scholarly journals Pathogenic and clinical trial of N-acetylcysteine (Fluimucil) in idiopathic pulmonary fibrosis

2005 ◽  
pp. 34-41
Author(s):  
E. N. Popova ◽  
B. M. Kornev ◽  
K. U. Reznikova ◽  
O. V. Kondarova

The aim of this study was to investigate free radical and lipid disorders in idiopathic pulmonary fibrosis (IPF), to assess clinical efficacy of Fluimucil and to substantiate its administration in different stages of the disease. We observed 127 IPF patients, of them 59 were treated typically with prednisolone, colchicines, or azathioprin, 68 ones received immunosuppressors and Fluimucil. The diagnosis of IPF was verified morphologically in open lung biopsy samples. The patients’ age was 25 to 74 yrs, the mean age, 46.7 ± 9.8 yrs. A control group included 20 healthy donors, the mean age, 41.2 ± 1.2 yrs. N-acetylcysteine (Fluimucil, Zambon group) was administered initially IV 1800 mg a day for 14 days, then orally 1 800 mg a day for a month, then 600 mg a day for 3 months. Clinical and free radical conditions were monitored before the treatment and 3, 6 and 12 months starting the therapy. Fluimucil improved thrombocyte antioxidant activity and plasma antioxidant activity, reduced CT signs of IPF, provided a stable growth in FEV1 and FVC (by 10 % and 12 % respectively) and DLco. Fluimucil was well-tolerated, adverse effects (nausea, stomach ache) were noted in 7 patients (10.2 %). Thus, the results confirmed antioxidant efficacy of Fluimucil in IPF. The long-term administration of Fluimucil combined with the immunosuppressive drugs in IPF patients was safe and reasonable as this inhibited progression of the disease.

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Ryo Yamazaki ◽  
Osamu Nishiyama ◽  
Sho Saeki ◽  
Hiroyuki Sano ◽  
Takashi Iwanaga ◽  
...  

AbstractSome patients with idiopathic pulmonary fibrosis (IPF) undergo recurrent acute exacerbations (AEs). This study aimed to elucidate the risk factors for recurrent AEs of IPF (AE-IPF). Consecutive patients with IPF admitted for their first AE-IPF between January 2008 and December 2018 were retrospectively recruited. Of 63 patients admitted for an AE-IPF and discharged alive, 9 (14.3%) developed a recurrence of AE within 1 year. The mean time to recurrence was 233 ± 103 days. Total doses (mg/month and mg/kg/month) of corticosteroids administered over day 1 to 30 after the AE were significantly higher in patients without recurrences of AE-IPF (5185 ± 2414 mg/month, 93.5 ± 44.0 mg/kg/month) than the doses in patients with recurrences (3133 ± 1990 mg/month, 57.2 ± 37.7 mg/kg/month) (p = 0.02 and p = 0.03, respectively). However, no differences were observed between the total doses of corticosteroids administered over days 31 to 60, 61 to 90, 91 to 120, and 151 to 180 after the AE. Furthermore, differences between the administration rates of immunosuppressive and antifibrotic treatments administered to the 2 patient groups were not significant. An increased total dose of corticosteroid administered over day 1 to 30 after an AE-IPF was associated with a decreased risk of subsequent recurrence of AE-IPF within 1 year after the first AE.


2020 ◽  
Vol 2020 ◽  
pp. 1-11
Author(s):  
Xiaozheng Wu ◽  
Wen Li ◽  
Zhenliang Luo ◽  
Yunzhi Chen

