sarcoid and sero negative disease

Question 1

how is sarcoidosis characterised

Answer 1

epithelia non caveating granuloma in involved organs

Question 2

how does sarcoidosis present

Answer 2

bilateral hilar lymphadenopathy
pulmonary infiltration
skin/eye lesions

Question 3

peak age of incidence

Answer 3

20s-40s

Question 4

how does it affect lung

Answer 4

parenchymal disease in 90% of cases

wheeze

Question 5

how does it affect skin

Answer 5

lupus pernio, plaques and nodules

Question 6

how does it affect eyes

Answer 6

uveitis
conjunctivitis
sicca

Question 7

how does it affect the lymphatic system

Answer 7

lymphadenopathy

splenomegaly

Question 8

how does it affect BM

Answer 8

infiltration

Question 9

how does it affect liver

Answer 9

failure
granulomata
portal HTN

Question 10

how does it affect kidneys

Answer 10

nephrocalcinosis
granulomata
glomerular disease

Question 11

how does it affect the heart

Answer 11

arteritis, cardiomyopathy, conduction abnormalities

Question 12

how does it affect the nervous system

Answer 12

central and peripheral neuropathy
intracerebral lesions
meningtis
seizures

Question 13

granulomata

Answer 13

endocrine and reproductive organs
GI tract
salivary/lacrimal glands
nose
tonsils
larynx

Question 14

staging of pulmonary disease

Answer 14

stage 0 - normal CXR
stage 1 - bilateral hilar lymphadenopathy
stage 2 - BHL + infiltrates
stage 3 - dense pulmonary infiltrates when functional limitation+ cor pulmonale
stage 4 - fibrosis

Question 15

MSK involvement

Answer 15

acute arthritis
chronic arthritis
bone infiltration
osteoporosis

Question 16

acute arthritis

Answer 16

oligoarthritis
large & small joint
lower limb > upper limb

Question 17

chronic arthritis

Answer 17

dactylitis - swelling and deformity of all fingers are present. some fingers demonstrate erythema and swelling of the nail folds and beds. Marked onychodystrophy.

non deforming small joint arthropathy

Question 18

what is lofgrens syndrome

Answer 18

eyrtheme nodosum
arthralgia/arthritis (bilateral ankle)
bilateral hilar adenopathy - check CXR

Question 19

serum findings in lofgrens

Answer 19

serum ACE level may be elevated - increased activity of activated pulmonary epithelial cells - AT1 converted to AT2 by ACE

Question 20

skin manifestations

Answer 20

• erythema nodosum
• lupus pernio (the most specific associated cutaneous lesion)
• violaceous rash on the cheeks or nose (common)

Question 21

ocular involvement

Answer 21

anterior or posterior granulomatous uveitis
bilateral
conjunctivitis lesions and scleral plaques

Question 22

cardiac involvement

Answer 22

heart block
sudden death
ventricular tachyarrhythmias 
cardiac MRI useful 
annual ECG + ECHO

Question 23

HRCT findings

Answer 23

active alveoli’s v fibrosis

Question 24

biopsy findings

Answer 24

endobronchial - culture for fungus and mycobacterium

Question 25

PFT findings

Answer 25

isolated DLCO
restrictive pattern - advanced disease
some patients have obstruction

Question 26

blood findings

Answer 26

elevated ACE
hypercalcaemia
ALKP

Question 27

biopsy

Answer 27

non caseating granuloma - central collection of modified mononuclear phagocytes which are called epithelioid cells

Question 28

pathophysiology of granuloma

Answer 28

initially CD4+ T cells - gradually become a granuloma

central areas become impacted and develop epithelioid appearance and multinucleate giant cells develop

Question 29

caveating granuloma?

Answer 29

TB

Question 30

treatment

Answer 30

NSAIDS
corticosteroids 
azathioprine - steroid sparing
infliximab
lung transplant