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Scleroderma is a rare, chronic autoimmune disease that causes skin to thicken and tighten. For some patients, it also causes life-threatening damage to internal organs. Although there's no cure for the disease, its impact on people's lives can be reduced.
The systemic forms of scleroderma cause fibrosis (scar tissue) to be formed in the skin and/or internal organs. The fibrosis eventually causes the involved skin or organs to harden, which is why scleroderma is popularly known as the "disease that turns people into stone."
There are two main types of scleroderma, localized and systemic. The systemic forms can affect any part of the body (skin, blood vessels, and internal organs.) They are also referred to as "systemic sclerosis" plus other terms such as diffuse, limited, CREST, and overlap. When systemic scleroderma affects the skin, it can cause the skin - most commonly on the hands and/or face - to harden. With affecting the blood vessels, it can cause Raynaud's phenomenon. When it affects the internal organs, it may cause disability or even death.
The localized forms are morphea and linear. They affect only the skin (and sometimes the underlying tissues) but do not affect the internal organs, or reduce one's life expectancy in any way.
Diffuse scleroderma is when skin tightening also occurs above the wrists or elbows. Limited scleroderma is when skin involvement is limited to the hands (although the face and neck may also be involved.) CREST stands for Calcinosis, Raynaud's, Esophagus, Sclerodactyly, and Telangiectasia. CREST may occur alone, or in combination with any other form of scleroderma (or even other autoimmune diseases) such as CREST with limited scleroderma, or CREST with lupus. If a scleroderma patient also has any other autoimmune disease (such as lupus, rheumatoid arthritis, etc.) it is referred to as overlap; as in "Diffuse scleroderma in overlap with lupus." Scleroderma symptoms can also be a part of Mixed Connective Tissue Disease (MCTD), or Undifferentiated Connective Tissue Disease (UCTD).
When any form of scleroderma (either localized or systemic) occurs in children, it is also called Childhood Scleroderma or Juvenile Scleroderma.
Incidence; Causes & Development
Scleroderma afflicts an estimated 150,000 to 500,000 Americans, primarily females who are 30 to 50 years old at onset. The overall occurrence is 30 people per 100,000 and the ratio of women to men is about four to one. Average age at diagnosis is significantly younger for blacks than for whites, and black patients are almost twice as likely to have diffuse disease than whites. Median survival is 11 years. [Wiley InterScience. Arthritis Rheum. 2003 Aug;48(8): pp.2246-55]
The cause of scleroderma is unknown. Some cases of scleroderma have been linked to chemical exposures.
Diagnosis & Tests
Proper diagnosis of scleroderma is often long and difficult, since it is a rare disease which few doctors are well-versed in, and in the early stages it may resemble many other connective tissue diseases, such as systemic lupus erythematosus, polymyositis, and rheumatoid arthritis, just to name a few.
Often the diagnostic uncertainty and frustration takes a huge toll on the psychological well-being of the patient, who sometimes describe their journey to diagnosis as being by far the most difficult part of their illness. Many other diseases may have a scleroderma-like appearance. In these diseases, abnormalities of nail folds, visceral involvement and immunological changes are usually different from those observed in systemic scleroderma. [PubMed. Rev Prat. 2002 Nov 1;52(17): pp.1884-90]
Treatment & Prevention
At present, there are no proven treatments or cure for any forms of scleroderma. However, there are effective therapies for many of the symptoms.
There are several "natural" things you can do for systemic scleroderma.- Avoid attacks of Raynaud's as much as possible. Try to learn various ways to prevent Raynaud's and to restore circulation. Then respond to every attack of Raynaud's as if it were a medical emergency, because even one prolonged attack can lead to gangrene, and possibly even amputation.
- One thing which will give you the greatest odds of living longer with systemic scleroderma, is to monitor your blood pressure at home every day or two. One of the most life-threatening, yet treatable, complications of systemic scleroderma (including CREST) is sudden kidney failure. If your blood pressure is elevated over a certain point (to be determined by your doctor) you should call your doctor immediately. You should also ask your doctor to take your blood pressure on every office visit.
- Stress can play havoc with your immune system, as well as trigger and worsen attacks of Raynaud's. Alternative therapies that help you deal more effectively with stress, such as biofeedback and yoga, may improve your symptoms.
- Strive for moderation in everything - just enough but not too much exercise, good sleep every night with sufficient rest breaks during the day, and well-balanced, regular meals.
