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The development of clinically progressive fibrosis complicates a wide array of interstitial lung diseases (ILDs). However, there are limited data regarding its prevalence and prognosis.We analysed consecutive patients seen for initial evaluation of a fibrosing form of ILD (FILD). Patients were evaluated for evidence of progressive fibrosis over the first 24 months of follow-up. We defined a progressive phenotype as the presence of at least one of the following: a relative decline in forced vital capacity (FVC) of ≥10%; a relative decline in FVC of ≥5%-10% with a relative decline in diffusing capacity of the lung for carbon monoxide of ≥15%, increased fibrosis on HRCT or progressive symptoms.Eight hundred and forty-four patients (397 with idiopathic pulmonary fibrosis [IPF] and 447 non-IPF FILD) made up the final analysis cohort. Three hundred and fifty-five patients (42.1%) met the progressive phenotype criteria (59.4% of IPF patients and 26.6% of non-IPF FILD patients, p 0.01). In both IPF and non-IPF FILD, transplantation-free survival differed between patients with a progressive phenotype and those without (p 0.01). Multivariable analysis showed that a progressive phenotype was an independent predictor of transplantation-free survival (hazard ratio [HR]: 3.36, 95% CI: 2.68-4.23, p 0.01). Transplantation-free survival did not differ between non-IPF FILD with a progressive phenotype and IPF (HR: 1.12, 95% CI: 0.85-1.48, p = 0.42).Over one-fourth of non-IPF FILD patients develop a progressive phenotype compared to approximately 60% of IPF patients. The survival of non-IPF FILD patients with a progressive phenotype is similar to IPF.

Introduction Monoclonal antibody therapy has been reported to be highly effective for preventing hospitalisation and severe cases in patients with Coronavirus Disease 2019 (COVID-19). However, since the drug is not readily available, it is important to rapidly and appropriately identify high-risk patients who can benefit most from therapy. Therefore, we designed a risk scoring system to identify at-risk COVID-19 patients in our region during the largest surge of COVID-19, from July to September 2021. Methods According to the risk scores, confirmed COVID-19 patients were introduced to receive REGN-CoV-2 to our hospital by regional health centre from 18th August (Term 3). The primary outcome was the comparison of the number of hospitalisation and severe condition with other periods, the 4th wave (Term 1) and the early part of the 5th wave (Term 2) in Japan. Results During Term 3, 115 patients were stratified with the scoring system and administered REGN-COV-2. The number of hospitalisation vs severe cases were 60 (5.2%) vs 14 (1.2%), 8 (1.5%) vs 3 (0.6%) and 21 (1.2%) vs 2 (0.1%), in term 1, 2 and 3, respectively. Among those aged

The clinical course of idiopathic pulmonary fibrosis (IPF) is characterized by a progressive decline in lung function; however, predicting changes in lung function is difficult. We sought to determine whether the prior 6-month trend in forced vital capacity (FVC) could predict mortality and the subsequent 6-month trend in FVC.We retrospectively analyzed consecutive patients with newly diagnosed IPF who underwent serial pulmonary function tests. The immediate two years after the initial evaluation were divided into four terms of six months each and stratified on the basis of presence or absence of a ≥10% relative decline in FVC at six months (declined and stable groups, respectively).We included 107 patients with %predicted FVC of 80.8% and %predicted diffusing capacity of the lung for carbon monoxide of 58.9%. In multivariate analysis, a decline in %predicted FVC in the initial six months was found to be an independent prognostic factor (hazard ratio 4.45, 95% confidence interval 2.62-7.56, p 0.01). Among the 46 terms in which the FVC declined during the initial 1.5-year study period, a decline in FVC was exhibited in 23 (50.0%) of the subsequent terms. Among 231 terms in which FVC remained stable, a decline was observed in 32 (13.9%) of the subsequent terms (relative risk 3.61, p 0.01). The frequency of FVC decline in each term was 16-27%. FVC was stable or declined in all four terms in 50.5% and 15.9% of cases, respectively.Six-month decline in FVC predicts subsequent FVC change and mortality in IPF patients in the era of antifibrotic agents.

