Scleroderma is a rare, chronic disease characterized by excessive deposits of collagen in the skin or other organs. The localized type of the disease tends not to be fatal. Diffuse scleroderma or systemic sclerosis, the generalized type of the disease, can be fatal as a result of heart, kidney, lung or intestinal damage. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... // M00-M99 - Diseases of the musculoskeletal system and connective tissue (M00-M25) Arthropathies (M00-M03) Infectious arthropathies (M00) Pyogenic arthritis (M01) Direct infections of joint in infectious and parasitic diseases classified elsewhere (M02) Reactive arthropathies (M023) Reiters disease (M03) Postinfective and reactive arthropathies in diseases classified elsewhere (M05-M14... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... MedlinePlus (medlineplus. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... A rare disease (sometimes known as an orphan disease) has such a low prevalence in a population that a doctor in a busy general practice would not expect to see more than one case a year. ... Medicine In medicine, a persistent and lasting condition is said to be chronic (from Greek chronos). ... Tropocollagen triple helix. ... In zootomy and dermatology, skin is the largest organ of the integumentary system made up of multiple layers of epithelial tissues that guard underlying muscles and organs. ...

Signs and symptoms

Scleroderma affects the skin, and in more serious cases it can affect the blood vessels and internal organs. The most evident symptom is the hardening of the skin and associated scarring. Typically, the skin appears reddish or scaly in appearance. Blood vessels may also be more visible. Where large areas are affected, fat and muscle wastage will weaken limbs and affect appearance. In zootomy and dermatology, skin is the largest organ of the integumentary system made up of multiple layers of epithelial tissues that guard underlying muscles and organs. ... f you all The blood vessels are part of the circulatory system and function to transport blood throughout the body. ...

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. A lesion is a non-specific term referring to abnormal tissue in the body. ...

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 fingers and toes. Raynauds phenomenon (RAY-noz), in medicine, is a vasospastic disorder causing discoloration of the fingers, toes, and occasionally other extremities, named for French physician Maurice Raynaud (1834 - 1881). ... Fingers of the human left hand A finger is a type of digit, an organ of manipulation and sensation found in the hands of humans and other primates. ...

Systemic scleroderma and Raynaud's can cause painful ulcers on the fingers or toes which are known as digital ulcers.

Calcinosis is also common in systemic scleroderma, and is often seen near the elbows, knees or other joints. Calcinosis is the formation of calcium deposits in any soft tissue. ... This article is about a joint in zootomical anatomy. ...

Complications

Diffuse scleroderma can cause musculoskeletal, pulmonary, gastrointestinal, renal and other complications.^[1] Patients with larger amounts of cutaneous involvement are more likely to have involvement of the internal tissues and organs. The musculoskeletal system is an organ system that gives animals the ability to physically move, by using the muscles and skeletal system. ... Human respiratory system The lungs flank the heart and great vessels in the chest cavity. ... The gastrointestinal tract (GI tract), also called the digestive tract, or the alimentary canal. ... The kidneys filter wastes (such as urea) from the blood and excrete them, along with water, as urine. ...

Musculoskeletal complications

The first joint symptoms that patients with scleroderma have are typically non specific joint pains, which can lead to arthritis, or cause discomfort in tendons or muscles.^[1] Joint mobility, especially of the small joints of the hand, may be restricted by calcinosis or skin thickening.^[2] Patients who have progressed later in their disease may develop muscle weakness, or myopathy, either from the disease, or its treatments.^[3] Arthralgia is a term used to describe pain in the joints. ... Arthritis (from Greek arthro-, joint + -itis, inflammation; plural: arthritides) is a group of conditions where there is damage caused to the joints of the body. ... Tenosynovitis is the inflammation of the fluid-filled sheath (called the synovium) that surrounds a tendon. ... Myalgia means muscle pain and is a symptom of many diseases and disorders. ... Calcinosis is the formation of calcium deposits in any soft tissue. ... In medicine, a myopathy is a neuromuscular disease in which the muscle fibers dysfunction for any one of many reasons, resulting in muscular weakness. ...