Objective. To systematically review the efficacy and safety of Ligustrazine in the treatment of idiopathic pulmonary fibrosis (IPF). Methods. The electronic literature databases (PubMed, EMbase, CNKI, WanFang database, and VIP) were retrieved through a computer to find out the randomized controlled trials (RCT) of Ligustrazine in the treatment of IPF according to the inclusion/exclusion criteria screening test. Cochrane’s bias risk table was also used to evaluate the quality of the study and to extract effective data. RevMan 5.3 was used for statistical analysis. Results. A total of 7 RCTs (a total of 366 patients, including 196 in experimental and 170 in control group). Compared with the control group, Ligustrazine could improve the clinical symptoms ([OR] = 2.20, 95% CI [1.40, 3.46], P = 0.0006 ), lung function (VC % [MD] = 3.92, 95% CI [0.68, 7.17], P = 0.02 ), (TLC% [MD] = 4.94, 95% CI [0.37, 9.52], P = 0.03 ), the pulmonary diffusion function (DLCO % [MD] = 9.12, 95% CI [5.70, 12.55], P < 0.00001 ), and arterial blood gas analysis (PaO2 [MD] = 7.11, 95% CI [1.96, 12.25], P = 0.007 ) (PaCO2 [MD] = −2.42, 95% CI [−4.36, −0.49], P = 0.01 ) of IPF patients, respectively. However, FEV1/FVC % ([MD] = 9.37, 95% CI [−1.23, 19.97], P = 0.08 ) and adverse reactions ([MD] = 0.35, 95% CI [0.02, 5.36], P = 0.45 ) were not significantly improved. Conclusion. Ligustrazine has certain clinical efficacy in the treatment of IPF, but the safety of applying it and the adverse reactions need to be further analyzed and determined. It can be considered as a new alternative and complementary medicine to be promoted and recommended for use in medical units in various countries in the world and it solved the difficult problem of conventional drug treatment of IPF; therefore, more research strength can be put in the treatment of the pathological mechanism of IPF for further exploration. The study was registered under registration number CRD42020193626.


2008 ◽  
Vol 53 (No. 5) ◽  
pp. 212-218 ◽  
Author(s):  
E. Straková ◽  
P. Suchý ◽  
I. Herzig ◽  
V. Šerman ◽  
N. Mas

120 selected layers (Bovans Goldline hybrid) aged 19 weeks with an average weight of 1 735 g were divided into two balanced groups: control group (Group C) and experimental (Group E). Layers were reared in three-tier cages with automatic watering, manual feeding, and at controlled light and temperature regimens. One cage accommodated two layers, the floor surface area was 0.1125 m<sup>2</sup> per layer. The experiment started after a 20-day adaptation period with layers aged 22 weeks (Week 22) and ended when layers were 68 weeks old (Week 68). In a period of initiation (i.e. Weeks 19–38), layers were fed the complete feeding mixture N1. Then they received the feeding mixture N2 until the end of the experiment. Feeding mixtures in both groups (C and E) had the same composition; the only difference between mixtures was that the feeding mixture in the experimental group was enriched with 1% clinoptilolite (i.e. the commercially available additive ZeoFeed). Layers received feeding mixtures and drinking water <I>ad libitum.</I> In the course of the experimental period, control layers laid 16 289 eggs while experimental layers laid 16 474 eggs. It follows from the results that the laying intensity in experimental layers was 1.7% higher as compared to control layers, i.e. the number of laid eggs in experimental layers increased by 5.6 eggs per layer. The mean weight of all laid eggs was 66.3 ± 6.25 g in the control group and 65.6 ± 5.44 g in the experimental group (<I>P</I> ≤ 0.01). Such performance was achieved at the consumption of feeding mixture being 141.7 g per laid egg in the control group and 137.6 g per laid egg in the experimental group. The consumption of feeding mixture in the experimental group was 4.1 g lower than that in the control group. The mean values of parameters monitored in blood plasma such as uric acid, cholesterol, glucose, lactose, calcium, phosphorus, ALP, and LDH in both groups of layers ranged within reference intervals, with no significant differences being detected between both groups.However, statistically significant differences between both groups were found in total plasma protein (<I>P</I> ≤ 0.01), triacylglycerol levels (<I>P</I> ≤ 0.05), and magnesium (<I>P</I> ≤ 0.01), which were elevated in the control group, and in AST (<I>P</I> ≤ 0.05) whose level in the control group was significantly lower than that in the experimental group. The results of haematological tests performed with layers’ blood revealed statistically significant changes in parameters such as the erythrocyte count (<I>P</I> ≤ 0.01), haemoglobin level (<I>P</I> ≤ 0.01), and MCHC (<I>P</I> ≤ 0.05), which were elevated in the experimental group, and in the leukocyte count (<I>P</I> ≤ 0.05), which was lower in the experimental group, as compared with the control. However, the values found varied within physiological ranges.


