RESUMO
In sporadic idiopathic pulmonary fibrosis (sIPF) and pulmonary fibrosis caused by a mutation in telomere (TRG-PF) or surfactant related genes (SRG-PF), there are a number of aberrant cellular processes known that can lead to fibrogenesis. We investigated whether RNA expression of genes involved in these processes differed between sIPF, TRG-PF, and SRG-PF and whether expression levels were associated with survival. RNA expression of 28 genes was measured in lung biopsies of 26 sIPF, 17 TRG-PF, and 6 SRG-PF patients. Significant differences in RNA expression of TGFBR2 (p = 0.02) and SFTPA2 (p = 0.02) were found between sIPF, TRG-PF, and SRG-PF. Patients with low (Assuntos
Fibrose Pulmonar Idiopática
, RNA
, Humanos
, RNA/genética
, Inclusão em Parafina
, Pulmão/patologia
, Fibrose Pulmonar Idiopática/metabolismo
, Chaperona BiP do Retículo Endoplasmático
, Formaldeído