Pearls from Dr. Claire Keating
Differential diagnosis of new-onset wheezing in adult
- Assess by anatomy:
- Extrathoracic upper airway obstruction: postnasal drip syndrome, paroxysmal vocal cord motion, hypertrophied tonsils, supraglottitis, laryngeal edem
- Intrathoracic upper airway obstruction: tracheal stenosis, foreign body aspiration, benign airway tumor, malignancie
- Lower airway obstruction: asthma/COPD, chronic obstructive pulmonary disease, pulmonary edema, aspiration, pulmonary embolism, bronchiolitis
Posted 09/22/16 09:25:32 AM by Matthew Cummings
Classic PFT patterns
- Obstruction: decreased/normal FVC, decreased FEV1, decreased FEV1/FVC ratio (<70%), normal/increase RV, TLC
- Restriction: decreased FVC, decreased/normal FEV1, normal FEV1/FVC, decreased TLC
Posted 09/22/16 09:30:14 AM by Matthew Cummings
Eosinophilic pulmonary diseases
- Infections (helminthic infections)
- Drug-induced pneumonitis (antibiotics, phenytoin, or l-tryptophan)
- Inhaled toxins (cocaine)
- Systemic disorders (eosinophilic granulomatosis with polyangiitis aka Churg-Strauss)
- Allergic bronchopulmonary aspergillosis
Posted 09/22/16 09:32:08 AM by Matthew Cummings
Strongyloidiasis (S. stercoralis)
- Endemic in tropics and subtropics (prevalence ~25%) , also occurs sporadically in temperate areas.
- Commonly asymptomatic, eosinophilia insensitive for diagnosis
- Symptoms often waxing, waning
- GI: Abd pain, bloating (70%)
- Pulm: SOB, wheeze (50-60%)
- Skin: pruritis (35%)
Posted 09/22/16 09:33:19 AM by Matthew Cummings
- Migratory phase
- Microfilariae migrate from pulmonary capillaries into the alveoli.
- Peripheral eosinophilia, elevated serum IgE levels.
- Pulmonary infiltrates with eosinophilia syndrome, asthma without infiltrates.
- Hyperinfection syndrome
- Accelerated autoinfection leading to wide-spread dissemination, typically in setting of immunosuppression.
- ARDS, cavitary lesions, interstitial infiltrates, and fibrotic disease.
- Management: PO vs. IV ivermectin depending on severity
Posted 09/22/16 09:35:37 AM by Matthew Cummings
For further reading...
Posted 09/22/16 09:40:30 AM by Matthew Cummings
Cystic Fibrosis (CF)
- Caused by a mutation in the CFTR protein that prevents normal passage of chloride resulting in dry mucosa with thick secretions
- Most common mutations is Delta F508 but others exist as well
- Affects multiple organs including sinuses, lungs, pancreas (exocrine and endocrine), GI trace/Liver and reproductive tract
- Nutrition and pulmonary hygiene are important --> Will often supplement with fat-soluable vitamins (ADEK) regardless of INR and even in those with diabetes, diet is never restricted
Posted 01/25/17 09:29:25 AM by Ying Liu
- DDx: bronchiectasis, parasites, cavitary PNA, malignancy, AVMs, epistaxis (must rule it out)
- Minor hemoptysis – common in patients with CF, esp during pulm exacerbations
- Massive hemoptysis – definition ranges from 100mL to > 600mL over a 24 hour period; typically > 500mL is used
- In CF, would recommend stopping NSAIDs/ASA and Chest PT
- Experts recommend against bronchoscopy given poor evidence that it helps to localize bleeding and that teh procedure can delay embolization and lead to more bleeding by promoting irritation/cough
- As Bronchial arteries are part of the arterial system, bleeding can quickly escalate, and you will often need IR to embolize in order to control bleeding
Posted 01/25/17 09:31:09 AM by Ying Liu
Pulmonary Exacerbations in CF
- Inhaled hypertonic saline – increase mucociliary clearance
- Beneficial when followed by chest physiotherapy
- Evidence for nebulized dose of 4mL of 7% saline to improve symptoms and lung function when compared to control of 0.12%
- More likely to return to their pre-exacerbation FEV1 with NNT = 6
- May cause transient airflow obstruction
- Variable response but up to 50% of patients have airway hyperresponsiveness
- Should be helpful in theory given chronic inflammation, but little data to support
- 2 small studies (n=44) showed small trend towards improvement in lung fx
- May have more benefit it patients with predominant asthma-symptoms
- Observational data that it can help in severe exacerbations
Posted 01/25/17 09:34:16 AM by Ying Liu
Approach to Antibiotics
- Target at minimum any mucoid P. aeruginosa and S. aureus
- Treat Achromobacterspecies if present à may be particularly inflammatory in nature and associated FEV1 deterioration
- There is more uncertainty regarding the importance of treating S. maltophilia
- Aspergillus generally not treated as unlikely cause of pulmonary exacerbations
- APBA however should be suspected in patients with wheezing and tx w/ steroids and elevated IgE
- When pan-resistant bug, select a combo of antibiotics that would otherwise be chosen empirically
- Oral azithromycin can be continued in an acute exacerbation if it is a component of the chronic pulmonary regimen
Posted 01/25/17 09:34:57 AM by Ying Liu
- 2 placebo controlled RCTs of 1108 patients
- Lumacaftor (VX-809), a CFTR corrector + Ivacaftor (VX-770), a CFTR potentiator
- Improved FEV1 ranged from 2.6 to 4.0 percentage points (P<0.001)
- 30-39% fewer pulmonary exacerbations in treatment group
- Rate of events leading to hospitalization or the use of IV abx lower as well
- Similar incidence of adverse events but higher rate of discontinuation → rates of hemoptysis similar in all groups
Posted 01/25/17 09:36:10 AM by Ying Liu
For further reading...
N Engl J Med 2015; 373:220-231
Posted 01/25/17 09:36:28 AM by Ying Liu
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It is not meant to replace the advice of a health professional.