Background: Although physical activity is associated with mortality in patients with idiopathic pulmonary fibrosis (IPF), reference values to interpret levels of physical activity are lacking. Objectives: This study aimed to investigate the prognostic significance of physical activity assessed by step count and its cutoff points for all-cause mortality. Methods: We measured physical activity (steps per day) using an accelerometer in patients with IPF at the time of diagnosis. Relationships among physical activity and mortality, as well as cutoff points of daily step count to predict all-cause mortality were examined. Results: Eighty-seven patients (73 males) were enrolled. Forty-four patients (50.1%) died during the follow-up (median 54 months). In analysis adjusting for Gender-Age-Physiology stage and 6-min walk distance, daily step count was an independent predictor of all-cause mortality (hazard ratio (HR) = 0.820, 95% confidence interval (CI) = 0.694–0.968, p = 0.019). The optimal cutoff point (receiving operating characteristic analysis) for 1-year mortality was 3,473 steps per day (sensitivity = 0.818 and specificity = 0.724). Mortality was significantly lower in patients with a daily step count exceeding 3,473 steps than in those whose count was 3,473 or less (HR = 0.395, 95% CI = 0.218–0.715, p = 0.002). Conclusions: Step count, an easily interpretable measurement, was a significant predictor of all-cause mortality in patients with IPF. At the time of diagnosis, a count that exceeded the cutoff point of 3,473 steps/day more than halved mortality. These findings highlight the importance of assessing physical activity in this patient population.

Background In Japan, the severity staging system for idiopathic pulmonary fibrosis (IPF) has been used to determine medical care subsidies. However, this system requires invasive procedures to measure arterial oxygen tension. Recently, noninvasive and simple measurements of oxygen saturation by pulse oximetry (SpO2) have been used for severity assessments. We propose a pulse oximetry saturation (POS) staging system consisting of SpO2 parameters to predict prognosis. Methods We developed four prototype staging systems based on SpO2 at rest and desaturation, and adopted the system with the highest C-statistic as the POS staging system. The cutoff SpO2 values at rest were 96% and 90%, and desaturation was defined as SpO2 Results Two-hundred and nineteen IPF patients were studied and the C-statistic values of models 1, 2, 3, and 4 were 0.633, 0.643, 0.630, and 0.673, respectively. We judged model 4 to be a superior POS staging system; it defined SpO2 ≥ 96% at rest without desaturation as stage Ⅰ; SpO2 ≥ 96% at rest with desaturation or SpO2 90%–95% at rest without desaturation as stage Ⅱ; and SpO2 90%–95% at rest with desaturation or SpO2 Conclusion A noninvasive and simple POS staging system defined by SpO2 can easily predict prognosis.

Computed tomography (CT) assessment of the cross-sectional area of the erector spinae muscles (ESMCSA) can be used to evaluate sarcopenia and cachexia in patients with lung diseases. This study aimed to confirm whether serial changes in ESMCSA are associated with survival in patients with idiopathic pulmonary fibrosis (IPF). Data from consecutive patients with IPF who were referred to a single centre were retrospectively reviewed. We measured the ESMCSA at the level of the 12th thoracic vertebra on CT images at referral and 6 months later (n = 119). The follow-up time was from 817–1633 days (median, 1335 days) and 59 patients (49.6%) died. A univariate Cox regression analysis showed that the decline in % predicted forced vital capacity (FVC) (Hazard ratios [HR] 1.041, 95% confidence interval [CI] 1.013–1.069, P = 0.004), the decline in body mass index (BMI) (HR 1.084, 95% CI 1.037–1.128; P CSA (HR 1.057, 95% CI 1.027–1.086; P CSA (HR 1.039, 95% CI 1.007–1.071, P = 0.015) was a significant prognostic factor, while those in % FVC and BMI were discarded. Early decrease in ESMCSA may be a useful predictor of prognosis in patients with IPF.

