Sarcoidosis 12/07

Question 1

What lung disease pattern?

Answer 1

Restrictive disease/interstitial lung disease

Question 2

What cells?

Answer 2

CD4 T helpers against unknown antigen

Question 3

What granulomas?

Answer 3

Noncaseating

Question 4

What granulomas involves most commonly?

Answer 4

Hillar lymph nodes and lung —> restrictive lung disease

BILATERAL HILLAR ADENOPATHY

Question 5

What are finding in granuloma?

Answer 5

Asteroid body

Question 6

Where is specifically asteroid body

Answer 6

WITHIN the giant cells of the granulomas

Question 7

If uvea involved -> ?

Answer 7

anterior uveatis

Question 8

If skin involved –> ?

Answer 8

Cutaneous nodules or erythema nodosum

Question 9

Is saliva and lacrimal involved?

Answer 9

mimics sjogren syndrome

Question 10

Clinical features?

Answer 10

Most commonly - Dyspnea or cough;
Elevated ACE
Hypercalcemia
Incr. CD4/CD8 ratio in bronchioalveolar lavage fluids.

Question 11

Why there is hypercalcemia?

Answer 11

1-alpha hydroxylase activity of epithelioid histiocytes (aka activated macrophages) converts vitamin D to its active form

Gaunasi, kad yra PTH-independent 1,26 dihydroxyvitamin D production

Question 12

Treatment?

Answer 12

Immunosupression. Usually revolves spontaneously.

Question 13

Activated macrophages produce whta? (2)

Answer 13

1-alpha-hydroxylase (in all granulomas)
ACE

Question 14

What produce CD4 T cells?

Answer 14

TNF-alfa -> drive macrophage activation and granuloma formation

Question 15

TNF-alfa -> drive macrophage activation and granuloma formation.
What cells?

Answer 15

CD4 T cells

Question 16

Where are present granulomas in liver?

Answer 16

Around portal veins

Question 17

What is hepatic presentation?

Answer 17

Asymptomatic hepatomegaly
incr. in alkaline phosphatase>aminotransferases

Question 18

If joints involved –> ?

Answer 18

arthralgias

Question 19

Presentation of erythema nodosum?

Answer 19

tender, subcutaneous, lover extremity nodules

Question 20

HP. What cells?

Answer 20

CD8

Question 21

HP. What type hypersensitivity?

Answer 21

III/IV

Question 22

HP. population?

Answer 22

Farmers and bird keepers

Question 23

HP mechanism?

Answer 23

exaggregated immunologic response to an inhaled antigen

Question 24

HP pattern?

Answer 24

restrictive

Question 25

HP. biopsy of both - acute and chronic?

Answer 25

Lymphocytic infiltrate (CD8) with poorly formed noncaseating granulomas

Question 26

Acute HP. presentation?

Answer 26

abrupt onset. Fever, chills, gouh, dyspnea, fatigue - THAT COINDICE with intermittent HIGH-DOSE antigen exposure. Auscultation - fine cracles

Question 27

Acute HP x ray?

Answer 27

normal or scattered micronodular opacification

Question 28

Chronic HP. presentation?

Answer 28

Gradually prgressive symptoms - cough, dyspnea, fatigue, WEIGHT LOSS, resulting from LONG-TERM MODERATE DOSE of antigen exposure. Auscultation - fine cracles X ray - difuse interstitial reticular opacities (fibrosis and interstitial inflammation) - consistent with development of pulmonary fibrosis

Question 29

HP. Changes in gas exchange?

Answer 29

hypoxemia, low DLCO

Question 30

Chronic HP is assoc with what?

Answer 30

Alveolar septal fibrosis

Question 31

Treatment?

Answer 31

Resolves with removal of the antigen exposure. However, chronic HP is often irreversible and leads to interstitial fibrosis.

Question 32

What is normal BAL?

Answer 32

85 proc. alveolar macrophages 10 proc. lymphocytes small percentage of neutrophils and eosinophils.

Question 33

chronic HP x-ray?

Answer 33

X ray - difuse interstitial reticular opacities (fibrosis and interstitial inflammation) - consistent with development of pulmonary fibrosis