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