Diffuse alveolar damage (DAD) is known to be a pathological hallmark of acute respiratory distress syndrome or acute interstitial pneumonia, and to have a poor prognosis. We report a case of anti‐melanoma differentiation‐associated gene 5 (MDA5) antibody‐positive clinically amyopathic dermatomyositis (CADM) with rapidly progressive interstitial lung disease (RP‐ILD), in which DAD was confirmed by transbronchial lung cryobiopsy at an early stage without respiratory failure. Although this patient initially did not show respiratory failure, his respiratory condition gradually worsened despite intensive immunosuppression therapy and he died 3 months later. Therefore, the early pathological findings of DAD did not match the clinical picture, which showed no respiratory failure. However, these findings were consistent with the subsequent course and poor outcome. Histological DAD, even in the absence of respiratory failure, may indicate a subsequent poor prognosis and explain the refractory course of RP‐ILD with anti‐MDA5 antibody‐positive CADM., We report a case showing diffuse alveolar damage in a patient with rapidly progressive interstitial lung disease (RP‐ILD) with anti‐melanoma differentiation‐associated gene 5 antibody‐positive clinically amyopathic dermatomyositis, diagnosed by transbronchial lung cryobiopsy (TBLC) at an early stage without respiratory failure. Although this patient initially did not show respiratory failure, his respiratory condition gradually worsened despite intensive immunosuppression therapy and he died 3 months later. In patients with RP‐ILD, TBLC has the potential to predict prognosis and treatment response by evaluating the pathological pattern.

Background Patients with connective tissue disease-associated interstitial lung disease (CTD-ILD) often experience impaired health status. In daily clinical practice, a short and easy instrument for assessing health status would be useful to help better understand the patient's condition. The COPD Assessment Test (CAT) is a simple questionnaire about respiratory symptoms and their impact. We aimed to examine the CAT's performance characteristics and to generate data to support its reliability and validity in patients with CTD-ILD. Methods We used data from 132 CTD-ILD patients evaluated at Tosei General Hospital from July 2011 to July 2016 to assess the cross-sectional and longitudinal validity of the CAT. Results The mean age of the patients was 64.5 years and 87 (66%) were women. There were no significant differences in CAT score between any of the CTD subgroups. Internal consistency (Cronbach's α = 0.881) and repeatability (intraclass correlation coefficient [ICC] = 0.803) for the CAT score were acceptable. At baseline, CAT score was significantly associated with clinically meaningful measures of physiologic function, exercise capacity, and dyspnea. Change in CAT score over 6–12 months was also associated with change in other measures. In the distribution- and anchor-based analyses, the estimated minimal clinically important difference of CAT score was 1–4 points. Conclusion These data support the validity and reliability of CAT as a sensitive measure for assessing health status in patients with CTD-ILD.

Background The beneficial effects of pulmonary rehabilitation (PR) for patients with idiopathic pleuroparenchymal fibroelastosis (IPPFE) remain unknown. This study aimed to examine the efficacy, feasibility, and safety of PR for IPPFE. Methods We retrospectively investigated 25 patients with IPPFE referred for PR between April 2007 and March 2017. The PR mainly consisted of a 10-week exercise training program. The primary outcome was a change in 6-min walk distance (6MWD). Secondary outcomes included changes in dyspnea (transition dyspnea index [TDI]), anxiety and depression (hospital anxiety and depression scale [HADS]), and health-related quality of life (HRQoL) (St George's respiratory questionnaire [SGRQ]). Results Thirteen patients participated in the PR program (PRP). Recurrent pneumothorax was the most common reason for patients not participating in the PRP. Four patients discontinued the PRP due to the recurrence of pneumothorax, new onset of pneumomediastinum, stroke, and another reason, respectively. Nine patients completed the PRP. Significant improvement was observed in 6MWD (median [interquartile range], 90 m [55–116 m]; P = 0.033). Clinically important improvements in the 6MWD, and TDI, HADS-anxiety, HADS-depression, and SGRQ total domain scores were observed in seven (78%), five (56%), four (44%), four (44%), and five (56%) of the nine patients, respectively. Conclusions Patients with IPPFE benefited from PR in terms of exercise capacity, dyspnea, anxiety, depression, and HRQoL. Pneumothorax and pneumomediastinum may impede the implementation of a PRP for patients with IPPFE. While careful patient selection is required, PR may be an efficacious non-pharmacological approach for managing disabilities in patients with IPPFE.

