Talk:Systemic scleroderma

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Heat is bad as is the cold for scleroderma. Heat causes excessive fatigue more so then you allready get with this disease. The harding of the skin can be very life threatening. I am not getting any blood to my rear end I can't stand the pain. Sleeping is very hard to do. It is hard to stand, sit or lie down. Because of the hard skin there is no blood flow along my skin between my two cheeks, it is raw and getting open sores much like the ones on fingers of others with scleroderma. We must get the word out about this disease, I did not know about it till I was told that I have it.

I agree. My mother has the disease and it took doctors years to arrive at this diagnosis. she does not show very much of the characteristic skin hardening, but she makes up for it with the other symptoms. --Morbid-o 12:44, 3 Jun 2005 (UTC)

Wikipedia is flawed. Information about something as obscure as scleroderma needs to come from a source that is current and updated often. It's impossible to get that on a site that not only requires you to write the update but to document it. Go to the source for current, medically-reviewed information about scleroderma -

I agree that this article should be based on authoratitive sources, preferably a recent systematic review. Many medical articles are indeed updated frequently, but there is clearly nobody around who is willing to nurture this article. Instead of criticism, how about you put some work in to improve this article? JFW | T@lk 00:45, 17 November 2005 (UTC)

I have it all ready, just haven't had the time or stamina (I have the systemic disease) to learn how to do the Wikipedia "documentation." I cite the places I get info from in the article and don't know why I have to use some unique coding system.

Coding system? It's Wiki markup and very easy to learn, see how to edit a page for more. JFW | T@lk 15:00, 22 November 2005 (UTC)

I'm happy it's easy for you to learn. If you had the disease, you'd understand why "easy" is relative.

I'm unsure how scleroderma symptoms should impair Wikipedia editing more than any other form of typing. I did not intend to offend you. JFW | T@lk 14:04, 15 December 2005 (UTC)

I heard colchicine is an effective treatment for schleroderma, but don't want to add it without proof. If anyone wants to look this up...

This study laments the absence of well-designed trials of colchicine in scleroderma. There's a nice subject for your PhD! JFW | T@lk 23:38, 25 April 2006 (UTC)


I wrote the pathogenesis section completely on the basis of Jimenez & Derk's excellent review. One wishes there were more systemic reviews of this type. Further sources would still be welcome. The review was in 2004. Has anything else come to the surface that is worth discussing? JFW | T@lk 23:38, 25 April 2006 (UTC)

Nice additions. The review article is quite good. -- Samir धर्म 21:07, 1 May 2006 (UTC)

Thanks. Have you anything to add with respect to the oesophageal dysmotility and malabsorption? JFW | T@lk 16:49, 2 May 2006 (UTC)

Added a bit on complications. -- Samir धर्म 08:22, 14 May 2006 (UTC)

Scleroderma : help me[edit]

If Scleroderma is a genetic diseases? my mother has the diseases, what are the chances that i will have it?(i mean is it dominant diseases?) what pecautions should i take to avoid the disease?

There does appear to be a genetic component to the disease as suggested by the following abstract:
Assassi S, Tan FK. Genetics of scleroderma: update on single nucleotide polymorphism analysis and microarrays. Curr Opin Rheumatol. 2005 Nov;17(6):761-7. Review. PMID 16224255.
I suggest you talk to your doctor. Nephron  T|C 08:34, 28 October 2006 (UTC)
Having a member of the family with scleroderma increases the rate to 1.3% from a few in a million. Rates among identical twins are about 20% (if one gets it, the chance the other will is about 20%). JoshNarins (talk) 12:53, 2 July 2013 (UTC)

Deleted BoTox reference[edit]

This cannot be correct. Scleroderma reduces Lower Esophageal Sphincter(LES) pressure by reducing the ability of the muscles to contract, causing the GE junction to be slack, and allowing the regurgitation of stomach acid into the esophagus. This causes esophageal erosion, Barrett's Esophagus, and esophageal strictures. Strictures are not muscular, they are fibrous connective tissue bands caused by the corrosive scarring of the stomach acid. The treatment for strictures is by esophageal dilitation with a device called a Bouge (among others).

Botulinum Toxin, maybe the most potent toxin known to man, is a paralytic substance. There is no need to use a paralytic on an already slackened LES. Since esophageal strictures are fibrous bands and not muscular, they cannot be treated with a paralytic. In fact, BoTox is one of the treatments for Achalasia, which is the failure of the LES to relax; the exact OPPOSITE effect of Scleroderma.

