What is Disease Scleroderma (Systemic Sclerosis)

Kolagen Sekleroderma a disease that can cause organ atrophy and sclerosis. Skin, gastrointestinal tract, especially esophageal, lung, no other muscle, bone, heart, kidney and other organs are systemic spread. Middle age is more common in women than in men.

Scleroderma Systemic Lupus Erythematosus disease, there are aspects of rheumatoid lung and similar diseases such as Sjogren's syndrome. A rare disease, the incidence is less than 3/100.000. Associated with the immune disorder of unknown cause. Histopathology 90% of cases there are changes in lung fibrosis. However, 25% of all cases and 15% of clinical symptoms, radiographic abnormalities are observed.

Pathology

In the early period are the connective tissue edema and cell infiltration. Develop the disease progresses, a fibrous reaction. Consists of more advanced periods of atrophy. Widespread interstitial fibrosis in the lungs, alveoli, thickening of the walls of the artery and vein is husule. Parenkiması further periods of lung connective tissue is infested with, narrowing and blockage of blood vessels develops. These changes followed the same as in idiopathic pulmonary interstitial fibrosis. Skleroderma'nın morphological abnormalities in other organs, causing the separation of idiopathic interstitial allows fibrosis'den.

Clinical symptoms of Scleroderma Lung

Clinical diagnosis of extrapulmonary organs, especially the skin is determined anormallirlerle. Thickened skin, the flexibility is lost, there is a view, especially the face and extremities, wax, bronze skin color would be like so many times. Most patients have Raynaud's syndrome, many times before skin lesions are seen. Raynaud's syndrome, cold, excitement, and factors such as smoking spasmıdır small vessels, especially the fingers. Raynaud's syndrome related to these factors, the fingers iskemiyle syanotik, consists of a purple appearance. Factors improves the color of the fingers disappeared.

Esophageal dilatation and dysphagia and dyspepsia due to a decrease of peristaltic movements occur. Lesions in the duodenum and the columns developed ileus, diverticulitis, constipation or diarrhea can cause such symptoms.

Scleroderma lung disease symptoms are not specific, as well as other organs. A mild cough, sputum, and there is a progressive dyspnea. In some cases, chest radiographs show a change in certain patients, but started to dyspnea.

Radiological findings

Period of the disease is about to change according to radiographic interstitial fibrosis, reticular or reticulonodular changes are seen. İdyo-booties interstisiel fibrosis as fine reticular pattern early in the disease has been localized in the lower lobes, upper lobes are normal. Next in the reticular and fibrous infiltration becomes more prevalent and increasing lung volume is decreased. Between reticular and nodular infiltrates and cystic fibrosis husule a change in income. Cysts are more localized to the lung periphery and the diameter is usually smaller than 1 part. Other kolagen pleuritis and pleural diseases, scleroderma, although rarely observed liquid.

Esophageal dilatation and swallowing difficulties as a result of peristaltic movements, reduction of income husule. In some cases, radiographic findings can be seen in the dilated esophagus with air contrast (air özofagogram'ı). Taken in the expansion of esophageal baryumla opera is better.
Phalanx joints such as rheumatoid artritis'deki 25% of the cases observed changes.

Lung function tests and laboratory findings

Function abnormalities, and especially the reduction in diffusion capacity has Skleroderma'da restrictive. Compliance is reduced. First, exertion, hypoxemia develops disease istirahatda proceed. In some cases, radiographic findings of pulmonary function abnormalities, especially before the observed decrease in diffusion.
In some cases, LE cells, latex test is positive and in some cases. Protein electrophoresis globülin'in gamma increases.

Scleroderma Treatment

Corticosteroid in the treatment of scleroderma lung disease have been some successful results. The dose and duration of the disease are the same as other kolagen. Salicylate joint symptoms, Raynaud's, such as reserpine vascular events has resulted in some cases.