Drug-induced lung injury (DLI) has become more common because of the increasing number of therapeutic agents in use. Mesalazine, also known as 5-aminosalicylic acid (5-ASA), is one of the key drugs for the treatment of ulcerative colitis (UC). Although mesalazine-induced lung injury has been previously reported, few cases have included severe respiratory failure. In this report, we present a case of mesalazine-induced lung injury with severe respiratory failure, which was improved by discontinuation of mesalazine and introduction of corticosteroid therapy and ventilation support with non-invasive positive pressure ventilation (NPPV). We also review the previous literature on mesalazine-induced lung injury.

Rice caryopses show different patterns of grain filling depending on position within a panicle. Caryopses located on the upper primary rachis branches generally accumulate larger amounts of starch at maturity than caryopses located on the secondary rachis branches of the lower primary rachis. In this study, the former and latter types of caryopses were defined as superior and inferior caryopses, respectively. Superior caryopses elongated soon after flowering, whereas inferior caryopses hardly elongated and were morphologically stagnant until the first 4 d after flowering (DAF). However, once inferior caryopses began elongation, their morphological development was the same as superior caryopses until the middle stage of grain filling. Cell division of the inner integument ceased before endosperm cellularization, pericarp functioned as a transient starch storage tissue until endosperm accumulated starch, and endosperm cell number was determined concomitantly with nucellus disintegration. These results implied the coordinated development of the endosperm with maternal tissues. In addition, differences of inner-integument cell number and endosperm cell number were related to a difference of endosperm size between superior and inferior caryopses.

In addition to advanced non-small cell lung cancer, nanoparticle albumin-bound paclitaxel (nab-PTX) may also harbor potential benefit for patients with relapsed small cell lung cancer (SCLC), since weekly paclitaxel (PTX) shows modest activity for relapsed SCLC. We evaluated the efficacy and safety of both weekly nab-PTX and PTX for relapsed SCLC.We retrospectively reviewed 52 consecutive relapsed SCLC patients who were treated with weekly nab-PTX or PTX at our hospital.The response rate, median progression-free survival and overall survival with nab-PTX and PTX were 5.6 vs 8.8%, 3.2 vs 1.7 months, and 5.4 vs 4.5 months, respectively. No statistically significant differences were observed. There was no statistical difference between the 2 groups for ≥Grade 3 adverse events.Weekly nab-PTX and PTX showed similar activity for relapsed SCLC. The toxicity profile of nab-PTX was equally tolerable to that of PTX.

Background The Pfizer-BioNTech COVID-19 vaccine has recently received emergency approval from the US FDA. The mRNA technology was used to manufacture the Pfizer vaccine; however, as a pioneering technology that has never been used in the manufacture of vaccines, many people have concerns about the vaccine’s side effects. Thus, the current study aimed to track the short-term side effects of the vaccine. Methods The information in this study was gathered by a Google Form-questionnaire (online survey). The results included the responses of 455 individuals, all of whom are Saudi Arabia inhabitants. Adverse effects of the vaccine were reported after the first and the second doses. Results The most common symptoms were injection site pain, headaches, flu-like symptoms, fever, and tiredness. Less common side effects were a fast heartbeat, whole body aches, difficulty breathing, joint pain, chills, and drowsiness. Rare side effects include Bell’s palsy and lymph nodes swelling and tenderness. Flu-like symptoms were more common among those under 60 years of age, while injection site pain was more frequent among recipients who were 60 years and older. The study revealed a significant increase in the number of females who suffered from the vaccine side effects compared to males. Difficulty of breathing was more reported among recipients who had been previously infected with the coronavirus compared to those who had not been previously infected. Conclusion Most of the side effects reported in this study were consistent with Pfizer’s fact sheet for recipients and caregivers. Further studies are required to determine the long-term side effects.