CHEST Journal ◽  
1995 ◽  
Vol 108 (1) ◽  
pp. 109-115 ◽  
Author(s):  
Jonathan B. Orens ◽  
Ella A. Kazerooni ◽  
Fernando J. Martinez ◽  
Jeffrey L. Curtis ◽  
Barry H. Gross ◽  
...  

2011 ◽  
Vol 2011 ◽  
pp. 1-7 ◽  
Author(s):  
Mostafa Ghanei ◽  
Marco Chilosi ◽  
Hassan Mohammad Hosseini Akbari ◽  
Rouzbeh Motiei-Langroudi ◽  
Ali Amini Harandi ◽  
...  

We performed a pathologic study with further using an immunohistochemical technique (using anti-p63 and anti-CK5) on tissues obtained by open lung biopsy from 18 patients with previous exposure to sulphur mustard (SM) as case group and 8 unexposed patients (control group). The most frequent pathologic diagnosis was constrictive bronchiolitis (44.4%), followed by respiratory (22.2%) and chronic cellular bronchiolitis (16.7%) in the case group, and hypersensitivity bronchiolitis (50%) in the control group. The pathologic diagnoses were significantly different in the case and control groups (P=0.042). In slides stained by anti-p63 and anti-CK5, the percent of stained cells and the mean number of epithelial cells were lower in the case group in comparison to the control group. This difference was significant for the mean number of cells stained by anti-CK5 (P=0.042). Furthermore, there was a significant correlation between pathologic diagnosis and total number of cells and mean number of cells stained with anti-p63 and anti-CK5 (P  value = 0.002, <0.001, 0.044). These results suggest that constrictive bronchiolitis may be the major pathologic consequence of exposure to SM. Moreover, decrease of p63 in respiratory tissues affected by SM may suggest the lack of regenerative capacity in these patients.


2021 ◽  
Vol 12 ◽  
Author(s):  
Yiliang Su ◽  
Li Shen ◽  
Fen Zhang ◽  
Xing Jiang ◽  
Xiaofeng Jin ◽  
...  

Background: Patients with idiopathic pulmonary fibrosis (IPF) often have irritating persistent dry cough. Possible correlations between dry cough and laryngopharyngeal reflux (LPR) remain unclear.Methods: 44 patients with IPF and 30 healthy individuals underwent 24 h laryngopharyngeal pH monitoring. Ryan index score was calculated. Patients’ demographic and clinical data were collected.Results: 44 patients with IPF and 30 healthy individuals were included. The proportions of men and smokers were significantly higher in IPF group than control group (All p &lt; 0.01). The average laryngopharyngeal pH value for 24 h was similar in the IPF (7.11 ± 0.08) group and control group (7.09 ± 0.06). According to the percentage duration of pH &lt; 6.5, pH6.5–7.5, and pH &gt; 7.5 in the overall measure duration, the patients were classified into three pH groups. In entire pH monitoring duration, the proportion of pH &gt; 7.5 group in IPF patients was higher than control group; at upright position, the proportion of pH &gt; 7.5 group in IPF patients was higher than control group; at supine position, the proportion of pH &lt; 6.5 group in IPF patients was higher than control group (All p &lt; 0.01). Seven patients had Ryan index score&gt;9.41 at upright position. All patients had Ryan index score&lt;6.79 at supine position. Four patients showed significantly higher and one patient had significantly lower average pH at coughing than the overall average pH (All p &lt; 0.05).Conclusions: Patients with IPF may have LPR. Basic and acidic LPR may likely occur at upright and supine position, respectively. Ryan index may not accurately reflect LPR in patients with IPF.


2020 ◽  
Vol 5 (2) ◽  
pp. 35-42
Author(s):  
Shehab Al-Ansari ◽  
Allen Borowski ◽  
Ali Fuad ◽  
Omar Alawadhi ◽  
Haris Riaz ◽  
...  

