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{{Scleroderma}}
{{Scleroderma}}


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==Overview==
==Overview==
==History==
The hallmark of scleroderma is [[sclerodactyly]]. A positive history of progressive [[skin]] tightening and hardening is suggestive of scleroderma. The most common symptoms of scleroderma include [[skin]] tightening or [[induration]], [[Raynaud's phenomenon]], and symptoms of [[gastroesophageal reflux disease]] (GERD). Less common symptoms of scleroderma include shiny [[skin]] appearance and restricted movement of affected areas of the [[skin]].
==Common Symptoms==
===Skin===
* Scleroderma affects the [[skin]], and in more serious cases it can affect the [[blood vessel]]s 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 [[limb]]s 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.
==History and Symptoms==
The hallmark of scleroderma is [[sclerodactyly]]. A positive history of progressive [[skin]] tightening and hardening is suggestive of scleroderma. The most common symptoms of scleroderma include [[skin]] tightening or [[induration]], [[Raynaud's phenomenon]] and symptoms of [[gastroesophageal reflux disease]] (GERD).<ref name="pmid28133631">{{cite journal |vauthors=Shreiner AB, Murray C, Denton C, Khanna D |title=Gastrointestinal Manifestations of Systemic Sclerosis |journal=J Scleroderma Relat Disord |volume=1 |issue=3 |pages=247–256 |date=2016 |pmid=28133631 |pmc=5267349 |doi=10.5301/jsrd.5000214 |url=}}</ref>


* 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 [[finger]]s and toes.
===History===
Patients with scleroderma may have a positive history of:
*Progressive [[skin]] tightening or hardening<ref name="pmid17017489">{{cite journal |vauthors=Pogorzelska-Antkowiak A, Antkowiak R |title=[Diagnostic and therapeutic problems of scleroderma] |language=Polish |journal=Wiad. Lek. |volume=59 |issue=5-6 |pages=392–5 |date=2006 |pmid=17017489 |doi= |url=}}</ref><ref name="pmid20453602">{{cite journal |vauthors=Hudson M, Fritzler MJ, Baron M |title=Systemic sclerosis: establishing diagnostic criteria |journal=Medicine (Baltimore) |volume=89 |issue=3 |pages=159–65 |date=May 2010 |pmid=20453602 |doi=10.1097/MD.0b013e3181dde28d |url=}}</ref>
*Skin [[induration]] affecting fingers and extending proximally ([[sclerodactyly]])<ref name="pmid17017489">{{cite journal |vauthors=Pogorzelska-Antkowiak A, Antkowiak R |title=[Diagnostic and therapeutic problems of scleroderma] |language=Polish |journal=Wiad. Lek. |volume=59 |issue=5-6 |pages=392–5 |date=2006 |pmid=17017489 |doi= |url=}}</ref><ref name="pmid20453602">{{cite journal |vauthors=Hudson M, Fritzler MJ, Baron M |title=Systemic sclerosis: establishing diagnostic criteria |journal=Medicine (Baltimore) |volume=89 |issue=3 |pages=159–65 |date=May 2010 |pmid=20453602 |doi=10.1097/MD.0b013e3181dde28d |url=}}</ref>
*Exaggerated response to cold temperatures or emotional stress causing [[numbness]], [[pain]] or color changes in fingers or toes ([[Raynaud's phenomenon]])<ref name="pmid20453602">{{cite journal |vauthors=Hudson M, Fritzler MJ, Baron M |title=Systemic sclerosis: establishing diagnostic criteria |journal=Medicine (Baltimore) |volume=89 |issue=3 |pages=159–65 |date=May 2010 |pmid=20453602 |doi=10.1097/MD.0b013e3181dde28d |url=}}</ref>
*[[Swallowing difficulty]]<ref name="pmid28133631">{{cite journal |vauthors=Shreiner AB, Murray C, Denton C, Khanna D |title=Gastrointestinal Manifestations of Systemic Sclerosis |journal=J Scleroderma Relat Disord |volume=1 |issue=3 |pages=247–256 |date=2016 |pmid=28133631 |pmc=5267349 |doi=10.5301/jsrd.5000214 |url=}}</ref>
*[[Heartburn]]
*[[Abdominal pain]]
*[[Nausea]]
*[[Vomiting]]
*[[Bloating]]
*[[Flatulence]]
*Altered [[Bowel movement|bowel movements]]
*[[Fatigue]]<ref name="pmid19106163">{{cite journal |vauthors=Sandusky SB, McGuire L, Smith MT, Wigley FM, Haythornthwaite JA |title=Fatigue: an overlooked determinant of physical function in scleroderma |journal=Rheumatology (Oxford) |volume=48 |issue=2 |pages=165–9 |date=February 2009 |pmid=19106163 |pmc=2638541 |doi=10.1093/rheumatology/ken455 |url=}}</ref>
*[[Weakness]]
*[[Weight loss]]
*[[Musculoskeletal pain]]