I hope that it is Ok that I delete the Botulinum toxin reference from the GI effects section.

Cochise11 00:01, 2 April 2007 (UTC)

8-) No this is NOT an April fools joke.

Is there a reason why Botulinum Toxin is still on the page? It is not a viable treatment option for esophageal scleroderma.

"Dysphagia due to esophageal dysfunction is common and results from abnormalities in motility and later from fibrosis."(Hellmann et al., 2007, p. 858)

"Esophageal reflux can be reduced and the risk of scarring diminished by avoidance of late-night meals and elevation of the head of the bed. In addition, proton pump inhibitors (eg, omeprazole, 20–40 mg/d orally), the only drugs that achieve near-complete inhibition of gastric acid production, are remarkably effective for refractory esophagitis. Patients with delayed gastric emptying maintain their weight better if they eat small, frequent meals and remain upright for at least 2 hours after eating."(Hellmann et al., 2007, p. 859)

"Gastroesophageal reflux disease affects 20% of adults, who report at least weekly episodes of heartburn, and up to 10% complain of daily symptoms. Although most patients have mild disease, esophageal mucosal damage (reflux esophagitis) develops in up to 50% and more serious complications develop in a few others. Several factors may contribute to gastroesophageal reflux disease." (McQuaid et al., 2007, p. 578)

"The antireflux barrier at the gastroesophageal junction depends on intrinsic lower esophageal sphincter pressure, the intra-abdominal location of the sphincter, and the extrinsic compression of the sphincter by the crural diaphragm. In most patients, baseline lower esophageal sphincter pressures are normal (10–30 mm Hg). In patients without hiatal hernias, about 70% of reflux episodes occur during relaxations of the lower esophageal sphincter that occur spontaneously ("transient relaxations") or as prolonged relaxation after swallowing. The remaining events occur during periods of low sphincter pressure ("hypotensive" sphincter). A small number of patients with more severe involvement (especially those with strictures) have chronically incompetent sphincters (< 10 mm Hg), resulting in free reflux or stress reflux during lifting, bending, or abdominal straining."(McQuaid et al., 2007, p. 578)

Note that scleroderma causes a slack, or hypotensive, lower esophageal sphincter (LES) due to the lack of the ability of the sphincter to contract as a result of the fibrous deposition in the muscle. Over time the disease progresses, and by definition, causes the LES to become chronically incompetent leading to esophageal strictures.

"Stricture formation occurs in about 10% of patients with esophagitis (see endoscopy). It is manifested by the gradual development of solid food dysphagia progressive over months to years. Often there is a reduction in heartburn because the stricture acts as a barrier to reflux. Most strictures are located at the gastroesophageal junction. Strictures located above this level usually occur with Barrett's metaplasia. Endoscopy with biopsy is mandatory in all cases to differentiate peptic stricture from malignant causes of esophageal stricture (esophageal carcinoma). Active erosive esophagitis is often present. Up to 90% of symptomatic patients are effectively treated with dilation with flexible weighted bougies (passed under fluoroscopic guidance), graduated polyvinyl catheters passed over a wire placed at the time of endoscopy or fluoroscopically, or balloons passed fluoroscopically or through an endoscope. Dilation is continued over one to several sessions. A luminal diameter of 13–17 mm is usually sufficient to relieve dysphagia. Long-term therapy with a proton pump inhibitor is required to decrease the likelihood of stricture recurrence. Some patients require intermittent dilation to maintain luminal patency, but operative management for strictures that do not respond to dilation is seldom required. Refractory strictures may benefit from endoscopic injection of triamcinolone into the stricture." (McQuaid et al., 2007, p. 580)

Triamcinolone is a corticosteroid used for its antiinflammatory effect. Refractory means obstinately resistant to authority or control.

Botulinum toxin is NEVER MENTIONED in this section of "Current Medical Diagnosis and Treatment". Why? Because like I said previously, it makes no sense to administer a paralytic substance to an already incompetent LES. The stricture is a fibrous band, with absolutely NO MUSCLE tissue whatsoever. If muscle tissue is not present, then there can be no muscular contraction (or relaxation). Therefore, fibrous bands cannot be treated with botulinum toxin at all. EVER.

