Prognosis: death usu. caused by medical complication (acute respiratory failure, severe pneumonia, pneumothorax, cardiac arrhythmia, pulmonary embolism) / survival with severe disease is from several

Prognosis: death usu. caused by medical complication (acute respiratory failure, severe pneumonia, pneumothorax, cardiac arrhythmia, pulmonary embolism) / survival with severe disease is from several up to 15 yrs

specific type of emphysema / panacinar versus basilar

Presentation: may present as bronchiectasis

Treatment: can give a-1 AT infusions (once weekly; to maintain target level > 80 mg/dL

mutations in cystic fibrosis transmembrane regulator (CFTR) / ΔF508 most common

Childhood: H influenzae, S. aureus

Adult: Pseudomonas, Aspergillus (50% colonized; occasionally cause disease), Burkholderia (always pathogenic), atypical mycobacteria (usu. colonizers)

Diagnosis: sweat chloride test (chloride > 70 meq/L positive; 1-2% false negative) / biopsies and imaging may show chronic tissue changes but not diagnostic

Treatment: patients do much better when followed by CF specializing centers
Bronchitis
Acute bronchitis (infectious) [NEJM]

Causes:

• 
  viral URI: influenza A, parainfluenza, adenovirus, RSV, rhinovirus, human metapneumonia virus
  
• 
  bacterial: Mycoplasma, B. pertussis, Chlamydia, B. dermatitidis
  

• 
  influenza assay if indicated
  
• 
  sputum culture likely unrevealing or showing underlying pulmonary disease (COPD, etc.)
  
• 
  CXR if pneumonia suspected
  
• 
  multiplex PCR testing being developed (for major causative bacteria)
  

• 
  cough suppressants / B2 agonists can be considered with bronchial hyperreactivity and steroids can be considered in patients with persistent cough
  
• 
  ABG as needed
  
• 
  antibiotics when there is concomitant COPD, purulent sputum, or persistent high fever
  
  • 
    PO macrolide or tetracycline or ampicillin 250 mg q 6 h
    
  • 
    trimethoprim-sulfamethoxazole 160/800 mg po bid may be given
    
• 
  antivirals if influenza A tests positive or if testing unavailable but during epidemic or clinically suspected or children with severe RSV (no antivirals yet effective for other players 11/06)
  

may be caused by various mineral and vegetable dusts; fumes from strong acids, ammonia, certain volatile organic solvents, chlorine, hydrogen sulfide, sulfur dioxide, or bromine; the environmental irritants ozone and nitrogen dioxide; and tobacco or other smoke.

definition: chronic productive cough at least 3 months in each of 2 successive yrs for which other causes, such as infection with Mycobacterium tuberculosis, carcinoma of the lung, or chronic heart failure, have been excluded

in which the degree of bronchoconstriction is not sufficient to produce overt wheezing, may be caused by allergen inhalation in an atopic person or chronic exposure to an irritant in a person with relatively mild airway hyperreactivity. Management is similar to that of ordinary asthma.

• 
  extrapulmonary
  

• 
  pulmonary
  

exudative stage

proliferative stage - may follow exudative - honeycomb lung - may only take 10 days
UIP - shorter course - fatal

DIP - longer course - amenable to steroid treatment - desquamation is actually histiocytes

talcosis birefringent talc particles

CWP

dust macules

Caplan’s syndrome fibrotic nodules

ferruginous body - amphibole fiber (big) / chrysotile fiber (small) / hemosiderin accretions

associated with malignant mesothelioma of pleural surface
Obstructive Sleep Apnea (OSA)

Epidemiology: 18 million Americans / EDS in 4% of men 2% women (30-65) / 82% of men and 93% of women with moderate to severe OSA are undiagnosed / 90% of men and 98% of women with mild to severe OSA and EDS are undiagnosed

prevalence comparable to asthma

• 
  HTN, change in voice, nasal obstruction, micrognathia,
  
• 
  retrognathia, macroglossia (cleft palate), tonsillar hypertrophy, uvulopalatal enlargement/elongation, submucosal infiltration
  

• 
  Obesity-hypoventilation syndrome (Pickwickian syndrome): obesity causes decreased compliance of chest wall / O₂ decreased all the time (not just when sleeping) (OSA may or may not be concurrently present) / screen for hypothyroid / usefulness of progesterone debated
  
