| Scleroderma also known as systemic | | | | Scleroderma affects approximately |
| sclerosis. Scleroderma is an autoimmune, | | | | 300,000 people in the United States. |
| connective tissue disease. Scleroderma | | | | Scleroderma affecting the esophagus |
| causes inflammation and thickening of | | | | leads to heartburn. This is directly a |
| the skin. Genes may play in leading to | | | | result of stomach acid backflowing up |
| the development of scleroderma. | | | | into the esophagus. Sometimes this can |
| Scleroderma is a diffuse connective | | | | lead to scarring of the esophagus with |
| tissue disease characterized by changes | | | | difficulty swallowing and/or localized |
| in the skin, blood vessels, skeletal | | | | pain in the central chest. It is four |
| muscles, and internal organs. A | | | | times as common in women than in men. |
| connective tissue disease is one that | | | | Incidence rates are estimated at 2-20 |
| affects tissues such as skin, tendons, | | | | per million per year in the United |
| and cartilage. In some cases, | | | | States. Scleroderma affecting the large |
| scleroderma also affects the blood | | | | bowel (colon) most often causes |
| vessels and internal organs. Scleroderma | | | | constipation, but can also lead to |
| is one of a group of arthritic | | | | cramping and diarrhea. When this is |
| conditions called connective tissue | | | | severe, it complete stool blockage |
| disorders. In these disorders, a | | | | (fecal impaction) can result. |
| person's antibodies are directed against | | | | Inflammation of the lungs in scleroderma |
| his or her own tissues. Scleroderma can | | | | can cause scarring, resulting in |
| be classified in terms of the degree and | | | | shortness of breath, especially with |
| location of the skin involvement. | | | | physical exertion. Elevated pressure in |
| Accordingly, scleroderma has been | | | | the arteries to the lungs (pulmonary |
| categorized into two major groups, | | | | hypertension) can also cause shortness |
| diffuse and limited. Diffuse form of | | | | of breath and difficulty getting an |
| scleroderma is involves symmetric | | | | adequate breath with activity. Elevated |
| thickening of skin of the extremities, | | | | blood pressure is potentially serious |
| face, trunk (chest, back, abdomen, or | | | | and can lead to kidney damage. Symptoms |
| flanks) which can rapidly progress to | | | | include headache, fatigue, and in severe |
| hardening after an early inflammatory | | | | cases, stroke. |
| phase.Organ disease can occur early on | | | | Scleroderma can affect many different |
| and be serious. Organs affected include | | | | organs and organ systems. Exposure to |
| the esophagus, bowels, lungs with | | | | industrial solvents or an environmental |
| scarring (fibrosis), heart, and kidneys. | | | | agent may play a role in predisposing to |
| Limited scleroderma typically has | | | | scleroderma. Medications that help with |
| gradual onset and is restricted to | | | | blood circulation such as, |
| certain areas of the skin. Raynaud's | | | | angiotensin-converting enzyme (ACE) |
| phenomenon is due to vasoconstriction of | | | | inhibitors and low-dose enteric-coated |
| the small arteries of exposed | | | | aspirin. Hydroxychloroquine (Plaquenil) |
| peripheries - particularly the hands and | | | | drug has relatively few side effects, |
| feet - in the cold. The skin changes and | | | | and it's also effective for the |
| other features of disease tend to occur | | | | arthritis that can be associated with |
| more slowly than in the diffuse form. | | | | scleroderma. Apart from |
| Because a characteristic clinical | | | | hydroxychloroquine's apparent ability to |
| pattern can occur in patients with the | | | | affect the way immune cells work, |
| limited form of scleroderma, this form | | | | scientists don't completely understand |
| has taken another name which is composed | | | | how it helps tame the disease process. |
| of the first initials of the common | | | | Cyclophosphamide (Cytoxan) is sometimes |
| components. Telangiectasia a condition | | | | used to treat pulmonary fibrosis. People |
| caused by the swelling of tiny blood | | | | with scleroderma-related lung disease |
| vessels, in which small red spots appear | | | | found cyclophosphamide modestly improved |
| on the hands and face. While not | | | | lung function and quality of life. |
| painful, these red spots can create | | | | Bosentan (Tracleer) is an oral |
| cosmetic problems. Genetic | | | | medication that has been approved for |
| predisposition appears to be limited: | | | | pulmonary hypertension in people with |
| genetic concordance is small; still, | | | | scleroderma. Esophagus irritation and |
| there often is a familial predisposition | | | | heartburn can be relieved with |
| for autoimmune disease. Polymorphisms in | | | | omeprazole (Prilosec), esomeprazole |
| COL1A and TGF-ß1 may influence severity | | | | (Nexium), or lansoprazole (Prevacid). |
| and development of the disease. There is | | | | Antacids can also be helpful. Elevating |
| limited evidence implicating | | | | the head of the bed can reduce backflow |
| cytomegalovirus (CMV) as the original | | | | of acid into the esophagus that causes |
| epitope of the immune reaction, and | | | | inflammation and heartburn. Avoiding |
| organic solvents and other chemical | | | | caffeine and cigarette smoking also |
| agents have been linked with | | | | helps. |
| scleroderma. | | | | |