Scleroderma

February 7, 2017

Scleroderma, also known as systematic sclerosis, is a chronic auto immune rheumatic disease. It affects skin and connective tissues of the body, which means that it is a condition where the body’s immune system acts abnormally. Hardening of the skin is one of the most visible effect of the disease. It also involves inflammation and scarring of many body parts, leading to problems in the lungs, kidneys, heart, intestinal system and other areas. Scleroderma is not contagious, infectious, cancerous or malignant.

The word “scleroderma” comes from two Greek words: “sclero” meaning hard, and “derma” meaning skin. The disease has been called “progressive systemic sclerosis,” but the use of that term has been discouraged as it has been found that scleroderma is not necessarily progressive. The disease varies from patient-to-patient.

Scleroderma is a rare disease. It is more common in women than men. Anyone can get it, even children. About 75,000 to 100,000 people in the U.S. have this disease.

Types of Scleroderma

There are two types of Sclerodera and each are sub classified.

Localized Scleroderma

The changes, which occur in localized scleroderma, are usually found in only a few places on the skin or muscles, and rarely spread elsewhere. It is relatively mild in nature. The internal organs are usually not affected, and persons with localized scleroderma rarely develop systemic scleroderma. It is classified in two types :

  • Morphea

Morphea is a form of localized scleroderma characterized by waxy patches on the skin of varying sizes, shapes and color. The skin under the patches may thicken. The patches may enlarge or shrink, and often may disappear spontaneously. It mostly occurs between the ages of 20 and 50, but is often seen in young children.

  • Linear scleroderma

It is a form of localized scleroderma which frequently starts as a streak or line of hardened, waxy skin on an arm or leg or on the forehead. Sometimes it forms a long crease on the head or neck, referred to as en coup de sabre because it resembles a saber or sword wound. This type tends to involve deeper layers of the skin as well as the surface layers, and sometimes affects the motion of the joints, which lie underneath and usually develops in childhood.

Systemic scleroderma (systemic sclerosis)

The changes occurring in systemic scleroderma may affect the connective tissue in many parts of the body. Systemic scleroderma can involve the skin, esophagus, gastrointestinal tract (stomach and bowels), lungs, kidneys, heart and other internal organs. It can also affect blood vessels, muscles and joints. It involve –

  • Limited Scleroderma – CREST Syndrome

Limited scleroderma means only limited areas of skin are thick; usually just the fingers and/or face. Limited scleroderma is the milder form of scleroderma. The CREST syndrome is a type of limited scleroderma. CREST stands for the following:

  • C – is for the calcium deposits under the skin and in tissues (calcinosis)
  • R – for Raynaud’s phenomenon.
  • E – esophageal dysmotility. This causes heartburn, which is often experienced by CREST patients.
  • S – is for sclerodactyly; that means thick skin on the fingers.
  • T – is for telangiectasias, which are enlarged blood vessels. These appear as red spots on the face and other areas.

Limited scleroderma causes less involvement of body organs than the more severe form. Some patients can develop lung and heart disease.

  • Diffuse Scleroderma

This type of scleroderma is called diffuse scleroderma. It means that more areas of the skin are involved and thickened, but there is a high degree of variability among patients. Skin of the arms, legs, and trunk are more likely to be involved. The tightened skin makes it difficult to bend fingers, hands, and other joints. There is sometimes inflammation of the joints, tendons and muscles. Tight skin on the face can reduce the size of a person’s mouth and make good dental care very important.

Diffuse scleroderma can have associated involvement of internal organs such as the gastrointestinal tract, heart, lungs, or kidneys. The degree of organ involvement is highly variable – some get none at all and other patients organs may be badly affected.

Causes

The cause of scleroderma is not known. We don’t yet know why people’s immune system becomes overactive and initiates excessive production of collagen or the reason for blood vessel abnormalities; but it is thought to be a combination of genetic and environmental factors.

Environmental Factors

  • Toxic Exposure – Exposure to cadmium, mercury (dental amalgam), solvents (such as paint thinners), radiation, and silica as either known or suspected causes of autoimmune diseases or scleroderma.
  • Artificial Joints and Silicon Implants – Some cases of connective tissue disease, such as scleroderma, related to artificial joints or silicone breast implants.
  • Asbestos – Asbestos is relatively harmless. However, when asbestos is become dry and brittle or is ground or broken up, the fibers become airborne and can be ingested through the mouth or inhaled through the lungs. As a result, once inhaled or ingested, asbestos fibers often remain in the body and can cause a multitude of medical problems like Scleroderma, lung cancer etc.
  • Air Pollution – Diseases such as scleroderma may be triggered by the inhalation of chemical solvents, herbicides and silica.
  • Drugs & Medications – Some medications and street drugs are known or thought to induce scleroderma or Raynaud’s, such as Bleomycin, Cocaine, Marijuana, and Paclitaxel (Taxanes).

Genetic Factors

Research has demonstrated that systemic sclerosis is a polygenic (involving more than one gene) autoimmune (involving the immune system response) disease. Several genes and gene-gene interactions have been identified as playing a role in systemic sclerosis.

