Short-Term Efficacy of Steroid and Immunosuppressive Drugs in Patients with Idiopathic Pulmonary Fibrosis and Pre-treatment Factors Associated with Favorable Response

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.

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High-flow nasal cannula oxygen therapy to treat acute respiratory failure in patients with acute exacerbation of idiopathic pulmonary fibrosis

Therapeutic Advances in Respiratory Disease ◽

10.1177/1753466619847130 ◽

2019 ◽

Vol 13 ◽

pp. 175346661984713 ◽

Cited By ~ 5

Author(s):

Andrea Vianello ◽

Giovanna Arcaro ◽

Beatrice Molena ◽

Cristian Turato ◽

Fausto Braccioni ◽

...

Keyword(s):

Respiratory Failure ◽

Mortality Rate ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Oxygen Therapy ◽

Treatment Algorithm ◽

High Flow ◽

High Flow Nasal Cannula ◽

Short Term ◽

Short Term Mortality

Background: Some patients with idiopathic pulmonary fibrosis (IPF) develop acute exacerbation (AE-IPF) leading to severe acute respiratory failure (ARF); despite conventional supportive therapy, the mortality rate remains extremely high. The aim of this study was to assess how a treatment algorithm incorporating high-flow nasal cannula (HFNC) oxygen therapy affects the short-term mortality of patients with AE-IPF who develop ARF. Method and design: A retrospective cohort analysis was conducted. Patients and interventions: The study consisted of 17 patients with AE-IPF admitted to a respiratory intensive care unit (RICU) for ARF managed using a treatment algorithm incorporating HFNC. The outcome measure was mortality rate during their stay in the RICU. Results: Implementation of the treatment algorithm led to a successful outcome in nine patients and to a negative one in eight patients (47.1%) who died within 39 days of being admitted to the RICU. The survival rate was 70.6% (±0.1 %) at 15 days, 52.9% (±0.1%) at 30 days, 35.3% (±0.1%) at 90 days, and 15.6% (±9.73 %) at 365 days. Overall, 4 out of 10 patients who did not respond to conventional oxygen therapy showed a satisfactory response to HFNC. Conclusions: Short-term mortality fell to below 50% when a treatment algorithm incorporating HFNC was implemented in a group of patients with AE-IPF admitted to a RICU for ARF. Patients not responding to conventional oxygen therapy seemed to benefit from HFNC. The reviews of this paper are available via the supplementary material section.

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Factors associated with the use of corticosteroids in the initial management of idiopathic pulmonary fibrosis

Pharmacoepidemiology and Drug Safety ◽

10.1002/pds.1959 ◽

2010 ◽

Vol 19 (7) ◽

pp. 756-762 ◽

Cited By ~ 5

Author(s):

Jeffrey C. Munson ◽

Maryl Kreider ◽

Zhen Chen ◽

Jason D. Christie ◽

Stephen E. Kimmel

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Initial Management ◽

Factors Associated

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Short-term outcomes of lung transplant in idiopathic pulmonary fibrosis

European Surgery ◽

10.1007/s10353-011-0618-6 ◽

2011 ◽

Vol 43 (5) ◽

pp. 284-294 ◽

Cited By ~ 1

Author(s):

A. Tk. Teo ◽

R. Pietrobon ◽

J. Atashili ◽

D. Rajgor ◽

J. Shah ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lung Transplant ◽

Short Term

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Short-term Clinical Experience on Interferon gamma-1b Therapy for Idiopathic Pulmonary Fibrosis

Tuberculosis and Respiratory Diseases ◽

10.4046/trd.2004.56.6.619 ◽

2004 ◽

Vol 56 (6) ◽

pp. 619

Author(s):

Jung Hye Hwang ◽

Man Pyo Chung ◽

Eun Hae Kang ◽

Kyung Chan Kim ◽

Byoung Hoon Lee ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Interferon Gamma ◽

Clinical Experience ◽

Short Term

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Short Term Outcomes of Pulmonary Rehabilitation in Idiopathic Pulmonary Fibrosis

CHEST Journal ◽

10.1378/chest.1991557 ◽

2014 ◽

Vol 146 (4) ◽

pp. 371A ◽

Cited By ~ 1

Author(s):

Nirmal Sharma ◽

Surya Bhatt ◽

Tracy Luckhardt ◽

Joao de Aandrade

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Pulmonary Rehabilitation ◽

Short Term

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Short-Term Effects of Comprehensive Pulmonary Rehabilitation and its Maintenance in Patients with Idiopathic Pulmonary Fibrosis: A Randomized Controlled Trial

Journal of Clinical Medicine ◽

10.3390/jcm9051567 ◽

2020 ◽

Vol 9 (5) ◽

pp. 1567

Author(s):

Inga Jarosch ◽

Tessa Schneeberger ◽

Rainer Gloeckl ◽

Michael Kreuter ◽

Marion Frankenberger ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Pulmonary Rehabilitation ◽

Controlled Trial ◽

Chronic Respiratory Disease ◽

Secondary Outcome ◽

Short Term ◽

Chronic Respiratory Disease Questionnaire

The recommendation for pulmonary rehabilitation (PR) in idiopathic pulmonary fibrosis (IPF) is weak with low-quality evidence. Therefore, the aim of this study is to investigate short-term PR effects and their maintenance after a 3-month follow-up. Fifty-four IPF patients were randomized into a group receiving a 3-week comprehensive, inpatient PR (n = 34, FVC: 74 ± 19% pred.) or usual care (UC) (n = 17, FVC: 72 ± 20%pred.). Outcomes were measured at baseline (T1), after intervention (T2), and 3 months after T2 (T3). A 6-min walk distance (6MWD) was used as the primary outcome and chronic respiratory disease questionnaire (CRQ) scores as the secondary outcome. Change in 6MWD from T1 to T2 (Δ = 61 m, 95% CI (18.5–102.4), p = 0.006) but not from T1 to T3 (∆ = 26 m, 95% CI (8.0–61.5), p = 0.16) differed significantly between groups. Higher baseline FVC and higher anxiety symptoms were significant predictors of better short-term 6MWD improvements. For the change in CRQ total score, a significant between-group difference from T1 to T2 (∆ = 3.0 pts, 95% CI (0.7–5.3), p = 0.01) and from T1 to T3 (∆ = 3.5 pts, 95% CI (1.5–5.4), p = 0.001) was found in favour of the PR group. To conclude, in addition to the short-term benefits, inpatient PR is effective at inducing medium-term quality of life improvements in IPF. PR in the early stages of the disease seems to provoke the best benefits.

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