scholarly journals Effectiveness of pirfenidone in idiopathic pulmonary fibrosis according to the autoantibody status: a retrospective cohort study

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Myung Jin Song ◽  
Sang Hoon Lee ◽  
Ji Ye Jung ◽  
Young Ae Kang ◽  
Moo Suk Park ◽  
...  

Abstract Background Pirfenidone is an anti-fibrotic agent shown to slow the progression of idiopathic pulmonary fibrosis (IPF). However, its effectiveness in association with serological autoimmune features in IPF remains unclear. Methods We retrospectively reviewed the medical records of patients with IPF treated at a tertiary care hospital in South Korea. The autoantibody status was defined as positive if we detected autoantibodies meeting the serological domain criteria for interstitial pneumonia with autoimmune features or anti-neutrophil cytoplasmic antibodies. Results We included 142 patients with IPF treated with pirfenidone for over six months (93 were autoantibody-positive and 49 were autoantibody-negative). The mean age was 69.5 ± 7.3 years, and 77.5% of the patients were male. The adjusted mean changes over one year were − 34.4 and − 112.2 mL (p = 0.168) in forced vital capacity (FVC), and − 0.53 and − 0.72 mL/mmHg/min (p = 0.356) in the lungs diffusion capacity for carbon monoxide (DLCO) in the autoantibody-negative and autoantibody-positive groups, respectively. Conclusions Reductions in FVC and DLCO were similar in autoantibody-positive and autoantibody-negative patients with IPF treated with pirfenidone. Pirfenidone is effective in attenuating the progression of IPF, irrespective of the autoantibody status.

2019 ◽  
Vol 2 (2) ◽  
pp. 90-95
Author(s):  
Mahesh N ◽  
◽  
Sadanand C D ◽  
Durga Lawande ◽  
◽  
...  

Author(s):  
Prem Singh ◽  
Achyut K. Pandey

Background: Psychiatric morbidity occurs more frequently in patients with epilepsy than in the general population. Routine evaluation and treatment of psychiatric morbidity can be helpful in improving epilepsy care but such data are relatively meagre from developing countries.Methods: The study was conducted in the Epilepsy Clinic of Department of Neurology at a tertiary care hospital over a period of one year.101 patients were included after fulfilling the inclusion criteria. All the patients seeking treatment in the OPD were screened, assessed and then all procedures were fully explained to them. History regarding name, age sex, socio-demographic profile and detailed history regarding seizure disorder was taken from both the patient and the reliable informant. Bengali version of SRQ-24 was used to screen for psychiatric morbidity.Results: One hundred and one patients with epilepsy consisting of 70 men (69.3%) and 31 women (30.7%) were included. Their ages ranged from 15 to 52, the mean age being 26.17 (SD = 7.84). Out of the 101 patients, 65 patients (64.4%) were suffering from partial epilepsies and 36 patients (35.6%) were suffering from generalized epilepsies. 50.49% of the subjects screened positive for psychiatric morbidity. Psychiatric morbidity was higher in unmarried, unemployed males from rural background who were suffering from generalized epilepsy and taking multiple antiepileptic medications. Psychiatric morbidity was statistically significant in people with poor education and those born at home (p<0.05) as compared to well educated, institutionally born persons.Conclusions: 50.49% of the subjects screened positive for psychiatric morbidity.


2017 ◽  
Vol 3 (3) ◽  
pp. 00019-2016 ◽  
Author(s):  
Akihiko Sokai ◽  
Kiminobu Tanizawa ◽  
Tomohiro Handa ◽  
Kumiko Kanatani ◽  
Takeshi Kubo ◽  
...  

The clinical significance of serial changes in serum biomarkers in patients with idiopathic pulmonary fibrosis (IPF) remains to be established. This retrospective study was conducted to clarify the associations of serial changes in serum Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) with changes in physiological indices and overall mortality in IPF.The study subjects were 75 patients with IPF. The 6 month change in serum KL-6 was significantly correlated with changes in the percentage of the predicted forced vital capacity (FVC % pred) and the percentage of the predicted diffusing capacity of the lung for carbon monoxide (% DLCO), while the 6 month change in serum SP-D was correlated only with % DLCO. During the mean follow-up period of 647 days, 22 (29.3%) patients died. An increase in serum KL-6 over a 6 month period was a significant predictor of mortality even after adjustment for %FVC, % DLCO and serum KL-6 at the baseline (hazard ratio 1.10 per 100 U·mL−1, 95% CI 1.01–1.18, p=0.03), whereas the 6 month increase in serum SP-D was not significant.Serial measurements of serum KL-6 may provide additional prognostic information compared to that provided by physiological parameters in patients with IPF.


