Interstitial Lung Diseases

Interstitial Lung Diseases
Objectives:
1-Classification of ILD.
2-Pathogenesis of ILD.
3-clinical Features of ILD.
4-Investigation of ILD.
5-Common causes of ILD.
6-Treatment of ILD.
Introduction:
The term interstitial lung disease (ILD) refers to a heterogeneous group of
diffuse parenchymal infiltrative disorders that impair the gas exchange
function of the lung by disrupting the alveolar walls.
In many ILDs, lung regeneration fails and interstitial fibrosis resulting from
fibroblastic proliferation and excessive collagen deposition is the dominant
pathologic process as a reparative response to injury of the gas-exchanging
units.
However, nonfibrotic processes can also cause ILDs by abnormal infiltration
of the lung parenchyma (interstitium and/or alveolar spaces) by cells (e.g.,
eosinophils, histiocytes) or substances (e.g., amyloid). Thus ILDs comprise a
broad spectrum of disorders of varying etiology with some similarities in
clinical, imaging, physiologic, and pathologic features.
ETIOLOGIC FACTORS AND PATHOGENIC MECHANISMS (IPF)
A widely held hypothesis is that IPF develops in susceptible individuals

following some unknown stimulus that initiates an uncontrolled epithelial-
driven cascade of events that evolve to the fibrotic process. Several potential
risk factors for the development of IPF have been identified. Smoking presents
the most significant association with IPF, particularly for individuals who were
ever smokers, with odds ratios varying from 1.6 to 9.4.
The same association has been found in familial pulmonary fibrosis. Some
occupational and environmental exposures—primarily to wood and metal
dusts—have also shown increased association with IPF. The presence of Epstein-
Barr virus found in some patients has led to speculation that chronic viral
infection might play a role in the etiology of IPF, but this virus has also been
found in other fibrotic lung diseases, as well as in many controls.
A higher incidence of GER(Gastroesophageal reflux) has been found in
patients with IPF. GER is frequent, however, in the normal population, as well
as in patients with other advanced lung diseases, including cystic fibrosis,
COPD, and fibrosis associated with scleroderma.
The role of host genetic factors and their interactions with environmental
factors leading to IPF is being elucidated. The first gene association revealed
that a common polymorphism in the promoter region of the MUC5B gene
increased the risk for sporadic IPF as well as familial pulmonary fibrosis,
although the link with disease pathogenesis is unclear.Paradoxically, it has
been suggested that this polymorphism in MUC5B may be associated with
improved survival.
Clinical features:
Symptoms in many patients with ILDs are present for months to years (i.e.,
chronic) and progress at varying rates.
Persistent cough and progressively worsening exertional dyspnea are common
presenting symptoms but are nonspecific and can be a feature of many
pulmonary and nonpulmonary disorders.
The presence of extrapulmonary symptoms may lead to the diagnosis of
connective tissue disease–associated ILD.
Symptoms of gastroesophageal reflux (GER) may suggest aspiration-related ILD.
Investigations:
1-Taking a careful history is of paramount importance in identifying the
clinical manifestations and their temporal evolution as well as potential
cause for the lung disease including exogenous agents and hereditary
disorders.
Eliciting a history of tobacco use as well as of electronic cigarettes is
important. Most adults presenting with PLCH, respiratory bronchiolitis,
or desquamative interstitial pneumonia are smokers. Smoking is a risk
factor for rheumatoid arthritis-related ILD.A subset of acute
eosinophilic pneumonia is smoking-related. Conversely, hypersensitivity
pneumonitis infrequently appears in the active smoker.
The occupational and environmental history is of obvious importance. It
should be thorough and detailed because a long latency period (e.g.,
asbestosis) may occasionally exist between exposure and the appearance of
clinical impairment and disability. Hypersensitivity pneumonitis can manifest
either as recurrent acute or subacute pneumonitis or as an insidious chronic
form with slowly progressive dyspnea.
A review of the medications used in the recent and distant past is important.
Family history is relevant for recognizing ILDs related to hereditary disorders
There are ILDs with a sex predilection. Sporadic lymphangioleiomyomatosis

