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Abstract: Introduction: The prognosis of patients with an acute exacerbation of interstitial pneumonia (AE‐IP) is poor. Pirfenidone (PFD) reduces the disease progression in idiopathic pulmonary fibrosis. Objectives: The purpose of this study was evaluating whether the administration of PFD improved the outcomes of AE‐IP. Methods: We conducted a retrospective study of 31 patients with AE‐IP who did not recover between 7 and 14 days after an initial treatment. Fourteen patients received PFD within 2 weeks (PFD group) of the AE, while 17 patients were treated without PFD (non‐PFD group). The patients’ clinical data and computed tomography (CT) scores were analyzed. Results: The survival rate in the PFD group was not significantly different from non‐PFD group at 30 (78.6% vs 64.7%, P = .46) and 90 days (64.3% vs 52.9%, P = .72). The white blood cell counts in the PFD group were significantly lower on PFD day 14 than on PFD days 1 and 7. The C‐reactive protein levels in the PFD group were also significantly lower on PFD day 7 than on PFD day 1. There were no significant differences regarding the changes of the CT scores. Conclusions: PFD may reduce the inflammation in AE‐IP patients undergoing corticosteroid treatment. [ABSTRACT FROM AUTHOR]

Background: Pirfenidone is one of the anti-fibrotic drugs used for patients with idiopathic pulmonary fibrosis. Pirfenidone exerts anti-inflammatory effects by inhibiting the influx of inflammatory cells. Objectives: The purpose of this study was to clarify the differences in the baseline parameters in responsive and unresponsive patients, and to assess the clinical and radiological changes after pirfenidone therapy. Methods: Patients with idiopathic pulmonary fibrosis who were treated with pirfenidone from April 2009 to March 2014 were retrospectively analyzed. The enrolled patients were classified into a good response group if they showed inhibition of progression, or were classified into a slowly progressive group on the basis of a decline in the vital capacity over a six-month interval after beginning treatment. The parameters of pulmonary function tests and laboratory findings were compared before and after treatment. The chest computed tomography findings were evaluated using the Sumikawa score. Results: Twenty patients were classified into seven good responders and eight cases with inhibition of progression. These groups had higher antinuclear antibody and autoimmune antibody values, and less ground glass attenuation at baseline. A chest computed tomography assessment at six-months after beginning pirfenidone administration showed a reduction of the ground glass attenuation findings in the good response group and an increase in airspace consolidation in the slowly progressing group compared with the baseline. Conclusions: Higher positive values for antinuclear antibodies and autoimmune antibodies at baseline and the location of ground glass attenuation at baseline, which indicates inflammatory lesions, may predict the efficacy of pirfenidone. (Sarcoidosis Vasc Diffuse Lung Dis 2017; 34: 290-299) ., (Copyright: © 2017.)

Purpose: Acute exacerbation (AE) is an important outcome of idiopathic pulmonary fibrosis (IPF) and nonspecific interstitial pneumonia (NSIP). Recombinant human soluble thrombomodulin (rhTM) is a new drug for the treatment of disseminated intravascular coagulation in Japan. The objective of this study was to evaluate the efficacy of rhTM for AE of IPF/NSIP., Methods: Twenty-two patients with AE-idiopathic interstitial pneumonia (16 patients with IPF and six patients with NSIP) were enrolled in our study. Among them, eleven patients were treated with rhTM (rhTM group), and eleven patients were treated without rhTM (non-rhTM group). Patients admitted to our hospital prior to December 2013 were treated with rhTM, while those admitted after January 2014 were treated without rhTM. The primary endpoint was mortality at 90 days after AE treatment. The secondary endpoint was the safety of rhTM for AE-IPF/AE-NSIP. In addition, we examined prognostic factors of AE-IPF/AE-NSIP., Results: The mortality rate was significantly lower in the rhTM group than in the non-rhTM group (mortality rate at 90 days: 36% vs 90%, P=0.023; median survival time: not reached vs 15.0 days, P=0.019). A univariate analysis revealed the respiratory rate (hazard ratio [HR] 1.09, 95% confidence interval [CI] 1.00-1.18, P=0.039) and rhTM administration (HR 0.21, 95% CI 0.06-0.77, P=0.013) as predictors of mortality at 90 days, and a multivariate analysis identified rhTM administration (HR 0.025, 95% CI 0.0006-0.94, P=0.046) as an independent predictor of mortality at 90 days. No serious adverse events were observed., Conclusion: The administration of rhTM is associated with reductions in mortality in patients with AE-IPF/NSIP, without causing adverse events.

Background: Limitations in airflow are detected in some patients with sarcoidosis in association with a poor prognosis. The impulse oscillation system (IOS) is used to treat patients with obstructive lung disease, as it can sensitively detect increased airway resistance., Objectives: To investigate the characteristics of parameters obtained with IOS in patients with sarcoidosis., Methods: Forty-six pulmonary sarcoidosis patients at Chiba University Hospital and 20 healthy controls were enrolled. The subjects underwent IOS, pulmonary function testing and multidetector computed tomography. We evaluated the correlations between these indices in the pulmonary sarcoidosis patients and compared the pulmonary sarcoidosis patients with the healthy controls., Results: The ratio of V50/V25, percentage of wall area (WA%), resistance at 5 Hz (R5) and difference between the R5 and R20 (R5-R20) values of the patients with pulmonary sarcoidosis were significantly increased compared to those observed in the controls. Inverse weak correlations were observed between the R5-R20 values and the forced expiratory volume in one second (r = -0.56; p <0.001). The R5-R20 values were correlated with the V50/V25 (r = 0.42; p < 0.005) and WA% (r = 0.43; p < 0.05) values. The WA% values were also significantly correlated with the V50/V25 (r = 0.32; p < 0.05) and R5 (r = 0.33; p < 0.05) values., Conclusions: IOS parameters were found to be significantly correlated with pulmonary function parameters and the airway wall thickness in pulmonary sarcoidosis patients. IOS is considered to be useful for detecting early manifestations of airflow limitation in pulmonary sarcoidosis patients.