Achados histológicos e sobrevida na fibrose pulmonar idiopática

Abstract

Background: Idiopathic pulmonary fibrosis was recently redefined as usual interstitial pneumonia of unknown etiology. Consequently, the prognostic value of histological findings needs to be reassessed. Objective: To correlate clinical, functional and histological findings with survival in patients with idiopathic pulmonary fibrosis. Method: Patients (n = 51; mean age: 66 ± 8 years; gender: 21 females/30 males) were evaluated. Of the 51, 26 were smokers or ex-smokers. Duration of symptoms, forced vital capacity and smoking habits were recorded. All patients presented usual interstitial pneumonia verified through histology. Degree of honeycombing, established fibrosis, desquamation, cellularity, myointimal thickening of blood vessels and number of fibroblastic foci were graded according to the semiquantitative method. Results: Median duration of symptoms was 12 months and initial forced vital capacity was 72 ± 21%. Cox multivariate analysis revealed that survival correlated inversely and significantly (p < 0.05) with duration of symptoms and fibroblastic foci score, as well as with myointimal thickening of blood vessels. Limited numbers of fibroblastic foci, as well as myointimal thickening involving less than 50% of blood vessels, were predictive of greater survival. No correlation with survival was found for gender, age, forced vital capacity, inflammation or degree of cellularity. Conclusion: Semiquantitative analysis of lung biopsies yields relevant prognostic information regarding patients with usual interstitial pneumonia.

Análise semiquantitativa de biópsias cirúrgicas de diferentes lobos pulmonares de pacientes com pneumonia intersticial usual/fibrose pulmonar idiopática

“…(2,27,28) In this study, this finding was significantly more common in the left lower lobes. However, one group of authors, having found no such association, questioned the prognostic value of fibroblastic foci in isolation.…”

Modelo experimental de doença pulmonar intersticial fibrosante associado à terapia celular utilizando células mononucleares de medula óssea

“…Os achados característicos são áreas microscópicas de pulmão normal alternadas com lesões em graus variáveis (GROSS et al, 2001;COLETTA et al, 2003;COTTIN et al, 2004).…”

“…O infiltrado celular consiste de macrófagos, linfócitos e células plasmáticas, com o agravamento da doença, são maiores a proliferação fibroblástica e o envolvimento pulmonar por cicatrização colagênica (GROSS et al, 2001;KING et al, 2001;COLETTA et al, 2003;SCOTT et al, 2004).…”

“…Doenças pulmonares intersticiais afetam pacientes em torno da quinta década de vida, mas pode ocorrer em qualquer idade. Após a confirmação diagnóstica a sobrevida média é de 3 anos (Fibrose Pulmonar Idiopática) e a morte acontece geralmente por falência respiratória irreversível (GROSS et al, 2001;COLETTA et al, 2003;COLLARD et al, 2003;COTTIN et al, 2004;SCOTT et al, 2004).…”

“…As possibilidades trazidas pela utilização do transplante autólogo de células-tronco podem responder à necessidade de se encontrar melhores alternativas para o tratamento de doenças Intersticiais, constituindo atualmente chance promissora de tratamento, visando melhora da qualidade de vida e aumento da sobrevida dos pacientes afetados. (COTRAN et al, 2000;GROSS et al, 2001;COLLARD et al, 2002;COLETTA et al, 2003).…”