Respiratory-related hospitalization, in particular acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF), is common and associated with increasing mortality in patients with IPF. We aimed to evaluate the implications of a newly proposed framework of acute respiratory deterioration (ARD) and AE-IPF in hospitalized patients.Using the data of an IPF cohort consisting of 225 consecutive patients, we retrospectively studied first hospitalizations from January 2008 to December 2017. We analysed the demographics and 90-day mortality of patients with AE-IPF and those with parenchymal cause of ARD other than AE.Among 122 patients with first hospitalization for ARD, 35 patients were diagnosed with AE-IPF, including 11 patients with triggered AE. Parenchymal cause of ARD other than AE was diagnosed in 71 patients, and extra-parenchymal cause in 16 patients. Almost all hospitalized patients (93%) underwent chest CT, and 83% of patients with AE-IPF underwent bronchoalveolar lavage. There was a significant difference in the anti-inflammatory therapy between the AE-IPF group and parenchymal cause of ARD other than AE group (p 0.001). AE-IPF was independently associated with poor survival in multivariate Cox proportional regression analysis.AE-IPF accounted for about 30% of first hospitalizations for ARD, and differentiation between AE-IPF and the other categories in ARD is important from a therapeutic and a prognostic point of view.

OBJECTIVES Little is known about the relationship between preoperative physical fitness and postoperative pneumonia after lung resection. We examined the association between preoperative 6-min walk distance (6MWD) and postoperative pneumonia. METHODS A retrospective study was conducted on patients with malignant lung tumours who were scheduled to undergo lung resection at Nagoya University Hospital from January 2014 to December 2015. Preoperative pulmonary function tests and the 6-min walk test were assessed. A logistic regression model and receiver operating characteristic curves were used to analyse clinical variables and compare the performance on 6MWD, forced expiratory volume in 1 s and diffusion capacity of the lung for carbon monoxide. RESULTS The data from a total of 321 patients including 283 with primary lung cancer and 38 with metastatic lung tumours were analysed. Pneumonia developed in 13 patients and caused longer hospital stays after surgery. The preoperative 6MWD of patients with pneumonia was significantly lower than that without pneumonia (425 vs 500 m, P = 0.002). In receiver operating characteristic analysis, 6MWD ≤ 450 m was a threshold for predicting postoperative pneumonia with 69.2% sensitivity and 71.1% specificity. A 6MWD ≤ 450 m, forced expiratory volume in 1 s

Background Anti-synthetase syndrome (ASS) is characterized by the presence of anti-aminoacyl-tRNA synthetase antibody and ASS-associated interstitial lung disease (ILD) often recurs. The effectiveness of remission induction therapy with corticosteroids and calcineurin inhibitor (CNI) and the predictive factors for ASS-ILD recurrence were examined. Methods We retrospectively identified consecutive patients with ASS-ILD treated with corticosteroids and CNI during 2006–2017 and evaluated the predictive factors for recurrence using logistic regression analysis. Results Of the 57 patients included in this study, 54 (94.7%) exhibited improved response to remission induction therapy. There were 32 recurrence patients during maintenance therapy. The median period until recurrence was 27 months. There were no significant differences in the baseline characteristics between the recurrence and nonrecurrence groups. In the recurrence group, respiratory function and St. George's Respiratory Questionnaire score deteriorated over the clinical course. The Krebs von den Lungen-6 (KL-6) level changed with disease behavior. The multivariate analysis revealed that KL-6 increase rate from remission (odds ratio: 3.21, 95% CI: 1.17–8.86, p = 0.02) and CNI discontinuation (odds ratio: 8.09, 95% CI: 1.39–47.09, p = 0.02) were independent predictive factors for recurrence. The receiver operating characteristics analysis revealed that the optimal cut-off point of KL-6 increase rate was 2.0. The positive predictive values of the KL-6 increase rate from remission of >2.0 and CNI discontinuation were 90.0 and 88.9%, respectively. The CNI treatment duration and recurrence were not related. Conclusions Recurrence influenced long-term deterioration. KL-6 was a serum biomarker for disease behavior and recurrence prediction. The results suggest the importance of CNI continuation.

