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26 Cards in this Set

  • Front
  • Back
causes of restrive lung disease
- Interstitial lung disease (most common)
- Abnormalities of the chest wall
- Weakness of the respiratory muscles
Abnormalities of the chest wall and PFTs
- Fibrothorax and Morbid obesity
– it will decrease FVC
Interstitial lung disease and PFTs
- Sarcoidosis and Idiopathic pulmonary fibrosis (IPF)
– it will decrease TLC, FVC and RV
Weakness of the respiratory muscles and PFTs
- Myasthenia Gravis and ALS
– it will decrease TLC and increase RV
interstitial lung disease mimicks
Pulmonary infection (esp PCP)
Lymphangitic carcinomatosis
interstitial lung disease path
- ↓ lung compliance (so ↓ FVC and FEV1, normal ratio)
- reduced lung volumes (TLC, FRC, RV)
- Diffusion impairment
- Destruction of small pulmonary vessels and alveolar-capillary interface by inflamm and fibrosis, so ↓ SA
– pulmonary Hypertension
interstitial lung disease Sx
- Dyspnea and cough
- Crackles (rales), Clubbing, Cor pulmonale (so JVD, Loud P2, TR, Lower extremity edema)
interstitial lung disease radiology
- CXR shows ↓ lung volumes
– also interstitial pattern, which is reticular (increased linear markings), and reticulonodular (linear and small nodules)
interstitial lung disease HRCT (high resolution CT)
– Detects sub-radiographic disease
- distinguishes inflam from fibrosis and suggests a dx
– ground glass (Means you can see BV’s and airways running through the infiltrates) suggests inflamm
– consolidation (you cant see BVs)
interstitial lung disease bronchoscopy
- to exclude infections
- BAL cellular differential can support a dx
– a transbronchial biopsy is for granulomatous disease or lymphangitic spread of cancer
interstitial lung disease VATS biopsy
- gold standard
– larger sample size and is required for differentiation of idiopathic interstitial pneumonias
– biopsy should be strongly considered in pts w/ DPLD when dx remains uncertain after clinical and radiological assessment
Idiopathic pulmonary fibrosis (IPF) definition
- a specific form of chronic fibrosing interstitial pneumonia limited to the lung and associated with the histologic appearance of UIP
- almost uniformly fatal (median survival 2-3 yrs)
Idiopathic pulmonary fibrosis (IPF) path
- involves a defect in injury repair
– some epi cells die and release GF’s and other stuff to regenerate
– in some people they stimulated fibroblasts to convert to myofibroblasts which lay down collagen in the lung
Idiopathic pulmonary fibrosis (IPF) clinical
- restrictive PFTs
- decreased DLCO
- Age >50 w/ a gradual onset of symptoms
– progressive dyspnea on exertion
– nonproductive paroxysmal cough
– Bibasilar crackles on auscultation
– Digital clubbing
Idiopathic pulmonary fibrosis (IPF) rad and hist
- show typical UIP pattern
– Fibroblastic foci, Temporal heterogeneity, Honeycombing appearance, Minimal inflammation
– there will be bilateral basilar opacities in CRX
– HRCT will show a reticular pattern w/ sub-pleural distribution and septal thickening
Idiopathic pulmonary fibrosis (IPF) diagnosis
- Transbronchial biopsy (bronchoscope down nose, outpatient) not useful, but can exclude others
– find UIP in surgical lung biopsy
Idiopathic pulmonary fibrosis (IPF) treatment
- O2 therapy to prevent or delay cor pulmonae
– IFN-g can also help by blocking TGF-B, which is part of the epithelial injury pathway
Sarcoidosis path
- lots of evidence that it is Ag driven
- Histiocytes, Dendritic cells (both APCs), and T-lymphocytes are big
– histiocytes are differentiated macros, that aggregate to look like an epithelium
Sarcoidosis and TH1
- TB and sarcoidosis (and virus and fungus) are mediated by TH1 cytokines (TNF-a, IL-12, IL-18, IL-2, IFN-g) -> progression to fibrosis
– asthma and parasites are by TH2
– the greatest linkage in genomic studies is to HLA region (the MHC genes)
Sarcoidosis Putative antigens
Mycobacteria and Propionibacteria (gram + thing in hair follicles)
Sarcoidosis and Lofgren’s syndrome
- fever, BHL, and polyarthritis
– erythema nodosum (raised red bumps on the back of the legs, esp in whites)
- lupus pernio (represents chronic - indurated plaques and discoloration of the nose, esp in blacks)
– Adjacent joints usually swollen and painful
Sarcoidosis Stages
- 1 presents has BLH and normal lung parenchyma (2/3 spontaneous resolution)
– 2 has parenchymal and BHL
- 3 has parenchymal and no BHL
- 4 has fibrosis with hilar retraction, honeycomb changes, and large bullae and cysts – lungs are usually clear on exam
Sarcoidosis HRCT
- nodules, ground glass opacification, intense opacification
- reticular patttern or honey combing
Sarcoidosis Serological clues
- Peripheral lymphopenia
- ↑ serum ACE
- Hypercalcemia
Sarcoidosis Dx
- need tissue
- must see granulomas and not other cause of them (TB, fungus, cancer)
Sarcoidosis Treatment
– Hydroxychloroquine (anti-malerial) alters the pH of the MHC containing peptides to reduce the amount of antigens presented
- Corticosteroids most effective and mainstay
- Immuonsuppressive agents
- Anti-TNF stops TNF from forming granulomas (bad in TB - will cause dissemination)