The role of nutritional status in the prognosis of subjects with severe chronic obstructive pulmonary disease was studied in a cohort of Canadian men and women followed for 3 to 5 yr. A total of 348 subjects who were recruited for a study of negative pressure ventilation were evaluated for lung function and body weight, and a subset who entered hospital for the study (n=184) had baseline measures of diffusing capacity, maximal inspiratory and expiratory mouth pressure (PImax and PEmax), and blood gases. Predictors of survival were analyzed using Cox regression models. In the total cohort, low body mass index (BMI) and use of home oxygen were independently associated with reduced survival. In the hospitalized group, predictors of respiratory mortality were elevated PaCO2 and low BMI, PImax, and diffusing capacity. PaO2 (measured on oxygen therapy), FEV1, PEmax, age, smoking behavior, and gender were not associated with survival. The predictors of total mortality were similar, except that BMI was no longer significant. In conclusion, low body weight, a potentially modifiable factor, was associated with respiratory mortality, but whether it has a casual effect or is a marker of declining health can only be resolved through an intervention trial.