Background Chronic hypersensitivity pneumonitis (CHP) is a fibrotic interstitial lung disease (ILD) caused by repeated exposure to a variety of organic particles. In November 2017, new criteria for CHP diagnosis were proposed by Morisset et al. based on a modified Delphi survey of ILD experts. However, it remains unclear whether these criteria are useful to accurately diagnose CHP. We aimed to evaluate the newly proposed CHP diagnostic criteria. Methods We retrospectively applied Morisset's CHP diagnostic criteria to consecutive Japanese patients who underwent surgical lung biopsy for diagnosis of ILD from 2008 to 2015. All patients underwent bronchoalveolar lavage and pulmonary function testing. Patients who had connective tissue disease complications or showed an acute or subacute disease onset were excluded. Results A total of 251 patients were included. The diagnoses based on multidisciplinary discussion (MDD) were CHP (n = 27), idiopathic pulmonary fibrosis (n = 117), unclassifiable interstitial pneumonia (IP) (n = 65), and other diagnoses (n = 42). Of the 27 MDD-CHP patients, 14 were classified as a CHP group with diagnostic confidence >50% and 13 were not categorized (sensitivity, 51.9%; specificity, 77.7%). Morisset's CHP diagnostic criteria could help avoid SLB for the diagnosis of CHP in seven patients. Of the 13 MDD-CHP patients who were not categorized in the CHP group with diagnostic confidence >50%, the reason for the exclusion was an inconsistent with UIP pattern without CHP features. Conclusions Half of the MDD-CHP patients were diagnosed with CHP using Morisset's CHP diagnostic criteria. Further investigation will be important for developing improved diagnostic criteria for CHP.

BACKGROUND AND OBJECTIVE Acute exacerbation (AE) is a severe complication of idiopathic pulmonary fibrosis (AE-IPF). In 2016, an international working group revised its definition and diagnostic criteria; however, few studies have assessed the frequency and prognosis of AE in patients with other fibrotic interstitial lung diseases (FILD). METHODS We used data from 1019 consecutive interstitial lung disease (ILD) patients initially evaluated between January 2008 and July 2015. All subject diagnoses were made by multidisciplinary discussion in December 2018. ILD was categorized as IPF (n = 462) and other FILD which included non-specific interstitial pneumonia (n = 22), chronic hypersensitivity pneumonitis (n = 29), connective tissue disease-associated ILD (n = 205) and unclassifiable ILD (n = 209). Using the 2016 definition of AE-IPF, we identified all subjects with an AE. RESULTS During the observational period, 193 patients experienced a first AE (AE-FILD n = 69, AE-IPF n = 124). The time to first AE was significantly longer in FILD than IPF (log-rank test, P

