|
|
| Line 7: |
Line 7: |
|
| |
|
| ==Medical Therapy== | | ==Medical 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.<ref>{{cite journal |author=Oliver GF, Winkelmann RK |title=The current treatment of scleroderma |journal=Drugs |volume=37 |issue=1 |pages=87-96 |year=1989 |pmid=2651089 |doi=}}</ref>
| |
|
| |
| 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.<ref>{{cite journal |author=Zandman-Goddard G, Tweezer-Zaks N, Shoenfeld Y |title=New therapeutic strategies for systemic sclerosis--a critical analysis of the literature |journal=Clin. Dev. Immunol. |volume=12 |issue=3 |pages=165-73 |year=2005 |pmid=16295521 |doi=}}</ref>
| |
|
| |
| ===Pharmacotherapy===
| |
|
| |
| ====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 [[NSAID]]s (nonsteroidal anti-inflammatory drugs) can be used to ease painful symptoms, such as [[naproxen]]. There is limited benefit from [[glucocorticoid|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.<ref name=Zandberg>{{cite journal |author=Zandman-Goddard G, Tweezer-Zaks N, Shoenfeld Y |title=New therapeutic strategies for systemic sclerosis--a critical analysis of the literature |journal=Clin. Dev. Immunol. |volume=12 |issue=3 |pages=165–73 |year=2005 |pmid=16295521 |doi=}} {{PMC|2275417}}</ref> The skin tightness may be treated systemically with [[methotrexate]] and [[cyclosporin]].<ref name=Zandberg/> 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 inhibitor]]s. The benefit of ACE inhibitors extends even to those who have to commence [[hemodialysis|dialysis]] to treat their kidney disease, and may give sufficient benefit to allow the discontinuation of renal replacement therapy.<ref name=Zandberg/> [[ACE inhibitors]] are also used for [[prophylaxis]],<ref name="pmid14706971">{{cite journal |author=Jimenez SA, Derk CT |title=Following the molecular pathways toward an understanding of the pathogenesis of systemic sclerosis |journal=[[Annals of Internal Medicine]] |volume=140 |issue=1 |pages=37–50 |year=2004 |month=January |pmid=14706971 |doi= |url=http://www.annals.org/article.aspx?volume=140&page=37 |accessdate=2012-08-30}}</ref><ref>{{cite journal |author=Steen VD, Mayes MD, Merkel PA |title=Assessment of kidney involvement |journal=Clin. Exp. Rheumatol. |volume=21 |issue=3 Suppl 29 |pages=S29-31 |year=2003 |pmid=12889219 |doi=}}</ref> 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.<ref>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.</ref>
| |
|
| |
| ====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.<ref>{{cite journal |author=Tashkin DP, Elashoff R, Clements PJ, ''et al'' |title=Cyclophosphamide versus placebo in scleroderma lung disease |journal=N. Engl. J. Med. |volume=354 |issue=25 |pages=2655–66 |year=2006 |month=June |pmid=16790698 |doi=10.1056/NEJMoa055120 |url=http://content.nejm.org/cgi/content/full/354/25/2655}}</ref><ref>{{cite journal |author=Hoyles RK, Ellis RW, Wellsbury J, ''et al'' |title=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 |journal=Arthritis Rheum. |volume=54 |issue=12 |pages=3962–70 |year=2006 |month=December |pmid=17133610 |doi=10.1002/art.22204 | url=http://www3.interscience.wiley.com/cgi-bin/fulltext/113490260/HTMLSTART}}</ref>
| |
|
| |
| Pulmonary hypertension may be treated with [[epoprostenol]], [[bosentan]] and possibly aerolized iloprost.<ref name=Zandberg/>
| |
|
| |
|
| ==References== | | ==References== |