FAM13A gene polymorphism showed a significant association with the susceptibility to IPF, with severity of lung function impairment and with poor prognosis.

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a major cause of morbidity and death in IPF. However, sensitive predictive factors of AE-IPF have not been well-investigated. To investigate whether high-resolution computed tomographic (HRCT) abnormalities predict AE-IPF in independent ethnic cohorts, this study included 121 patients with IPF (54 German and 67 Japanese; mean age, 68.5 ± 7.6 years). Two radiologists independently visually assessed the presence and extent of lung abnormalities in each patient. Twenty-two (18.2%) patients experienced AE-IPF during the follow-up. The incidence of AE-IPF was significantly higher in the Japanese patients (n = 18, 26.9%) than in the German patients (n = 4, 7.3%, p < 0.01). In the Kaplan–Meier analysis, patients with a larger extent of ground glass opacity (GGO), fibrosis, and traction bronchiectasis experienced an earlier onset of AE-IPF (p = 0.0033, 0.0088, and 0.049, respectively). In the multivariate analysis, a larger extent of GGO and fibrosis on HRCT were independent predictors of AE-IPF (p = 0.026 and 0.037, respectively). Additionally, Japanese ethnicity was independently associated with the incidence of AE-IPF after adjustment for HRCT findings (p = 0.0074). In conclusion, a larger extent of GGO and fibrosis on HRCT and Japanese ethnicity appear to be risk factors for AE-IPF.

The GGA area on HRCT is a risk factor for chemotherapy-related AE-ILD. Therefore, this parameter can be used to predict the risk of AE-ILD before administering chemotherapy.

BackgroundMucin 1 (MUC1) contributes to the growth and metastasis of various cancers, including lung cancer, and MUC1 gene length polymorphisms are associated with susceptibility to lung cancer and its prognosis. In contrast, the association between rs4072037, a single nucleotide polymorphism in MUC1, and lung cancer has not been well studied.MethodsIn the present study, we determined the rs4072037 genotype and measured serum KL-6 levels to evaluate the association between lung adenocarcinoma (ADC) and rs4072037 or serum KL-6 levels. DNA samples were available for 172 patients and these were included in the genomic analyses. In addition, 304 patients were included in the serum analyses. Furthermore, 276 healthy volunteers were included in both genomic and serum analyses.ResultsThe rs4072037 genotype was not associated with susceptibility to lung ADC or its prognosis. Interestingly, serum KL-6 levels significantly differed according to rs4072037 genotype in those with T1 or T2 (P < 0.001), N0 or N1 (P = 0.002) and M0 (P < 0.001), but not in those with T3 or T4 (P = 0.882), N2 or N3 (P = 0.616) and M1a or M1b (P = 0.501). Serum KL-6 levels were significantly associated with the presence of lung ADC, as well as with its progression and prognosis, indicating the crucial involvement of KL-6/MUC1 in the development of lung cancer and its progression.ConclusionBased on these findings, we conclude that rs4072037 does not have a significant impact on the pathogenesis or prognosis of lung ADC, whereas serum KL-6 levels, which might reflecting the molecular length of MUC1, are significantly associated with lung ADC.Electronic supplementary materialThe online version of this article (doi:10.1186/s12885-017-3272-y) contains supplementary material, which is available to authorized users.