I challenge anyone in this entire english Wiki to find proof of at least equal strength to the evidence that I present here, that will actually endorse the use of Botulinum Toxin for esophageal strictures. Now I will present additional information which will prove my case beyond any reasonable doubt.

"Achalasia is an idiopathic motility disorder characterized by loss of peristalsis in the distal two-thirds (smooth muscle) of the esophagus and impaired relaxation of the lower esophageal sphincter. There appears to be denervation of the esophagus resulting primarily from loss of nitric oxide-producing inhibitory neurons in the myenteric plexus. The cause of the neuronal degeneration is unknown."(McQuaid et al., 2007, p. 592-593) Idiopathic just means that there is no known cause. Peristalsis is the creation of wave-like contractions for the purpose of moving material farther through a tube like structure. Nitric Oxide has the effect of inducing relaxation in smooth muscle structures. Note that the end effect of the vastly different disease processes, HAVE THE EXACT OPPOSITE EFFECT ON THE LES.

"Chest x-rays may show an air-fluid level in the enlarged, fluid-filled esophagus. Barium esophagography discloses characteristic findings, including esophageal dilation, loss of esophageal peristalsis, poor esophageal emptying, and a smooth, symmetric "bird's beak" tapering of the distal esophagus. Without treatment, the esophagus may become markedly dilated ("sigmoid esophagus").(McQuaid et al., 2007, p. 593) The dilated esophagus is primarily the result of ingested material. That material is stopped by LES that is unable to relax, and is in fact clenching down on itself with about 2-3 times the normal force of contraction, preventing anything from passing into the stomach. As the esophagus becomes distended by the ingested material, the smooth muscles in the esophageal wall stretches beyond the ability of the actin and myosin fibers in the majority of the individual muscle cells to connect with each other. This causes the complete lack of peristalsis in contrast to the lack of relaxation in the LES. So how can this problem be solved? Well it all starts with getting the LES to open up somehow.

"Endoscopically guided injection of botulinum toxin directly into the lower esophageal sphincter results in a marked reduction in lower esophageal sphincter pressure with initial improvement in symptoms in 65–85% of patients. However, symptom relapse occurs in over 50% of patients within 6–9 months and in all patients within 2 years. Three-fourths of initial responders who relapse have improvement with repeated injections. Because it is inferior to pneumatic dilation therapy and surgery in producing sustained symptomatic relief, this therapy is most appropriate for patients with comorbidities who are poor candidates for more invasive procedures."(McQuaid et al., 2007, p. 593) The rest of the section goes on to talk about additional treatment options involving pneumatic balloon dilation, and surgical myotomy. If any of you are motivated and able, and you can find it, you can look that up on your own.

Hellmann, D. B., & Stone, J. H. (2007). Systemic Sclerosis (Scleroderma). In L. M. Tierney, Jr., S. J. McPhee, & M. A. Papadakis (Eds.), Current Medical Diagnosis and Treatment (pp. 858-859). San Francisco: McGraw-Hill Companies, Inc.

McQuaid, K. R. (2007). Gastroesophageal Reflux Disease. In L. M. Tierney, Jr., S. J. McPhee, & M. A. Papadakis (Eds.), Current Medical Diagnosis and Treatment (pp. 578-583). San Francisco: McGraw-Hill Companies, Inc.

McQuaid, K. R. (2007). Esophageal Motility Disorders. In L. M. Tierney, Jr., S. J. McPhee, & M. A. Papadakis (Eds.), Current Medical Diagnosis and Treatment (pp. 592-595). San Francisco: McGraw-Hill Companies, Inc.

Now that I have proven my point beyond a shadow of a doubt, I will delete the offending term "Botulinum Toxin" from the page.

Cochise11 01:49, 5 April 2007 (UTC)

Cleanup of my own mess[edit]

Sorry about this. When I deleted the BoTox reference, it caused the paragraph to not make sense. Additionally, there is no mention in the literature about the development of Megaesophagus from Sclreoderma. Megaesophagus is a sequele of Chaga's disease, and Achalasia. "Chagas' disease is associated with esophageal dysfunction that is indistinguishable from idiopathic achalasia and should be considered in patients from endemic regions (Central and South America); it is becoming more common in the southern United States." (McQuaid et al., 2007, p. 593) "The chronic stage is usually manifested by cardiac disease in the third and fourth decades of life, characterized by arrhythmias, congestive heart failure (often with prominent right-sided findings), ventricular aneurysms, and systemic or pulmonary embolization originating from mural thrombi. Valvular lesions are absent. Sudden cardiac arrest in young persons may occur and is attributed to ventricular fibrillation. Megacolon and megaesophagus, caused by damage to nerve plexuses in the bowel or esophageal wall, occur in some areas of Chile, Argentina, and Brazil; findings include dysphagia, regurgitation, constipation, sigmoid volvulus, bacterial overgrowth in the small intestine, and parotid gland hypertrophy. Megasyndromes can also affect the urinary tract."(Jacobs et al., 2007, p. 1497)