2015 ◽  
Vol 41 (4) ◽  
pp. 299-304 ◽  
Author(s):  
Adalberto Sperb Rubin ◽  
Douglas Zaione Nascimento ◽  
Letícia Sanchez ◽  
Guilherme Watte ◽  
Arthur Rodrigo Ronconi Holand ◽  
...  

AbstractObjective: To evaluate the changes in lung function in the first year after single lung transplantation in patients with idiopathic pulmonary fibrosis (IPF).Methods: We retrospectively evaluated patients with IPF who underwent single lung transplantation between January of 2006 and December of 2012, reviewing the changes in the lung function occurring during the first year after the procedure.Results: Of the 218 patients undergoing lung transplantation during the study period, 79 (36.2%) had IPF. Of those 79 patients, 24 (30%) died, and 11 (14%) did not undergo spirometry at the end of the first year. Of the 44 patients included in the study, 29 (66%) were men. The mean age of the patients was 57 years. Before transplantation, mean FVC, FEV1, and FEV1/FVC ratio were 1.78 L (50% of predicted), 1.48 L (52% of predicted), and 83%, respectively. In the first month after transplantation, there was a mean increase of 12% in FVC (400 mL) and FEV1 (350 mL). In the third month after transplantation, there were additional increases, of 5% (170 mL) in FVC and 1% (50 mL) in FEV1. At the end of the first year, the functional improvement persisted, with a mean gain of 19% (620 mL) in FVC and 16% (430 mL) in FEV1.Conclusions: Single lung transplantation in IPF patients who survive for at least one year provides significant and progressive benefits in lung function during the first year. This procedure is an important therapeutic alternative in the management of IPF.


2018 ◽  
Vol 52 (6) ◽  
pp. 1801130 ◽  
Author(s):  
Ganesh Raghu ◽  
Luca Richeldi ◽  
Bruno Crestani ◽  
Peter Wung ◽  
Raphael Bejuit ◽  
...  

A phase 2b trial (NCT02345070) was conducted to evaluate the efficacy and safety of two dose levels/regimens of SAR156597 (a bispecific IgG4 antibody that binds and neutralises both circulating interleukin-4 and interleukin-13), in comparison with placebo, administered to patients with idiopathic pulmonary fibrosis (IPF) over 52 weeks.DRI11772 was a multinational randomised double-blind placebo-controlled phase 2b trial. Patients aged >40 years with a documented diagnosis of IPF received SAR156597 200 mg once every week (QW), SAR156597 200 mg once every 2 weeks (Q2W) or placebo, over 52 weeks. The primary efficacy end-point was absolute change from baseline in forced vital capacity (FVC) % predicted at 52 weeks.Of 327 randomised patients, 325 received treatment with placebo (n=109), SAR156597 Q2W (n=108) or SAR156597 QW (n=108). The mean change from baseline in FVC % pred at 52 weeks was –5.8%, –5.2% and –6.3% for the placebo, Q2W and QW arms, respectively (Q2W versus placebo, p=0.59; QW versus placebo, p=0.63). The safety profile observed in the three treatment arms was generally similar, although serious adverse events were more common in the QW arm than in the other arms.The DRI11772 study failed to demonstrate benefit of SAR156597 in the treatment of IPF.


2014 ◽  
Vol 21 (3) ◽  
pp. e55-e60 ◽  
Author(s):  
Arturo Cortes-Telles ◽  
Lutz Forkert ◽  
Denis E O’Donnell ◽  
Onofre Morán-Mendoza

BACKGROUND: The lung function of patients with idiopathic pulmonary fibrosis (IPF) has not been characterized in detail.OBJECTIVE: To characterize the heterogeneous physiological abnormalities that exist in patients with IPF during their initial clinical evaluation.METHODS: Lung function tests from 93 patients, performed within six months of the initial diagnosis of IPF, were obtained from a referral pulmonary function laboratory at a tertiary care hospital in Canada. A restrictive pattern was defined as total lung capacity (TLC) <95th percentile of predicted value. Patients with obstructive lung disease, lung cancer, emphysema and other restrictive lung diseases were excluded.RESULTS: On diagnosis, 73% of patients with IPF had a restrictive pattern, with a mean TLC of 72% of predicted. Mean forced vital capacity (FVC) was 71% and 44% of patients had an FVC <95th percentile. Mean diffusing capacity for carbon monoxide (DLco) was 60% and DLco/alveolar volume (VA) 92% of predicted. Increased severity of restriction – based on TLC – was associated with lower DLco(74% of predicted in mild restriction and 39% of predicted in severe restriction) and higher forced expiratory volume in 1 s (FEV1)/FVC ratio (82% of predicted in mild restriction and 90% of predicted in severe restriction) but not with age (76 years in mild restriction and 69 years in severe restriction). Regardless of severity of restriction, the average DLco/VA (≥86% of predicted) remained within normal limits.CONCLUSIONS: One in four patients with IPF had normal TLC and more than one-half had a normal FVC during initial evaluation. As the severity of the restriction increased, FEV1/FVC increased, DLcodecreased but DLco/VA remained normal.


