Diseases Flashcards
(19 cards)
Pulm HTN Class I (Name, Dx, Path, Lesion location, epidemiology, tx)
Pulmonary Arterial HTN (PAH)
Dx: mPAP > 25mmHg; PCWP < 15mmHg; PVR > 3 woods (high resistance)
Path: smooth muscle hypertrophy, neointima formation, endothelial cell proliferation
Location: PA
this causes Right Sided Heart Changes!
Epi: RARE, least common, but most research
tx: Pulm VDs: Endothelin Path blockers, NO path promoters (receptor stimulators, and prevent NO breakdown - viagra/cialis), prostacyclin analogues
Pulm HTN Class II (Name, Dx, Path, Lesion location, epidemiology)
Pulmonary Venous HTN (PVH)
Dx: mPAP > 25mmHg; PCWP > 15mmHg
Path: medial thickening, occlusive venopathy
Location: PV
Left Sided Heart Changes cause this!
Epi: MOST Common, due to high CHF prevalence
Tx: HTN meds, diuresis (Pulm VDs will increase pulmonary edema)
Pulm HTN Class III (Name, Dx, Path, Lesion location, epidemiology)
Pulmonary HTN assoc w/ Lung Disease
Dx: mPAP > 25mmHg + underlying lung disease
Path: non-proliferative smooth muscle hypertrophy, mix of arterial/venous changes
Location: Near Capillary Bed/Lung Interstitium
Epi: 2nd most common
tx: Lung Disease Meds (Pulm VDs worsen V/Q mismatch by negating hypoxic VC)
Pulm HTN Class IV (Name, Dx, Path, Lesion location, epidemiology)
Chronic Thrombo-Embolic Pulmonary HTN (CTEPH) Dx: mPAP > 25mmHg; PCWP < 15mmHg; Evidence of V/Q mismatch (needs V/Q scan) Location: PA (thromboembolism) Epi: 3rd most common,
Tx: Pulm VDs treat sx but CURATIVE with thromboendarterectomy
Virchow’s Triad (what disease)
Pulmonary Embolism
- Venous Stasis
- Coagulation Alterations (Hypercoagulability)
- Vascular Injury
Well’s Score (what disease, values)
Pulmonary Embolism
WELLS > 4 (PE LIKELY)
WELLS < 4 (PE UNLIKELY)
Christopher Study for PE Dx
1. Wells if greater than 4 --> CT-Pulm Angio if less than 4 --> D-dimer 2. D-Dimer if greater than 500 --> CT-Pulm Angio if less than 500 --> PE excluded
PE Tx Acute vs. Chronic
Acute + Stable: Heparins (UH or LMWH)
Acute + “Massive”: PE + Shock sx
Tx: Fibrinolytics or IVC filter
Chronic: warfarin, LMWH, novel oral anti-coags for 3 mo. if provoked, indefinite if unprovoked
Light’s Criteria (disease, what they are, how many you need to meet)
Dx an exudative Pleural Effusion if:
- (Pleural Fluid Protein):(Serum Total Protein) > 0.5
- (Pleural LDH):(Serum LDH) > 0.6
- (Pleural LDH) > 0.67*(Upper Normal Value of Serum LDH
Transudative Pleural Effusion if otherwise
DDx for the two types of pleural effusions
transudative
- CHF
- Cirrhosis
- Nephrosis
exudative
- Infectious - Neutrophilic Fluid
- Autoimmune/C.T. Disease/Malignancy/TB - Lymphocytic
Three Types of PTX
- Primary Spontaneous
No inciting event, common in young, thin patients - Secondary Spontaneous
In setting of underlying lung disease (Infection - PJP/cavitation/malignancy/CF) - Traumatic/Iatrogenic
Due to blunt trauma (see broken ribs)
Due to medical treatment (complication of bipsy, feeding tube, bronchoscopy)
Three Physiologies of PTX
- Open PTX: Pb = Pip - lung collapses, relatively safe
- Closed PTX: one-way flow, Pip rises dangerously, may exceed pressure of venous return
- Tension PTX: shifts heart and mediastinum, dangerous if closed and Pip exceeds pressure of venous return
Six Questions for IIPs
- Are you sure it’s idiopathic?
Rule out occupational exposure/non-smoking trigger/c.t. disease/meds toxicities/HP panel - Is it sarcoidosis?
CXR: upper lobe predominant nodules/bilateral hilar adenopathy
Path: well-formed tightly packed non-caseating granulomas - Are there lots of eosinophils?
Use BAL, if yes - eosinophilic pneumonia (steroid responsive) - Is patient acutely ill w/o hx of lung disease?
If yes, AIP or COP (Path: DADs or OP) - Actively smoking with ground glass opacities?
Yes - Respiratory Bronciolitis-ILD or desquamative interstitial pneumonia - IPF?
WORSE OUTCOMES, DIFF THERAPIES (immunosuppression = harmful)
Needs UIP on HRCT (Sub-pleural basal dominant fibrosis, reticulation, honeycombing, traction bronchiectasis)
Use lung biopsy if inconsistent physical/radiographic findings
What are the two treatments for IPF?
Antifibrotics
- Perfenidone: inhibits TGF-beta mediated collagen synthesis (SE: Nausea, Photosensitivity rash, dyspepsia)
- Nintedanib: multiple tyrosine kinase inhibitor (SE: N/V/D)
Non-Pharm
Supp O2, PPV, Flu Vax, Pulm Rehab, Lung Tx, Palliative care
What is the strongest predictor of morbidity/mortality in CF?
How well can genes predict phenotype?
Pulmonary manifestations of disease
GI/Genital Tract: can be predicted on genotype
Pulm: Function of both genes and environment - harder to predict
What are the mutation classes of CF?
I: premature stop codons - more severe phenotype - G542X
II: misfolding/termination before PM - MOST COMMON - F508-del
III: abnormal gating - G551D
IV: channelopathy
V: abnormal splicing
VI: faster turnover
Pathophysiology of CF
Abnormal CFTR = Less Cl- transport = less water transport = hyperviscous mucous = dampens cilia and more difficult to clear
Inciting Event: Infection
Infection - Tissue Damage - Inflammation - Altered Airway Secretions - more susceptible to infections
Medications for CF, and special new ones
- Mucolytics (DNAse - pulmozyme)
- High Freq Chest Wall Oscillation device
- Exercise
- Bronchodilators
- Anti-inflammatory Agents (Corticosteroids/NSAIDS)
- ABx
- Nutrition (Pancreatic Enzymes, Vitamins)
RESTORE CFTR FUNCTION
Correctors (Lumacaftor, Tezacaftor) - help protein fold correctly - targets F508-del
Potentiators (Ivacaftor) - help gate stay open longer - targets G551D
What is the definition of Occupational Lung Disease?
- Occupational exposure is sole cause of disease
OR - Occupational exposure was one of the factors causing the disease
OR - Occupational exposure aggravated, accelerated, or exacerbated the condition/disease
