Interstitial lung disease in scleroderma
نویسنده
چکیده
Pulmonary involvement is quite frequent in the development of progressive systemic sclerosis (PSS), a disease that affects the skin and internal organs and may present in various forms, especially as interstitial lung disease (ILD) or pulmonary hypertension (PH).(1) The incidence of pulmonary involvement varies depending on the population and on the methodology used for the diagnosis, reaching up to 70% for ILD and 50% for PH in autopsy studies.(2) The presence of pulmonary disease is a determining factor for the quality of life, morbidity and mortality.(3-4) In the most comprehensive study found in the literature, comprising more than 1000 patients, pulmonary involvement was responsible for 33% of deaths.(5) In the past few years, this theme has been extensively studied from various aspects. However, a number of doubts persist. A prospective multicenter study is currently being conducted in the United States.(4) Interstitial disease is more often accompanied by the diffuse cutaneous form of PSS, whereas PH is more common in the limited cutaneous form, but both diseases may occasionally be associated with both forms. Proper management of ILD accompanied by PSS is still uncertain. The fact that the disease is rare contributes to this problem since few centers have a relevant sample under long-term follow-up treatment. In addition, evolution is slow and the disease may remain stable for long periods. Furthermore, the presence of PH or involvement of other organs may cause confusion in the diagnosis.(1-3) Factors that may signal a worse prognosis for ILD include presence of positive antitopoisomerase (anti-Scl-70) autoantibodies, diffuse cutaneous involvement, being AfricanAmerican, severe forms of the disease (revealed by computed tomography and pulmonary function tests) and rapid evolution to systemic disease (< 5 years), as well as functional and radiological worsening during follow-up.(6) A routine for the diagnostic evaluation of ILD should therefore be followed, especially in cases presenting the disseminated cutaneous form and Editorial
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