Pulmonary complications

Some impairment in lung function is almost universally seen in patients with diffuse scleroderma on pulmonary function testing;^[4] 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. Spirometry, also known as Pulmonary Function Testing (PFT), is the measurement of lung function, specifically by measuring the volume and speed of air that can be inhaled and exhaled. ... In medicine, pulmonary hypertension (PH) is an increase in blood pressure in the pulmonary artery or lung vasculature, leading to shortness of breath, dizziness, fainting, and other symptoms, all of which are exacerbated by exertion. ... The pulmonary arteries carry blood from the heart to the lungs. ... In biology, diffusion capacity is a measurement of the lungs ability to absorb and excrete gases, notably, oxygen and carbon dioxide. ...

Other pulmonary complications in more advanced disease include aspiration pneumonia, pulmonary hemorrhage and pneumothorax.^[1] Aspiration pneumonia is a specific form of lung infection (pneumonia) that develops when oral or gastric contents (including food, saliva, or nasal secretions) enter the bronchial tree. ... This page meets Wikipedias criteria for speedy deletion. ... Left-sided pneumothorax (on the right side of the image) on CT scan of the chest with chest tube in place. ...

Gastrointestinal complications

Endoscopic image of peptic stricture, or narrowing of the esophagus near the junction with the stomach due to chronic gastroesophageal reflux. This is the most common cause of dysphagia, or difficulty swallowing, in scleroderma.

Diffuse scleroderma can affect any part of the gastrointestinal tract.^[5] The most common manifestation in the esophagus is reflux esophagitis, which may be complicated by peptic stricturing, or benign narrowing of the esophagus.^[6] This is best initially treated with proton pump inhibitors for acid suppression,^[7] but may require bougie dilatation in the case of stricture.^[5] Image File history File links Peptic_stricture. ... Image File history File links Peptic_stricture. ... In medicine (gastroenterology), esophagogastroduodenoscopy (EGD) or upper endoscopy is a diagnostic endoscopic procedure that visualises the upper part of the gastrointestinal tract. ... The esophagus (also spelled oesophagus/Å“sophagus, Greek ), or gullet is an organ in vertebrates which consists of a muscular tube through which food passes from the pharynx to the stomach. ... In anatomy, the stomach is a bean-shaped hollow muscular organ of the gastrointestinal tract involved in the second phase of digestion, following mastication. ... Gastroesophageal Reflux Disease (GERD, or GORD when -oesophageal, the BE form, is substituted) is injury to the esophagus that develops from chronic exposure of the esophagus to acid coming up from the stomach (reflux). ... Dysphagia () is a medical term defined as difficulty swallowing. ... The esophagus (also spelled oesophagus/Å“sophagus, Greek ), or gullet is an organ in vertebrates which consists of a muscular tube through which food passes from the pharynx to the stomach. ... Esophagitis (or Oesophagitis) is inflammation of the esophagus. ... Proton pump inhibitors (or PPIs) are a group of drugs whose main action is pronounced and long-lasting reduction of gastric acid production. ... This does not cite its references or sources. ...

Scleroderma can decrease motility anywhere in the gastrointestinal tract.^[5] 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. The small intestine can also become involved, leading to bacterial overgrowth and malabsorption, of bile salts, fats, carbohydrates, proteins, and vitamins. The colon can be involved, and can cause pseudo-obstruction or ischemic colitis.^[1] Motility is a biological term which refers to the ability to move spontaneously and independently. ... Dysphagia () is a medical term defined as difficulty swallowing. ... Esophagitis (or Oesophagitis) is inflammation of the esophagus. ... Skírnir tries to woo Gerd for Freyr as related in Skírnismál. ... Endoscopic image of Barretts esophagus, which is the area of red mucosa projecting like a tongue. ... In biology the small intestine is the part of the gastrointestinal tract (gut) between the stomach and the large intestine and includes the duodenum, jejunum, and the ileum. ... Bacterial overgrowth is when theres too much bacteria in a certain place. ... Malabsorption is the state of impaired absorption of nutrients in the small intestine. ... Bile is also another name for Belenus, a god in Brythonic mythology. ... Fats is the plural for fat, a generic term for a class of lipids in biochemistry. ... Carbohydrates (literally hydrates of carbon) are chemical compounds that act as the primary biological means of storing or consuming energy, other forms being fat and protein. ... A representation of the 3D structure of myoglobin, showing coloured alpha helices. ... Retinol (Vitamin A) Vitamins are nutrients required in very small amounts for essential metabolic reactions in the body [1]. The term vitamin does not encompass other essential nutrients such as dietary minerals, essential fatty acids, or essential amino acids. ... For the article about the punctuation symbol, see Colon (punctuation). ... Ogilvie syndrome is the acute pseudoobstruction and dilation of the colon in the absence of any mechanical obstruction in severely ill patients. ... Ischemic colitis is inflammation of the intestine (colitis) caused by inadequate blood supply (ischemia) to meet the metabolic demands. ...

