INTERSTITIAL LUNG DISEASE (ILD) IN SYSTEMIC SCLEROSIS (SSc)

Authors:

Srđan Novak

Summary

Intersitial lung disease is a frequent complication of systemic sclerosis that often has a poor pognosis and together with pulmonary arterial hypertension are the most common cause of death in scleroderma patients. For detection and evaluation of interstitial lung disease, high-resolution CT and pulmorary functional tests are pivotal. The decision about whether to start treatments often the most difficult challenge. Patients with short duration of systemic disease with recent deterioration in DCO are the candidates for immunosupressive therapy. Best current initial treatment is intravenous monthly cyclophosphamide together with low-dose oral glucocorticoids although azathioprine and mycophenolate mofetil are also widelly used.

Sažetak
Intersticijska bolest pluća je česta komplikacija sistemske skleroze koja nerijetko ima lošu prognozu te je zajedno s plućnom arterijskom hipertenzijom najčešći uzrok smrti u ovoj bolesti. U otkrivanju i praćenju iste, najvažnije metode su CT visoke rezolucije te funkcionalni testovi plućne funkcije. Odluka o započinjanju liječenja često je vrlo teška. Kandidati za imunosupresivno liječenje su obično bolesnici u kojih sistemska bolest kratko traje i u kojih dolazi do naglog pada DCO. Kao lijek izbora najčešće se spominje cikolofosfamid primjenjen intavavenski u mjesečnim intervalima zajedno s malim dozama glukokortikoida iako se u liječenju primjenjuju i drugi imunosupresivi, najčešće azatioprin ili mikofenolat mofetil.

Vol.: 57

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