• 
  Allergic rhinitis: 40% present (20% incidence in general population)
  
• 
  Hypothyroidism: 5% present (decreased response to CO2, deposition of material increases obstruction)
  

• 
  HTN, LVH and heart failure, dilated cardiomyopathy and CHF, MI
  
• 
  nocturnal cardiac disrhythmias (usu. bradycardia), sudden nocturnal death
  
• 
  nocturnal pulmonary HTN, early sustained pulmonary HTN and RV failure with diurnal decreased paO₂ and increased paCO₂ (10% incidence) / Note: rarely causes RV dysfunction without underlying COPD
  
• 
  association with renal disorders
  

• 
  CPAP (continuous positive airway pressure) [80% effective, eliminates mortality: treatment of choice; BIPAP is more expensive, doesn’t improve compliance, not shown to be more efficacious]
  
• 
  uvulopalatopharyngoplasty, tonsillectomy, other nasal and airway surgeries (40-50% effective), protriptyline 20-30 mg qhs, tracheostomy
  

• 
  defect in respiratory muscle or metabolic control mechanism
  
• 
  decreased respiratory drive: sleep onset, hyperventilation (idiopathic, hypoxic, pulmonary edema of CHF, CNS), prolonged circulation time of heart failure [CNS over-corrects before chemoreceptors appreciate signals]
  
• 
  upper airway reflex inhibition: esophageal reflux, aspiration, upper airway collapse
  

• 
  nocturnal supplemental oxygen
  
• 
  acidification with acetazolamide
  
• 
  CPAP (under investigation, effective in CSA secondary to CHF, may increase paCO2)
  

severe pneumonia: Klebsiella, Staphylococci, influenza A virus, fungi, mycobacteria, mycoplasma (rarely) / pneumonia complicating measles, pertussis, or certain adenovirus

bronchial obstruction from any cause (foreign body, adenopathy, mucus inspissations, lung tumors)

chronic fibrosing lung diseases (e.g., aspiration pneumonia, injurious gases or particles (e.g., silica, talc, or bakelite)

• 
  direct bronchial wall destruction
  

• 
  mechanical alterations
  

• 
  CXR: may show increased bronchovascular markings from peribronchial fibrosis and intrabronchial secretions, crowding from an atelectatic lung, tram lines (parallel lines outlining dilated bronchi due to peribronchial inflammation and fibrosis), areas of honeycombing, or cystic areas with or without fluid levels, but occasionally x-rays are normal
  
• 
  HRCT (with or without contrast): dilated airways, indicated by tram lines, by a signet ring appearance with a luminal diameter > 1.5 times that of the adjacent vessel in cross section, or by grapelike clusters in more severely affected areas. These dilated medium-sized bronchi may extend almost to the pleura because of the destruction of lung parenchyma. Thickening of the bronchial walls, obstruction of airways (evidenced by opacification--e.g., from a mucus plug--or by air trapping), and, sometimes, consolidation are other findings.
  
• 
  Bronchoscopy: rule out tumor, foreign body, or other localized endobronchial abnormality.
  

• 
  treat active infections or underlying disorders / prophylactic or suppressive antimicrobial regimens (talk to pulmonologist)
  
• 
  avoid smoking and irritants
  
• 
  surgical resection – rarely necessary but can be considered
  

failure to develop proper bronchi is a rare disease / Mounier-Kuhn syndrome, Williams-Campbell syndrome

bronchiectasis, situs inversus and sinusitis / accounts for 50% of a subgroup of primary ciliary dyskinesia (PCD) syndromes (defective mucociliary clearance) / chronic rhinitis, serous otitis media, male sterility, corneal abnormalities, sinus headaches, and a poor sense of smell

obstructive azoospermia, chronic sinopulmonary infections, normal spermatogenesis, a dilated epididymal head filled with spermatozoa, and amorphous material without spermatozoa in the region of the corpus / does not have ciliary defect as in PCD

• 
  Diffuse microatelectasis (seen with early ARDS)
  

• 
  Rounded atelectasis (folded lung syndrome)
  

complication of asbestos-induced or other pleuropulmonary disease.