Rarer complications include pneumatosis cystoides intestinalis, or gas pockets in the bowel wall, wide mouthed diverticula in the colon and esophagus, and liver fibrosis. Patients with severe gastrointestinal involvement can become profoundly malnourished.^[6] Diverticulosis, otherwise known as diverticular disease, is the condition of having diverticula in the large colon which are outpocketings of the colonic mucosa and submucosa through weaknesses of muscle layers in the colon wall. ... The esophagus (also spelled oesophagus/Å“sophagus, Greek ), or gullet is an organ in vertebrates which consists of a muscular tube through which food passes from the pharynx to the stomach. ... Cirrhosis of the liver is a consequence of chronic liver disease characterized by replacement of liver tissue by fibrotic scar tissue as well as regenerative nodules, leading to progressive loss of liver function. ... Percentage of population affected by malnutrition by country, according to United Nations statistics. ...

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.^[6] Gastric antral vascular ectasia (GAVE, also called watermelon stomach) is an uncommon cause of chronic gastrointestinal bleeding or iron deficiency anemia. ... From Greek pylorus; pyl- = gate, -orus = guard. ... Endoscopic image of a posterior wall duodenal ulcer with a clean base, which is a common cause of upper GI hemorrhage. ... Iron deficiency anemia is the most common type of anemia, and the most common cause of microcytic anemia. ...

Renal complications

Renal involvement, in scleroderma, is considered a poor prognostic factor and not infrequently a cause of death in patients with scleroderma.^[8]

The most important clinical complication of scleroderma involving the kidney is scleroderma renal crisis.

Symptoms of scleroderma renal crisis are:^[9]

• Malignant hypertension,
• Hyperreninemia,
• Azotemia,
• Microangiopathic hemolytic anemia and
• Renal failure.

Complications of renal crisis include:^[10] Malignant hypertension is a complication of hypertension characterized by very elevated blood pressure, and organ damage in the eyes, brain, lung and/or kidneys. ... Azotemia is a medical condition characterized by abnormal levels of urea, creatinine, various body waste compounds, and other nitrogen-rich compounds in the blood as a result of insufficient filtering of the blood by the kidneys. ... In medicine (hematology) microangiopathic hemolytic anemia (MAHA) is a subgroup of hemolytic anemia (anemia, loss of red blood cells through destruction) caused by factors in the small blood vessels. ... Renal failure is the condition in which the kidneys fail to function properly. ...

• Hematuria (blood in the urine),
• Hypertension and
• Proteinuria.

In the past scleroderma renal crisis was almost uniformily fatal.^[11] While outcomes have improved significantly with the use of ACE inhibitors^[12][13] 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.^[14] Patients that have rapid skin involvement have the highest risk of renal complications.^[14] In medicine, hematuria (or haematuria) is the presence of blood in the urine. ... This article or section does not cite any references or sources. ... Proteinuria (from protein and urine) means the presence of an excess of serum proteins in the urine. ... ACE inhibitors, or inhibitors of Angiotensin_Converting Enzyme, are a group of pharmaceuticals that are used primarily in treatment of hypertension and congestive heart failure, in most cases as the drugs of first choice. ... Renal failure is the condition in which the kidneys fail to function properly. ...

