Term
| patients with sarcoidosis present at what age? |
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Definition
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Term
| patients with idiopathic pulmonary fibrosis present at what age? |
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Definition
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Term
| which diffuse infiltrative lung disease is mostly found in premenopausal women? |
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Definition
| lymphangioleiomyomatosis (LAM) |
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Term
| what is a family-inherited mutation that leads to several patterns of interstitial pneumonia including UIP? |
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Definition
| mutations in surfactant protein C gene |
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Term
| in which 3 interstitial lung diseases is smoking a big factor? |
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Definition
| desquamative interstitial pneumonia, respiratory bronchiolitis and 2/3 of patients with idiopathic pulmonary fibrosis |
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Term
| what might a sudden worsening of dyspnea indicate? |
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Definition
| spontaneous pneumothorax (esp if assoc with acute chest pain), as in LAM and neurofibromatosis |
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Term
| what are the most common physical examination findings for diffuse interstitial lung disease? |
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Definition
| tachypnea and bibasilar end-inspiratory dry crackles |
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Term
| what is a nonspecific lab finding elevated in infiltrative lung diseases? |
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Definition
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Term
| what enzyme level would be elevated in sarcoidosis? |
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Definition
| serum angiotensin-converting enzyme |
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Term
| what enzyme level would be elevated in hypersensitivity pneumonitis? |
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Definition
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Term
| which antibodies are elevated if vasculitis is suspected? |
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Definition
| antineutrophil cytoplasmic antibodies or anti-basement membrane antibodies |
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Term
| what are some nonspecific findings we'd see on a CXR for diffuse infiltrative lung disease? |
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Definition
| bibasilar reticular pattern; nodular or mixed pattern of alveolar filling and increased reticular markings; honeycombing (small cystic spaces) |
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Term
| what imaging modality alone can tell what the dx is? |
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Definition
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Term
| most all of infiltrative lung diseases cause restrictive changes in spirometry results. what few diseases cause obstructive results? |
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Definition
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Term
| would we expect to see changes in diffusion capacity in infiltrative lung disease? |
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Definition
| yes, reduction is common but nonspecific. severity does not correlate with disease stage |
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Term
| what is the most effective means of confirming the dx and assessing disease activity and prognosis? |
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Definition
| bx! adequate sized bx from multiple sites, usually from 2 lobes |
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Term
| what is the mainstay of rx for infiltrative lung disease? |
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Definition
| glucocorticoids (low success rate) lung transplant may have to be considered |
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Term
| what is the most common form of idiopathic interstitial pneumonia? what age group is most affected and what is the prognosis? |
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Definition
| idiopathic pulmonary fibrosis (usual interstitial pneumonitis). usually men >70yo. poor response to rx, 5 year survival only 20-40% |
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Term
| what is the characteristic histologic finding for idiopathic pulmonary fibrosis/usual interstitial pulmonitis? |
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Definition
| (need surgical bx) heterogeneous appearance at low mag, alternating areas of normal lung and interstitial inflammation, foci of proliferating fibroblasts, dense collagen fibrosis and honeycomb changes (peripheral, subpleural) |
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Term
| "burnt out lung" assoc with what disease? |
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Definition
| IPF/UIP (destroyed lung, lots of holes) |
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Term
| what is the histological hallmark of desquamative interstitial pneumonia? |
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Definition
| (exclusively in cig smokers) extensive accumulation of macrophages in intraalveolar spaces with minimal interstitial fibrosis. |
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Term
| what age group is most affected by desquamative interstitial pneumonia? whats the prognosis? |
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Definition
| 4th and 5th decades. 10 year survival rate 70% (good prognosis, good response to smoking cessation and systemic glucocorticoids) |
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Term
| describe what respiratory bronchiolitis-associated ILD is and what the histological hallmark is |
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Definition
| subset of desquamative interstitial pneumonia (exclusively in smokers), characterized by accumulation of macrophages in peribronchial alveoli |
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Term
| what 3 drugs cause drug induced ILD? what are the specific features of each disease? |
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Definition
| amiodarone, bleomycin, carmustine; bleomycin (d/t oxygen toxicity - exposed to bleomycin 10 years ago, then has surgery, exposed to oxygen, respiratory failure and pulmonary infiltrate); amiodarone (foamy macrophages, has iodine so CT scan looks like there was IV contrast injected) |
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Term
| describe who is at risk for acute interstitial pneumonia and what the prognosis is like |
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Definition
| >40 yo, abrupt onset in previously healthy individual; mortality >60% |
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Term
| what do we need for dx of acute interstitial pneumonia? |
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Definition
| clinical syndrome of idiopathic ARDS, pathologic confirmation of organizing diffuse alveolar damage (bx necessary) |
