Idiopathic Pulmonary Fibrosis: A Study of 46 Patients from Western India: Clinical Presentations and Survival

Natarajan, S.; Subramanian, Poonam

doi:10.5152/ttd.2015.4584

Cited by 4 publications

(3 citation statements)

References 29 publications

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“…In the present study we found that patients with NSIP had a better survival as compared to patients with IPF. 14 Female predominance was seen in our study which is in contrast with earlier studies 4,8,9,11,[15][16][17] almost all other studies on NSIP; except in the study by Travis et al 5 which showed a male predominance (15 out of 22). The recent systematic review of 12 studies by Travis et al 16 showed that the mean age at presentation was 52 years with age ranged from 26 to 73 years.…”

Section: Discussioncontrasting

confidence: 96%

Clinical Profile of Idiopathic Non-specific Interstitial Pneumonia: A Retrospective Study from Western India

Natarajan¹,

Subramanian²

2022

The Indian Journal of Chest Diseases and Allied Sciences

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Background. Non-specific interstitial pneumonia (NSIP) has now been accepted to be a distinct clinical entity. However, very limited data is available in western Indian population regarding its clinical presentation, treatment options and survival of patients diagnosed with idiopathic NSIP.Methods. A retrospective, observational analysis of clincial, radiological, treatment and survival of patients data collected from hospital records who were diagnosed with idiopathic NSIP over the six years was carried out.Results. Out of a total of 146 patients of interstitial lung disease (ILD), 46 (31.5%) patients diagnosed with idiopathic non-specific interstitial pneumonia were included in the study. There were 12 male patients. Mean age of female was 60.6 years and 60.5 years for male patients. Clubbing was seen in 12 (26%) patients. Fifteen (32.6%) patients were on anti-tuberculosis medications prior to the diagnosis of idiopathic NSIP. The mean forced vital capacity (FVC) in the present study was 56%. High resolution computed tomography (HRCT) of the chest revealed reticulation with basal predominance in 35 (76%) patients and ground-glass opacities in 9 (19%). Median survival from initial visit was 29.6 months; while that from symptom onset was 46.2 months (p=0.03). Stabilisation of disease was seen in 26 (56.5%). Twenty patients died during the study period.Conclusions. Idiopathic NSIP is a disease of the elderly with female predominance with a five-year mortality of 25%. Late presentation worsened the prognosis.

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Section: Discussioncontrasting

confidence: 96%

Clinical Profile of Idiopathic Non-specific Interstitial Pneumonia: A Retrospective Study from Western India

Natarajan¹,

Subramanian²

2022

The Indian Journal of Chest Diseases and Allied Sciences

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“…These findings are similar to those found in existing articles from both developing and developed countries. [13,14,[35][36][37] While baseline FVC and FEV1 in this cohort were similar to those found by Collard et al, [37] RV and TLCO were markedly lower in our patients. This adds further evidence to the presence of advanced disease on presentation.…”