Treatments for scleroderma renal crisis include ACE inhibitors, which are also used for prophylaxis,^[14][13] 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.^[15] ACE inhibitors, or inhibitors of Angiotensin_Converting Enzyme, are a group of pharmaceuticals that are used primarily in treatment of hypertension and congestive heart failure, in most cases as the drugs of first choice. ... Prophylaxis refers to any medical or public health procedure whose purpose is to prevent, rather than treat or cure, disease. ... Kidney transplantation or renal transplantation is the organ transplant of a kidney in a patient with chronic renal failure. ...

Diagnosis

Diagnosis is by clinical suspicion, presence of autoantibodies (specifically anti-centromere and anti-scl70/anti-topoisomerase) and occasionally by biopsy. Of the antibodies, 90% have a detectable anti-nuclear antibody. Anti-centromere 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 prone to the systemic form).^[16] Anti-nuclear antibodies (ANAs, also known as anti-nuclear factor or ANF) are detected in a large group of autoimmune disorders. ... Languages Predominantly American English Religions Protestantism (chiefly Baptist and Methodist); Roman Catholicism; Islam Related ethnic groups Sub-Saharan Africans and other African groups, some with Native American groups. ...

In 1980 the American College of Rheumatology agreed upon diagnostic criteria for scleroderma.^[17] Year 1980 (MCMLXXX) was a leap year starting on Tuesday (link displays the 1980 Gregorian calendar). ...

Types

There are three major forms of scleroderma: diffuse, limited (CREST syndrome) and morphea/linear. Diffuse and limited scleroderma are both a systemic disease, whereas the linear/morphea form is localized to the skin. (Some physicians consider CREST and limited scleroderma one and the same, others treat them as two separate forms of scleroderma.) There is also a subset of the systemic form known as "systemic scleroderma sine scleroderma", meaning the usual skin involvement is not present.

Diffuse scleroderma

Diffuse scleroderma is the most severe form - it has a rapid onset, involves more widespread skin hardening, will generally cause much internal organ damage (specifically the lungs and gastrointestinal tract), and is generally more life threatening. Human respiratory system The lungs flank the heart and great vessels in the chest cavity. ... The gastrointestinal tract (GI tract), also called the digestive tract, or the alimentary canal. ...

Limited scleroderma/CREST syndrome

The limited form is much milder: it has a slow onset and progression, skin hardening is usually confined to the hands and face, internal organ involvement is less severe, and a much better prognosis is expected.

In typical cases of limited scleroderma, Raynaud's phenomenon may precede scleroderma by several years. Raynaud's phenomenon is due to vasoconstriction of the small arteries of exposed peripheries - particularly the hands and feet - in the cold. It is classically characterised by a triphasic colour change - first white, then blue and finally red on rewarming. The scleroderma may be limited to the fingers - known as sclerodactyly.

The limited form is often referred to as CREST syndrome. "CREST" is an acronym for: In medicine, the term syndrome is the association of several clinically recognizable features, signs, symptoms, phenomena or characteristics which often occur together, so that the presence of one feature alerts the physician to the presence of the others. ...

• Calcinosis
• Raynaud's syndrome
• Esophageal dysmotility
• Sclerodactyly
• Telangiectasia

These are the five major signs that make up the CREST syndrome. Calcinosis is the formation of calcium deposits in any soft tissue. ... Raynauds phenomenon (RAY-noz), in medicine, is a vasospastic disorder causing discoloration of the fingers, toes, and occasionally other extremities, named for French physician Maurice Raynaud (1834 - 1881). ... The esophagus (also spelled oesophagus/Å“sophagus, Greek ), or gullet is an organ in vertebrates which consists of a muscular tube through which food passes from the pharynx to the stomach. ... Sclerodactyly is a localized thickening and tightness of the skin of the fingers or toes. ... Telangiectasias Are small enlarged blood vessels near the surface of the skin, usually they measure only a few millimetres. ...