* Systemic scleroderma and [[Raynaud's phenomenon]] can cause painful [[ulcer]]s on the fingers or toes which are known as digital ulcers.
===Common Symptoms===
Common symptoms of scleroderma include:
*[[Skin]] tightening and [[induration]] (hardening)<ref name="pmid17017489">{{cite journal |vauthors=Pogorzelska-Antkowiak A, Antkowiak R |title=[Diagnostic and therapeutic problems of scleroderma] |language=Polish |journal=Wiad. Lek. |volume=59 |issue=5-6 |pages=392–5 |date=2006 |pmid=17017489 |doi= |url=}}</ref><ref name="pmid20453602">{{cite journal |vauthors=Hudson M, Fritzler MJ, Baron M |title=Systemic sclerosis: establishing diagnostic criteria |journal=Medicine (Baltimore) |volume=89 |issue=3 |pages=159–65 |date=May 2010 |pmid=20453602 |doi=10.1097/MD.0b013e3181dde28d |url=}}</ref>
*Exaggerated response to cold temperatures or emotional stress causing [[numbness]], [[pain]] or color changes in fingers or toes ([[Raynaud's phenomenon|Raynaud's Phenomenon]])<ref name="pmid20453602">{{cite journal |vauthors=Hudson M, Fritzler MJ, Baron M |title=Systemic sclerosis: establishing diagnostic criteria |journal=Medicine (Baltimore) |volume=89 |issue=3 |pages=159–65 |date=May 2010 |pmid=20453602 |doi=10.1097/MD.0b013e3181dde28d |url=}}</ref>
*Non-productive [[cough]]<ref name="pmid20453602">{{cite journal |vauthors=Hudson M, Fritzler MJ, Baron M |title=Systemic sclerosis: establishing diagnostic criteria |journal=Medicine (Baltimore) |volume=89 |issue=3 |pages=159–65 |date=May 2010 |pmid=20453602 |doi=10.1097/MD.0b013e3181dde28d |url=}}</ref>
*[[Chest]] tightness
*[[Chest pain]]
*[[Joint pain]]
*[[Dysphagia]]<ref name="pmid28133631">{{cite journal |vauthors=Shreiner AB, Murray C, Denton C, Khanna D |title=Gastrointestinal Manifestations of Systemic Sclerosis |journal=J Scleroderma Relat Disord |volume=1 |issue=3 |pages=247–256 |date=2016 |pmid=28133631 |pmc=5267349 |doi=10.5301/jsrd.5000214 |url=}}</ref><ref name="pmid20453602">{{cite journal |vauthors=Hudson M, Fritzler MJ, Baron M |title=Systemic sclerosis: establishing diagnostic criteria |journal=Medicine (Baltimore) |volume=89 |issue=3 |pages=159–65 |date=May 2010 |pmid=20453602 |doi=10.1097/MD.0b013e3181dde28d |url=}}</ref>
*[[Abdominal pain]]
*[[Diarrhea]]
*[[Constipation]]


* [[Calcinosis]] is also common in systemic scleroderma, and is often seen near the elbows, knees or other [[joint|joints]].
===Less Common Symptoms===
 
Less common symptoms of scleroderma include:
===Lungs===
*Shiny [[skin]] appearance
 
*Restricted movement of affected area of [[skin]]
Some impairment in lung function is almost universally seen in patients with diffuse scleroderma on [[pulmonary function test]]ing;<ref>{{cite journal |author=Steen VD |title=The lung in systemic sclerosis |journal=Journal of clinical rheumatology |volume=11 |issue=1 |pages=40-6 |year=2005 |pmid=16357695 |doi=}}</ref> 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]].<ref name=Primer>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.</ref>
 
===Musculoskeletal===
 
The first joint symptoms that patients with scleroderma have are typically non specific [[arthralgia|joint pains]], which can lead to [[arthritis]], or cause discomfort in [[tenosynovitis|tendons]] or [[myalgia|muscles]].<ref name=Primer/>  Joint mobility, especially of the small joints of the hand, may be restricted by [[calcinosis]] or skin thickening.<ref>{{cite journal |author=Valentini G, Black C |title=Systemic sclerosis |journal=Best practice & research. Clinical rheumatology |volume=16 |issue=5 |pages=807-16 |year=2002 |pmid=12473275 |doi=}}</ref>  Patients who have progressed later in their disease may develop muscle weakness, or [[myopathy]], either from the disease, or its treatments.<ref>{{cite journal |author=Olsen NJ, King LE, Park JH |title=Muscle abnormalities in scleroderma |journal=Rheum. Dis. Clin. North Am. |volume=22 |issue=4 |pages=783-96 |year=1996 |pmid=8923596 |doi=}}</ref>
 
