Interstitial lung disease (ILD)

• Interstitial lung disease (ILD), also called

diffuse parenchymal lung disease, refers to
more than 200 lung disorders in which the
tissue between the air sacs of the lungs (the
interstitium) is affected by inflammation or
scarring (fibrosis).
• ILD is a general term used to describe these
diseases that involve the supporting structures
of the lung – connective tissue, alveolar
septae, and lymphatic space.
◾ Diffuse parenchymal lung diseases include a
large number (>200) of heterogeneous
conditions that affect the lung parenchyma
with varying degrees of inflammation and
fibrosis
1. Primary ILD: Idiopathic in origin. No
secondary cause or association is identifiable.

2. Secondary ILD: Have either a known

etiological cause or an association with
another disease of known etiology.
◾ Usual interstitial pneumonia (UIP); i.e. Idiopathic
pulmonary fibrosis (IPF)
◾ Non specific interstitial pneumonia (NSIP)
◾ Cryptogenic organizing pneumonia (COP)
◾ Respiratory bronchiolitis associated ILD (RBILD)
◾ Acute interstitial pneumonia (AIP)
◾ Desquamative interstitial pneumonia (DIP)
◾ Lymphocytic interstitial pneumonia (LIP)
 Usual interstitial pneumonia (UIP); i.e.
Idiopathic pulmonary fibrosis (IPF)
• Inflammatory change ---- remodeling of the lung
parenchyma -----patchy with areas of normal lung.
• As a part of excessive immune response, lung is
trying to heal by itself but instead overresponds
resulting in extensive scarring.
• Clinical manifestations
• Slow onset of dyspnoea particularly with
exercise –hypoxia with exercise.
• Dry cough
• Crackles
• Finger clubbing
 Restrictive pulmonary disease
• The inflammation and scarring result in a stiff lung.
• Increased recoil and decreased compliance
• TLC, RV & VC are reduced
• No slowing of respiration – defect in the lung
parenchyma not in the airway
• Gas exchange is affected
• FEV1—redduced
• FEV1: FVC ratio is normal
• Expiration time is short
c/m
• Dyspnoea on exercise
• increased respiratory rate
• Arterial oxygenation adequate at rest.
• Hyperventilate to maintain Pao2
• Oxy gen saturation decreases on exertion
Known causes:
◾ Infections: tuberculosis, fungal, bacterial, viral,
parasitic.
◾ Non infectious causes: hypersensitivity
pneumonitis, pneumoconiosis (silicosis,
absetosis),coal workers –black lung, drug
induced, radiation induced, malignancies-
tumor, drugs-bleomycin,
Association with diseases of inflammatory or
unknown aetiology:
◾ Sarcoidosis
◾ Connective tissue disorders: SLE, Rheumatoid
arthritis
◾ Chronic eosinophilic pneumonia
◾ Miscellaneous
◾ Known causes of ILD include occupational
exposures (e.g., asbestosis), medications
(e.g., nitrofurantoin), and those related to
an underlying systemic disease (e.g.,
cryptogenic organizing pneumonia [COP]
in the setting of polymyositis).
 Hypersensitivity pneumonitis (allergic
alveolitis)
• It is a specific reaction to an inhaled antigen.
• Eg . Farmers lung –antigen in moldy hay
• Bird fanciers lung – antigen in bird products
• c/m – fever, chills, cough, dyspnoea
The reaction will begin several hours after
exposure and is usually self limited. With
continued exposure a chronic form of disease
with fibrotic changes in the lung occurs.
 Sarcoidosis
• Age 20-40yrs
• No known etiology
• The initial lung lesion is hilar
lymphadenopathy.
• The pathologic change is one of noncaseating
granuloma formation ,some wwill regress and
others progress to fibrotic changes.
• Clumps of inflammatory cells in the lung.
• It can affect multiple organs eyes, skin, heart,
spleen –due to impaired immunity.
• Patients are anergic – lack of reaction by the
body defense mechanisms to foreign
substances.
 Pathophysiology
• Injury to the lung tissue ---- inflammatory
reaction in the lung, an influx of inflammatory
cells into the interstitium of the lung ---
stimulates interstitial fibroblasts----
development of lung fibrosis.
• Chronic changes ---- lung tissue damage ---
inflammation of alveoli with scarring and
fibrosis. Stiffening of the interstitium --- limits
oxygen transport through scared alveolar
capillary membrane into the blood stream.
◾ No clear understanding of disease pathogenesis.
◾ Inflammatory hypothesis: inflammation, lack of
response to corticosteroids.
◾ Vascular hypothesis: increased angiogenesis
important role in pulmonary fibrosis.
◾ Alveolar epithelial cells: Abnormalities in
alveolar type II cell injury and repair .
◾ Matrix remodelling: imbalance between the
production and degradation of extracellular
matrix, which leads to excessive production of
extracellular matrix molecules including
collagens, proteoglycans, glycosaminoglycans
etc.
◾ Fibroblast activation and dysfunction:
◾ Insidious onset
◾ Symptoms of progressive breathlessness with
exertion (dyspnea) or a persistent non-
productive cough.
◾ Pulmonary symptoms associated with another
disease, such as a connective tissue disease
◾ Hemoptysis
◾ Lung function abnormalities ---- restrictive
ventilatory pattern (i.e., reduced total lung
capacity and forced vital capacity).
◾ Fatigue
 Clinical indicators of potential hypoxemia

