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Respiratory symptoms started while he received high\u2010dose prednisone for persistent proteinuria. Bilateral, diffuse ground\u2010glass infiltrates corresponded to the presence of inflammatory cells in the bronchioalveolar lavage fluid. Empiric antibiotic treatment including clarithromycin was given, but the microbiological work\u2010up remained negative. Serum IgE, C3, and C4 concentrations were normal. He recovered within 3 weeks after onset.<\/jats:p><jats:p>We systematically reviewed 23 reports describing 30 additional cases of rituximab\u2010associated lung disease. Twenty eight patients had received rituximab for B\u2010cell malignancies, one for graft\u2010versus\u2010host disease and one for immune thrombocytopenia. Median age was 64 years (interquartile range [IQR] 58\u201369 years). Seventy one percent received concomitant chemotherapy. Time to onset from the last rituximab dose was 14 days (IQR 11\u201322 days). Eleven of 31 patients required mechanical ventilation, and 9 died (29%). Ventilation was a significant predictor of fatal outcome (odds ratio 46.7; confidence interval 9.5\u2013229.9). High dose glucocorticoid therapy did not improve survival or prevent severe lung disease or death.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>With the expanding use of rituximab for novel indications, additional cases of RALI affecting younger age groups are expected to emerge. Mechanical ventilation predicts poor outcome. Glucocorticoids may not be protective. 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