interstitial lung disease Flashcards
what is The term Interstitial lung disease (ILD) ?
refers to a broad group of
inflammatory lung disorders
what is ILD charaterzied by?
by acute, sub acute, or chronic inflammatory infiltration of alveolar walls by cells, fluid, and connective tissue
how is ILD grouped?
based on
- occupational or environmental exposure,
- disease associations, and specific pathology.
what can untreated ILD lead to?
irreversible pulmonary firbosis
what are the 3 main categories of IDL?
- interstitial inflammation with granulomas
- idiopathic
- interstitial inflammation without granulomas
- miscellaneous diffuse lung disease
what is the defintion of IDL
***restrictive lung diseases!!
Definition: a group of diseases that cause
chronic interstitial inflammation of the lung
leading to
1) collagen deposits (scar
tissue) and
2) pulmonary fibrosis
what is the Known Risk factors for ILD
- occupation • environment • exposure to dusts • cigarette smoke • gender • age
What is the pathophysiology of ILD?
- abnormalities involve pulmonary interstitium
- –between capillary and alveolar space - Injury to the lung caused by
- chronic inflammation
- accumulation of inflammatory cells within the interstitial space
3.Leads to scarring and fibrosis
charateristic of IPF?
What:
** specific form of chronic progressive fibrosing interstitial pneumonia. affecting mainly the lower lobes and periphery of lungs
Cause
- unknown cause
Who
- occurs in older adult
how many types of IPF and what are they?
2 types
1) DIP (desquamative interstitial
pneumonia
2)UIP (usual interstitial pneumonia
What are the risk factors of IPF
Smoking
- strong association particularly
if history > 20 years, 75% are smokers
or ex-smokers
GERD
-micro-aspiration of gastric
contents pulmonary fibrosis in
susceptible individuals
Viruses:
- role in initiation, progression
and acute exacerbations
What are the ILD disease in the interstitial inflammation without granulomas?
- Hypersensitivity Pneumonitis
- Sarcoidosis
- Wegner’s granulomatosis
- Pneumoconiosis
- ->Silicosis
- ->Coal Worker’s
- ->Asbestosis
What are the ILD disease in the Interstitial
disorders without granulomas
- Drug-induced
Interstitial Disease
2.Radiation-induced
Interstitial Lung
Disease
3.Goodpasture’s
Syndrome
4.Bronchiolitis
Obliterans with
Organizing
Pneumonia
5.Alveolar
Proteinosis
6.Collagen Vascular Disease -->Rheumatoid Arthritis -->Systemic Lupus Erythematosus
What is the Anatomic Alterations of the Lungs in IDL?
- Destruction of the alveoli and adjacent pulmonary
capillaries - Fibrotic thickening of the respiratory bronchioles,
alveolar ducts, and alveoli - granuloma
- Honeycombing and cavity formation
- bronchial secretion
- bronchospasm
What is the clinical manifestations that lead
to a diagnosis of ILD initially?
- Non-productive cough (dry) >80% of patients,
- progressive dyspnea –> often hypoxemia (tachypnea, ↑HR, ), rapid shallow breathing,
- bibasilar late inspiratory velcro like crackles >90% cases,
- dull percussion, increased tactile and vocal fremitus
What are the manifestations in late stage ILD?
Non Respiratory: - heart disease - pulmonary hypertension (RHF), - Rheumatoid: joint pain & swelling
the key diagnosis finding in ILD?
1 CXR/CT – bilateral reticulo-nodular
opacity
–>secretion, atelectasis
2 .PFT’s – volumes, decrease DLCO,decrease FEV1, decrease FVC,
same FEV1%
3.ABG’s Hypoxemia at rest later on
what are the exception to the expected decrease diffusion capacity?
- Goodpasture’s syndrome
- Idiopathic pulmonary hemosiderosis
– DLCo is elevated response to the
increased amount of blood retained in the
alveolar spaces that is associated with these two disorders.
what are the chest Radiograph findings?
Bilateral reticulonodular pattern Irregularly shaped opacities Granulomas Cavity formation Honeycombing Pleural effusion
what are the Pharmacologic Therapy for ILD?
Pirfenidone Esbriet
–> antifibrotic agent with anti-inflammatory & antioxidant effects
Nintedanib OFEV
–> a tyrosine kinase inhibitor (TKI) inhibit vascular endothelial, fibroblast
& platelet derived growth factor receptors
dose/frequency of Pirfenidone Esbriet
dose
- 267mg/pill
freq
- 3 tid
dose/frequency of Nintedanib OFEV?
dose
150mg
freq
- BID
what is the outcome for both tx?
Improves FVC & disease progression &
quality of life
What is the goal of ILD treatment?
- early dx to slow disease progression
- manage co-morbidities
- prevent exacerbations to optimize QofL and increase survival
what are other non-pharmcological treatment
• Oxygen Therapy • Broncho-pulmonary Hygiene Therapy • Avoidance of inflammatory triggers (GERDPPinhibitors, OSA-CPAP) decrease lung injury • Pulmonary rehabilitation • Transplant (end-stage) less than 55 y.o • Vaccinations (preventative) • Palliative care (home care LTOT)
what are the top 5 occupational that cuase ILD
- Printing (Metal Working Fluids)
- Healthcare/Nursing
- Wood Working
- Agriculture
- Cleaners
what is Pneumoconiosis ?
Pneumoconiosis is a form of lung
fibrosis caused by inhalation of dusts
such as asbestos, coal and silica
what is Mesothelioma?
Mesothelioma is a malignant tumour
of the pleura caused by inhalation of
asbestos 20 40 years previously