CPAP at 5 to 15 cm H₂O

PEEP

bronchoscopy

chest physical therapy – encouraged to cough –IPPB or an incentive spirometer

aspiration of inert fluids or particulate matter

children: vegetal (e.g., peanuts) esp. in

adults: meat

high obstruction is obvious, but more distal can present more insidiously, clues to diagnosis:

• 
  CXR (taken during exhalation) may show atelectasis or hyperinflation of the affected lung partial obstruction with air trapping causes the cardiac shadow to shift away from the abnormal lung during this phase of respiration
  
• 
  recurrent parenchymal infections in the same segment of lung
  

putrid (due to anaerobic bacteria) or nonputrid (due to anaerobes or aerobes).

Single > multiple (usu. unilateral; S. aureus becoming more common, esp. IVDA; suppurative venous thrombophlebitis due to aerobic or anaerobic bacteria)

Causes:

• 
  aspiration (alcohol, other drugs, CNS disease, general anesthesia, coma, or excessive sedation)
  
• 
  usually anaerobes (esp. if periodontal disease) but can be variety of organisms (Klebsiella, Staphylococcus aureus, Actinomyces israelii, B-hemolytic strep, S. milleri (and other aerobic or microaerophilic streptococci), Legionella, or H. influenzae is sometimes complicated by abscess formation)
  
• 
  immunocompromised (Nocardia, Cryptococcus, Aspergillus, Phycomycetes, atypical mycobacteria (primarily Mycobacterium avium-intracellulare or M. kansasii), or gram-negative bacilli, blastomycosis, histoplasmosis, coccidioidomycosis)
  
• 
  septic pulmonary emboli, secondary infection of pulmonary infarcts, and direct extension of amebic or bacterial abscesses from the liver through the diaphragm into the lower lobe of the lung
  

• 
  bronchogenic carcinoma
  
• 
  cavitary TB
  

• 
  CXR, CT, sputum (usu. only useful if transtracheal aspiration, transthoracic aspiration, or bronchoscopy with a protected brush)
  

• 
  antibiotics (clindamycin or metronidazole) and/or as per suspected organisms / continue until pneumonitis has resolved and cavity has disappeared, leaving only a small stable residual lesion, a thin-walled cyst, or clear lung fields (usu. requires several weeks or months of treatment, much of which is given oral/outpatient)
  
• 
  postural drainage, bronchoscopy and/or surgical drainage as indicated (always drain if empyema suspected)
  
• 
  pulmonary resection if resistant to drugs, particularly if bronchogenic carcinoma is suspected / lobectomy versus segmental resection versus pneumonectomy (if really needed; mortality 5-10%)
  

mechanism: underlying lung process (e.g., pneumonia, infarction, TB) / direct entry of infectious agent or irritating substance into the pleural space (e.g., with a ruptured esophagus, amebic empyema, or pancreatic pleurisy) / entry of infectious, noxious agent or neoplastic cells via the bloodstream or lymphatics / parietal pleural injury (e.g., trauma, especially rib fracture, or epidemic pleurodynia [due to coxsackievirus B]) / asbestos-related pleural disease / pleural effusion related to drug ingestion (rarely)

• 
  sudden pain is dominant symptom (usu. only when patient breathes deeply or coughs) / visceral pleura is insensitive (pain from inflammation of the parietal pleura innervated by intercostal nerves; usu. felt over pleuritic site but may be referred to distant regions; irritation of posterior and peripheral portions of the diaphragmatic pleura, supplied by the lower six intercostal nerves, may cause pain referred to the lower chest wall or abdomen and may simulate intra-abdominal disease; irritation of the central portion of the diaphragmatic pleura, innervated by the phrenic nerves, causes pain referred to the neck and shoulder)
  
• 
  respiration is usually rapid and shallow / motion of the affected side may be limited / breath sounds may be diminished
  
• 
  pleural friction rub, although infrequent, is characteristic sign (varies from a few intermittent sounds that may simulate crackles to a fully developed harsh grating, creaking, or leathery sound synchronous with respiration, heard on inspiration and expiration. Friction sounds due to pleuritis adjacent to the heart (pleuropericardial rub) may vary with the heartbeat as well
  
• 
  pleural effusion (usu. occurs along with decrease in pleuritic pain) / larger effusion with all attendant clinical implications
  

• 
  clinical findings and picture (may get relief by pressure on chest wall by mechanical factors) / rule out other causes (many)
  

• 
  CXR: cannot show pleurisy but may elucidate any underlying pulmonary infection, process