The bold part below is what doesn't make sense:

"Scleroderma can decrease motility anywhere in the gastrointestinal tract.[5] The most common source of motility involvement is the esophagus, which can also lead to difficulty swallowing or dysphagia, or to chest pain. The esophagus can become massively dilated (megaesophagus).[1] This can be treated with esophageal dilatation."

I'm going to make the bold part say:

"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 dilitation, and Barrett's esophagus."

McQuaid, K. R. (2007). Esophageal Motility Disorders. In L. M. Tierney, Jr., S. J. McPhee, & M. A. Papadakis (Eds.), Current Medical Diagnosis and Treatment (pp. 592-595). San Francisco: McGraw-Hill Companies, Inc.

Jacobs, R. A. (2007). American Trypanosomiasis (Chaga's Disease). In L. M. Tierney, Jr., S. J. McPhee, & M. A. Papadakis (Eds.), Current Medical Diagnosis and Treatment (pp. 1496-1498). San Francisco: McGraw-Hill Companies, Inc Cochise11 22:02, 5 April 2007 (UTC)

Cochise11 22:28, 5 April 2007 (UTC)

Some issues[edit]

Localised scleroderma... does it needs its own article given that this article is presently mainly about systemic sclerosis?

doi:10.1186/ar2191 is another nice resource that summarises recent trial results.

doi:10.1111/j.1572-0241.2007.01758.x suggests bacterial overgrowth is rather common, and that rifaximin may be of benefit. JFW | T@lk 11:30, 11 May 2008 (UTC) - management of lung disease. JFW | T@lk 11:57, 11 May 2008 (UTC)
doi:10.1111/j.1365-2036.2008.03739.x - when to suspect watermelon stomach. JFW | T@lk 09:22, 25 May 2008 (UTC)
Agree. If no one objects, I'll move this article to "systemic scleroderma", and generate a stub for "localised scleroderma". --Arcadian (talk) 04:58, 17 February 2009 (UTC)
  • This plan sounds good to me. Also, just so you are aware, there are some stubs on the various types of scleroderma (see Scleroderma#Types). kilbad (talk) 12:44, 17 February 2009 (UTC)

I'd like to help[edit]

I'm particularly interested in Pulmonary Hypertension secondary to Scleroderma. I can provide references and will keep everything according to current resources. The field is changing daily but since there is so little published here, I see it as an opportunity share what I have learned by necessity. I'm a former medical researcher, no longer able to do lab work. If I put up a suggested outline I hope other scientists would give me feedback and editors would help me learn how to do the article properly. The first decision would be what to put under scleroderma, and what to put under pulmonary hypertension. Jo47phox (talk) 19:48, 7 June 2008 (UTC)

What The?[edit]

I can't tell if this article is just written poorly or what, but after stumbling through the first few paragraphs and being thoroughly confused, I jumped ahead to sections I would hope to enlighten me, and was balked. Almost everything here reads like pseudo-scientific rambling. I honestly can't tell from this article: Is this a real, recognized disease or just a class of symptoms caused by something the author of this page was disinclined to mention? NOTHING on this page describes clearly what the cause of this "disease" is, nor does it acknowledge this fact. This makes me highly suspect. If it is so poorly understood that the opening paragraph cannot explain the nature of this disease outside of mentioning a few vague symptoms, I think a note to that order would be justified.

