National Registry of Interstitial Lung Disease from Pakistan

Zubairi, Ali Bin Sarwar; Ansarie, Mosavir; Mahmud, Talha; Ashraf, Saadia; Rao, Nisar Ahmed; Javaid, Arshad; Shaheen, Zubair; Khan, Suhail Ahmad; Khan, Afzaalullah

doi:10.7759/cureus.14684

Cited by 3 publications

(5 citation statements)

References 14 publications

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“…Among the enrolled IIPs, IPF (36.4%) was the most frequent pathological variant. This is relatively similar to the data from some registries (18.9-38.6%) [6,37,47,[51][52][53][55][56][57][58]. Importantly, 14.6% of the IPAF entity has a UIP pattern based on HRCT.…”

Section: Discussionsupporting

confidence: 85%

“…These data represent the results of a single center, while certain registers relating to multicenter studies [6,37,45,47,49,[51][52][53][54][55][56][57][58][59] have showed the existence of differences in the plans, number, and duration of studies, however, some extrapolations can still be made. Indeed, IPF and sarcoidosis were the most common diseases in some registries [37,47,49,[52][53][54]60,61], which differs from our results.…”

Section: Discussionmentioning

confidence: 99%

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Clinical and Functional Characteristics of Interstitial Lung Disease in Algeria: A Single-Center Prospective Study

Ketfi,

Selatni,

Djouadi

et al. 2023

JoR

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Introduction: There are a limited number of epidemiological studies describing the global burden of chronic diffuse interstitial lung diseases (ILD) and their subtypes’ heterogeneity worldwide. Our main is to characterize new-onset ILDs in Algeria and compare our results with data from other populations. Materials and Methods: Newly diagnosed ILDs were prospectively collected in a single-center observational cohort study including all patients diagnosed as ILDs in the pulmonology, phthisiology, and allergology departments between 2015 and 2019. Detailed anamnestic and clinical data were collected at the time of diagnosis. The results of high-resolution computed tomography (HRCT), serological tests, biology data, and respiratory functional exploration were systematically performed and collected. Results: A total of 455 cases were included. The mean age was 59.4 ± 13.2 years. There was a slight predominance of females (300; 65.9%). The most common disease was ILD secondary to connective tissue disease (CTD) or ILD-CTD (48.1%), followed by idiopathic interstitial pneumonias (IIPs) (23.5%), sarcoidosis (16.9%), interstitial pneumonia with autoimmune features (IPAF) (12.1%), and hypersensitivity pneumonitis (HP) (2.4%). Idiopathic pulmonary fibrosis (IPF) was present in 8.6% and unclassifiable ILD in 4.6% of the total ILD cases. Conclusions: ILD-CTD, IIP, and sarcoidosis were the most frequently observed ILDs in this Algerian population. Similarities and many differences were found compared to previous data from other countries.

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Section: Discussionsupporting

confidence: 85%

Section: Discussionmentioning

confidence: 99%

Clinical and Functional Characteristics of Interstitial Lung Disease in Algeria: A Single-Center Prospective Study

Ketfi,

Selatni,

Djouadi

et al. 2023

JoR

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“…Baseline characteristics of these patients are recorded in Table . Majority of the patients were males 49(62.8%), and the average age was 69.1±9.7 years, which is similar to other regional and international data. 1,4,5 A total of 51 (65.4%) patients were non-smokers, 25 (32.1%) were ex-smokers, and only 2 (2.6%) were current smokers. These observations were incongruent with most literature, which report that a majority of patients with IPF have a history of cigarette smoking.…”

Section: Methods and Resultsmentioning

confidence: 99%

Challenges in the management of idiopathic pulmonary fibrosis from a low- and middle-income country

Chundrigar,

Ali Khan,

Ansari

et al. 2024

J Pak Med Assoc

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Idiopathic pulmonary fibrosis (IPF) is the most common progressive form of interstitial lung disease (ILD) that leads to gradual deterioration of lung function and ultimately death. Data from low- and middle-income countries (LMIC) on IPF is scarce. In this communication, we report the challenges encountered in managing IPF from Pakistan’s largest tertiary care centre. A total of 108 patients with IPF were evaluated at the Aga Khan University Hospital in Karachi, Pakistan from January 2017 to March 2020. A significant concern was that most patients with IPF presented late during their disease. A bigger challenge encountered in clinical practice was the cost and non-availability of antifibrotic therapy in the country until mid-2020. Successfully addressing these limitations, it is anticipated that better care will be available for the patients suffering from IPF in this part of the world. Keywords: Interstitial Lung Disease, Pulmonary Fibrosis; Idiopathic Pulmonary Fibrosis, LMIC.

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“…However, this gender difference in IPF patients in our study is not unique. For example, a slight female predominance, or near equal gender distribution, were demonstrated in cohort studies from India (54% and 50%) [ 33 , 34 ], Saudi Arabia (51%), [ 35 ] and Pakistan (46%) [ 21 ]. On the one hand, heterogeneity of the patients in different registries should be regarded as one of registries’ strengths [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

“…The establishment of patient registries has enabled the collection of data from cohorts of patients with IPF with a broader spectrum of disease severity and comorbidities managed in clinical practice, thereby providing a better understanding of the real world behavior and impact of IPF [ 9 ]. In recent years, a number of IPF registries have been established to describe the epidemiology, the natural course, and the clinical management of real patient populations with IPF [ 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 ]. Data from patient registries have already improved knowledge of the clinical course and impact of IPF, and of diagnostic and treatment practices.…”

Section: Introductionmentioning

confidence: 99%

Russian Registry of Idiopathic Pulmonary Fibrosis: Clinical Features, Treatment Management, and Outcomes

Чикина

Черняк

Merzhoeva

et al. 2023

Life

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A registry of patients with idiopathic pulmonary fibrosis (IPF) was founded in Russia in 2016. The aim of this study was to analyze the demographic, clinical, functional, radiological, and morphological data of the patients included in this registry. Methods. This was a prospective multicenter, observational, non-interventional study. Patients’ risk factors, demographics, clinical data, results of high-resolution computed tomography (HRCT) of the chest and pulmonary function testing, and lung tissue biopsy findings were analyzed. We also analyzed the exercise tolerance (6-min walking test) of patients, serological markers of systemic connective tissue diseases, treatment, clinical course, and outcomes of the disease. Multidisciplinary discussion (MDD) was used as needed. Results. One thousand three hundred and fifty-three patients were included in the registry from 2016 to 2020. The mean age was 64.4 ± 10.7 years, most patients were active smokers or ex-smokers. Antifibrotic therapy was administered to 90 of 948 patients (9.5%). Since starting the registry in 2016, the incidences of IPF have increased and the time period from manifestation of the disease to making the diagnosis has shortened, the number of patients on antifibrotic therapy has increased and the number of patients taking systemic steroids decreased. Conclusion. The registry of patients with IPF was helpful to improve IPF diagnosis and to implement antifibrotic agents in clinical practice. Further analysis of the clinical course and prognostic markers of IPF in the Russian population is needed. An analysis of the long-term efficacy of antifibrotic therapy in this population is also important.

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National Registry of Interstitial Lung Disease from Pakistan

Cited by 3 publications

References 14 publications

Clinical and Functional Characteristics of Interstitial Lung Disease in Algeria: A Single-Center Prospective Study

Clinical and Functional Characteristics of Interstitial Lung Disease in Algeria: A Single-Center Prospective Study

Challenges in the management of idiopathic pulmonary fibrosis from a low- and middle-income country

Russian Registry of Idiopathic Pulmonary Fibrosis: Clinical Features, Treatment Management, and Outcomes

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