Restrictive & Obstructive Lung Diseases Flashcards
(195 cards)
What is the qualitative definition of Interstitial Lung Disease?
inflammatory-fibrotic infiltration of the alveolar septa
What are the biophysical consequences of Chronic diffuse ILD?
decrease in diffusion capacity, FVC, lung volume, lung compliance
What are radiographic hallmarks of chronic diffuse ILD?
hazy gray shadows; glass opacities; End-stage: honey-comb” lung
Compare & contrast differences b/t COPD & pulmonary fiborsis?
Emphysema (COPD): pt. can’t exhale b/c lungs have decreased ability to recoil (lungs prefer to stay inflated); Pulmonary fibrosis: pt. can’t inhale b/c recoil pressure is too high (lungs prefer to stay deflated)
Describe the epidemiology of Idiopathic pulmonary fibrosis?
most common in elderly men > 60 yrs. especially those w/ familial risk for pulmonary fibrosis
what are the clinical presentations of IPF?
insidious progressive onset of unexplained dyspnea on exertion; bibasilar inspiratory crackles; cyanosis, myositis & clubbing may also be present; PE findings: augmented P2, S3 gallop
What are the histological hallmarks of IPF?
follows a UIP pattern: honeycombing cysts w/ reticular septal thickening; minimal glass opacities & basal predominance
What would a pulmonary function test show for a pt. w/ suspected IPF?
reduced VC, increased FEV1/FVC ration; decreased DLCO
What are pre-existing risk factors for pulmonary fibrosis?
neoplasm; mutations of TGF-beta; collagen vascular diseases including RA, SLE, & progressive systemic sclerosis
What are environmental factors of pulmonary fibrosis?
smoking; metal fumes; wood dust; occupational exposure
How do fibroblasts contribute to IPF?
fibroblasts are activated in response to epithelial injury and deposit collagen in interstitial spaces leading to interstitial fibrosis and respiratory failure; abnormal increased signaling through PI3K/AKT
Describe the pathogenesis of Familial IPF?
mutant telomerase: mutations in TERT & TERC leading to pathologically short telomeres; MUC5B mutation: increases secretion of mucin and alter mucociliary clearance
What histological findings can be used to distinguish UIP & IPF from other restrictive lung diseases?
honeycomb interstitial fibrosis w/ fibroblast hyperplasia; no necrosis or substantial inflammation; mostly lymphocytes and very few neutrophils
What are major histological distinctions b/t IPF & NSIP?
NSIP: no fibroblastic foci; notable inflammation & diffuse fibrotic lesions rather than honeycomb
What are clinical hallmarks of desquamative interstitial pneumonia?
large collections of pigmented macrophages containing remnants of necrotic type 2 pneumocytes; Smoking is the biggest risk factor; presents in 4th & 5th decade of life
What other interstitial lung disease is assoc. w/ smoking
respiratory bronchiolitis: similar histological patterns as DIP
What is the proposed etiology of sarcoidosis?
cell-mediated response to an unidentified Ag
what lab findings are indicative of sarcoidosis?
increased CD4/CD8 ratio; increased levels of TH1 cytokines (IL-2 & IFN-gamma) promotes T-cell expansion and macrophage activation; increased levels of IL-8 & TNF promotes formation of granulomas; polyclonal hypergammaglobulinemia (manifestation of helper T-cell dysregulation; impaired dendritic cell function
What histologic characteristics would you expect to find if sarcoidosis is suspected?
GRANULOMATOUS INFLAMMATION; giant cell nodular aggregates; NO NECROSIS
What are other more non-specific histological findings that can also be found for sarcoidosis?
schaumann bodies: basophilic laminated concretions of calcium & proteins; asteroid bodies: stellate inclusions enclosed in giant cells
What other lung abnormality is commonly seen in pts. w/ sacoidosis?
bilateral hilar lymphadenopathy
What are other conditions besides sarcoidosis that are assoc. w/ pulmonary granulomas?
Histoplasmosis; TB; neoplasm; lymphoma; foreign bodies
What skin test is performed for diagnosis of sarcoidosis?
cell mediated immunity to tuberculin
What are clinical presentations of sarcoidosis?
progressive dry cough & dyspnea; polyarthralgia; T-cell alveolitis w/ activated Mo-Ma