- In general, most doctors recommend that scleroderma patients follow a normal, well-balanced diet, without the addition of any herbs, vitamins, or minerals. Do not cut out any food groups from your diet, nor go on any "cleansing" diets, since this may accelerate the disease process.
- Don't go on any diets that eliminate certain food groups.
- Avoid high-protein diets because they are very hard on the kidneys.
- For relief of symptoms such as Raynaud's and heartburn, you may want to particularly avoid:
- caffeine (can trigger Raynaud's and heartburn)
- chocolate (can trigger Raynaud's and heartburn)
- peppermint (can trigger heartburn)
- ginger (can trigger heartburn)
Many herbs and over-the-counter medications can interact with prescription medications, and many of them make scleroderma symptoms worse. Even simple over-the-counter cold remedies often have ingredients that can trigger Raynaud's or heartburn in systemic scleroderma patients.
The immune system does not need to be improved in scleroderma, because it is already overactive (and not underactive, as in AIDS.) Doing things that enhance the immune system can actually make scleroderma worse in some cases, as it gives the body more power to attack itself.
Research shows that Raynaud's can cause an abnormal processing of certain vitamins and minerals, leading to worsening scleroderma. So excessive intake of any vitamin is to be avoided.
Prognosis
In the past, only the worst cases of scleroderma were diagnosed, thus leading to the widespread belief that systemic scleroderma was always progressive and fatal. Now that the full range of types and severity of scleroderma are being recognized and diagnosed, there is increasing awareness that many people have a milder type of illness with a much better long term outlook.
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Conditions that suggest Scleroderma:
Risk factors for Scleroderma:
Recommendations and treatments for Scleroderma: | | | | | Skin |  DMSO Topically | In an older study, later confirmed by others, Arthur Scherbel, MD, of the Department of Rheumatic Diseases and Pathology at the Cleveland Clinic Foundation, used DMSO on 42 scleroderma patients who had already exhausted all other possible therapies without relief. The investigators concluded that 26 of the 42 showed good or excellent improvement. Histotoxic changes were observed together with healing of ischemic ulcers on fingertips, relief from pain and stiffness, and an increase in strength. The investigators noted, "It should be emphasized that these have never been observed with any other mode of therapy." [Ann NY Acad Sci 141: pp.613-29, 1967] |
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Vitamins |
 Vitamin Paba |
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KEY | | Weak or unproven link |  | | Proven definite or direct link | | | Likely to help | | | Highly recommended |
GLOSSARY
AIDS
Acquired Immune Deficiency Syndrome. An immune system deficiency disorder that suddenly alters the body's ability to defend itself. The AIDS virus invades the T4 helper/inducer lymphocytes and multiplies, causing a breakdown in the body's immune system, eventually leading to overwhelming infection and/or cancer, with ultimate death.
Arthritis (Arthritic)
Inflammation of a joint, usually accompanied by pain, swelling, and stiffness, and resulting from infection, trauma, degenerative changes, metabolic disturbances, or other causes. It occurs in various forms, such as bacterial arthritis, osteoarthritis, or rheumatoid arthritis. Osteoarthritis, the most common form, is characterized by a gradual loss of cartilage and often an overgrowth of bone at the joints.
Autoimmune Disease (Autoimmune, Autoimmunity)
One of a large group of diseases in which the immune system turns against the body's own cells, tissues and organs, leading to chronic and often deadly conditions. Examples include multiple sclerosis, rheumatoid arthritis, systemic lupus, Bright's disease and diabetes.
Chronic (Chronicity)
Usually referring to chronic illness: Illness extending over a long period of time.
Chronic Renal Failure (Chronic Renal Insufficiency, Kidney Failure, Renal Insufficiency)
(CRF) Irreversible, progressive impaired kidney function. The early stage, when the kidneys no longer function properly but do not yet require dialysis, is known as Chronic Renal Insufficiency (CRI). CRI can be difficult to diagnose, as symptoms are not usually apparent until kidney disease has progressed significantly. Common symptoms include a frequent need to urinate and swelling, as well as possible anemia, fatigue, weakness, headaches and loss of appetite. As the disease progresses, other symptoms such as nausea, vomiting, bad breath and itchy skin may develop as toxic metabolites, normally filtered out of the blood by the kidneys, build up to harmful levels. Over time (up to 10 or 20 years), CRF generally progresses from CRI to End-Stage Renal Disease (ESRD, also known as Kidney Failure). Patients with ESRD no longer have kidney function adequate to sustain life and require dialysis or kidney transplantation. Without proper treatment, ESRD is fatal.
