BACKGROUND: @entity2416 ( @entity2416 ) is characterized by the insidious onset of @entity1353 or cough. However, a subset of @entity1 has a short duration of symptoms with rapid progression to end-stage disease. In this study, we evaluated clinical and molecular features of "rapid" and "slow" progressors with @entity2416 . METHODS AND FINDINGS: 26 @entity1 with <6 months of symptoms before first presentation [rapid progressors] and 88 @entity1 with >24 months of symptoms [slow progressors] were studied. Survival was analyzed by the Kaplan-Meyer method and proportional hazard's model. Lung microarrays and tissue proteins were measured in a subset of @entity1 . No differences were found in age, physiologic impairment and bronchoalveolar lavage (BAL) cellular profile. There were more males (OR = 6.5; CI:1.4-29.5; p = 0.006) and smokers (OR = 3.04; CI:1.1-8.3; p = 0.04) in the rapid progressors group. Survival from the beginning of symptoms was significantly reduced in rapid progressors (HR = 9.0; CI:4.48-18.3; p<0.0001) and there was a tendency for decreased survival from the time of diagnosis (HR = 1.5; CI:0.81-2.87; p = 0.18). We identified 437 differentially expressed genes. Lungs of rapid progressors overexpressed genes involved in morphogenesis, oxidative stress, migration/proliferation, and genes from fibroblasts/smooth muscle cells. Upregulation of two of these genes, @entity859 -2B receptor and @entity5439 / @entity5439 , was validated by immunohistochemistry and were expressed by alveolar epithelial cells. BAL from rapid progressors showed a >2-fold increase of active @entity1495 , and induced a higher fibroblast migration compared with slow progressors and controls [238+/-98% versus 123+/-29% (p<0.05) and 30+/-17% (p<0.01)]. CONCLUSIONS/SIGNIFICANCE: A subgroup of @entity2416 @entity1 , predominantly smoking males, display an accelerated clinical course and have a gene expression pattern that is different from those with slower progression and longer survival. These findings highlight the variability in the progression of @entity2416 , and may explain, in part, the difficulty in obtaining significant and reproducible results in studies of therapeutic interventions in @entity1 with @entity2416 .

Accelerated variant of XXXX : clinical behavior and gene expression pattern.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity859 :: ('MESH:D000241', 'Chemical') :: ['adenosine'] @entity5439 :: ('8842', 'Gene') :: ['CD133', 'prominin-1'] @entity1353 :: ('MESH:D004417', 'Disease') :: ['dyspnea'] @entity2416 :: ('MESH:D054990', 'Disease') :: ['Idiopathic pulmonary fibrosis', 'IPF'] @entity1495 :: ('4318', 'Gene') :: ['matrix metalloproteinase-9']
@entity2416 :: (MESH:D054990,Disease) :: ['idiopathic pulmonary fibrosis']
