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Systemic sclerosis Symptoms, Diagnosis and Treatment

Sunday, February 24th 2013. | Diseases & Condition

What is Systemic sclerosis :

Connective tissue or connective are those whose primary function is to provide support to the body and systemic integration. These structures allow to differentiate tissues and organs within areas, separate between them, achieving cohesion that allows the different components of the body are not stacked on each other without any form and are the means through which the circulating vessels and nerves .

There are various types of connective tissue, some specialized, such as bone or cartilage, and other less specialized, such as adipose tissue or serous lining the muscles, organs and joints. If a connective tissue disorder such that this occurs in excess and therefore constrict vessels, organs, skin and various tissues, they undergo fibrosis, which alters both its shape and functionality. When this occurs in some particular tissues and vascular disorders, immunological and collagen is said that the patient has systemic sclerosis.

How to produce

Systemic sclerosis is caused by alterations in the vessels, which clog and become more fragile, to the fibroblasts, which produce collagen in excess and will form plaques which affect the organs and tissues that support and immunological level therefore have detected antibodies that attack body tissues that do not recognize as their own, as the antibodies ANA, Scl-70 antibodies called or anticentromere antibodies, these antibodies is that systemic sclerosis is considered an autoimmune disease.

Scleroderma Systemic Sclerosis Systemic sclerosis Symptoms, Diagnosis and Treatment

In systemic sclerosis are two different ways:

  • Diffuse systemic sclerosis, with a rapidly evolving cutaneous involvement that starts distally and progresses to more proximal areas. It is associated with gastrointestinal, lung, kidney, heart and joints.
  • Limited systemic sclerosis, slowly progressive and exclusive involvement of fingers and backs of hands and forearms. Associated with Raynaud syndrome and with the passage of time also occurs pulmonary and gastrointestinal. Occasionally appears in the context of so-called CREST syndrome.

We can say that there is a form of sclerosis whose involvement is merely skin, called localized scleroderma. Its mechanism of action is similar but only affects the skin, subcutaneous tissue, muscle and sometimes the periosteum. It usually appears in early childhood and rarely affects other organs.

Systemic sclerosis Symptoms

This disease affects many organ systems, hence the name of systemic sclerosis. For devices, the different manifestations of this condition include:

  •  Skin
    • Distal fingers sclerosis
    • edematous fingers
    • Raynaud syndrome
    • loss of skin folds, which limits the mobility and reduced facial expression characteristic of these patients
    • ulcerations on the fingertips, conventionally called scars “rat bite”
    • pigmentation disorders
    • telangiectasias and soft tissue calcifications
  • Musculoskeletal
    • joint pain and swelling from inflammation of the synovial
    • tendon and muscle involvement
  • Digestive
    • altered mobility of the esophagus and lower sphincter, which leads gastroesophageal reflux
    • slowing of gastric and duodenal motility
    • intestinal malabsorption due to fibrosis
    • at least one fifth of the patients is associated with biliary cirrhosis
  •  Lung
    • interstitial disease, especially in the lower part of the lung, with progressive respiratory difficulty is the leading cause of death in these patients
    • pleural inflammation with possible spill
    • Accordingly alveolar fibrosis with pulmonary hypertension
  •  Heart
    • pericarditis and fibrosis of the myocardium, thereby hinders the filling and emptying of the heart
    •  Hypertension
    •  arrhythmias and conduction disturbances
  •  Kidney
    •  renal failure, one of the major causes of death in these patients
    •  malignant hypertension
  • Neurological
    • polyneuropathies
    • trigeminal neuralgia
    • paresthesias

Sometimes systemic sclerosis is associated with other diseases, such as Sjogren’s syndrome, Hashimoto’s thyroiditis and primary biliary cirrhosis.

 

Systemic sclerosis Diagnosis

The diagnosis of systemic sclerosis is based predominantly on the patient’s chart.

The blood tests will be appreciated an elevated erythrocyte sedimentation rate (ESR). It will look specifically antibodies that can be elevated in this disease, as Scl-70, anticentromere or ANA.

Capillaroscopy is a technique that allows to assess the involvement of the capillaries of the nail bed, which in the case of systemic sclerosis show a characteristic pattern of dilated capillary loops with or without loss and destruction thereof depending on the type of damage , diffuse or limited respectively.

With all this, we can say that the existence of scleroderma proximally in the fingers is enough to diagnose systemic sclerosis. There are also some minor criteria, which are lower bilateral pulmonary fibrosis, sclerodactyly or the presence of scars “rat bite” on the fingers, if two of these three criteria can also be given the diagnosis of systemic sclerosis.

Systemic sclerosis Treatment

Treatment of systemic sclerosis is based largely on treating symptoms, drugs that may inhibit collagen synthesis, to avoid external factors that may aggravate symptoms, such as cold or snuff, and enhance physical exercise moderate.

Drugs such as penicillamine, colchicine or interferon gamma can inhibit collagen synthesis and slowing the progression of the disease. To prevent Raynaud’s clinic should avoid exposure to cold and can be treated with vasodilators prostaglandins or calcium antagonists.

Corticosteroids are administered to treat synovitis, edema and alveolar inflammation. In case of pulmonary fibrosis will be treated with corticosteroids and cyclophosphamide.

Esophagitis and gastroesophageal reflux are treated with dietary measures and drugs antacids.

Hypertension and renal involvement, we will try to control with antihypertensive treatment IECA.El type of systemic sclerosis is based largely on treating symptoms, drugs that may inhibit collagen synthesis, to avoid external factors may aggravate symptoms, such as cold or snuff, and promote moderate physical exercise.

Precautions

Systemic sclerosis is an autoimmune disease, in which there are no preventive measures to avoid it.

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