Esophagus (Esophageal)
Commonly called the "food pipe", it is a narrow muscular tube, about nine and a half inches long, that begins below the tongue and ends at the stomach. It consists of an outer layer of fibrous tissue, a middle layer containing smoother muscle, and an inner membrane, which contains numerous tiny glands. It has muscular sphincters at both its upper and lower ends. The upper sphincter relaxes to allow passage of swallowed food that is then propelled down the esophagus into the stomach by the wave-like peristaltic contractions of the esophageal muscles. There is no protective mucosal layer, so problems can arise when digestive acids reflux into the esophagus from the stomach.
Herbs (Herb, Herbal)
Herbs may be used as dried extracts (capsules, powders, teas), glycerites (glycerine extracts), or tinctures (alcohol extracts). Unless otherwise indicated, teas should be made with one teaspoon herb per cup of hot water. Steep covered 5 to 10 minutes for leaf or flowers, and 10 to 20 minutes for roots. Tinctures may be used singly or in combination as noted. The high doses of single herbs suggested may be best taken as dried extracts (in capsules), although tinctures (60 drops four times per day) and teas (4 to 6 cups per day) may also be used.
Immune System (Immune Response, Immunity)
A complex that protects the body from disease organisms and other foreign bodies. The system includes the humoral immune response and the cell-mediated response. The immune system also protects the body from invasion by making local barriers and inflammation. The process may involve acquired immunity (the ability to learn and remember a specific infectious agent), or innate immunity (the genetically programmed system of responses that attack, digest, remove, and initiate inflammation and tissue healing).
Ischemia (Ischemic)
Localized tissue anemia due to obstruction of the inflow of arterial blood. A decrease in the blood supply to a bodily organ, tissue, or part caused by constriction or obstruction of the blood vessels.
Kidneys (Kidney, Renal)
Bean-shaped organs, each about the size of a fist. They are located near the middle of the back, just below the rib cage. The kidneys are sophisticated reprocessing machines, each day handling about 50 gallons of blood to sift out about half a gallon of waste products and extra water. The waste and extra water become urine, which flows to the bladder through tubes called ureters. The actual filtering occurs in tiny units inside the kidneys called nephrons. Every kidney has about a million nephrons. In a nephron, a glomerulus -- which is a tiny blood vessel, or capillary -- intertwines with a tiny urine-collecting tube called a tubule. A complicated chemical exchange takes place, as waste materials and water leave your blood and enter your urinary system. The kidneys recycle chemicals such as sodium, phosphorus, and potassium and thus regulate their levels. Renal: Pertaining to the kidneys.
Mineral (Minerals)
Plays a vital role in regulating many body functions. They act as catalysts in nerve response, muscle contraction and the metabolism of nutrients in foods. They regulate electrolyte balance and hormonal production, and they strengthen skeletal structures.
Over-The-Counter (OTC, Over The Counter)
A drug or medication that can legally be bought without a doctor's prescription being required.
Pathology (Pathologist)
Disease, particularly one with clear and obvious changes in structure or function; the study of same.
Raynaud's Phenomenon (Raynaud's, Raynauds Disease, Raynaud's Disease, Raynauds Phenomenon, Raynauds Syndrome, Raynaud's Syndrome)
Raynaud's disease or syndrome is a disorder of blood circulation, mainly in the fingers and toes. It is of unknown cause and characterized by changes of the skin that are aggravated by exposure to cold: first, becoming white with numbness and pain as a result of inadequate oxygenation of the blood, then red/purple with a burning sensation. The sudden constriction of blood vessels causes decreased blood flow to the extremities and can, in extreme cases, lead to gangrene. Also called "white finger", "wax finger" or "dead finger".
Rheumatoid Arthritis
A long-term, destructive connective tissue disease that results from the body rejecting its own tissue cells (autoimmune reaction).
Scar Tissue
Fibrous tissue replacing normal tissues destroyed by injury or disease.
Scleroderma (Systemic Sclerosis)
An autoimmune disease of the connective tissue, characterized by the formation of scar tissue (fibrosis) in the skin and organs of the body. This leads to thickness and firmness of involved areas.
Telangiectasia
Small, unsightly red, purple or blue blood vessels found along the surface on the face, upper chest, neck and rarely on other parts of the body. Similar veins are found on the legs but called spider veins.
Ulcer (Ulceration, Ulcers)
Lesion on the skin or mucous membrane.
Last updated: Apr 13, 2008
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