Diffuse Parenchymal Lung Disease

Front 1

Diffuse parenchymal lung disease (DPLD)

Back 1

"interstitial lung diseases"
-encompass ~200 entities in which the lung is altered by a combination of interstitial inflammation, granulomatous inflammation, or fibrosis
-most common group: idiopathic interstitial pneumonias

Front 2

H&P

Back 2

H:
-dyspnea: slowly progressive, intermittent, acute/fulminant
-cough
-exposure hx: asbestos, silica, beryillium
PE:
-dry "velcro" crackles
-hypoxemia
-cyanosis
-clubbing

Front 3

Testing in DPLD

Back 3

PFTs: measuring severity
Radiography (HRCT): hints about dx
-xray patterns: nodules, adenopathy, pleural effusions)
-is the disease likely to respond to therapy? honeycomb vs. ground glass
Lung Biopsy: can make dx; granulmoas or malignancy

Front 4

PFTs of restrictive lung disease

Back 4

-restrictive pattern:
dec FEV1 & FVC
Increased FEV-1/FVC ratio (b/c both come down proportionally)
Decreased Total Lung Capacity (TLC)
Decreased Diffusion Capacity (DLCO) (use CO)

Front 5

pathophys of DPLD

Back 5

-low PaO2: V/Q mismatch
-chronic hypoxemia: pulmonary vascular constriction
-leads to right-heart pump overload: cor pulmonale
-lung stuffness: dyspnea

Front 6

DPLP algorthium

Back 6

-look at slide 12

Front 7

Usual interstitial pneumonia (UIP)

Back 7

-pathological finding of idiopathic pulmonary fibrosis
-characterized by interstitial fibrosis that is temporarily heterogeneous
Fibroblastic foci
Subpleural thickening
honeycompbing

Front 8

Idiopathic pulmonary fibrosis (IPF)

Back 8

-characterized by reticular infiltrates on radiology that are predominantly in the lower lobes and subpleural areas
-honeycombing
-Traction Bronchiectasis (dilated vessels)
-no inciting cause

Front 9

Treatment of IPF/UIP

Back 9

1. Steroids w/w/out immunomodulator (methotrexate)
-only 20% pts respond
2. CT scan may predict who will respond
-ground glass -->yes
-honeycomb changes --> no
3. N-acetylcycteine: study-NAC + standard IPF therapy led to slower decline in vital capacity and DLCO over 1 yr

Front 10

Diagnosis of IPF Major Criteria

Back 10

1. Exclusion of other known causes of ILD
2. Abnormal PFTs
3. Bibasilar reticular abnormalities on HRCT scan
4. No histologic or cytologic features on transbronchial lung biopsy or BAL of alt dx

Front 11

Diagnosis of IPF minor criteria

Back 11

Age >50 yr
Insidious onset of otherwise unexplained exertional dyspnea
Duration of illness 3 months
Bibasilar, dry (“Velcro”) inspiratory crackles

Front 12

Nonspecific interstitial pneumonia (NSIP)

Back 12

-histology: termporally uniform, homogenous appearance of either inflamm or fibrosis
2 subtypes: cellular and fibrosing patterns
-radiology: ground class opacities (hazy)
-tx: steroids

Front 13

Desquamative Interstitial Pneumonia (DIP)

Back 13

-alveolar macrophage pneumonia
-diffuse airspace disease with mild fibrosis

Front 14

Interstitial Lung Disease (RB- ILD)

Back 14

-A mild ILD characterized histologically by chronic bronchiolitis in which pigmented macrophages accumulate within respiratory bronchioles and adjacent alveolar spaces.

Front 15

Tx of DIP and RB-ILD

Back 15

STOP SMOKING!

Front 16

Cryptogenic Organizing Pneumonia (COP)AKA: Bronchiolitis Obliterans Organizing Pneumonia (BOOP)

Back 16

-nonspecific rxn to lung injury
-mimic CAP: nonproductive cough, dyspnea
-radiology: patchy airspace consolidation
-tx: corticosteroids--> recurrences common after stopping therapy

Front 17

Sarcoidosis

Back 17

-multi-organ disease affects lungs, lymph nodes, heart, eye, liver and others
-see noncaseating granulomas
-radiology: initially has bilateral hilar lymphadenopathy, eventually fibrosis can develop
-mimic: Berylliosis

Front 18

Sarcodosis tx

Back 18

-monitoring to high dose corticosteroids +/- immunomodulators

Front 19

Lymphangioleiomyomatosis (LAM)

Back 19

-disease of young women of childbearing age
-70% b/t 20-40
-assoc with tuberous sclerosis
-suspect in young women with recurrent pneumothoracies and chylous pleural effusion
-Radiology shows cystic destruction of lung parenchyma

Front 20

Langerhans’ Cell Histiocytosis

Back 20

-can involve other organs, esp bone and lymph nodes
-multiple nodular infiltrates with a stellate lesion
-Radiology: symmetric, bilateral nodular pattern with irregular shaped cysts, that are predominantly in the upper lobes
-smokers disease, stabilizes quit smoking

Front 21

Pulmonary Alveolar Proteinosis

Back 21

-thought to be due to an AB against GM-CSF: Causes dysfunction of alveolar macrophages leading to reduced surfactant clearance.
-radiology: crazy paving
-secondary cause: silica exposure --> silicoproteinosis

Front 22

Tx of Pulmonary Alveolar Proteinosis

Back 22

-whole lung lavage (flooding lung and draining it out with water)- opens up the lung so they can breath
-GM-CSF (experimental)