Background Interstitial lung diseases (ILDs) may occur secondary to connective tissue diseases (CTDs) and increase morbidity and mortality due to ventilation impairment.
Objectives To reveal clinical, laboratory and imaging features of CTD -ILDs and to analyze treatment approaches.
Methods One hundred thirty two consecutive patients were included in this cohort. Demographic characteristics, laboratory and HRCT results and treatment results were analyzed. More than 10% increase of pulmonary function tests were categorized as improvement, >10% decrease were categorized as deterioration and ±10% changes were categorized as stable disease.
Results There were 111 ILD patients (86.5% CTD-ILD) with follow-up time for more than 6 months. Median follow up time was 48 (Min-Max:6–260) months and mean age was 54.5±11.9 years. HRCT scan was repeated in median 3 (1–10) times per patients, with median 12.5 month intervals. The most frequent HRCT findings were ground-glass opacities, interlobular septal thickening and honeycomb patterns. 89.6% of CTD-ILD patients recieved corticosteroids and 44.8% recieved hydroxylchloroquine sulphate. Azathioprine and cyclophosphamide were the most common used immunosupresive drugs. Eighty four patients recieved at least one immunosuppressive agent. There were only 2 patients treated with four different immunosuppressive drugs. After treatment, mean pulmonary function tests did not significanty differ from baseline (p>0.05) (Table). 35% of the patients improved forced vital capacity(FVC >%10 increase) by treatment whereas 31% had decreased and 34% were stable.
Conclusions It is not detected to have significant improvement by current immunosuppressive drugs in patients with CTD-ILDs. There is need more effective novel drugs to treat for CTD-ILD.
Disclosure of Interest None declared
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