Recent studies have suggested that in patients with an idiopathic interstitial pneumonia (IIP), a probable usual interstitial pneumonia (UIP) pattern on chest computed tomography (CT) is sufficient to diagnose idiopathic pulmonary fibrosis (IPF) without histopathology.We retrospectively compared the prognosis and time to first acute exacerbation (AE) in IIP patients with a UIP and a probable UIP pattern on initial chest CT.One hundred and sixty IIP patients with a UIP pattern and 242 with a probable UIP pattern were identified. Probable UIP pattern was independently associated with longer survival time (adjusted hazard ratio 0.713, 95% CI 0.536–0.950; p=0.021) and time to first AE (adjusted hazard ratio 0.580, 95% CI 0.389–0.866; p=0.008). In subjects with a probable UIP pattern who underwent surgical lung biopsy, the probability of a histopathological UIP pattern was 83%. After multidisciplinary discussion and the inclusion of longitudinal behaviour, a diagnosis of IPF was made in 66% of cases. In IPF patients, survival time and time to first AE were not associated with CT pattern. Among subjects with a probable UIP pattern, compared to non-IPF patients, survival time and time to first AE were shorter in IPF patients.In conclusion, IIP patients with a probable UIP pattern on initial chest CT had a better prognosis and longer time to first AE than those with a UIP pattern. However, when baseline data and longitudinal behaviour provided a final diagnosis of IPF, CT pattern was not associated with these outcomes. This suggests diagnostic heterogeneity among patients with a probable UIP pattern.

Background and objective The COPD Assessment Test (CAT) has been reported to have potential utility for measuring health status of idiopathic pulmonary fibrosis (IPF). Although the CAT has been developed for the assessment of COPD patients, it has not been fully evaluated exclusively in IPF. This study was designed to evaluate the validity of the CAT in IPF. Methods The clinical data for 106 consecutive subjects with newly diagnosed IPF who completed pulmonary function tests, partial pressure of oxygen in arterial blood (PaO2 ) at rest, 6-min walk test (6MWT), CAT, St George's Respiratory Questionnaire (SGRQ), modified Medical Research Council (mMRC) dyspnoea grade and Hospital Anxiety and Depression Scale (HADS), were analysed. We assessed the validity of the CAT in comparison with the SGRQ. Results The present subjects showed mild to moderate restrictive impairment on spirometry. Mean CAT score and total SGRQ were 12.8 ± 8.0 and 30.8 ± 17.7, respectively. The concurrent validity of the CAT score in comparison with the SGRQ total score was significant (r = 0.72, P

The aim of this study was to examine long-term changes in pulmonary function and respiratory impedance (Zrs) as assessed by forced oscillation technique (FOT) of rheumatoid arthritis (RA)-related pulmonary disorders. Data of 42 RA patients who underwent pulmonary function tests and Zrs measurements at least twice at a >900-day interval were retrospectively reviewed. Zrs, respiratory resistance (Rrs) and reactance (Xrs), were measured as a function of oscillatory frequency from 4 to 36 Hz. The Rrs and difference between inspiratory and expiratory phases of Xrs were significantly decreased. Annual changes in Xrs parameters significantly correlated with those of spirometric parameters. Zrs parameters were significantly different between the low (the lower 75 percentile of incidence) and high (the top quartile) frequency of adverse respiratory event groups. The Zrs combined with spirometry may be beneficial to evaluate alterations in respiratory functions of RA.

BACKGROUND Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) presents as episodes of acute respiratory worsening closely associated with endothelial damage and disordered coagulopathy. Recombinant human soluble thrombomodulin (rhTM) regulates the coagulation pathway mainly by reducing thrombin-mediated clotting and enhancing protein C activation. We investigated the efficacy of rhTM for the treatment of patients with AE-IPF. METHODS This historical control study comprised 40 patients with AE-IPF. Twenty patients treated with rhTM (0.06 mg/kg/d) for about 6 days (rhTM group) and 20 patients treated without rhTM (control group) were evaluated. The predictors of 3-month mortality (logistic regression model) were evaluated. RESULTS There was no difference in baseline characteristics between the control group and the rhTM group. Three-month mortality of the rhTM group and control group was 30.0% and 65.0%, respectively. In univariate analysis, C-reactive protein and rhTM therapy were significant determinants for 3-month survival. In multivariate analysis, rhTM therapy (OR, 0.219;95% CI, 0.049-0.978; P = 0.047) was an independent significant determinant for 3-month survival. CONCLUSIONS We found that rhTM therapy improved 3-month survival of AE-IPF. The results observed here warrant further investigation of rhTM in randomized control trials.