Restrictive Disorders

Question 1

Group of lung dz affecting the interstitium
Restrictive deficits
Diffuse Parenchymal Lung Dz (DPLD); newer term

Answer 1

= Interstitial Lung Dz (ILD); older term

Question 2

MC Dx of ILD/DPLD
Cough, DOE
PFTs, serial CXRs (bilat progressive fibrosis), CT (honeycombing, small ground glass)
Dx of exclusion (Bx excludes others)
Tx prednisone + azathioprine 3-6 months

Answer 2

Idiopathic Pulmonary Fibrosis (IPF)

Question 3

Multisystem dz c granulomatous inflamm
Non-infectious
Swed, Denmark, Norway, AA (female), Japan

Answer 3

Sarcoidosis

Question 4

Signs/Sxs (Sarcoid)

Answer 4

>90% pulm findings (DOE, dry non-productive cough)
Also: 
Skin: Erythema NODOSUM, lupus pernio, 
Heart: Ventricular arrhythmias, 
Ocular: UVEITIS, Iritis
Parotid gland enlargement, LAD
ARTHRALGIA
CNS
Granulomatous hepatitis (hepatomegaly)

Question 5

Dx (Sarcoid)

Answer 5

Bx: NON-CASEATING GRANULOMAS
CXR: bilateral perihilar adenopathy
Inc ACE, ESR, CRP, +RF
HYPERCALCEMIA (2/2 inc calcitriol)
Hypergammaglobulinemia
CBC - leukopenia, eosinophilia

Question 6

Tx (Sarcoid)

Answer 6

Oral corticosteroids

Question 7

Group of lung disorders based on environmental exposure

Answer 7

Pneumoconioses

Question 8

Chronic fibrotic lung
Inhal of coal dust ingested by alv macrophages
Coal, graphite, WV, PA
CXR mult UL nodular opacities, coalesce

Answer 8

Coal Worker’s Pneumoconiosis

Question 9

Formation of small rounded opacities
Prolonged inhalation of silica
Sand, rock, stone, quarry, brick, mortar
INC incidence of pulmonary TB
CXR UL > LL, Calcified hilar LN, "egg shell"

Answer 9

Silicosis

Question 10

Nodular interstitial fibrosis
Progressive dyspnea
Smoking accel disease & inc risk of lung ca
Assoc c MESOTHELIOMA
PLEURAL PLAQUE, clubbing
CXR (fibrosis>cysts>honeycombing); LL > UL
Insulation, construction, shipyard, plumber

Answer 10

Asbestosis