Scleroderma is an autoimmune disease of the connective tissue featuring skin thickening, spontaneous scarring, blood vessel disease, varying degrees of inflammation, associated with an overactive immune system. Autoimmune diseases are illnesses that occur when the body's tissues are attacked by its own immune system. The formation of scar tissue (fibrosis) in the skin and organs of the body characterize scleroderma. This leads to thickness and firmness of involved areas. Scleroderma, when it is diffuse or widespread over the body, is also referred to as systemic sclerosis.
The cause of scleroderma is unknown. Researchers have found some evidence that certain genes are important factors, but the environment also seems to play a role. The result is activation of the immune system in a susceptible individual, causing injury to tissues that result in injury similar to scar-tissue formation. The fact that genes seem to cause a predisposition to developing scleroderma means that inheritance at least plays a partial role. It is not uncommon to find other autoimmune diseases in families of scleroderma patients. Some evidence for the role genes may play in leading to the development of scleroderma comes from the study of Choctaw Native Americans who are the group with the highest reported prevalence of the disease. The disease is more frequent in females than in males.
Scleroderma is a chronic systemic autoimmune disease (primarily of the skin) characterized by fibrosis (or hardening), vascular alterations, and autoantibodies. There are two major forms:
Limited systemic sclerosis/scleroderma involves cutaneous manifestations that mainly affect the hands, arms and face. Previously called CREST syndrome in reference to the following complications: Calcinosis, Reynaud’s phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasias. Additionally, pulmonary arterial hypertension may occur in up to one third of patients and is the most serious complication for this form of scleroderma.
Diffuse systemic sclerosis/scleroderma is rapidly progressing and affects a large area of the skin and one or more internal organs, frequently the kidneys, esophagus, heart and lungs. This form of scleroderma can be quite disabling. There are no treatments for scleroderma itself, but individual organ system complications are treated.accordinglly. Other forms of scleroderma include systemic scleroderma, which lacks skin changes, but has systemic manifestations, and two localized forms that affect the skin, but not the internal organs: morphea, and linear scleroderma.
The cause is unknown. Scleroderma runs in families, but the genes have not been identified. It affects the small blood vessels (arterioles) in all organs. First, the endothelial cells of the arteriole die off, along with smooth muscle cells, by a process of apoptosis. They are replaced by collagen and other fibrous material. Inflammatory cells, particularly CD4+ helper T cells, infiltrate the arteriole, and cause further damage. Many of the inflammatory and destructive protein signals have been identified, and they are potential targets for drugs that could interrupt the process.
Individuals with morphea or limited scleroderma have a relatively positive outlook. They will usually die from another disease, not the scleroderma. Those with very widespread skin and organ involvement (systemic) have a negative prognosis. More women have scleroderma, but the disease kills more men. Following diagnosis, two-thirds of patients live at least 11 years. The higher the patient's age at diagnosis, the more likely they are to die from the disease. People with scleroderma have very different life expectancies. For example, those with limited or mild diffuse disease—can expect to live 20 to 50 years after diagnosis, just like anyone else. Others with severe, rapidly progressive disease—a group which makes up less than 10% of the total number of patients with diffuse scleroderma—might have a 50% chance of a five-year survival.
Some types of scleroderma affect only the skin, while others affect the whole body.
- Localized scleroderma usually affects only the skin on the hands and face. It develops slowly, and rarely, if ever, spreads throughout the body or causes serious complications.
- Systemic scleroderma, or sclerosis, may affect large areas of skin and organs such as the heart, lungs, or kidneys. There are two main types of systemic scleroderma: Limited disease (CREST syndrome) and diffuse disease.
Skin symptoms of scleroderma may include:
- Fingers or toes that turn blue or white in response to hot and cold temperatures)(Reynaud’s)
- Hair loss
- Skin hardness
- Skin that is abnormally dark or light
- Skin thickening, stiffness, and tightness of fingers, hands, and forearm
- Small white lumps beneath the skin, sometimes oozing a white substance that looks like toothpaste
- Sores (ulcers) on the fingertips or toes
- Tight and mask-like skin on the face
Bone and muscle symptoms may include:
- Joint pain
- Numbness and pain in the feet
- Pain, stiffness, and swelling of fingers and joints
- Wrist pain
Breathing problems may result from scarring in the lungs and can include:
- Dry cough
- Shortness of breath
Digestive tract problems may include:
- Bloating after meals
- Difficulty swallowing
- Esophageal reflux or heartburn
- Problems controlling stools (fecal incontinence)
There is no specific treatment for scleroderma. Your doctor will prescribe medicines and other treatments to control your symptoms and prevent complications.
Medicines used to treat scleroderma include:
- Power anti-inflammatory medicines called corticosteroids
- Immune-suppressing medications such as methotrexate and Cytoxan
- Nonsteroidal anti-inflammatory drugs (NSAIDs)
Other treatments for specific symptoms may include:
- Medicines for heartburn or swallowing problems
- Blood pressure medications (particularly ACE inhibitors) for high blood pressure or kidney problems
- Light therapy to relieve skin thickening
- Medicines to improve breathing
- Medications to treat Reynaud’s phenomenon
Treatment usually also involves physical therapy.
Some people with scleroderma have symptoms that develop quickly over the first few years and continue to get worse. However, in most patients, the disease slowly gets worse.
People who only have skin symptoms have a better outlook. Widespread (systemic) scleroderma can damage the heart, kidney, lungs, or GI tract, which may cause death.
Lung problems are the most common cause of death in patients with scleroderma.
The most common cause of death in people with scleroderma is scarring of the lungs, called pulmonary fibrosis.
Other complications of scleroderma include:
- Heart failure
- High blood pressure in the lungs (pulmonary hypertension)
- Kidney failure
- Problems absorbing nutrients from food (malabsorption)
There is no known prevention. Reducing your exposure to silica dust and polyvinyl chloride may lower your risk for this disease.
Because scleroderma is an autoimmune disease, one of the major pillars of treatment involves the use of immunosuppressive agents.
How Medical Marijuana (Cannabinoids) Can Help Symptoms
- Anti-fibroid effect
- protect the immune system
- anti-inflammatory effects
- analgesic effects
- cannabinoids used as anti-itch
- anti-immune suppressive properties
- improve sleep
- improve mood (anti-anxiety)
- improve appetite
- help relieve hardened esophagus
- relieve painful joints
- help with gastrointestinal problems (diarrhea)
- lower blood pressure
Use as adjunct to other prescription drugs. You may find that you will begin to use less and less of your prescription (NSAIDS and opiates) drugs. You may be able to cut them out completely. Medical marijuana will provide relief from many of your symptoms and with little or no side effects.
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2. Varga J, Denton CP. Systemic sclerosis and the scleroderma-spectrum disorders. In: Firestein GS, Budd RC, Harris ED Jr., et al., eds. Kelley's Textbook of Rheumatology. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2008: chap 77.
3. Clouse RE, Diamant NE. Esophageal motor and sensory function and motor disorders of the esophagus. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2006: chap 41.