restrictive lung disease

Question 1

what are the two general causes of extrapulmonary restrictive disease?

Answer 1

1. neuromuscular (Guillain-Barre, Myasthenia Gravis, Poliomyelitis, Postpolio syndrome, ALS, Diaphragmatic disease)
2. diseases of the chest wall (kyphoscoliosis, obesity, pickwickian syndrome)

Question 2

what is pickwickian syndrome?

Answer 2

a serious consequence of obesity-hypoventilation which is characterized b a reduced central drive to breathe.

Question 3

4 requirements for ARDS and ALI (acute lung injury)

Answer 3

1. reduced art O2 to inspired O2 ratio (ALI, 200 < PaO2/FIO2 < 300 mmHG, in ARDS 200 < PaO2/FIO2… normal = 500)
2. Acute onset
3. Bilat. lung infiltrates
4. pulm cap wedge P < 18 mmHg (or normal LAP) to rule out left-seided CHF as a cause of the lung infiltrates

Question 4

what is ARDS and ALI (acute lung injury)

Answer 4

acute-onset diffuse alveolar damage and leakage of fluid out of pulm cap. into interstitium and alveoplar spaces (a restrictive disease)

Question 5

what are hyaline membranes?

Answer 5

pockets of eosinophilic, acellular material that are seen inside the alveoli is ARDS and ALI (acute lung injury) and NRDS (neonatal resp distress synd) in histology

Question 6

predisposing factors to NRDS (neonatal resp. distress synd) (3)

Answer 6

1. prematurity
2. maternal diabetes mellitus
3. delivery by c-section

Question 7

How can you assess fetal pulm. maturity?

Answer 7

measure the ratio of surfactant lecithin to sphingomyelin in the amniotic fluid. A ratio of 2:1 or greater indicates lung maturity

Question 8

4 txt for NRDS

Answer 8

1. exogenous surfactant admin
2. mech. vent w/PEEP
3. Inhaled NO
4. Antenatal maternal corticosteroid therapy to promote surfactant production

Question 9

4 common inorganic causes of Pneumoconiosis (a group of ILD (intersitial lung dis) caused by inhalation of inorganic and organic particulate matter)

Answer 9

1. silicosis
2. coal workers pneumoconiosis (CWP)
3. Asbestosis
4. Berylliosis

Question 10

silicosis is assoc with an increased risk of…

Answer 10

TB

Question 11

berylliosis can mimic what disease?

Answer 11

sarcoidosis (with granulomas in multiple organs)

Question 12

silicosis is associated with what 3 finidings?

Answer 12

1. lung nodules
2. “egg shell calcification in hilar nodes
3. TB

Question 13

CWP (coal worker’s pneumoconiosis) is assoc with what histo finding?

Answer 13

“dust ells” (alveolar macrophages with anthracotic pigment)

Question 14

asbestosis is associated with what two things?

Answer 14

branchogenic carcinoma > > malignant mesothelioma

Question 15

berylliosis is assoc with what findings?

Answer 15

granulomas mimicking sarcoidosis

Question 16

Ferruginous bodies (yellow-brown rods with clubbed ends) are seen in what disease?

Answer 16

asbestosis: the asbestos fibers are taken up by alveolar macrophages and coated with iron (which stains with prussian blue) to form ferruginous bodies that cause diffuse interstitial fibrosis

Question 17

lab findings in sarcoidosis:
calcium
calciuria
IgG
ACE

Answer 17

calcium: hypercalcemia (due to increased 1-alpha-hydroxylase production of activated macrophages leading to increased 1,25-(OH)-Vit. D)
calciuria: hypercalciuria
IgG: hypergammaglobulinemia
ACE: increased ACE activity

Question 18

what on biopsy is suggestive of sarcoidosis?

Answer 18

noncaseating granulomas (core macrophages sur. by T-lymphocytes) in the lung w/neg microbio workup

Question 19

what lung sounds are heard in Idiopathic pulm. fibrosis?

Answer 19

dry cackles or rales

Question 20

“honeycombing” is seen in what?

Answer 20

Usual Interstitial pneumonia (UIP)

Question 21

honeycombing is seen in ______ whereas ground glass opacities are seen in ____

Answer 21

honeycombing: Usual Interstitial pneumonia (UIP)

ground-glass opacities: nonspecific interstitial pneumonia (NSIP)

Question 22

causes of Hypercalcemia “Chimpanzees”

Answer 22

Calcium excess intake (milk-alkali syndrome)
Hyperparathyroidism and Hyperthyroidism 
Iatrogenic (thiazides)
Multiple myeloma
Paget disease
Addison disease
Neoplasm (PTH, PTHrP etc)
z
Excess Vit D
Excess vit A
Sarcoidosis

Z can remind you that both hyperparathyroidism and Zollinger-Ellioson syndrome can be seen in multiple endocrine neoplasia (MEN1)

Question 23

what is goodpasture syndrome?

Answer 23

autoimmune disease targeting the lungs and kidney

Question 24

what is goodpasture syndrome caused by?

Answer 24

T2 hypersensitivity against the alpha3-chain of type IV collagen, located in the bsement membranes of alveoli and glomeruli

Question 25

two causes of rapidly progressive glomerularnephritis, which presents as nephritic syndrome. Histology will reveal crescent-shaped proliferation of glomerular parietal cells and accumulation of fibrin in glomeruli

Answer 25

1. goodpasture syndrome

2. Wegener granulomatosis (GPA)

Question 26

Wegener Granulomatosis (GPA)

Answer 26

an autoimmune vasculitis affecting primarily the URT, lungs and kidneys, but can also affect the joints, skin, eyes or nervous system

Question 27

GPA (wegener’s) affects what size vessels?

Answer 27

vasculitis of small and medium blood vessels, with granulomas surrounding the vessels

Question 28

Wegener granulomatis is a _____-associated granulomatous vasculitis

Answer 28

Antineutrophil cytoplasmic antibody (ANCA)

Question 29

presentation in wegener’s granulomatis

Answer 29

varied!
cough
dyspnea
hemoptysis
persisten rhinorrhea
bloody/purulent nasal disharge
nasal pain
nephritic syndrome
eye and ear symp
arthritis
cutaneous vasculitis

Question 30

diagnosis of wegener’s can be made with (3)

Answer 30

CT: one/several nodules (coin lesions) and infiltrates, often w/cavitation
c-ANCA positive (antiproteinase 3 autoaAb
Biopsy: necrotizing granulomatous vasculitis