Morphea/linear scleroderma

Morphea/linear scleroderma involves isolated patches of hardened skin - there generally is no internal organ involvement.^[18] Her first appearance was in Atari Force #1, Morphea is a DC Comics character who is an alien Canopian in the DC Universe. ...

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.^{[citation needed]}

A range of NSAIDs (nonsteroidal anti-inflammatory drugs) can be used to ease symptoms, such as naproxen. 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. Non-steroidal anti-inflammatory drugs, usually abbreviated to NSAIDs, are drugs with analgesic, antipyretic and anti-inflammatory effects - they reduce pain, fever and inflammation. ... Naproxen (trade names: Aleve, Anaprox, Naprogesic, Naprosyn, Naprelan) is a non-steroidal anti-inflammatory drug (NSAID) commonly used for the reduction of mild to moderate pain, fever, inflammation and stiffness caused by conditions such as osteoarthritis, rheumatoid arthritis, psoriatic arthritis, gout, ankylosing spondylitis, injury, menstrual cramps, tendinitis, bursitis, and the... Proton pump inhibitors (or PPIs) are a group of drugs whose main action is pronounced and long-lasting reduction of gastric acid production. ... Omeprazole (INN) (IPA: ) is a proton pump inhibitor (brand names Antra® in Italy) used in the treatment of dyspepsia, peptic ulcer disease (PUD), gastroesophageal reflux disease (GORD/GERD) and Zollinger-Ellison syndrome. ...

Immunosuppressant drugs, such as mycophenolate mofetil (Cellcept®) or cyclophosphamide are sometimes used to slow the progress. Mycophenolate mofetil (MMF, trade name Cellcept®) is an immunosuppresant drug used to prevent rejection in organ transplantation. ... Cyclophosphamide (the generic name for Cytoxan, Neosar) is a nitrogen mustard alkylating agent, used to treat various types of cancer and some autoimmune disorders. ...

Digital ulcerations can be helped by prostacyclin (iloprost) infusion. Iloprost being a drug which increases blood flow by relaxing the arterial wall. Prostacyclin is a member of the family of lipid molecules known as eicosanoids. ...

Causes

There is no clear obvious cause for scleroderma and systemic sclerosis. Genetic predisposition appears to be limited: genetic concordance is small; still, there often is a familial predisposition for autoimmune disease. Polymorphisms in COL1A and TGF-β1 may influence severity and development of the disease. There is limited evidence implicating cytomegalovirus (CMV) as the original epitope of the immune reaction, and organic solvents and other chemical agents have been linked with scleroderma.^[16] Transforming growth factor (TGF) is one of many characterized growth factors that exist in nature. ... species see text Cytomegalovirus (CMV), is a genus of Herpes viruses; in humans the species is known as Human herpesvirus 5 (HHV-5). ... A solvent is a liquid that dissolves a solid, liquid, or gaseous solute, resulting in a solution. ...

One of the suspected mechanisms behind the autoimmune phenomenon is the existence of microchimerism, i.e. fetal cells circulating in maternal blood, triggering an immune reaction to what is perceived as "foreign" material^[19].^[16] In zoology, a chimera is an animal which has two or more different populations of genetically distinct cells that originated in different zygotes; if the different cells emerged from the same zygote, it is called a mosaicism. ...

Pathophysiology

The overproduction of collagen is thought to result from an autoimmune dysfunction, in which the immune system would start to attack the kinetochore of the chromosomes. This would lead to genetic malfunction of nearby genes. T cells accumulate in the skin; these are thought to secrete cytokines and other proteins that stimulate collagen deposition. Stimulation of the fibroblast, in particular, seems to be crucial to the disease process, and studies have converged on the potential factors that produce this effect.^[16] Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ... The kinetochore is the protein structure in eukaryotes which assembles on the centromere and links the chromosome to microtubule polymers from the mitotic spindle during mitosis. ... T cells are a subset of lymphocytes that play a large role in the immune response. ... Cytokines are a group of proteins and peptides that are used in organisms as signaling compounds. ... Mouse Embryonic Fibroblasts (MEF) A fibroblast is a type of cell that synthesizes and maintains the extracellular matrix of many animal tissues. ...

