결체조직질환을 동반한 간질성 폐렴
Connective Tissue Disease-Associated Interstitial Lung Disease
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초록
결체조직질환에 동반된 역류성 식도염이나 폐동맥고혈압이 환자의 증상 및 심폐기능에 영향을 주므로 이에 대한 치료도 반드시 이루어져야 한다. 결론적으로 결체조직질환에 의한 간질성 폐렴은 특발성 간질성 폐렴과 임상적, 영상의학적 및 병리학적으로 매우 유사하지만 특발성 간질성 폐렴보다 예후가 좋으므로 감별이 꼭 필요하고, 간질성 폐렴은 기회감염성 폐렴, 약제 유발성 간질성 폐렴과 구분하여 치료 방향을 결정하는 것이 매우 중요하며, 임상적으로 의미 있는 호흡기 증상과 폐기능 저하가 있거나 진행성 간질성 폐렴 환자들에게만 약물 치료를 시작해야 하는데 주로 스테로이드 및 cyclophosphamide, azathioprine과 같이 부작용이 많은 약물이 투여되므로 그 효과 및 부작용을 잘 관찰하면서 치료를 시행해야 한다.
Abstract
Interstitial lung disease (ILD) is the most common pulmonary manifestation of connective tissue disease (CTD). It may develop in the patients with preexisting connective tissue diseases or may be the initial lone manifestation of an underlying CTD. Since ILD causes a potentially substantial morbidity and mortality in the patients with CTD, close collaboration among pulmonologists, rheumatologists, radiologists and pathologists is very essential to optimize the diagnostic evaluation and treatment. Important information on the pattern and extent of the ILD can be obtained by chest HRCT imaging. In general, surgical lung biopsy is not recommended for CTD-associated ILD. All patients with CTD-associated ILD do not require pharmacologic treatment. The decision to treat CTD-associated ILD often depends on 1) whether the patient is clinically impaired by the ILD, 2) whether the ILD is progressive, and 3) what contraindications exist. Differential diagnosis from superimposed pneumonia and drug-induced ILD is quite important. Immunosuppressive therapy is the mainstay of treatment. Recent success of cyclophosphamide in the clinical trials of systemic sclerosis-associated ILD is promising.
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