Diseases of Unknown Origin/Miscellaneous Disorders

Question 1

What % of patients with Sarcoidosis have pulmonary involvement?

Answer 1

90%

Question 2

What are the stages of sarcoidosis?

Answer 2

Stage 0: no radiographic findings
Stage 1: hilar adenopathy only
Stage 2: hilar adenopathy with parenchymal involvement
Stage 3: parenchymal involvement only
Stage 4: Fibrotic changes – pulmonary insufficiency

Question 3

What is the distribution of sarcoidosis?

Answer 3

Apical

Question 4

What syndrome is associated with sarcoidosis when erythema nodosum is also present and what is the triad?

Answer 4

Lofgren’s syndrome

Triad:

• erythema nodosum
• fever
• hilar adenopathy

Question 5

Which stages have the best prognosis?

Answer 5

Stage 0 & 1

Question 6

Sarcoidosis affects which organs/body systems?

Answer 6

• Pulmonary
• Cardiovascular
• Ocular
• Cutaneous
• Liver/Spleen
• Renal
• Nervous system

Question 7

What are the radiographic findings associated with usual interstitial pneumonia (UIP)?

Answer 7

• reticular opacities
• honeycombing
• traction bronchiectasis
• irregular fibrosis
• ground-glass opacity

Question 8

What is the pathology with desquamative interstitial pneumonia?

Answer 8

Alveoli that fill with macrophages. There is a strong association with smoking.

This pneumonia is thought to represent the end stage of respiratory bronchiolitis interstitial lung disease.

Question 9

Lymphocytic interstitial pneumonia (LIP) most commonly occurs in patients with which pre-existing disease?

Answer 9

Autoimmune (particularly Sjogrens syndrome and AIDS)

Question 10

Bronchiolitis obliterans organizing pneumonia is now referred by what name?

Answer 10

Cryptogenic Organizing pneumonia (COP)

Question 11

What characterizes cryptogenic organizing pneumonia from other pneumonia?

Answer 11

Presence of granulation tissue in the alveolar ducts, alveoli AND bronchiolar lumen.

Question 12

What is the preferred distribution of BOOP and what is its appearance?

Answer 12

Peripheral, subpleural and peribronchovascular.

Patchy consolidation or ill-defined nodules.

Question 13

What is a rare, yet pathognomonic radiographic sign for BOOP?

Answer 13

Reverse halo sign (atoll sign) – ground glass opacity surrounded by denser ring of consolidation.

Question 14

Luminal occlusion/stenosis of the bronchioles as a result of submucosal and peribronchiolar inflammation & fibrosis (WITHOUT granulation tissue or polyps/polyposis) is the pathology for which condition?

Answer 14

Obliterative bronchiolitis (not to be confused with BOOP which does have granulation tissue).

Question 15

What is the M/C/C for cryptogenic organizing pneumonia?

Answer 15

Idiopathic mostly (but pulmonary infection and collagen vascular diseases are also causes).

Question 16

Which syndrome is an aggressive form of idiopathic pulmonary pneumonitis and fibrosis and has a poor prognosis (death within 1 year)?

Answer 16

Hamman-Rich syndrome (Acute interstitial pneumonitis)

Question 17

Which condition is almost exclusively found in women of child-bearing age and can present with chylous pleural effusions and pneumothorax from ruptured cysts?

Answer 17

Lymphangioleiomyomatosis

Question 18

What is the pathogenesis of lymphangioleiomyomatosis and which other condition can the pulmonary findings mimic?

Answer 18

Progressive proliferation of spindle cells, resembling immature smooth muscle. Proliferation of the cells along the bronchioles causes air trapping –> cysts.

Radiographically, can present very similar to pulmonary tuberous sclerosis.

Question 19

Pulmonary histiocytosis X:

a) How does it present?
b) Who does it present in?
c) Where does it present?

Answer 19

a) Granulomatous nodules along peribronchial tracts –> reticular/nodular/cystic pattern
b) Young or middle-aged; 90% are smokers
c) Mid to upper lobes

Question 20

Which idiopathic condition presents with diffuse microliths?

What is its clinical presentation and what is the component of the microliths?

Answer 20

Pulmonary alveolar microlithiasis.

Usually an incidental finding (until late stage where there may be respiratory failure).

Microliths = calcium phosphate.

Question 21

What is the aka for Riley-Day syndrome?

Answer 21

Familial dysautonomia

Question 22

What is the pathogenesis of familial dysautonomia?

Where is its common distribution?

Answer 22

Malfunction of the autonomic nervous system resulting in hypersecretion of mucous glands - causing repeated bouts of pneumonia. Seen exclusively in Jewish descent.

M/C seen in right upper lobe.

Question 23

What is the M/C complication of blunt chest trauma?

Answer 23

Parenchymal contusion

Question 24

What is the name of the condition where the whole lung/lobe is twisted 180 degrees?
What are 3 causes for this?

Answer 24

Lung torsion

3 Causes:

a) Spontaneous – have other pulmonary/diaphragmatic abnormalities
b) Post-traumatic – severe compressive trauma to thoracic cage
c) After thoracic surgery

Question 25

Where is the M/C location for a fracture of the trachea/bronchi?

Answer 25

Right side – main stem bronchi; 1-2cm distal to carina; parallel to cartilage rings

Question 26

Fractures of the trachea and bronchi are usually accompanied by what other finding (other than pneumomediastinum)?

Answer 26

Fracture upper 3 ribs.

Question 27

What is the main source of mediastinal hemorrhage?

Answer 27

Bleeidng from aorta

Question 28

Where is the M/C location for an aortic rupture?

Answer 28

Aortic isthmus (90%) – immediately distal to the origin of the left subclavian artery.

Question 29

What is the non-traumatic mechanism for a ruptured diaphragm?

Answer 29

Sudden increase in intra-thoracic or intra-abdominal pressure against a fixed diaphragm.

Question 30

What side is a ruptured diaphragm M/C?

Answer 30

Left (will see stomach and colon in thorax)

Question 31

What is the M/C/C for acute mediastinitus?

Answer 31

Esophageal rupture

Question 32

Where is the M/C location for an esophageal rupture?

Answer 32

Below the cricopharyngeal muscle.

Question 33

What is the pathomechanism with alpha-1-antitrypsin?

Answer 33

Alpha-1-antitrypsin inhibits the action of neutrophil elastase which degrades normal lung tissue. Deficiency of this will cause BASAL emphysema.