AbstractAim: Pirfenidone is a novel anti-fibrotic agent utilized in the treatment of idiopathic pulmonary fibrosis (IPF). It has been implicated in mitigating myocardial fibrosis and left ventricular (LV) systolic and diastolic dysfunction in animal models. However, its impact on LV mechanics in humans remains unknown. The aim of this study was to retrospectively evaluate the effects of pirfenidone on echocardiographic parameters of LV function and structure in patients with IPF.Methods: A total of 124 patients with IPF were included in this study: 64 patients treated with pirfenidone (treatment group) and 60 patients not taking pirfenidone (control group), who had serial pretreatment/baseline and posttreatment/follow-up echocardiograms done within a time frame of four years. Changes in the means of parameters of LV function (systolic, diastolic, and global longitudinal strain) and LV structure (mass and volume indices) were compared between the treatment and control groups. This was followed by a subgroup analysis that included only 88 patients (47 treated, 41 controls) with echocardiographic evidence of myocardial dysfunction at baseline (defined as an ejection fraction of ≤45, or diastolic dysfunction stage 1 or more) in addition to a known clinical diagnosis of congestive heart failure. To account for potential confounders, a secondary adjusted analysis by way of 1:1 propensity score matching (PSM) was carried out. This yielded a sample consisting of 62 patients with 56 patients in the subgroup cohort.Results: Patients in the treatment group were significantly younger (69.4 vs. 77 years, p<0.001) and had relatively lower forced vital capacity (69.9% vs. 80.6%, p = 0.005) in comparison to the control group. However, after PSM, the age demographics were comparable between both groups (72.18 vs. 72.15, p = 0.9). In the primary unadjusted analysis, there was no statistically significant change in any of the mean parameters of LV function and structure after pirfenidone administration when compared to the control group. Furthermore, no significant differences were noted in the subgroup cohort. Such findings were re-demonstrated after a secondary analysis with PSM.Conclusion: From an echocardiographic perspective, pirfenidone had no significant effects on LV structure and function in patients with IPF, even in patients with more overt cardiac dysfunction.


2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Stéphane Jouneau ◽  
Bruno Crestani ◽  
Ronan Thibault ◽  
Mathieu Lederlin ◽  
Laurent Vernhet ◽  
...  

Abstract Background Nintedanib is an approved therapy for idiopathic pulmonary fibrosis (IPF). Some patients treated with nintedanib experience weight loss. Exploratory data suggest that low body mass index or weight loss are associated with worse outcomes in patients with IPF. We investigated whether BMI at baseline or weight loss over 52 weeks was associated with FVC decline, or influenced the effect of nintedanib, in patients with IPF. Methods Using pooled data from the two INPULSIS trials, we analysed the rate of decline in FVC (mL/yr) over 52 weeks in patients treated with nintedanib and placebo in subgroups by baseline BMI (< 25; ≥25 to < 30; ≥30 kg/m2) and by weight loss over 52 weeks (≤5; > 5%) using random coefficient regression. Results In the placebo group, the mean rate of FVC decline over 52 weeks was numerically greater in patients with lower baseline BMI (− 283.3 [SE 22.4], − 207.9 [20.9] and − 104.5 [21.4] in patients with BMI < 25 kg/m2, ≥25 to < 30 kg/m2 and ≥ 30 kg/m2, respectively). Nintedanib reduced the rate of FVC decline versus placebo in all subgroups by BMI, with a consistent treatment effect across subgroups (interaction p = 0.31). In the placebo group, the mean rate of FVC decline was numerically greater in patients with > 5% than ≤5% weight loss over 52 weeks (− 312.7 [SE 32.2] versus − 199.5 [SE 14.4] mL/year). Nintedanib reduced the rate of FVC decline versus placebo in both subgroups by weight loss, with a greater treatment effect in patients with > 5% weight loss (interaction p = 0.0008). The adverse event profile of nintedanib was similar across subgroups. Conclusions In patients with IPF, lower BMI and weight loss may be associated with faster decline in FVC. Nintedanib reduces the rate of FVC decline both in patients who lose weight on treatment and those who do not. Trial registration ClinicalTrials.gov; Nos. NCT01335464 and NCT01335477; URL: www.clinicaltrials.gov.


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