autosomal recessive inherited d/o defective chloride transport leads to buildup of mucous in lungs, pancreas, liver, intestines, & repro tract
cystic fibrosis
how is cystic fibrosis diagnosed
sweat chloride test >60mmol/L on 2 occasions; pilocarpine (cholinergic)
what would you expect to find on a CXR c/ CF
hyperinflated, c/ bronchiectasis (prox/medial airway dilation, lack of tapering & thick walls “Tram Track” appearance; pulm art dil- “signet ring sign”
young pt c/ bronchiectasis, pancreatic insufficiency, growth delay and infertility
classic CF
what clinical manifestations would you expect to find in the lungs and 2/2 pancreatic insufficiency in a CF pt
recurrent resp infx (pseudomonas, staph aureus) productive cough, chest pain, dyspnea, chronic sinusitis; dec fat absorb, steatorrhea (bulky, pale, foul smelling stool) fat soluble vit deficiency
what you you expect to find on PFT with an obstructive dz like CF
dec flow and inc volume; dec FEV1 and dec FEV1/FVC
chronic multisys inflamm granulomatous dsrdr affecting lungs, skin lymph nodes, eyes
sarcoidosis
what are the most common sx 2/2 granulomas taking up space and disrupting fxn in sarcoidosis
dry cough dyspnea, chest pain, hilar lymphadenopathy, erythema nodosum, LUPUS PERINO, parotid enlargement, uveitis, myocardial arrhythmias, rheum, neuro
what would you expect to see on bx with sarcoidosis
noncaseating (no central clearing) granulomas
what would you expect to see on CXR c/ sarcoidosis
bilat HILAR lymphadenopathy, R paratracheal, interstitial lung dz, reticular opacities, fine ground glass, eggshell nodal calcifications
would you expect a obstructive or restrictive pattern c/ sarcoidosis
restrictive pattern- norm or inc FEV1/FVC; dec lung vol, VC, RV, FRC, TLC
how do you manage CF
bronchodil, mucolytics, abx, decongestants, replace pancreatic enzymes, supplement A,D,E,K, transplant
if you wanted to rule out ifx (TB) in sarcoid what could you do
bronchiolar lavage- inc CD4: dec CD8
what would you expect to see on labs c/ sarcoid
inc ACE, hypercalciuria, eosinophilia, cutaneous anergy (diminished skin allergy test)
how do you manage sarcoid
oral corticosteroids, methotrexate, NSAIDs
if you see hyperlucent lower lung fields on CXR what would be on your DDx
alpha 1 antitrypsan deficiency
what is alpha 1 antitrypsan deficiency ass c/
liver dz, and genetic RF for COPD
chronic progressive interstitial scarring (fibrosis) from persistent inflamm, loss of pulm fxn c/ restrictive component unknown cause M 40-50yrs
idiopathic pulmonary fibrosis
what would you expect to find on exam c/ IPF
fine bibasilar insp crackles, clubbing of fingernails
what would you expect to see on CXR c/ IPF
diffuse reticular opacities (honeycombing) ground glass opacities
is IPF obstructive or restrictive
restrictive -dec lung vol (TLC, RV) norm FEV1/FVC
what are some other names for pulmonary vasculitis
granulomatosis c/ polyangitis, wegner granulomatosis
what is the classic triad c/ pulmonary vasculitis
upper and lower resp tract dz, glomerulonephritis
stone/pottery worker c/ DOE, cough and small nodular opacities in upper lobes and eggshell calcifications of hilar and mediastinal nodes
silicosis
cotton/textile worker c/ dyspnea, wheeze, cough, chest tightness- sx improve as week progresses
byssinosis
coal mine, small upper lobe nodules, hyperinflation of lower lobes resembles emphysema, massive progressive fibrosis+ RA
coal workers pneumocoinosis
construction, renovation old buildings, insulation, pleural plaques, interstitial fibrosis (honeycomb lung) lower lobes, dry velcro crackles, streaking at lung bases, ferriginous body
asbestosis
generalized lung inflamm of alveoli & resp bronchioles 2/2 organic antigens
hypersensitivity pneumonitis
what would you expect on CXR c/ hypersensitivity pneumonitis
small nodular densities sparing apices and bases
what meds are 1st line for persistent asthma
inhaled corticosteroids (beclomethasone, fluticasone, triamcinolone) don’t affect s.m. target inflamm, dec cap perm, inhibit leukotriene release, red hyperresponse
what are some common ADE of inhaled corticosteroids and how prevent
candidiasis, hoarse voice, local immune suppression, nose bleed, sore throat; swish and spit
should you use mast cell stabilizers (cromolyn) for acute attack
no- blocks initiation, good for pretreatment of allergen or exercise induced bronchoconstriction
what are some common ADE of Beta agonists
tachycardia, hyperglycemia, hypokalemia, hypomagnesemia, tremor
what are the long acting B agonists
salmeterol, xenafoate, formeterol - should not be used alone ;should+ ICS
if you have asthma and COPD what kind of inhaled bronchodilator would be best
anticholinergic- ipratropium, tiotropium
what are some ADE of leukotriene receptor antagonists -
inc liver enzymes, inc warfarin effects