===Gastrointestinal===
 
Diffuse scleroderma can affect any part of the gastrointestinal tract.<ref name=Sallam>{{cite journal |author=Sallam H, McNearney TA, Chen JD |title=Systematic review: pathophysiology and management of gastrointestinal dysmotility in systemic sclerosis (scleroderma) |journal=Aliment. Pharmacol. Ther. |volume=23 |issue=6 |pages=691-712 |year=2006 |pmid=16556171 |doi=10.1111/j.1365-2036.2006.02804.x}}</ref>  The most common manifestation in the [[esophagus]] is [[esophagitis|reflux esophagitis]], which may be complicated by peptic stricturing, or benign narrowing of the esophagus.<ref name=Rose>{{cite journal |author=Rose S, Young MA, Reynolds JC |title=Gastrointestinal manifestations of scleroderma |journal=Gastroenterol. Clin. North Am. |volume=27 |issue=3 |pages=563-94 |year=1998 |pmid=9891698 |doi=}}</ref>  This is best initially treated with [[proton pump inhibitor]]s for acid suppression,<ref>{{cite journal |author=Hendel L, Hage E, Hendel J, Stentoft P |title=Omeprazole in the long-term treatment of severe gastro-oesophageal reflux disease in patients with systemic sclerosis |journal=Aliment. Pharmacol. Ther. |volume=6 |issue=5 |pages=565-77 |year=1992 |pmid=1420748 |doi=}}</ref> but may require [[esophageal dilatation|bougie dilatation]] in the case of stricture.<ref name=Sallam/>
 
Scleroderma can decrease [[motility]] anywhere in the gastrointestinal tract.<ref name=Sallam/> 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 [[Gastroesophageal reflux disease|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 (anatomy)|colon]] can be involved, and can cause [[Ogilvie's syndrome|pseudo-obstruction]] or [[ischemic colitis]].<ref name=Primer/>
 
Rarer complications include pneumatosis cystoides intestinalis, or gas pockets in the bowel wall, [[diverticulosis|wide mouthed diverticula]] in the colon and [[esophagus]], and [[cirrhosis|liver fibrosis]].  Patients with severe gastrointestinal involvement can become profoundly [[malnutrition|malnourished]].<ref name=Rose/>
 
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.<ref name=Rose/>
 
===Renal===
 
Renal involvement, in scleroderma, is considered a poor prognostic factor and not infrequently a cause of death in patients with scleroderma.<ref>{{cite journal |author=Ruangjutipopan S, Kasitanon N, Louthrenoo W, Sukitawut W, Wichainun R |title=Causes of death and poor survival prognostic factors in thai patients with systemic sclerosis |journal=Journal of the Medical Association of Thailand |volume=85 |issue=11 |pages=1204-9 |year=2002 |pmid=12546318 |doi=}}</ref>
 
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), [[Renin|hyperreninemia]] (high renin levels), [[azotemia]] (kidney failure with accumulation of waste products in the blood) and [[microangiopathic hemolytic anemia]] (destruction of red blood cells).<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> Apart from the high blood pressure, [[hematuria]] (blood in the urine) and [[proteinuria]] (protein loss in the urine) may be indicative.<ref>{{cite journal |author=Steen VD |title=Renal involvement in systemic sclerosis |journal=Clin. Dermatol. |volume=12 |issue=2 |pages=253-8 |year=1994 |pmid=8076263 |doi=}}</ref>
 
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==


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}


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Latest revision as of 00:05, 30 July 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: M. Khurram Afzal, MD [2]

Overview

The hallmark of scleroderma is sclerodactyly. A positive history of progressive skin tightening and hardening is suggestive of scleroderma. The most common symptoms of scleroderma include skin tightening or induration, Raynaud's phenomenon, and symptoms of gastroesophageal reflux disease (GERD). Less common symptoms of scleroderma include shiny skin appearance and restricted movement of affected areas of the skin.

History and Symptoms

The hallmark of scleroderma is sclerodactyly. A positive history of progressive skin tightening and hardening is suggestive of scleroderma. The most common symptoms of scleroderma include skin tightening or induration, Raynaud's phenomenon and symptoms of gastroesophageal reflux disease (GERD).[1]

History

Patients with scleroderma may have a positive history of:

Common Symptoms

Common symptoms of scleroderma include:

Less Common Symptoms

Less common symptoms of scleroderma include:

  • Shiny skin appearance
  • Restricted movement of affected area of skin

References

  1. ↑ 1.0 1.1 1.2 Shreiner AB, Murray C, Denton C, Khanna D (2016). "Gastrointestinal Manifestations of Systemic Sclerosis". J Scleroderma Relat Disord. 1 (3): 247–256. doi:10.5301/jsrd.5000214. PMC 5267349. PMID 28133631.
  2. ↑ 2.0 2.1 2.2 Pogorzelska-Antkowiak A, Antkowiak R (2006). "[Diagnostic and therapeutic problems of scleroderma]". Wiad. Lek. (in Polish). 59 (5–6): 392–5. PMID 17017489.
  3. ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Hudson M, Fritzler MJ, Baron M (May 2010). "Systemic sclerosis: establishing diagnostic criteria". Medicine (Baltimore). 89 (3): 159–65. doi:10.1097/MD.0b013e3181dde28d. PMID 20453602.
  4. ↑ Sandusky SB, McGuire L, Smith MT, Wigley FM, Haythornthwaite JA (February 2009). "Fatigue: an overlooked determinant of physical function in scleroderma". Rheumatology (Oxford). 48 (2): 165–9. doi:10.1093/rheumatology/ken455. PMC 2638541. PMID 19106163.

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