Section: Discussionsupporting

confidence: 91%

Idiopathic pulmonary fibrosis in Soweto, South Africa: A descriptive study

Aitchison

Blydenstein

Wong

2023

JPATS

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Objectives: Idiopathic pulmonary fibrosis (IPF) is a specific form of age-related fibroproliferative interstitial pneumonia that is chronic, progressive, and carries a poor prognosis, with median survival of just 2.5–3.5 years from diagnosis. The exact etiology is unknown, but smoking is known to be risk factor. Symptoms and signs include progressive dyspnoea, cough, inspiratory “Velcro” crackles, and clubbing. At present, treatment options are limited; but include pulmonary rehabilitation, long-term domiciliary oxygen therapy, and the conditionally recommended pharmacological therapies pirfenidone and nintedanib. This study sought to describe the cohort of patients that attended the respiratory outpatient services at a tertiary-level hospital in South Africa during the period 2007– 2016. To the best of the authors’ knowledge, this is the first such descriptive study performed in Africa. Materials and Methods: This was a retrospective, descriptive, and record review, that included patients ≥18 years of age who fulfilled 2011 ATS/ERS/JRS/ALAT diagnostic criteria for IPF. Results: Data from 74 patients were used for analysis in this study, of which 60.8% were female. The mean age standard deviation was 64.4 (10.9) years and the majority (79.7%) were Black. Over half of the patients (40/74, 54.1%) were current or previous smokers, although there was no correlation between smoking history and age or baseline pulmonary function testing. All patients reported dyspnea, which was modified Medical Reseach Council (mMRC) Grade 3 or 4 in 80% of patients. High resolution computed tomography chest was reported as radiological usual interstitial pneumonia (UIP) in 72 patients (97.3%) and three patients underwent lung biopsy, all of which showed a UIP pattern. Fifty-eight patients (78.4%) had spirometry results available, with median forced vital capacity 67.3% of predicted; this was significantly higher in females. Median transfer factor of the lung for carbon monoxide was 39% predicted. Twenty-five patients (33.8%) received corticosteroids, of whom five (6.8%) received the prednisone-azathioprine-N-acetylcysteine regime. Three patients (4.1%) received nintedanib; two of whom showed slowing of decline in lung function, although no significant symptomatic improvement was reported. Mean duration of follow-up was 13.3 months, although females had significantly longer duration of follow-up than males. Conclusion: Despite a fairly small sample size and retrospective nature, this study contributes to the body of literature on IPF and highlights the need for additional studies in developing countries, particularly in Africa.

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“…It was observed, for example, in a study from western India, that the median delay in diagnosis of IPF was 20 months, and >20% of patients had wrongly received anti-tuberculosis drugs before they were diagnosed with IPF. [ 10 ] These observations indicate the need to increase awareness of IPF and other ILDs. General practitioners are usually the first point of medical contact and should be trained adequately to differentiate between ILDs from other respiratory disorders, to allow prompt referral of such patients to expert centers.…”

Section: Discussionmentioning

confidence: 99%

Real world efficacy and safety of nintedanib in idiopathic pulmonary fibrosis: A single center, observational study from India

Talwar¹,

Prajapat²,

Talwar³

2022

Lung India

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Background: Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis (IPF) has been established by multiple clinical trials. This study aims to assess the efficacy and safety of nintedanib in real-world IPF patients in India. Methods: Clinical records of IPF patients (prescribed with nintedanib) visiting tertiary pulmonary care center, between June 2016 and December 2019, were analyzed retrospectively. Data were analyzed for forced vital capacity (FVC), Diffusing capacity of lung for carbon monoxide(DLCO), 6-min walk distance (6-MWD). Acute exacerbations and adverse events were also analyzed. Results: A total of 76 IPF patients were prescribed with nintedanib. Drug was prescribed at 100 and 150 mg BD dose to 37 and 39 patients. Ten patients (13.1%), of which eight were over the age of 60 years, died during the study period. Only 42 patients visited for follow-up. Mean baseline FVC was 1.67 L and mean annualized absolute change in FVC and FVC % predicted was −0.07 L and −1.80%, respectively. Mean baseline DLCO was 37.21% and mean annualized absolute change in DLCO % predicted was-2.20%. At follow-up, 1 (2.38%), 17 (40.47%), and 24 (57.14%) patients were at Deparatment of Internal Medicine stage I, II, and III, respectively. Acute exacerbations and adverse events were reported by 48 and 6 patients, respectively. Conclusion: Our results support the findings from previous studies, that nintedanib leads to annual decline in parameters such as FVC and DLCO and increased 6-MWD. It was found to be well tolerated in the Indian patients with IPF.

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Idiopathic Pulmonary Fibrosis: A Study of 46 Patients from Western India: Clinical Presentations and Survival

Cited by 4 publications

References 29 publications

Clinical Profile of Idiopathic Non-specific Interstitial Pneumonia: A Retrospective Study from Western India

Clinical Profile of Idiopathic Non-specific Interstitial Pneumonia: A Retrospective Study from Western India

Idiopathic pulmonary fibrosis in Soweto, South Africa: A descriptive study

Real world efficacy and safety of nintedanib in idiopathic pulmonary fibrosis: A single center, observational study from India

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