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Scleroderma, Word that derives from the Greek adjective sklērodermos, which means 'of hard skin', is an autoimmune disease that is characterized by the thickening and hardening of the skin. This change may be limited, especially affecting hands and the closest to them part of the forearms, feet, face and neck, with a slow progression over the years; or may be diffuse, spreading to the trunk, progressing, in general, more quickly in the first three years and then parked it.
The thickening and hardening makes losing wrinkles, skin folds and the expression of the face. These changes in themselves do not produce pain, but alterations companions or precedents to these changes in the circulation of the blood in the fingers can produce pain and ulcers on the skin.
Scleroderma has an incidence of 4 to 12 new cases per million inhabitants per year, and is three to four times more frequent in women than in men, occurs in all races and is universal. The disease often starts at age 40.

Types of Scleroderma

Scleroderma includes several clinical forms ranging from localized disease, with involvement of the skin, until systemic sclerosis, widespread disease that affects not only the skin, but also to various internal organs, as the digestive tract, kidney, heart and lung. Thus, we can say that the clinical forms of scleroderma are:
  • Preesclerodermia: there is Raynaud's phenomenon (you can find an explanation of what this phenomenon in the section signs and symptoms of Scleroderma), there are alterations capilaroscopicas (study of capillaries), antinuclear antibodies are positive and there is ischemic lesions in the fingers.
  • Limited: there are several years of evolution Raynaud's phenomenon, there is a skin condition that is limited to the hands and the face or forearms, or both. In addition the visceral involvement appears late, there are anticentromere antibodies (in approximately 75% of patients) and in the laroscopy large capillaries and dilated capillary loops are observed, but there is no hair loss.
  • Diffuse form: exist recent emergence Raynaud's phenomenon, there is a skin condition of the trunk and extremities, there are early condition of internal organs, some patients (30%) have anti-Scl70 antibodies and in the laroscopy loss of capillaries and dilated loops are observed.
  • Scleroderma sine Scleroderma: there may be or not Raynaud's phenomenon, there is no involvement of the skin and other viscera (lung, heart, kidney, gastrointestinal tract) and antinuclear antibodies are positive.

Prognosis for Scleroderma

The prognosis for Scleroderma depends primarily on whether the disease is only skin, limited or diffuse. Diffuse forms of the disease present with pulmonary, cardiac and renal involvement, which often accompany a worse prognosis.
In the majority of patients Scleroderma evolves slowly and survive for many years after diagnosis. Only a few develop a disease that quickly advance to a general deterioration and death.

Causes and risk factors of Scleroderma

The exact cause of scleroderma, is not known although it is considered that there are three elements that influence in its origin and development:
  • A disorder of the synthesis of collagen that causes fibrosis.
  • Certain disorders of the blood vessels.
  • Certain immunological abnormalities.
The most accepted idea about generation of disease is that an altered immune activity, which would consist of an increased production of antibodies and a type of lymphocyte, T-lymphocytes, against a specific antigen. This would lead to a vascular disorder that would be the first step and also the cause of fibrosis.
In addition, there are some cases in which Yes has established a relationship between the contact with certain substances, and the appearance of similar to Scleroderma clinical pictures:
  • Polyvinyl chloride: handling during polymerization.
  • Silica: Scleroderma is 25 times more common in miners exposed to it.
  • Silicone: breast prostheses.
  • ( Treatment with bleomycin: chemotherapy drug) or with pentazocine (opioid drug).
  • Certain organic solvents: trichloroethylene and aromatic hydrocarbons.
  • -Toxic oil: the toxic oil syndrome or syndrome of rape was a mass poisoning happened in Spain in 1981, caused by the ingestion of rapeseed oil denatured.

Prevention of Scleroderma

Apart from the clinical pictures induced by chemical substances, at the moment there is no prevention of scleroderma. These cases, due to contact with substances that carry this risk, would be prevented if avoiding such contact.

Signs and symptoms of Scleroderma

In some cases the Scleroderma begins with widespread pain, fatigue, loss of weight and stiffness. In others it starts with symptoms caused by the condition of the viscera, even without alterations in the skin. But the most common Scleroderma is that the affected person present during a variable number of years a progressive skin tightening, especially of the hands (sclerodactyly), accompanied by Raynaud's phenomenon.
The mean age at onset of Scleroderma is 40 years. As we said, the most characteristic clinical manifestation is the involvement of the skin, with three phases: first tend to inflate the fingers of the hands, which adopt a form of sausage, in a second phase hardens the skin - is not pinching skin folds and wrinkles disappear, there is little mobility in fingers, the face has no expression and the mouth opening is less - andFinally, skin thinning occurs.