arises almost exclusively in women. In addition, many connective tissue
diseases more commonly affect women.
2-Chest imaging:
Although standard chest radiography is not as sensitive for detection of
ILD as the high-resolution computed tomography (HRCT) scan, it is the
logical starting point and the initial way of identifying and defining
disease. Chest radiography allows for the initial assessment of pattern
and distribution of parenchymal abnormalities and associated features
such as pleural effusion or mediastinal lymphadenopathy.
3- PULMONARY FUNCTION TESTING:
Assessment of ventilatory function and the mechanical properties of the lungs, as
well as gas exchange, particularly during exercise, are vital components of the initial
evaluation of patients with suspected ILD . Although limited in diagnostic
usefulness, the pulmonary function results are an important component of
assessing the severity of ILD. In addition, serial measurements of function enable
the physician to determine progression of the disease and the response to
therapeutic interventions. Most patients with ILDs manifest a restrictive pulmonary
impairment with a reduced diffusion capacity for carbon monoxide (DlCO). In most
patients, gas exchange is disturbed during exertion, even in those with normal gas
exchange at rest. In early or mild ILD, however, pulmonary function results may be
entirely normal. Obstructive pattern of impairment is commonly seen in some ILDs,
including PLCH, lymphangioleiomyomatosis, sarcoidosis, and ILD superimposed on
preexisting COPD/emphysema.
4-laboratory test:
Laboratory tests performed in patients with suspected ILD generally include a
complete blood count with differential leukocyte counts, metabolic panel, and
urinalysis. Anemia is frequently seen in patients with diffuse alveolar
hemorrhage syndromes. Peripheral eosinophilia raises suspicion for
eosinophilic pneumonias or drug reactions.
Hypercalcemia is often seen in patients with sarcoidosis. Abnormal urinary
sediment suggests possible pulmonaryrenal syndrome such as systemic
vasculitis. Serologic testing for connective tissue disease, vasculitis, or
hypersensitivity pneumonitis may be useful depending on the clinical context
and imaging findings.
5- BRONCHOALVEOLAR LAVAGE:
BAL is a technique used to sample the distal airways via the instillation of sterile
saline through a wedged fiberoptic bronchoscope . After aspiration, the
contents, which are thought to represent the cellular, immunologic, and
biochemical milieu of the alveolar structures, can be analyzed.
Cytologic analysis of BAL fluid can be diagnostic for malignancies and infections
that mimic ILD in presentation.
6- LUNG BIOPSY:
The final step in the evaluation of a patient with ILD is to decide whether
histologic evaluation is necessary for diagnosis. As previously noted, the
diagnosis of connective tissue, occupational, or drug-related ILD is often
obvious after a careful history has been taken.
In cases of idiopathic ILDs , the diagnosis may not be easily reached. If the
clinical, imaging, and laboratory results are inconclusive, lung
histopathology may be needed to reach a specific ILD diagnosis.
Treatment:
Generally the treatment different based on the type of
ILD and if it is secondary to other disease should treat the primary
Disease and the decision of antifibrotics based on progression of disease like if
the patient have connective tissue disease and in appropriate medications but
he have progression of disease in this time we can start Antifibrotic Therapy.
1- Pirfenidone.
As a result of these positive trials, pirfenidone is approved for the
treatment of IPF in the United States, Canada, Europe, Japan, and many
other countries. The usual dosing is 267 mg 3 times daily by mouth,
which is increased weekly to 801 mg 3 times daily by the third week.
The main adverse effects are gastrointestinal and hepatic; patients
often develop a photosensitivity skin rash.
2- Nintedanib.
As a result of these data, nintedanib has been approved for the treatment of
IPF in the United States, Canada, Europe, Japan, and many other countries. The
usual dosing of nintedanib is 150 mg by mouth twice a day. The main adverse
effects are gastrointestinal and hepatic.
In sum, both pirfenidone and nintedanib reduce the rate of FVC decline
by approximately 50% over 1 year of treatment in patients with IPF.
Pooled data analyses and post hoc analyses suggest these drugs may
improve quality of life, reduce the rate of hospitalizations and acute
exacerbations, and prolong survival.A recent real-world retrospective
cohort analysis using a large insurance database suggested the use of
these medications reduced risk of allcause mortality (hazard ratio 0.77)
for up to 2 years with no significant difference between the two
drugs.157 Studies to date suggest the adverse effects of these
medications are manageable for the majority of patients with a
reasonable long-term tolerability profile. Combination therapy with
both pirfenidone and nintedanib is being explored.

Interstitial Lung Diseases

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Interstitial Lung Diseases

A widely held hypothesis is that IPF develops in susceptible individuals

There are ILDs with a sex predilection. Sporadic lymphangioleiomyomatosis

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