Looking at other webpages that speak of this disease, it is pretty clear to me that is is insanely rare and that what causes it are absolutely unknown. This needs to be reflected in this page, and the useless technical jargon removed IMO. It certainly didn't help me understand this issue any more than I did looking for this page. (talk) 11:24, 4 July 2008 (UTC)

It is not that rare, unfortunately. Not "insanely" rare anyway. And even though it is relatively rare, it is a nasty chronic disease that is not infrequently lethal due to its associated organ damage.
When I wrote the section on pathophysiology I thought it was reasonably clear that there is no definite known cause for this disease. Rather, there is some knowledge on the way it causes tissue damage, but the relative importance of several known risk factors is not clear at all. I'll see if I can clarify this. JFW | T@lk 06:01, 18 September 2008 (UTC)


I'm not a professional and I'm not American either so I don't want to mess with the article, but I was told by a rheumatologist that in the UK use of the CREST definition is falling out of favour and it is not longer used as an official classification. She told me that what would previously have been called CREST is now lumped in as limited cutaneous sclerosis.

Also, I understand that that subtype does have a significant incidence of pulmonary hypertension (so people with scleroderma get yearly echos and pulmonary function tests) and also renal crisis with malignant hypertension. So you can't really say that people with CREST (which doesn't officially exist any more) don't get pulmonary and renal sequelae. Most don't, but enough do that it is an important complication.

Whrrivers (talk) 22:52, 19 November 2008 (UTC)

CREST is a form of limited SSc, that's right. The rate of death, I've read, is 1-4%/annum for CREST's, 7-10%/annum for diffuse SSc forms. It certainly isn't a fun disease, but the dSSc forms are far more deadly. CREST, the term, might go away, but this form of SSc is very specific. People who have the anti-centromere antibodies (ACA) have CREST's, and many patients express one of the 9 anti-bodies that distinguish the 9 disease sub-types (e.g. ACA, Scl-70, Anti-Topoisomerase, Anti-RNP III, Anti-Th/To). Incidences of the four, major, internal organ complications (PAH, pulmonary fibrosis, scleroderma renal crisis, and GI complications) are much worse in dSSc than CREST's. By the way, using lSSc has its own problems. CREST's and Anti-Th/To can be classified as lSSc, because of the limited skin involvement, but Anti-Th/To acts like the progressive dSSc in every other respect. CREST's really is in its own boat, and represents 2/3rds of annual SSc cases. JoshNarins (talk) 13:07, 2 July 2013 (UTC)

systemic sclerosis[edit]

I think that most medical sources refer to systemic scleroderma as systemic sclerosis.Therefore,to be consistent,i propose renaming the page systemic scleroderma systemic sclerosis.I am a bit reluctant to do it my self because i have little experience renaming pages.Immunize (talk) 16:15, 16 January 2010 (UTC)

Systemic Sclerosis[edit]

I agree completely: This page should be named Systemic Sclerosis. Systemic Scleroderma is NOT a medical term.

Altes2009 (talk) 17:59, 1 February 2010 (UTC)

Review articles[edit]

I just searched Pubmed for review articles in my favorite journals on systemic sclerosis and I found two (plus an interesting epidemiological study) that weren't cited in the article. Unfortunately they're not as current as one would like given how fast the immunological treatments are changing. Scleroderma. Gabrielli A, Avvedimento EV, Krieg T. N Engl J Med. 2009 May 7;360(19):1989-2003. doi: 10.1056/NEJMra0806188. Review. No abstract available. PMID: 19420368 Systemic sclerosis: hypothesis-driven treatment strategies. Charles C, Clements P, Furst DE. Lancet. 2006 May 20;367(9523):1683-91. Review. doi:10.1016/S0140-6736(06)68737-0 PMID:16714190

Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients. Ferri C, Valentini G, Cozzi F, Sebastiani M, Michelassi C, La Montagna G, Bullo A, Cazzato M, Tirri E, Storino F, Giuggioli D, Cuomo G, Rosada M, Bombardieri S, Todesco S, Tirri G; Systemic Sclerosis Study Group of the Italian Society of Rheumatology (SIR-GSSSc). Medicine (Baltimore). 2002 Mar;81(2):139-53. Review. No abstract available. PMID: 11889413

--Nbauman (talk) 23:38, 14 September 2014 (UTC)

I achieve the best result by searching for the related MESH term as Major heading. In this case the search term is "Scleroderma, Systemic"[MAJR]. Having done that, I select "Review" to exclude all primary research. This still gives >1000 citations, but it is possible to find key references purely by looking at the title. There's been a number of reviews on systemic sclerosis within the last five years.
doi:10.1002/art.38098 is a recent key document. JFW | T@lk 23:03, 16 September 2014 (UTC)

Stem cell transplants[edit]

doi:10.1111/bjh.13201 JFW | T@lk 00:15, 12 November 2014 (UTC)