Editing Systemic scleroderma (section)

== Treatment ==
No cure for scleroderma is known, though treatments exist 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 |vauthors=Oliver GF, Winkelmann RK |title=The current treatment of scleroderma |journal=Drugs |volume=37 |issue=1 |pages=87–96 |year=1989 |pmid=2651089 |doi=10.2165/00003495-198937010-00006|s2cid=25010323 }}</ref>  Holistic care of patients comprising patient education tailored to patients' education level is useful in view of the complex nature of the disease symptoms and progress.<ref>{{cite book |author1=Philip J. Clements |author2=Daniel E. Furst |title=Systemic Sclerosis, 2nd ed., Chapt. 23 |url=http://www.hopkinsscleroderma.org/downloads/SystemicSclerosis_Chapter23.pdf}}</ref>

===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|nonsteroidal anti-inflammatory drugs]], such as [[naproxen]], can be used to ease painful symptoms.{{Citation needed|date=May 2008}} The benefit from [[glucocorticoid|steroids]] such as prednisone is limited.{{Citation needed|date=May 2008}} 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 |vauthors=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=10.1080/17402520500233437 |pmc=2275417}}</ref> Skin tightness may be treated systemically with [[methotrexate]] and [[ciclosporin]].<ref name=Zandberg/> and the skin  thickness can be treated with penicillamine.

===Kidney disease===
Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis that may be the initial manifestation of the disease.  Renal vascular injury (due in part to collagen deposition) leads to renal ischemia, which results in activation of the renin-angiotensin-aldosterone system (RAAS).  This raises blood pressure and further damages the renal vasculature, causing a vicious cycle of worsening hypertension and renal dysfunction (e.g., elevated creatinine, edema).  Hypertensive emergency with end-organ dysfunction (e.g., encephalopathy, retinal hemorrhage) is common.  Thrombocytopenia and microangiopathic hemolytic anemia can be seen.  Urinalysis is usually normal but may show mild proteinuria, as in this patient; casts are unexpected.{{citation needed|date=September 2021}}

The mainstay of therapy for SRC includes ACE inhibitors, which reduce RAAS activity and improve renal function and blood pressure.  Short-acting ACE inhibitors (typically captopril) are used because they can be rapidly uptitrated.  An elevated serum creatinine level is not a contraindication for ACE inhibitors in this population, and slight elevations in creatinine are common during drug initiation.

Scleroderma renal crisis, the occurrence of [[acute kidney injury]], and [[malignant hypertension]] (very high blood pressure with evidence of organ damage) in people with scleroderma are effectively treated with drugs from the class of the ACE inhibitors. 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/>

===Lung disease===
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  |vauthors=Tashkin DP, Elashoff R, Clements PJ, etal |title=Cyclophosphamide versus placebo in scleroderma lung disease |journal=N. Engl. J. Med. |volume=354 |issue=25 |pages=2655–66 |date=June 2006 |pmid=16790698 |doi=10.1056/NEJMoa055120 |doi-access=free }}</ref><ref>{{cite journal  |vauthors=Hoyles RK, Ellis RW, Wellsbury J, etal |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 |date=December 2006 |pmid=17133610 |doi=10.1002/art.22204 |doi-access=free }}</ref>

Pulmonary hypertension may be treated with [[epoprostenol]], [[treprostinil]], [[bosentan]], and possibly aerolized iloprost.<ref name=Zandberg/> [[Nintedanib]] was approved for use in the United States [[Food and Drug Administration]] on September 6, 2019, to slow the rate of decline in pulmonary function in patients with systemic sclerosis-associated [[interstitial lung disease]] (SSc-ILD).<ref>{{Cite web|url=http://www.fda.gov/news-events/press-announcements/fda-approves-first-treatment-patients-rare-type-lung-disease|archive-url=https://web.archive.org/web/20190906201437/https://www.fda.gov/news-events/press-announcements/fda-approves-first-treatment-patients-rare-type-lung-disease|url-status=dead|archive-date=September 6, 2019|title=FDA approves first treatment for patients with rare type of lung disease|last=Commissioner|first=Office of the|date=2020-02-20|website=FDA|language=en|access-date=2020-02-25}}</ref><ref>{{Cite web |title=FDA approves Ofev® as the first and only therapy in the U.S. to slow the rate of decline in pulmonary function in patients with systemic sclerosis-associated ILD |url=https://www.boehringer-ingelheim.com/press-release/fda-approves-nintedanib-ssc-ild |access-date=2022-11-30 |website=www.boehringer-ingelheim.com}}</ref>

===Other===
Some evidence indicates that [[plasmapheresis]] (therapeutic plasma exchange) can be used to treat the systemic form of scleroderma. In Italy, it is a government-approved treatment option. This is done by replacing [[blood plasma]] with a fluid consisting of [[albumin]], and is thought to keep the disease at bay by reducing the circulation of scleroderma autoantibodies.<ref>{{cite journal|title=Therapeutic plasma exchange for the treatment of systemic sclerosis: A comprehensive review and analysis|doi=10.1177/2397198318758606|year=2018|last1=Harris|last2=Meiselman|last3=Moriarty|last4=Metzger|last5=Malkovsky|first1=Edward|first2=Herbert|first3=Patrick|first4=Allan|first5=Miroslav|journal=Journal of Scleroderma and Related Disorders|volume=3|issue=2|pages=132–152|pmid=35382237 |pmc=8892860 |s2cid=79950781|doi-access=free}}</ref>