No abstract

Grading the strength of recommendations and the quality of underlying evidence enhances the usefulness of clinical practice guidelines. Professional societies and other organizations, including the American Thoracic Society (ATS), should reach consensus about whether they will use one common grading system and which of the numerous grading systems they would apply across all guidelines. The profusion of guideline grading systems confuses consumers of guidelines, and undermines the value of the grading exercise in conveying a transparent message. In response to this dilemma, the international GRADE working group has developed an approach that is useful for many guideline contexts, and that several national and international organizations have adopted. The GRADE system classifies recommendations as strong or weak, according to the balance of the benefits and downsides (harms, burden, and cost) after considering the quality of evidence. The quality of evidence reflects the confidence in estimates of the true effects of an intervention, and the system classifies quality of evidence as high, moderate, low, or very low according to factors that include the study methodology, the consistency and precision of the results, and the directness of the evidence. On recommendation of the ATS Documents Development and Implementation Committee, the ATS adopted the GRADE approach for its guidelines in line with many other organizations that have recently chosen the GRADE approach. This article informs ATS guideline developers, investigators, and those interpreting future ATS guidelines that follow the GRADE approach about the methodology and applicability of ATS guidelines and recommendations.

Background-Approximately 60 million people in the United States live with one of four chronic conditions: heart disease, diabetes, chronic respiratory disease, and major depression. Anxiety and depression are very common comorbidities in COPD and have significant impact on patients, their families, society, and the course of the disease.

Rationale: Pulmonary lymphangioleiomyomatosis is a progressive cystic lung disease that is associated with infiltration of atypical smooth muscle-like cells. Previous descriptions of clinical characteristics of subjects with lymphangioleiomyomatosis have been based on a limited number of patients. Objectives: To describe the clinical characteristics of subjects with pulmonary lymphangioleiomyomatosis, both sporadic and tuberous sclerosis-related forms. Methods: Over a 3-yr period, from 1998 to 2001, 243 subjects with pulmonary lymphangioleiomyomatosis were enrolled into a national registry; 13 subjects who had already undergone lung transplantation were excluded for the purposes of this report. Measurements and Main Results: All 230 subjects were women, aged 18 to 76 yr (mean Ϯ SE, 44.5 Ϯ 0.65 yr). The average age at onset of symptoms was 38.9 Ϯ 0.73 yr and at diagnosis was 41.0 Ϯ 0.65 yr. Tuberous sclerosis complex was present in 14.8% of subjects. Pulmonary manifestations, most commonly spontaneous pneumothorax, were the primary events leading to the diagnosis in 86.5% of cases. Nearly 55% of the subjects were being treated with a progesterone derivative. An obstructive pattern on pulmonary function testing was observed in 57.3% of the subjects, whereas 33.9% had normal spirometric results. Women with tuberous sclerosis-related lymphangioleiomyomatosis were younger and had less impaired lung function compared with those with the sporadic form. Conclusions: The age range of women afflicted with pulmonary lymphangioleiomyomatosis is broader than previously appreciated and the degree of pulmonary function can be quite variable, with one-third of subjects having normal spirometry at enrollment into this registry.