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Interstitial Lung Diseases Last

Interstitial lung diseases (ILDs) are a diverse group of conditions characterized by inflammation and fibrosis of the lung interstitium, presenting with symptoms such as cough and exertional dyspnoea. Diagnosis involves clinical assessment, imaging, and sometimes serologic testing, with idiopathic pulmonary fibrosis being a notable type. Treatment options include antifibrotic drugs, pulmonary rehabilitation, and management of complications, with lung transplant considered for end-stage disease.
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0% found this document useful (0 votes)
26 views21 pages

Interstitial Lung Diseases Last

Interstitial lung diseases (ILDs) are a diverse group of conditions characterized by inflammation and fibrosis of the lung interstitium, presenting with symptoms such as cough and exertional dyspnoea. Diagnosis involves clinical assessment, imaging, and sometimes serologic testing, with idiopathic pulmonary fibrosis being a notable type. Treatment options include antifibrotic drugs, pulmonary rehabilitation, and management of complications, with lung transplant considered for end-stage disease.
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© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Interstitial lung diseases

BY

Shaimaa abdelghani elbialy


This heterogeneous group of conditions are
also referred to as diffuse parenchymal lung
diseases

They are characterized by varying degrees of


inflammation and fibrosis, initially affecting
the interstitium of the lung.

They typically presenting with exertional


dyspnoea, with or without cough.
Features common to the diffuse parenchymal
lung diseases

Clinical presentation
• Cough: usually dry, persistent and distressing
• Breathlessness: usually slowly progressive; insidious
onset; acute in some cases

Examination findings
• Crackles: typically bilateral and basal
• Clubbing: common in idiopathic pulmonary fibrosis
but also seen in other types, e.g. asbestosis
• Central cyanosis and signs of right heart failure in
advanced disease
Radiology

Chest X-ray: typically small lung volumes


with reticulo nodular shadowing but may be
normal in early or limited disease
High-resolution computed tomography
Pulmonary function Flow volume curve
 Typically restrictive (Restrictive pattern)
ventilatory defect with
reduced lung volumes
and impaired gas
transfer;
 Spirometry (Volume
time curve)
PATHOGENESIS

The ILDs are non malignant disorders and


are not caused by identified infectious agents.

The precise pathway(s) leading from injury to


fibrosis is not known

The two major histopathologic patterns are a


granulomatous pattern and a pattern in
which inflammation and fibrosis predominate.
Granulomatous Lung Disease

This process is characterized by an accumulation


of T lymphocytes, macrophages, and epithelioid
cells organized into discrete structures
(granulomas) in the lung parenchyma.

The granulomatous lesions can progress to


fibrosis. .

The Main differential diagnosis is between


sarcoidosis and hypersensitivity
pneumonitis
Inflammation and Fibrosis

The initial insult is an injury to the epithelial


surface that causes inflammation in the air
spaces and alveolar walls.

If the disease becomes chronic, inflammation


spreads to adjacent portions of the
interstitium and vasculature.
Idiopathic pulmonary fibrosis

is defined as a progressive fibrosing


interstitial pneumonia of unknown cause,
 occurring in adults and associated with the
histological or radiological pattern of UIP.
CT chest demonstrating idiopathic pulmonary fibrosis.
There is subpleural reticulation and honeycombing at both lung
bases
Diagnosis of interstitial lung disease (ILD)

History and clinical examination


Initial assessment
 Chest radiography
 Spirometry
Confirmation of ILD
 Computed tomography (CT) scan
 Serologic testing for autoantibodies
 Serum-specific IgG antibodies

Multidisciplinary team assessment


 Members from pulmonology and radiology
Assessment and treatment of ILD

Assess disease severity


 Pulmonary function testing
 6-min walk test
Exclude complications
 Echocardiogram
Treatment

Idiopathic pulmonary fibrosis

Antifibrotic drugs :Nintedanib

:
Pirfenidone
Treatment for disease complications

Pulmonary rehabilitation
Pulmonary hypertension
Respiratory failure: oxygen
End-stage disease: lung transplant
Management of symptoms (eg, cough and
breathlessness

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