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Term
| what is the clinical presentation like for acute interstitial pneumonia? |
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Definition
| abrupt onset in previously healthy individual, flu-like prodrome for 7-14 days, then fever cough dyspnea, bilateral patchy symmetric ground glass attenuation, bilateral areas of air-space consolidation --> resp failure, need mechanical ventilation |
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Term
| what labs do we run when checking for progressive systemic sclerosis assoc with ILD? |
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Definition
| +anti-centromere abs (pulm htN); +scl-70 (pulm fibrosis); in pts with PSS, clinical evidence if ILD found in ~50% pts, ressitant to current modes of rx |
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Term
| who's most at risk for ILD associated with rheumatoid arthritis? and what are the clinical findings? |
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Definition
| men; pleurisy w/o effusion, necrobiotic nodules (forearms and lungs, w/or w/o cavities), pulm htn secondary to rheumatoid pulmonary vasculitis, BOOP, upper airway obstruction; CAPLAN's syndrome (RA, nodules, occupational exposure to coal) |
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Term
| what are clinical features assoc with ILD in SLE? |
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Definition
| acute lupus pneumonitis (pulmonary capillaritis --> alveolar hemorrhage --> hemoptysis) need to r/o pulmonary infection! |
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Term
| what ab level do we check in patients suspected of having ILD assoc with PM/DM? |
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Definition
| anti-jo1 antibody (radiographically similar to IPF) |
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Term
| what are the unique features of cryptogenic organizing pneumonia? |
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Definition
| (aka idiopathic BOOP) unknown etiology. recurrent and migratory opacities in the periphery and lower lung zone. granulation tissue in small airways, alveolar ducts, airspaces, chronic inflammation, air-space consolidation, ground glass opacities, bronchial wall thickening and dilation |
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Term
| how do we treat cryptogenic organizing pneumonia? |
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Definition
| glucocorticoid therapy induces clinical recovery in 2/3 (some pts have rapidly progressive courses with fatal outcomes despite rx) |
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Term
| what are the unique features of pulmonary alveolar proteinosis? |
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Definition
| caused by macrophage defect --> impaired ability to process surfactant (spills into alveoli, becomes PAS+ material in distal air spaces) --> (m>f) expectoration of chunky gelatinous material, polycythemia, hypergammaglobulinemia, increased LDH, elevated levels of lung surfactant proteins A and D, "bat-wing" distribution or "fluffy" appearance on radiograph, "crazy paving" on CT |
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Term
| describe pathogenesis of pulmonary LAM and its unique features |
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Definition
| proliferation of abn smooth muscle cells --> invade and destroy parenchyma (airways, lymphatics) and cause cyst formation --> spontaneous pneumothorax (50%). disease accelerates during pregnancy. hemoptysis life threatening. |
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Term
| how do we tx pulmonary LAM? |
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Definition
| oophorectomy, progesterone tamoxifen and LHRH analogues, lung transplant only hope for cure (reports of recurrent disease in transplanted lungs) |
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Term
| what is the tx for syndromes of ILD with diffuse alveolar hemorrhage? |
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Definition
| IV corticosteroids with or without immunosuppressive therapy |
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Term
| how does goodpasture's syndrome present? |
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Definition
| diffuse alveolar hemorrhage with an associated glomerulonephritis (anti-basement membrane antibodies present) plasmapharesis rec as adjunctive tx |
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Term
| name some inherited disorders associated with ILD |
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Definition
| phakomatoses, tuberous sclerosis, neurofibromatosis, lysosomal storage diseases, niemann-pick disease, gaucher disease, hermansky-pudlak syndrome |
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Term
| inhalation of organic or inorganic dusts, causing hypersensitivity pneumonitis causes what kind of reaction? |
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Definition
| granulomatous inflammatory reaction |
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Term
| what are the unique features of pulmonary langerhans cell histiocytosis |
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Definition
| smoking related, men 20-40, UL disease sparing costophrenic angles, parenchyma infiltrated by abn histiocytes (cleft nucleoli) --> star-shaped/stellate nodules; combination of stellate nodules + thin walled cysts are diagnostic of PLCH; need to discont smoking. death by resp failure 10% |
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Term
| what are granulomatous vasculitides characterized by? and name some |
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Definition
| pulmonary angiitis with granuloma formation. wegeners granulomatosis, allergic angiitis, churg-strauss syndrome, necrotizing sarcoid granulomatosis, benign lymphocytic angiiti |
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Term
| describe the etiology of sarcoidosis |
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Definition
| disordered immune regulation in genetically predisposed individuals exposed to certain environmental agents (--> noncaseating granulomas, bilateral hilar lymphadenopathy) |
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Term
| what are the diff ways a pt with sarcoidosis could present? |
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Definition
| bilateral hilar adenopathy on CXR, peripheral lymphadenoapthy, cutaneous lesions, eye involvment, splenomegaly, hepatomegaly, insidious onset of respiratory abnormalities |
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Term
| 3 tests we like to do on pts suspected of having sarcoidosis |
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Definition
| 1. neuro exam (MRI if abn) 2. EKG (r/o heart block) 3. opthalmologic exam (r/o eye disease) |
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Term
| describe lymphocytic interstitial pneumonitis |
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Definition
| assoc with sjogrens syndrome and HIV (AIDS defining illness in kids). lymphocyte and plasma cell infiltration of lung parenchyma |
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