Raynaud's phenomenon

The most frequent in Scleroderma initial manifestation is the Raynaud's phenomenon - occurs in 90% of cases, which usually persist throughout the disease. It is so characteristic that their absence makes it unlikely the diagnosis of scleroderma. Raynaud's phenomenon - can appear in different diseases, not only in the Scleroderma — is a disorder that is characterized by the reduction or lack of blood supply (ischemia) in the fingers, episodic way. Manifested by certain changes in the coloration of the fingers of the hands and feet: pallor, followed by bluish discoloration of the skin (cyanosis) after exposure to cold, and acontinuación redness after heating.
Fingers, after being exposed to the cold, pale represents the ischemic phase of the phenomenon, and is due to the closure of the arteries that carry blood to the fingers. During this phase are dilated small blood vessels (capillaries and venules) and appears blue fingers staining because there are deoxygenated blood in these vessels. Phases of pallor and cyanosis is accompanied by numbness, tingling and feeling of cold. With warming blood circulated rapidly resulting in redness and heat, which patients usually experience as intense pain in the fingers. These alterations in the blood supply in the fingers can cause ulcers on the skin.

Involvement of joints

It begins with stiffness and pain, then produced a limitation of mobility, especially in the fingers of the hands.

Condition of the viscera

Within the visceral manifestations, the most common are gastrointestinal, especially affecting the esophagus, but any part of the digestive tract can be altered. Problems of the esophagus are due mostly to loss of its mobility, which alters the advance of food from the mouth to the stomach.
The following visceral change in frequency is the pulmonary condition. Affected patients have difficulty breathing and, in general, to suffer a gradual deterioration in lung function due to fibrosis of the lungs, which is the leading cause of death in these patients.

Heart condition

The involvement of the heart consists, among others, angina-like chest pain and arrhythmias. The disease can affect both the cardiac muscle, as blood vessels, the heart's conduction system.

Kidney condition

When the disease affects the kidney is spoken of nephropathy, which usually occur during the first few years. Some patients also have severe hypertension, precisely, induced nephropathy.

Other manifestations

Other manifestations may occur in Scleroderma as diseases of the endocrine system, disorders of the nerves or dry syndrome or Sjogren (disease of autoimmune origin whose essential feature is the lower secretion of Exocrine glands, such as, for example, the sweat producing).

Diagnosis of Scleroderma

Some features of the disease (discoloration of the fingers) Raynaud's phenomenon, sclerodactyly (hardening of the skin of the fingers) and esophageal disorder should consider the diagnosis of Scleroderma.
In addition, the results of some tests such as the detection of some autoantibodies specific to this disease or the alteration of circulation in the capillaries through the Wadi laroscopy (evaluation of Microcirculation under the nail) nail can help to orientate the diagnosis of scleroderma.
In laboratory tests detected unspecified disorders - that is, that can appear in many other diseases, such as: the sedimentation speed, anemia, rheumatoid factor positive or the increase of immunoglobulins.
It is oriented more in the diagnosis of the esclerdodermia the study of antinuclear antibodies, but they are also indicative of other systemic diseases. These autoantibodies are very frequent in Scleroderma, are detected in 90% of patients and the most common pattern is mottled, although nucleolar pattern is the more specific disease. These patterns are observed using a fluorescence microscopy, the type. Other autoantibodies as the anticentromere, the anti-Scl70 or antinucleolo can be detected.

Treatment of Scleroderma

As a general measure for the treatment of Scleroderma is often used D-penicillamine, a drug that is capable of interfering with the synthesis of collagen in the early stages of the disease, a dose of 125 grams on alternate days. In this phase also used low-dose glucocorticoids to treat swelling (edema), since high doses can trigger a renal crisis.
For the treatment of Raynaud's phenomenon is recommended to avoid the cold and given the (nifedipine and diltiazem) calcium channel blocking drugs; drugs such as prostacyclin, the bosentan or the antiplatelet agents (e.g., acetylsalicylic acid) can be used in cases of ulcers on the fingers. The use of moisturizing creams for the skin is also recommended.
In patients with esophageal symptoms (such as omeprazole) Proton pump inhibitor drugs are administered. In cases of overgrowth of intestinal bacteria is usually given some kind of antibiotic. The stiffness and pain in the joints are treated with nonsteroidal anti-inflammatory drugs (e.g., ibuprofen).
The Interstitial Pulmonary condition is treated with oxygen therapy, bronchodilators, antibiotics and cyclophosphamide and glucocorticoids in the early stages (alveolitis). In cases with pulmonary hypertension, vasodilators (e.g. nifedipine), prostacyclin and bosentan, among other treatments are used.
The heart condition is treated depending on the manifestations that arise. The fámacos for the treatment of high blood pressure are ACE Inhibitors (angiotensin, as for example the enalapril-converting enzyme inhibitors).
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