Scleroderma
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]
For patient information click here
Synonyms and keywords: Systemic sclerosis
Click here for The Heart in Scleroderma
Overview
Epidemiology
Pathophysiology
Etiology
Types of Scleroderma
Diagnosis
Diagnosis is by clinical suspicion, presence of autoantibodies (specifically anti-centromere and anti-scl70/anti-topoisomerase antibodies) and occasionally by biopsy. Of the antibodies, 90% have a detectable anti-nuclear antibody. Anti-centromere antibody is more common in the limited form (80-90%) than in the systemic form (10%), and anti-scl70 is more common in the diffuse form (30-40%) and in African-American patients (who are more susceptible to the systemic form).[1]
In 1980 the American College of Rheumatology agreed upon diagnostic criteria for scleroderma.[2]
Diffuse scleroderma can cause musculoskeletal, pulmonary, gastrointestinal, renal and other complications.[3]Patients with larger amounts of cutaneous involvement are more likely to have involvement of the internal tissues and organs.
Cardiovascular Symptoms
Skin Symptoms
Musculoskeletal System Related Symptoms
Respiratory System Symptoms
Gastrointestinal System Related Symptoms
Renal Symptoms
Therapy
There is no cure for every patient with scleroderma, though there is treatment for some of the symptoms, including drugs that soften the skin and reduce inflammation. Some patients may benefit from exposure to heat.[4]
Digital ulcerations and pulmonary hypertension can be helped by prostacyclin (iloprost) infusion. Iloprost being a drug which increases blood flow by relaxing the arterial wall.[5]
Topical/symptomatic
Topical treatment for the skin changes of scleroderma do not alter the disease course, but may improve pain and ulceration. A range of NSAIDs (nonsteroidal anti-inflammatory drugs) can be used to ease painful symptoms, such as naproxen. There is limited benefit from steroids such as prednisone. Episodes of Raynaud's phenomenon sometimes respond to nifedipine or other calcium channel blockers; severe digital ulceration may respond to prostacyclin analogue iloprost, and the dual endothelin-receptor antagonist bosentan may be beneficial for Raynaud's phenomenon.[6] The skin tightness may be treated systemically with methotrexate and cyclosporin.[6] If there is esophageal dysmotility (in CREST or systemic sclerosis), care must be taken with NSAIDs as they are gastric irritants, and so a proton pump inhibitor (PPI) such as omeprazole can be given in conjunction.
Kidney disease
Scleroderma renal crisis, the occurrence of acute renal failure and malignant hypertension (very high blood pressure with evidence of organ damage) in people with scleroderma, is effectively treated with drugs from the class of the ACE inhibitors. The benefit of ACE inhibitors extends even to those who have to commence dialysis to treat their kidney disease, and may give sufficient benefit to allow the discontinuation of renal replacement therapy.[6] ACE inhibitors are also used for prophylaxis,[7][8] and renal transplantation. Transplanted kidneys are known to be affected by scleroderma and patients with early onset renal disease (within one year of the scleroderma diagnosis) are thought to have the highest risk for recurrence.[9]
Lung disease and pulmonary hypertension
Active alveolitis is often treated with pulses of cyclophosphamide, often together with a small dose of steroids. The benefit of this intervention is modest.[10][11]
Pulmonary hypertension may be treated with epoprostenol, bosentan and possibly aerolized iloprost.[6]
Experimental treatments
Case Studies
References
- ↑ Jimenez SA, Derk CT (2004). "Following the molecular pathways toward an understanding of the pathogenesis of systemic sclerosis". Ann. Intern. Med. 140 (1): 37–50. PMID 14706971.
- ↑ "Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee". Arthritis Rheum. 23 (5): 581–90. PMID 7378088. Text "year:1980 " ignored (help) Available online at "Criteria for the Classification of Systemic Sclerosis 1980". Text " accessdate:5 August 2007" ignored (help)
- ↑ Invalid
<ref>tag; no text was provided for refs namedPrimer - ↑ Oliver GF, Winkelmann RK (1989). "The current treatment of scleroderma". Drugs. 37 (1): 87–96. PMID 2651089.
- ↑ Zandman-Goddard G, Tweezer-Zaks N, Shoenfeld Y (2005). "New therapeutic strategies for systemic sclerosis--a critical analysis of the literature". Clin. Dev. Immunol. 12 (3): 165–73. PMID 16295521.
- ↑ 6.0 6.1 6.2 6.3 Zandman-Goddard G, Tweezer-Zaks N, Shoenfeld Y (2005). "New therapeutic strategies for systemic sclerosis--a critical analysis of the literature". Clin. Dev. Immunol. 12 (3): 165–73. PMID 16295521. PMC 2275417
- ↑ Invalid
<ref>tag; no text was provided for refs namedjimenez - ↑ Invalid
<ref>tag; no text was provided for refs namedsteen11033587 - ↑ Pham PT, Pham PC, Danovitch GM, Gritsch HA, Singer J, Wallace WD, Hayashi R, Wilkinson AH. Predictors and risk factors for recurrent scleroderma renal crisis in the kidney allograft: case report and review of the literature. Am J Transplant. 2005 Oct;5(10):2565-9. PMID 16162209.
- ↑ Tashkin DP, Elashoff R, Clements PJ; et al. (2006). "Cyclophosphamide versus placebo in scleroderma lung disease". N. Engl. J. Med. 354 (25): 2655–66. doi:10.1056/NEJMoa055120. PMID 16790698. Unknown parameter
|month=ignored (help) - ↑ Hoyles RK, Ellis RW, Wellsbury J; et al. (2006). "A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma". Arthritis Rheum. 54 (12): 3962–70. doi:10.1002/art.22204. PMID 17133610. Unknown parameter
|month=ignored (help)
External links
- Goldminer: Scleroderma
- DermnetNZ: Systemic sclerosis
- Juvenile Scleroderma Network
- Juvenile Systemic Sclerosis
- Scleroderma Foundation
- Scleroderma Society of Ontario
- International Scleroderma Network
- The Scleroderma Research Foundation
- UK Scleroderma Society
- Scleroderma Information from Johns Hopkins University
Template:Diseases of the musculoskeletal system and connective tissue
de:Sklerodermie it:Sclerodermia he:סקלרודרמה nl:Sclerodermie sk:Sklerodermia sv:Sklerodermi