.
- ↑ 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.
- ↑ Ruangjutipopan S, Kasitanon N, Louthrenoo W, Sukitawut W, Wichainun R (2002). "Causes of death and poor survival prognostic factors in thai patients with systemic sclerosis". Journal of the Medical Association of Thailand. 85 (11): 1204–9. PMID 12546318.
- ↑ Steen VD, Mayes MD, Merkel PA (2003). "Assessment of kidney involvement". Clin. Exp. Rheumatol. 21 (3 Suppl 29): S29–31. PMID 12889219.
- ↑ Steen VD (1994). "Renal involvement in systemic sclerosis". Clin. Dermatol. 12 (2): 253–8. PMID 8076263.
- ↑ Steen VD (2003). "Scleroderma renal crisis". Rheum. Dis. Clin. North Am. 29 (2): 315–33. PMID 12841297.
- ↑ Rhew EY, Barr WG (2004). "Scleroderma renal crisis: new insights and developments". Current rheumatology reports. 6 (2): 129–36. PMID 15016343.
- ↑ Steen VD, Medsger TA (2000). "Long-term outcomes of scleroderma renal crisis". Ann. Intern. Med. 133 (8): 600–3. PMID 11033587.
- ↑ Invalid
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