NEW YORK (Reuters Health) – The rare interstitial lung disease, pulmonary Langerhans'-cell histiocytosis, in adults is associated with decreased survival. A substantial number of deaths are attributable to respiratory failure, according to a report published in the February 14th issue of The New England Journal of Medicine.
Dr. Robert Vassallo and colleagues, from the Mayo Clinic and Foundation in Rochester, Minnesota, reviewed the records of 102 adults with pulmonary Langerhans'-cell histiocytosis. Among surviving patients, health status was determined using the 36-Item Short-Form General Health Survey.
The patients were followed for a median of 4 years. Thirty-three deaths occurred, 15 of which were due to respiratory failure, the authors note. The median survival period was 12.5 years, significantly shorter than expected among people of the same sex and year of birth in the general population (p < 0.001). Lower carbon monoxide diffusing capacity, lower FEV1, higher residual volume, and former smoking status were predictive of shorter survival.
Fourteen patients developed noncutaneous malignant neoplasms, the researchers report. Six patients developed hematologic cancers, a much higher frequency than would be expected in the general population.
Also, compared with matched controls, patients had lower scores for physical functioning, ability to perform role-related activities, general health, vitality, and overall physical well being.
The findings indicate that "among adults with pulmonary Langerhans'-cell histiocytosis, long-term survival is shorter than in the general population, health is substantially affected, and death is frequently due to respiratory complications," the authors conclude. Furthermore, the findings suggest that the disease is a risk factor for hematologic neoplasms.
N Engl J Med 2002;346:484-490.
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