• Tachycardia
• Tachypnoea
• Restlessness
• Cyanosis
• Impaired judgement
 Diagnosis
◾ History: demographics, family history,
occupational/environmental
exposures – birds, drugs.
◾ History of vascular disease, immune
suppression…..
◾ Physical examination: Typical ‘velcro’
crepts, clubbing, inspiratory squeaks.
◾ Skin involvement, arthritis, eye
changes (conjunctivitisuveitis), muscle
weakness, lymphadenopathy etc..
◾ Chest radiography
◾ Interstitial infiltrates are seen as a discrete
linear, nodular or reticulonodular
shadows diffusely distributed in both the
lungs.
 ◾ Bibasilar reticular and /or nodular infiltrates,
honey combing

Honeycombing can be
diagnosed via HRCT
by the presence of
thick-walled, air-filled
cysts, usually between
the size of 3mm to 1cm
in diameter.
Ground glass opacification/
Reticular opacities and traction
appearance (GGO)- hazy
bronchiectasis predominate although
area of increased attenuation
ground-glass opacity is also visible in the
abnormal areas.
◾ Restrictive defects in spirometry: Airway
obstruction, reduced forced vital capacity
(FRV), forced expiratory flow.
◾ Bronchoalveolar lavage
• Ventilation perfusion scan (V/Q mismatch)
• Bronchoscopy and biopsy
◾ Other investigations: ESR, CRP, TC, DC, RFT,
Platelets, CPK, SACE, radionuclide scanning,
antibody testing (IgE, IgG)
◾
Six-minute walk tests detect the presence
of oxygen desaturation during ambulation
and distance walked.
• PFT – Decreased TLC,RV, & VC
• FEV1/FEV – normal
• ABG maintained at rest, but falls in exercise
• Ventilation perfusion scan impaired
(mismatch)
• Bronchoscopy and biopsy – broncho alveolar
lavage or trans bronchial biopsy is performed
to get a sample of inflammatory cells in the
alveoli or an actual piece of lung tissue.
• Open lung biopsy or thoracoscopic biopsy
 Other tests
• In sarcoid skin tests are performed to
determine if the pt is anergic
• In hypersensitivity pneumonitis titers of
antibody to the offending antigen can be
identified.
• In collagen vascular disease RA can be
checked.
 COMPLICATIONS
• Severe hypoxemia
• Corpulmonale
Objectives:
◾ Provide symptom relief
◾ Slow down disease progression
◾ Treating the underlying disease process
◾ Prevent complications
◾ Improve quality of life
◾ Prolong survival
◾ Prevent treatment complications
◾ End-of-life care and palliative treatment
◾ Treatment options are limited
◾ Supportive and symptomatic therapy
◾ Supportive treatment for respiratory failure,
pulmonary HTN, corpulmonale, CCF when
indicated.
◾ Corticosteroids – prednisolone 60mg/day
◾ Corticosteroids with in conjunction with Cytotoxic
drugs – Azathioprine, cyclophosphamide,
methotrexate
◾ Monitor WBC level

Anti-fibrotic agents
◾ Colchicin (inhibits alveolar collagen formation),
pentoxifylline, D-penicillamine, Interferron-gamma
• Nintedanib 100mg BD --Nintedanib is in a class
of medications called kinase inhibitors.
• It works by blocking the action of enzymes
involved in causing fibrosis.
◾ Antioxidants: N-acetyl cysteine –Mucinac
600mg BD
◾ Anti-Apoptosis agents -Navitoclax is one of
the B-cell lymphoma 2 (BCL-2) family protein
inhibitors
◾ Anti angiogenesis agents -Bevacizumab (
 • Supportive and symptomatic drugs
◾ Ambulatory Oxygen therapy
◾ Pulmonary vasodilators - Epoprostenol
◾ Bronchodilators
◾ Diuretics
◾ Antibiotics (if infection)
MANAGEMENT
◾ Lung Tansplant
 Rehabilitation
◾ In advanced disease

◾ Nutrition and dietary supplements

◾ Graded and regulated exercises

◾ Pulmonary physical rehabilitation - Pulmonary

rehabilitation improved the six-minute walk
distance, improved oxygen consumption,
reduced dyspnoea. Ultimately quality of life
improved