Inflammatory Response and Autoimmunity

The disease cause leading to scleroderma seems to occur as an autoimmune response, in which an abnormal immune system attacks the body itself. In scleroderma, this response produces swelling (inflammation) and too much production of collagen. Collagen is the tough protein that helps build connective tissues such as tendons, bones, and ligaments. Collagen also helps scar tissue form. When normal tissue from skin, lungs, the esophagus, blood vessels, and other organs is replaced by this type of abnormal tissue, none of these body parts work as well, and many of the symptoms previously described occur.

Other Factors

  • Proteins – Increased amount of S100A8 (calcium- and zinc-binding protein) and S100A9 (Calcium Binding Protein) in patients with diffuse coetaneous systemic sclerosis. A correlation with organ involvement and immunological abnormalities. These two proteins may play important roles in the development of systemic sclerosis.
  • Oxidative Stress- Lipid Peroxidation – Oxidative Stress is an imbalance between pro-oxidants and antioxidants that can result in cellular degeneration.
  • Gluten – Studies suggest, of several rheumatological disorders , including Raynaud’s phenomenon in people with celiac disease, but this seems to be the first report of extensive microvascular damage, similar to capillary changes in scelroderma, documented by nailfold capillaroscopy in a patient with celiac disease.
  • Thyroid – It is common for people with systemic scleroderma to also have other health problems, and thyroid disease can often be associated with it. However, thyroid disease is very common in the general population, whereas systemic scleroderma is very rare, and thyroid disease is not considered to be a symptom of any type of scleroderma.
  • B and T Cells – B and T cells are white blood cells that help stimulate an immune response to infections. In the thymus gland, lympohocytes are matured into these cells. Sometimes these cells become overactive, which is suspected as being part of the process that leads to autoimmune diseases like Scleroderma.

Symptoms

Symptoms vary greatly from person to person depending on what part of the body is involved.

For some people, following symptoms are among the early signs of scleroderma:

  • Raynaud’s phenomenon – The fingers or toes turn white, then blue in the cold, and then red as blood flow returns. This is caused by narrowing of the blood vessels. It is possible to have Raynaud’s without having scleroderma, but most people with scleroderma will have symptoms of Raynaud’s at some time and it’s often one of the first symptoms to appear.
  • Thickening and hardening of the skin on the hands, arms and face
  • Stiffness and pain in the muscles and/or joints
  • Swelling of hands and feet, especially in the morning
  • Thinning of the pads at the finger tips
  • Small white chalky lumps (calcium deposits) under the skin
  • Indigestion or heartburn
  • Diarrhoea or constipation
  • Shortness of breath or reduced ability to exercise
  • Kidney problems and high blood pressure
  • Gut Problems – The gastrointestinal (GI) tract is primarily responsible for the processes of digestion and excretion, and is frequently affected by manifestations of Scleroderma.
  • Dryness – It is characterized by a decrease in secretions of the tear glands and the salivary glands, which provide lubrication for the eyes and mouth. The unusual dryness of the eyes resulting from this condition can lead to serious irritation and inflammation. Excessive dryness of the mouth may lead to difficulties in swallowing and speaking, a pronounced increase in tooth decay and cavities, and a reduced sense of taste. Dryness may also involve vagina and other areas of the body.

Related Complications

  • Digestive Systems – Digestive problems associated with scleroderma can lead to acid reflux and difficulty swallowing — some describe feeling as if food gets stuck midway down the esophagus.
  • Heart – Scarring of heart tissue increases the risk of abnormal heartbeats (arrhythmias) and congestive heart failure, and can cause inflammation of the membranous sac surrounding the heart (pericarditis). Scleroderma also can raise the pressure on the right side of the heart and cause it to wear out.
  • Teeth – Severe tightening of facial skin can cause the mouth to become smaller and narrower, which may make it hard to brush teeth or to even have them professionally cleaned.
  • Kidneys – When scleroderma affects the kidneys, it can develop an elevated blood pressure and an increased level of protein in the urine.
  • Lungs – Scarring of lung tissue (pulmonary fibrosis) can result in reduced lung function, reduced ability to breathe and reduced tolerance for exercise.
  • Sexual function – Men who have scleroderma often experience erectile dysfunction. Scleroderma may also affect the sexual function of women, by decreasing sexual lubrication and constricting the vaginal opening.
  • Gut – Evidence suggests that the involuntary muscle of the gastrointestinal tract (smooth muscle) can be affected in scleroderma. When this muscle is involved, abnormal motor function of the esophagus, stomach, small or large bowel results.

Treatment

  • Medications

Calcium-channel blockers are the standard drugs to open the blood vessels, and may be used for pulmonary artery hypertension and Raynaud’s phenomenon. This may involve – diltiazem (Cardizem, Dilacor), dihydropyridine medications (felodipine, amlodipine, and isradipine).

Side effects may include fluid buildup in the feet, constipation, fatigue, gingivitis, erectile dysfunction, flushing, and allergic symptoms.

