Lecture 21:ILD 2 Flashcards
what are the Interstitial lung diseases?
- -Heterogeneous group of diseases
- -Confusing and duplication of disease names
- -Poor classification
- -Fibrotic lung disease
- -Restrictive defect on pulmonary function
- -Diagnostically & therapeutically challenging
what is the definition of ILDs?
group of disorders that result in disruption of the distal lung parenchyma
what is the interstitium?
alveolar walls and septae and contains the lung connective tissue elements
what is the hallmark of ILDs?
- -FIBROSIS
- -An extensive alteration of alveolar/airway architecture
What is Pulmonary Fibrosis?
- -Pulmonary Fibrosis = scarring of the lung.
- -Gradually, the alveoli become replaced by fibrotic tissue.
- -When the scar forms, the tissue becomes thicker causing a loss of the tissue’s ability to transfer oxygen into the bloodstream.
classification of ILDs?
1) Aetiological
2) Radiological: most used clinically
2) Histological: (if tissue specimen available)
- -In general the more cellular the picture, the more treatment responsive;
- -Treatment is typically steroids or other immunosuppression
etiological classification of ILDs?
1)Idiopathic:
–Idiopathic pulmonary fibrosis, sarcoidosis, Langerhans cell histiocytosis
2)Collagen vascular disease associated:
–Systemic disease e.g. scleroderma, rheumatoid arthritis
3)Drug/treatment-related:
–E.g. amiodarone, nitrofurantoin, bleomycin, MTX, radiation
4)Occupational or environmental exposures:
–Inorganic: e.g. asbestosis, silicosis.
–Organic dust: (hypersensitivity pneumonitis)
pigeon-fanciers, farmers lung, etc
5)Post-infectious: e.g. TB
radiological classification of ILDs?
- -Based on zones most affected on CX
- -Upper zones
- -Lower zones
Upper versus Lower zone fibrosis?
1)CHHARTS (top of the charts = Upper lobe)
Coalworkers, Hypersensitivity, Histiocytosis X, –Ankylosing spondylitis, Radiation, Tuberculosis, Silicosis
2)RAIDS (for lower lobe)
–Rheumatoid arthritis, Asbestosis, IPF, Drugs, Scleroderma,
histological classification of ILDs?
- -Usual interstitial pneumonitis (UIP)
- -Non-specific interstitial pneumonitis (NSIP)
- -Desquamitive interstitial pneumonitis (DIP)
- -Respiratory Bronchiolitis interstitial lung disease (RB-ILD)
- -Lymphocytic interstitial pneumonitis (LIP)
- -Acute interstitial pneumonia (AIP)
- -Bronchiolitis obliterans organizing pneumonia (BOOP)
list drugs causing pulmonary fibrosis
amiodarone, nitrofurantoin, bleomycin, MTX, radiation
Rheumatoid arthritis, Asbestosis, IPF, Drugs, Scleroderma predominantly affect upper lobes. T/F
False
1)CHHARTS (top of the charts = Upper lobe)
Coalworkers, Hypersensitivity, Histiocytosis X, –Ankylosing spondylitis, Radiation, Tuberculosis, Silicosis
2)RAIDS (for lower lobe)
–Rheumatoid arthritis, Asbestosis, IPF, Drugs, Scleroderma,
what is the bronchiolitis obliterans?
A chronic disease of the small airways, usually caused by repeated cycles of inflammation and scarring. Over time, scarring builds up and causes obstructive lung disease. Common etiologies include inhalation of toxic fumes and severe adenovirus pneumonia.
in history taking of patients with ILD, what are the most likely findings to be discovered?
1) progressive exertional dyspnoea
2) Gradual history 6-12 months or more
3) ± Dry cough - Productive cough is rare for interstitial lung disease
* * No wheeze
4) Occasionally there may be chest findings and impressive radiology findings with minimal SOB e.g. 5)Sarcoidosis
6) Haemoptysis unusual
7) New hemoptysis in old ILD; consider malignancy
why wheeze is not seen in ILDs?
because wheezing is a sign of obstructive diseases, whereas ILDs are restrictive.
does hemoptysis is common finding in ILD?