A significant player in the process is transforming growth factor (TGFβ). This protein appears to be overproduced, and the fibroblast (possibly in response to other stimuli) also overexpresses the receptor for this mediator. An intracellular pathway (consisting of SMAD2/SMAD3, SMAD4 and the inhibitor SMAD7) is responsible for the secondary messenger system that induces transcription of the proteins and enzymes responsible for collagen deposition. Sp1 is a transcription factor most closely studied in this context. Apart from TGFβ, connective tissue growth factor (CTGF) has a possible role.^[16] Transforming growth factor (TGF) is one of many characterized growth factors that exist in nature. ... The introduction to this article provides insufficient context for those unfamiliar with the subject matter. ... The introduction to this article provides insufficient context for those unfamiliar with the subject matter. ... The introduction to this article provides insufficient context for those unfamiliar with the subject matter. ... SMAD7 or Mothers against decapentaplegic homolog 7 is a polypeptide that, as its name describes, is a homolog of the Drosophila gene: Mothers against decepentaplegic. It belongs to the SMAD family of proteins, which belong to the TGFβ superfamily of ligands. ... A micrograph of ongoing gene transcription of ribosomal RNA illustrating the growing primary transcripts. ... In molecular biology, a transcription factor is a protein that binds DNA at a specific promoter or enhancer region or site, where it regulates transcription. ...

Damage to endothelium is an early abnormality in the development of scleroderma, and this too seems to be due to collagen accumulation by fibroblasts, although direct alterations by cytokines, platelet adhesion and a type II hypersensitivity reaction have similarly been implicated. Increased endothelin and decreased vasodilation has been documented.^[16] The endothelium is the layer of thin, flat cells that lines the interior surface of blood vessels, forming an interface between circulating blood in the lumen and the rest of the vessel wall. ... A 250 ml bag of newly collected platelets. ... Endothelin is a 21-amino acid vasoconstricting peptide that plays a key part in vascular homeostasis. ... The blood vessels are part of the circulatory system and function to transport blood throughout the body. ...

Jimenez & Derk^[16] describe three theories about the development of scleroderma:

• The abnormalities are primarily due to a physical agent, and all other changes are secondary or reactive to this direct insult.
• The initial event is fetomaternal cell transfer causing microchimerism, with a second summative cause (e.g. environmental) leading to the actual development of the disease.
• Physical causes lead to phenotypic alterations in susceptible cells (e.g. due to genetic makeup), which then effectuate DNA changes which alter the cell's behavior.

Epidemiology

Scleroderma affects approximately 300,000 people in the United States. It is four times as common in women than in men. Incidence rates are estimated at 2-20 per million per year in the United States.

Juvenile Scleroderma affects approximately 7000 children in the United States. Most common form of Juvenile Scleroderma is Localized Scleroderma - Morphea and/or Linear.

Patients' advocacy

The Juvenile Scleroderma Network is a volunteer-staffed 501(c)(3) non-profit organization dedicated to provide emotional support and educational information to parents and their children living with Juvenile Scleroderma; to support pediatric research to identify the cause of and the cure for Juvenile Scleroderma; to enhance the public's awareness of Juvenile Scleroderma.

The Scleroderma Foundation is a leading organization dedicated to raising awareness of the disease and assisting those who are affected. Its national spokesperson is Jason Alexander. Bob Saget is also very active in advocacy through the Scleroderma Research Foundation. Mr. Saget's sister died of the disease. Jason Alexander (born Jason Scott Greenspan on September 23, 1959) is a Jewish American television, cinema and musical theatre actor, best known for his role as George Costanza on the hit television series Seinfeld. ... Robert Lane Saget (born May 17, 1956) is an American actor, stand-up comedian and game show host best known for his role as Danny Tanner in the ABC sitcom Full House from 1987 to 1995, as host of Americas Funniest Home Videos from 1989 to 1997 and as...

