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What is ILD?

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What is ILD?

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fibroblast_activity_1Interstitial Lung Disease (ILD) is a heterogeneous group of non-infectious, non-malignant diffuse parenchymal disorders of the lower respiratory tract, affecting the alveolar wall structure and often small airways and blood vessels of the lung parenchyma. The most important of these is the idiopathic pulmonary fibrosis.

There are more than 300 different types of ILDs present. For practical purposes, they are broadly classified into either those with an identifiable cause or idiopathic i.e. without a known etiology. Known causes include genetics/hereditary, drug-induced, collagen vascular related, hypersensitivity pneumonitis and occupational exposures such as asbestos and silica. Etiology in a majority of cases remains unidentified and such ILD types include idiopathic pulmonary fibrosis, non-specific interstitial pneumonias, sarcoidosis, respiratory bronchiolitis ILD and so on.

Usual specific symptoms include difficulty in breathing, especially on exertion and persistent dry cough, but these may vary. In addition, systemic signs and auscultatory findings help to guide clinically towards the diagnosis of ILD. Diagnostic investigations include imaging studies such as chest x-ray and high resolution computed tomography (HRCT) of the chest. Each type of ILD manifests a different pattern on the HRCT. Pulmonary function tests (PFTs) typically show a restrictive pattern and a ventilatory defect. However, confirmation of diagnosis warrants a biopsy of the lung tissue. Ideally, the diagnosis of ILD should be based on a multidisciplinary approach involving a pulmonologist, radiologist and histopathologist.

Current therapy includes new anti-fibrotic agents aimed at slowing the progression of the disease. Many clinical trials are underway, raising hope of having better treatment options in the future for patients with ILD.

Epidemiology

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Epidemiology of the ILDs has always been uncertain. As in the beginning these were not understood and identified for a long time until the advent of HRCT, when more clear identification and classifications could be made. A landmark study in the epidemiology of ILD was the first registry in Bernalillo County, New Mexico was published in 1994 and then much later a number of registries in major European countries were established. However, the results of these registries and individual research work remained confounded until 2011, when definitions and identifiable clinical features became more clear and stratified, leading to the possibility of a global approach of identification and classification of the disease.

The European registries in Italy, Belgium, Germany, Spain, Greece, Finland, Denmark added invaluable information to the epidemiology of the subject. This led to the information that various types of ILD had different incidences and prevalences in these registries.

The global data available estimates the prevalence of all ILDs in the US at about 500,000 out of which IPF afflicts between a 132000 to 200000 people. Approximately 50,000 new cases are diagnosed each year and as many as 40,000 people die from IPF each year. Another recent study estimates IPF affects 1 out of 200 adults over the age of 65 in the United States. The current estimate of the incidence of IPF in the EU is between 30,000 to 40,000 people and in the UK more than 5,000 new cases are diagnosed each year. Data from a local registry suggests that the burden of ILD stands at 4.1% of all pulmonary referrals and IPF constitutes 33% of these. Extrapolating the latest US estimation of prevalence, it can be assumed that there are 67,000 IPF patients over the age of 55 years in Pakistan. The total estimated ILD population would hence be much higher.

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Epidemiology

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+ Interstitial Lung Disease (ILD)

What is ILD?

null

fibroblast_activity_1Interstitial Lung Disease (ILD) is a heterogeneous group of non-infectious, non-malignant diffuse parenchymal disorders of the lower respiratory tract, affecting the alveolar wall structure and often small airways and blood vessels of the lung parenchyma. The most important of these is the idiopathic pulmonary fibrosis.

There are more than 300 different types of ILDs present. For practical purposes, they are broadly classified into either those with an identifiable cause or idiopathic i.e. without a known etiology. Known causes include genetics/hereditary, drug-induced, collagen vascular related, hypersensitivity pneumonitis and occupational exposures such as asbestos and silica. Etiology in a majority of cases remains unidentified and such ILD types include idiopathic pulmonary fibrosis, non-specific interstitial pneumonias, sarcoidosis, respiratory bronchiolitis ILD and so on.

Usual specific symptoms include difficulty in breathing, especially on exertion and persistent dry cough, but these may vary. In addition, systemic signs and auscultatory findings help to guide clinically towards the diagnosis of ILD. Diagnostic investigations include imaging studies such as chest x-ray and high resolution computed tomography (HRCT) of the chest. Each type of ILD manifests a different pattern on the HRCT. Pulmonary function tests (PFTs) typically show a restrictive pattern and a ventilatory defect. However, confirmation of diagnosis warrants a biopsy of the lung tissue. Ideally, the diagnosis of ILD should be based on a multidisciplinary approach involving a pulmonologist, radiologist and histopathologist.

Current therapy includes new anti-fibrotic agents aimed at slowing the progression of the disease. Many clinical trials are underway, raising hope of having better treatment options in the future for patients with ILD.

+ Epidemiology

Epidemiology

null

Epidemiology of the ILDs has always been uncertain. As in the beginning these were not understood and identified for a long time until the advent of HRCT, when more clear identification and classifications could be made. A landmark study in the epidemiology of ILD was the first registry in Bernalillo County, New Mexico was published in 1994 and then much later a number of registries in major European countries were established. However, the results of these registries and individual research work remained confounded until 2011, when definitions and identifiable clinical features became more clear and stratified, leading to the possibility of a global approach of identification and classification of the disease.

The European registries in Italy, Belgium, Germany, Spain, Greece, Finland, Denmark added invaluable information to the epidemiology of the subject. This led to the information that various types of ILD had different incidences and prevalences in these registries.

The global data available estimates the prevalence of all ILDs in the US at about 500,000 out of which IPF afflicts between a 132000 to 200000 people. Approximately 50,000 new cases are diagnosed each year and as many as 40,000 people die from IPF each year. Another recent study estimates IPF affects 1 out of 200 adults over the age of 65 in the United States. The current estimate of the incidence of IPF in the EU is between 30,000 to 40,000 people and in the UK more than 5,000 new cases are diagnosed each year. Data from a local registry suggests that the burden of ILD stands at 4.1% of all pulmonary referrals and IPF constitutes 33% of these. Extrapolating the latest US estimation of prevalence, it can be assumed that there are 67,000 IPF patients over the age of 55 years in Pakistan. The total estimated ILD population would hence be much higher.

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Epidemiology

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