- -no,
- -New hemoptysis in old ILD; consider malignancy
list some history clues suggesting ILD.
1) Age:
- -Most Idiopathic Pulmonary Fibrosis patients are >60 years old at presentation
- -Most Sarcoidosis and Connective Tissue associated ILD present between 20-40 years old
2) Smoking:
- -Idiopathic Pulmonary Fibrosis (IPF)
- -Respiratory bronchiolitis-ILD
3) Gender: Connective Tissue associated ILD is commoner in women
- -Duration: acute/subacute Interstitial lung disease(ILD) often confused with pneumonia
4) Meds: include OTC meds, protein supplements, and remote drug hx (e.g. amiodarone)
smoking is a risk factor for IPF. T/F
True
Risk factors: cigarette smoking, environmental or occupational exposures, chronic aspiration, genetic predisposition
connective tissue disorder associated ILDsare more common in males. T/F
False
Connective Tissue associated ILD is commoner in women
what occupations predispose to ILDs?
- -sand blasting (silica), coal miners
- -asbestos
- -hay farming, mushroom farming – hypersensitivity pneumonitis (HP) (symptoms may lessen if away for a week and return upon re-exposure).
- -Hobbies – pigeon fancier (HSP)
what are the typical signs of symptoms of CTDs that can be acquired during history taking?
- -Musculoskeletal pain, weakness, fatigue, fever, joint pain or swelling, photosensitivity, pleuritis, dry eyes/mouth
- -Scleroderma symptoms – tightening of the skin over hands, mouth, nose. Raynaud’s phenomenon, swallowing difficulties.
what are the possible signs on P/E of a patient with ILD?
- -General summary of signs:
- -Abnormal but non-specific
1) Digital Clubbing: common in IPF, asbestosis. Rare in sarcoidosis
2) Crackles (“velcro”) common in all ILD - -May occur even with normal CXR
3) Cor pulmonale: due to pulmonary hypertension (loud P2, parasternal heave, elevated JVP if TR).
digital clubbing is common in sarcoidosis. T/F
False
It is common in asbestosis
can crackles be heard in a patient with ILD and normal CXR?
Yes
what are the P/E signs on hands that can suggest ILD or its predisposing disease?
- -Clubbing (IPF & asbestosis)
- -Sclerodactaly (Scleroderma)
- -Vasculitic phenomena: splinter hemorrhages (RA, SLE)
- -Raynaud’s phenomenon (Scleroderma, SLE)
- -Symmetrical small joint arthropathy, onycholysis (RA)
- -Gottren’s papules (Dermatomyositis)
what are the Gottron’s papules?
Erythematous papules on the extensor surfaces of the hands, typically over the metacarpophalangeal (MCP) and interphalangeal joints. A characteristic dermatological finding in dermatomyositis.
what are the P/E signs on the head and neck that can suggest ILD or its predisposing disease?
- -Butterfly rash: SLE
- -Lupus pernio, parotid swelling, LMN VII palsies, iritis: sarcoidosis
- -Keratoconjunctivitis sicca: Sarcoidosis, RA
- -Uveitis: painful red eye, irregular pupil
- -Telangiectasia, microstomia, ‘beaking’ of the nose: Scleroderma
what is the lupus pernio?
An extrapulmonary manifestation of sarcoidosis that most commonly manifests with erythematous to violaceous, indurated lesions on the central face.
what are the P/E signs on the chest that can suggest ILD or its predisposing disease?
1) Rapid shallow breathing
2) ± Central cyanosis
3) ± Tracheal deviation (TB)
4) decreased chest diameter and chest expansion
5) Tactile fremitus: normal or increased
6) Percussion: normal or dull
7) Auscultation: bilateral fine end-inspiratory crepitations/crackles (velcro type)
what happens to tactile fremitus in ILDs?
normal or increased
what is heard on auscultation of patients with ILD?
bilateral fine end-inspiratory crepitations/crackles (velcro type)