The Road Back Foundation, founded as a non-profit, charitable organization in 1993, is a peer-to-peer endeavor run entirely by volunteers -- professionals who are dedicated to providing information and support to people with rheumatic diseases.

References

1. ^ ^{a b c d} Systemic sclerosis and related syndromes. Primer on the rheumatic diseases, 11th edition, The Arthritis Society, p. 269, 1997
2. ^ Valentini G, Black C. Systemic sclerosis. Best Pract Res Clin Rheumatol. 2002 Dec;16(5):807-16. PMID 12473275
3. ^ Olsen NJ, King LE Jr, Park JH. Muscle abnormalities in scleroderma. Rheum Dis Clin North Am. 1996 Nov;22(4):783-96. PMID. 8923596.
4. ^ Steen VD. The lung in systemic sclerosis. J Clin Rheumatol 2005 Feb;11(1):40-6. PMID 16357695
5. ^ ^{a b c} Sallam H, McNearney TA, Chen JD. Systematic review: pathophysiology and management of gastrointestinal dysmotility in systemic sclerosis (scleroderma). Aliment Pharmacol Ther. 2006 Mar 15;23(6):691-712. PMID 16556171
6. ^ ^{a b c} Rose S, Young MA, Reynolds JC. Gastrointestinal manifestations of scleroderma. Gastroenterol Clin North Am. 1998 Sep;27(3):563-94. PMID 9891698
7. ^ Hendel L, Hage E, Hendel J, Stentoft P. Omeprazole in the long-term treatment of severe gastro-oesophageal reflux disease in patients with systemic sclerosis. Aliment Pharmacol Ther. 1992 Oct;6(5):565-77. PMID 1420748
8. ^ Ruangjutipopan S, Kasitanon N, Louthrenoo W, Sukitawut W, Wichainun R. Causes of death and poor survival prognostic factors in thai patients with systemic sclerosis. J Med Assoc Thai. 2002 Nov;85(11):1204-9. PMID 12546318.
9. ^ Steen VD, Mayes MD, Merkel PA. Assessment of kidney involvement. Clin Exp Rheumatol. 2003;21(3 Suppl 29):S29-31 PMID 12889219.
10. ^ Steen VD. Renal involvement in systemic sclerosis. Clin Dermatol. 1994 Apr-Jun;12(2):253-8. PMID 8076263.
11. ^ Steen VD. Scleroderma renal crisis. Rheum Dis Clin North Am. 2003 May;29(2):315-33. Review. PMID 12841297.
12. ^ Rhew EY, Barr WG. Scleroderma renal crisis: new insights and developments. Curr Rheumatol Rep. 2004 Apr;6(2):129-36. Review. PMID 15016343.
13. ^ ^{a b} Steen VD, Medsger TA Jr. Long-term outcomes of scleroderma renal crisis. Ann Intern Med. 2000 Oct 17;133(8):600-3. PMID 11033587. [Free Full Text http://www.annals.org/cgi/reprint/133/8/600.pdf].
14. ^ ^{a b c} Jimenez S, Koenig AS. Scleroderma. eMedicine.com. URL: http://www.emedicine.com/MED/topic2076.htm. Accessed: May 22, 2006.
15. ^ 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.
16. ^ ^{a b c d e f g} Jimenez SA, Derk CT. Following the molecular pathways toward an understanding of the pathogenesis of systemic sclerosis. Ann Intern Med 2004;140:37-50. PMID 14706971.
17. ^ http://www.rheumatology.org/publications/classification/systsclr.asp.
18. ^ Morpea CNN.com, (May 05, 2006).
19. ^ Bianchi DW. Fetomaternal cell trafficking: a new cause of disease? Am J Med Genet 2000;91:22-8. PMID 10751084.

CNN.com is the news website maintained by CNN. The website debuted on August 30, 1995, and it describes itself as the first major news and information website on the Internet. ...

External links

• Juvenile Scleroderma Network
• Juvenile Systemic Sclerosis
• Scleroderma Foundation
• Scleroderma Society of Ontario
• International Scleroderma Network
• The Scleroderma Research Foundation
• UK Scleroderma Society