intestitial lung diseases Flashcards
What labs/bronchioalveolar lavage findings are expected in idiopathic pulmonary fibrosis? what about on biopsy?
restrictive disease; lavage shows increased PMNs; biopsy shows extensive fibrosis and loss of parenchymal architecture
What are the radiographic findings of idiopathic pulmonary fibrosis?
reticulonodular pattern and honycomb lung; CT shows ground glass
What is the treatment for idiopathic pulmonary fibrosis?
corticosteroids plus either azathioprine or cyclophosphamide help in some pts- follow with PFTs to determine how effective they are; lung transplant
What are the key features of sarcoidosis?
noncaseating granulomas, bilateral hilar adenopathy, pulmonary infiltrates, skin lesions, pulmonary infiltrates
What are the key historical features of sarcoidosis?
cough, malaise, wieght loss, dyspnea, arthritis, chest pain, fever, erythema nodosum LAD, vision loss, cranial nerve palsies
What are key lab findings of sarcoidosis?
increased serum ACE, high calcium, incr alk phos, decr WBC, incr ESR
What are the key features on lab, radiology and complications for asbestosis?
-asbestos fibers on pleural biopsy
-Radiology shows multinodular opacities, pleural effusions, blurring of heart/diaphragm
CT shows linearl pleural and parenchymal fibrosis
-incr risk of mesothelioma and lung CA, esp. in conjunction with tobacco use
What are the key features on radiology and what are the key complications of silicosis?
radiology: small apical and nodular opacities; eggshell calcifications of lymph nodes, hilar adenopathy
complications: fibrosis, incr. risk of TB infection
What are the key features on radiology and what are the key complications of berylliosis?
pulmonary edema, diffuse granuloma formation, hilar adenopathy
incr. risk of lung CA. may need chronic corticosteroid treatment
What is goodpasture’s syndrome?
disease of lungs and kidneys- antiglomerular basement membrane antibodies
What is the presentation of goodpasture’s disease?
hemoptysis, dyspnea, recent infection with positive anti-GBM antibodies, restrictive pattern with increased DlCO. proteins and granular case on UA. renal biopsy shows crescentic glomerulonephritis and IgG deposition
What is granulomatosis with polyangiitis?
rare- granulomatous inflammation and necrosis of lung and other organ systems due to systemic vasculitis that mainly affects lungs and kidneys. causes formation of noncaseating granulomas and destruction of the lung parenchyma. Often causes ulcertaions of the nasopharynx.
What are key clinical features of granulomatosis with polyangiitis?
ulcerations of nasopharyns, chronic sinusitis, hemoptysis, dyspnea, renal sx, CNS sx, eye sx, arrhythmias
-cANCA positive with non-caseating granulomas
What is the tx for granulomatosis with polyangiitis?
cyclophsophamide, corticosteroids
