ILD Flashcards
Name the 7 idiopathic interstitial pneumonias
Idiopathic pulmonary fibrosis, non-specific interstital pneumonia, acute interstital pneumonia, cryptogenic organising pneumonia, desquamative interstital pneumonia, respiratory bronchiolitis associated ILD, lymphocytic interstitial pneumonitis
Name then two idiopathic interstital pneumonias particularly associated with smoking
DIP (desquamative intersitial pneumonia) and respiratory bronchiolitis associated ILD.
Name 3 pulmonary haemorrhage syndromes
Granulomatosis with polyangiitis (formerly Wegners)
Goodpastures (anti-glomerular basement membrane)
Idiopathic pulmonary haemosideroisis
What are 3 typical characteristics of pulmonary lymphangioleiomyomatosis?
Affects pre-menopausal women
Present with recurrent pneumothorax
Chylous effusions
Name 5 connective tissue disorders associated with ILD
Progressive systemic sclerosis Rheumatoid arthritis Systemic lupus erythrmatosis Polymyositis/dermatomyositis Sjogrens
What are 3 extra-pulmonary signs or conditions associated with pulmonary langerhans cell histiocytosis?
Diabetes insipidus (Central)
Lyric bone lesions
Exophthalmos
What is the other name for acute interstital pneumonia?
Hamman-rich syndrome
What are the characteristic radiological findings in idiopathic pulmonary fibrosis?
Usual interstitial pneumonia pattern
- predominantly basal changes
- sub pleural reticular opacities
- honeycombing
- traction bronchiectasis
What ILDs are associated with basal changes?
IPF, asbestosis, NSIP
What ILDs are associated with upper lobe changes?
Sarcoid
Hypersensitivity pneumonitis
What findings on PFTs for ILD?
Restrictive
- reduced TLC, FRC, RV
- reduced FEV1 and FVC - ratio preserved
- DLCO decreased
What is the most common idiopathic interstital pneumonia?
IPF
Who typically gets IPF?
males
often ex smokers
age greater then 60 years
What is the classic finding of IPF on imaging?
Honeycombing
What is the pathological pattern of IPF?
Usual interstital pneumonia - UIP
- fibroblastic foci
- lymphoplasmacytic infiltrate
What are some new therapies for IPF?
Nintedanib - multiple tyrosine kinase receptor blocker, slows rate of disease progression and increases time to first exacerbation
Pirfenidone - antifibrotic agent, slows rate of decline and reduces mortality
When to consider lung transplant for IPF?
When DLCO less then 40%
- IPF do best of all disease transplanted
What is the imaging features of NSIP?
basillar subpleural ground glass opacities
*No honeycombing
What is NSIP often associated with?
Connective tissue disease related lung disease
What is AIP?
acute onset, similar to ARDs, high mortality, often requires mechanical ventilation
pathological pattern is diffuse alveolar damage (DAD)
Often have hyaline membrane (which is also present in ARDS)
What is COP?
Cryptogenic organizing pneumonia (COP)
- usually follows an infection
- basilar subpleural ground glass opacities and consolidation
- good prognosis, 2/3 respond to steroids
What is DIP?
Desquamative interstital pneumonia
typical in smokers
diffuse ground glass opacity, basal and peripheral
lots of reactive macrophages in intraalvelor spaces
good prognosis with smoking cessation
What is RB-ILD?
Respiratory bronchiolitis associated ILD
- centrilobular, micronodular disase
- occurs in smokers, resolves with cessation of smoking
What is pulmonary alveolar proteinosis?
most are aquired
autoimmune process with igG antibody against GM-CSF which causes dysfunctional alveolar macrophages
leads to decreased surfactant clearence and occumulation of acid schiff positive proteinaceous material
Treatment of PAP?
whole lung lavage
What is the most common connective tissue disease leading to ILD?
Scleroderma - occurs in half RA typically in men, 20% SLE - uncommon to get progressive ILD Poly/dermatomyositis - 10% Sjogrens - get lymphocytic interstital pneumonia
Name some exposure related ILD?
Amiodarone - typically occurs in first year
Methotrexate - uncommon
