Lungs 7

Front 1

causes of restrive lung disease

Back 1

- Interstitial lung disease (most common)
- Abnormalities of the chest wall
- Weakness of the respiratory muscles

Front 2

Abnormalities of the chest wall and PFTs

Back 2

- Fibrothorax and Morbid obesity
– it will decrease FVC

Front 3

Interstitial lung disease and PFTs

Back 3

- Sarcoidosis and Idiopathic pulmonary fibrosis (IPF)
– it will decrease TLC, FVC and RV

Front 4

Weakness of the respiratory muscles and PFTs

Back 4

- Myasthenia Gravis and ALS
– it will decrease TLC and increase RV

Front 5

interstitial lung disease mimicks

Back 5

CHF
Pulmonary infection (esp PCP)
Lymphangitic carcinomatosis

Front 6

interstitial lung disease path

Back 6

- ↓ 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

Front 7

interstitial lung disease Sx

Back 7

- Dyspnea and cough
- Crackles (rales), Clubbing, Cor pulmonale (so JVD, Loud P2, TR, Lower extremity edema)

Front 8

interstitial lung disease radiology

Back 8

- CXR shows ↓ lung volumes
– also interstitial pattern, which is reticular (increased linear markings), and reticulonodular (linear and small nodules)

Front 9

interstitial lung disease HRCT (high resolution CT)

Back 9

– 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)

Front 10

interstitial lung disease bronchoscopy

Back 10

- to exclude infections
- BAL cellular differential can support a dx
– a transbronchial biopsy is for granulomatous disease or lymphangitic spread of cancer

Front 11

interstitial lung disease VATS biopsy

Back 11

- 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

Front 12

Idiopathic pulmonary fibrosis (IPF) definition

Back 12

- 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)

Front 13

Idiopathic pulmonary fibrosis (IPF) path

Back 13

- 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

Front 14

Idiopathic pulmonary fibrosis (IPF) clinical

Back 14

- 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

Front 15

Idiopathic pulmonary fibrosis (IPF) rad and hist

Back 15

- 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

Front 16

Idiopathic pulmonary fibrosis (IPF) diagnosis

Back 16

- Transbronchial biopsy (bronchoscope down nose, outpatient) not useful, but can exclude others
– find UIP in surgical lung biopsy

Front 17

Idiopathic pulmonary fibrosis (IPF) treatment

Back 17

- 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

Front 18

Sarcoidosis path

Back 18

- 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

Front 19

Sarcoidosis and TH1

Back 19

- 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)

Front 20

Sarcoidosis Putative antigens

Back 20

Mycobacteria and Propionibacteria (gram + thing in hair follicles)

Front 21

Sarcoidosis and Lofgren’s syndrome

Back 21

- 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

Front 22

Sarcoidosis Stages

Back 22

- 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

Front 23

Sarcoidosis HRCT

Back 23

- nodules, ground glass opacification, intense opacification
- reticular patttern or honey combing

Front 24

Sarcoidosis Serological clues

Back 24

- Peripheral lymphopenia
- ↑ serum ACE
- Hypercalcemia

Front 25

Sarcoidosis Dx

Back 25

- need tissue
- must see granulomas and not other cause of them (TB, fungus, cancer)

Front 26

Sarcoidosis Treatment

Back 26

– 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)