A variety of interstitial Lung Diseases (ILD) have been described in patients with myelodysplastic syndromes (MDS) with possible etiologies including autoimmunity, drug related toxicity, and recurrent infections. A comprehensive study of ILD in MDS patients has not been previously performed. Out of 827 consecutive biopsy proven MDS patients seen at our institution from June 1970-May 2010, 18 (2%) were found to have ILD. There was no statistical significance in baseline characteristics between patients with ILD (ILD +) vs those without ILD (ILD-). Cytogenetic studies were reported in 14 ILD+patients out of whom 43% had 5q- abnormalities (21% isolated and 22% part of complex karyotype). Prevalence of high risk MDS was similar between both groups (22% vs 29% in ILD-) with similar overall survival. ILD was diagnosed prior to MDS in the majority of cases (72%) with a median time to MDS diagnosis of 22.3 months. Our study suggests that ILD are present in a higher percentage than anticipated in the MDS population. Deletion 5q was frequent in ILD+ cases and this requires further study. Prior MDS treatment and autoimmunity seemed to play no significant role in ILD development.
Keywords: Azacitidine; Hypomethylating agents; Interstitial lung disease; Lenalidomide; Myelodysplastic syndrome.
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