2020 ◽  
Author(s):  
Sho Saeki ◽  
Osamu Nishiyama ◽  
Ryo Yamazaki ◽  
Kazuya Yoshikawa ◽  
Ken Shirahase ◽  
...  

Abstract Background Antifibrotic agents suppress the decline in forced vital capacity (FVC) and disease progression in idiopathic pulmonary fibrosis (IPF) patients. However, their effect on survival prognosis and differences in this effect according to baseline lung function have been unexplored. Therefore, this study aimed to examine the effect of antifibrotics on survival prognosis and whether this effect differed according to baseline FVC. Methods Consecutive IPF patients from January 2008 to May 2019 were examined retrospectively. FVC and effect of pirfenidone or nintedanib therapy were assessed. FVC at registration was used to categorize the patients into mild: FVC % predicted ≥ 80%, moderate: FVC % predicted 50–80%, and severe: FVC % predicted < 50% IPF groups. Results In total, 172 IPF patients were included. The mean FVC % predicted was 77.4 ± 22.2%. The median survival periods of patients in the mild, moderate, and severe IPF groups were 1,452, 1,305, and 481 days, respectively. Significant differences were observed in survival between the mild and severe groups and the moderate and severe groups (p < 0.0001), but not between the mild and moderate groups (p = 0.20). The survival was longer in patients on antifibrotic therapy in the mild (p = 0.18) and moderate groups (p = 0.04), but not in the severe group (p = 0.93). Conclusions Antifibrotics extended the survival of IPF patients. The effect was obvious in patients with FVC % predicted of 50–80%, a tendency was observed in patients with FVC % predicted ≥ 80%, while no effect was observed in patients with FVC % predicted < 50%.


2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Hong Yeul Lee ◽  
Jaeyoung Cho ◽  
Nakwon Kwak ◽  
Jinwoo Lee ◽  
Young Sik Park ◽  
...  

Abstract No studies on idiopathic pulmonary fibrosis (IPF) have investigated the prognostic impact of extrapulmonary cancers in patients with IPF. We aimed to determine the prognostic impact of malignancies in patients with IPF. We retrospectively reviewed the medical records of patients diagnosed with IPF between 2001 and 2015. Patients were divided into three groups: IPF without cancer (n = 440), IPF with lung cancer (n = 69), and IPF with extrapulmonary cancer (n = 70). Of the 579 patients with IPF, 139 (24%) had cancer; the three most common types were lung (11.9%), gastric (2.4%), and colorectal (1.9%). Survival was significantly worse in patients with lung cancer than in those without cancer (hazard ratio [HR] = 1.83, 95% confidence interval [CI], 1.35–2.48) or those with extrapulmonary cancer (HR = 1.70, 95% CI, 1.14–2.54). The rate of hospitalisation for cancer-related complications was significantly higher in IPF patients with lung cancer than in those with extrapulmonary cancer. The annual rates of decline in percent predicted forced vital capacity and diffusion capacity for carbon monoxide did not differ among the groups. Physicians should pay attention to the development and progression of cancer and its prognostic impact in patients with IPF.


Author(s):  
Sarvepalli Vijaya Kumar ◽  
Krishnan Ramalingam

Background: Anaemia defined as reduction in the concentration in Haemoglobin is one of the key health indicators of health care system of the country. Accurate screening methods are required to estimate the levels of haemoglobin for diagnosing the cause of anaemia. Objectives of the study was to analyze and compare the results of haemoglobin concentrations estimated with automated haematology analyzer and point of care device HemoCue Hb301.Methods: It is a prospective cross-sectional study was conducted for one year after ethical approval. Non fasting capillary and venous blood samples were collected from the selected cases of children and Haemoglobin concentrations were estimated by automated analyzer and HemoCue Hb301 system and the values were noted. Quality control checks were performed for both. Statistical analysis was done using IBM SPSS Version 24.0.Results: Mean Hb% concentration was estimated in 108 children with 44 female and 64 males. The mean value of Automated hematology analyzer (11.965±1.012) was significantly higher when compared with the mean value of HemoCue Hb301 (11.697±1.312) (p=0.002). There was a significantly strong correlation between HemoCue Hb301and Automated hematology analyzer (r-value = 0.732, p <0.0001).Conclusions: The HemoCue is useful in many different settings and remains a widely used method in field settings as it has several advantages and is relatively inexpensive compared with automated haematology analysers. Further studies are needed to better understand potential sources of error in the Hb assessment by HemoCue with the aim to better train phlebotomists and implement appropriate standardised procedures.


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