  • ACE Inhibitors

Many medications are available for controlling blood pressure, but ACE inhibitors appear to be the most effective for scleroderma patients because of their protective actions in the kidney. This includes – captopril (Capoten), enalapril (Vasotec), quinapril (Accupril), benazepril, and lisinopril (Prinivil, Zestril). Side effects may include irritating cough, large drops in blood pressure, and allergic reactions.

  • Angiotensin Receptors – This involves losartan, candesartan cilexetil, and valsartan. They have positive effects on blood vessels. Small studies showing improvement in Raynaud’s phenomenon warrant further research.
  • Nitrates – Nitrates relax smooth muscles and open arteries, and are therefore sometimes used for the short-term management of Raynaud’s phenomenon. Side effects of nitrates include headaches, dizziness, nausea, blurred vision, fast heartbeat, and sweating.
  • Cyclophosphamide (Cytoxan) – Cyclophosphamide is the most important immunosuppressant currently used for scleroderma. It blocks some of the destructive actions of scleroderma in the lungs. Intravenous cyclophosphamide can be life-saving for patients with pneumonia caused by interstitial lung disease. Side effects may include – hair loss, infection, and bleeding into the urinary tract.
  • Other Drugs – D-penicillamine (which may be useful for skin symptoms), methotrexate (Rheumatrex), sirolimus (rapamycin), antithymocyte globulin (ATG), corticosteroids, cyclosporine A, and chlorambucil (Leukeran). All of these drugs have potentially severe side effects.

Surgeries

  • Sympathectomy and Hand Surgeries – This uses procedures that block or remove the nerve responsible for narrowing blood vessels in the hand. The result is increased blood flow in the hand.
  • Other Surgeries – Disabling deformity of the hand is a common feature of scleroderma. Various surgical procedures can relieve pain, prevent tissue loss, protect hand function, and improve the appearance of the hands.

Alternative Treatment

Environmental Medicine is a branch of medicine whose domain is not limited by anatomical boundaries but, rather, is concerned with the whole person and the way that a person reacts to his/her total environment. At our center we first carry out Comprehensive Approach of studying the patient’s medical history, where we are able to get hold of the root cause and treat accordingly.

  • Bio-detoxification Programme – The Center’s Bio-detoxification Program utilizes clinical procedures that safely reduce the body’s burden of toxic chemicals, including chemicals stored following occupational, accidental, and/or chronic airborne exposures.
  • Alka Vita Supplements – This has the tremendous advantage that it alkalises, therefore increases oxygenation, counteracts free radical attack and damage and therefore undermines the basis of disease. It is also directly ‘anti-septic’ against fungal infections, including Candida and possibly all harmful micro-organisms and parasites, although there is not enough information on this, we do know that all anaerobic infections (the harmful ones) are attacked by free electrons that Alka- vita supplies and are eventually destroyed or severely limited by an alkaline environment.
  • AA and DHA – The essentiality of arachidonic acid (AA) and docosahexaenoic acid (DHA), when dosed appropriately, fish oil-based lipid emulsions contain sufficient essential fatty acid (EFAs) to prevent essential fatty acid deficiency.
  • Curcumin (Tuermeric) – The varied biological properties of curcumin and lack of toxicity even when administered at higher doses makes it attractive to explore its use in various disorders like tumors of skin, colon, duodenum, pancreas, breast and other skin diseases.
  • Vitamin B – A growing body of research is drawing a link between low B12 and early cognitive decline, a condition that often leads to auto immune disease.
  • Probiotics – Yogurt may be helpful for combatting bowel involvement with systemic scleroderma, especially small bowel bacterial overgrowth. It may also be particularly helpful when taking antibiotics.
  • Lipioc acid – Dihydrolipoic acid (DHLA) not only acts as an antioxidant but also an antifibrotic since it has the ability to reverse the profibrotic phenotype of Sclerodermal fibroblasts.
  • Vitamins & Minerals – Different Vitamins are essential to aid the immune system and many biochemical processes including the utilisation of calcium and magnesium. Its deficiency is almost universal in northern climates and even more so since the introduction of sun screen. Its deficiency has been linked strongly to auto-immune diseases. Zinc has critical effect in homeostasis, immune function, oxidative stress, apoptosis, aging and in chronic diseases, including atherosclerosis, several malignancies, neurological disorders, autoimmune diseases. Vitamin D is essential for promoting calcium absorption in the gut, build and preserve bone, helps prevent osteoporosis and helps decrease fracture risk.
  • Herbs – Herbs such as Cleavers and Red Clover help to cleanse toxins from the lymphatic system and to purify the blood, allowing much needed nutrients to reach the skin and tissues.

 

Referneces –

www.sclero.org

www.medicinenet.com

www.hopkinsscleroderma.org

www.mayoclinic.org

www.raynauds.org.uk

www.rheumatology.oxfordjournals.org

www.arthritis-research.com

www.todaysdietitian.com

www.scleroderma.ca

www.aarda.org

www.niams.nih.gov