Connective Tissue

Question 1

autoimmune multisystem disorder which causes
lymphocytic infiltration if the skin,
immune complex deposition
and thickened wire loop of capillary walls in the glomeruli

Answer 1

SLE

Question 2

autoimmune multisystem disorder associated with anti-centromere abs. histology sshows increased collagen in skin and organs as well as onion skin thickening of the arterioles

Answer 2

limited scleroderma

Question 3

autoimmune infla,mmatory dosirder of the muscles and skin, cutaneous features include heliotrope rash and gottrons papules.

Answer 3

Dermatomyositis

Question 4

anti-Jo1 abs and myofibre damage

Answer 4

polymyositis

Question 5

anti-scl70, anti fibrillarin and anti RNA pol. histology shows inflammation within or around muscle fibres

Answer 5

diffuse scleroderma

Question 6

speckled pattern on ANA test

Answer 6

mixed connective tissue disease

Question 7

pulseless disase with bruits and claudication. occurs in large vessels

Answer 7

Takayasu’s arteritis

Question 8

occurs in the elderly with scalp tenderness, temporal headache, jaw claudication and raised ESR. histology shows granulomatous transmural inflamm, giant cells.

Answer 8

Giant cell arteritis (temporal arteritis)

Question 9

vasculitis affection the medium sized vessels, with renal failure as main feature. 30% have underlying hepB. microaneurysm beads on angiography.

Answer 9

PAN polyarteritis nodosa

SPARES the lungs

Question 10

red palms and soles with later desquamation, conjunctiviits, fever >5 days. coronary arteries may be involved in aneurysm formation.

Answer 10

Kawasaki

Question 11

medium vessel disease resulting from inflammation of the arteries of the extremities - leads to pain and ulceration on extremities. common in heavy smokers <35.

Answer 11

Buergers disease (thromboangitis obliterans)

Question 12

small vessel disease that presents as a triad of:

1. URT sx - sinusitis, epistaxis, saddle nose
2. LRT sx - cavitation, pulm haemorrhage
3. kidney - crescenteric GN

Answer 12

Wegeners (GPA_

Question 13

small vessel vasculitis with asthma and eosinophilia. can have later systemic involvement.

Answer 13

eGPA

Churg Strauss

Question 14

small vessel vasculitis and pulmonary renal syndrome of 1. pulm haemorrhage 2. GN. p-ANCA positive.,

Answer 14

microscopic polyangiitis

Question 15

IgA mediated small vessel vasculitis in choldren, preceded by an URTI. presents with a palpaple purpuric rash, glomerulonephritis and colicky abdo pain.

Answer 15

HSP

Question 16

c-ANCA for proteinaise 3

Answer 16

Wegeners

we gunna cus we can-ca

Question 17

p-anca for myeloperoxidaseq

Answer 17

churg strauss, MPA

Question 18

multisystem disorder caused by abnormal folding of proteins that are deposited as amyloid fibrils in tissues, disrupting their normal function.

Answer 18

amyloidosis

Question 19

deposition of AL protein, most commonly associated with plasma cell dyscrasias such as multiple myeloma.
most have monoclonal Ig and free light chains (bence jones proteins) in the urine.

Answer 19

primary amyloidosis - AL

Question 20

protei found in secondary amyloidosis

Answer 20

AA protein

produced secondary to chronic infections and inflammation as the A protein is an acute phase protein.

Question 21

common conditions causing secondary AA amyloidosis

Answer 21

1. autoimmune - RA, ankspond, IBD
2. chronic infections - TB, osteomyelitis, IVDU (skin infections)
3. non-immune causes of inflamm - renal cell carcinoma, Hodgkins

Question 22

deposition of b2-microglobulin

Answer 22

haemodialysis associated amyloidosis

Question 23

most common cause of familial amyloidosis

Answer 23

familial mediterranean fever
AR defect causing incresed production of IL-1, attacks of fever and inflamma of serosal surfaces such as pluera, peritoneum, and synovium.

Question 24

what protein is deposited most commonly in FMF and where

Answer 24

AA, renal

Question 25

most common presentation of amyloidosis

Answer 25

nephrotic syndrome

also caues conduction defects, heart failure, macroglossia, HSM, carpal tunnel

Question 26

histology of amyloidosis

Answer 26

apple green birefringence with congo red stain under polarised light
caused by beta pleated sheet configuration

Question 27

non-caseating granulomas and histiocytes, schaumann and asteroid bodies (protein and ca inclusions)

Answer 27

sarcoidosis

Question 28

skin manifestations of sarcoid

Answer 28

lupus pernio on nose, erthema nodosum, skin nodules

Question 29

eye manifestations of sarcoid

Answer 29

anterior uveitis, progressive visual loss, keratoconjunctivitis

Question 30

biochemical manifestations

Answer 30

high Ca, high ACE, high Ig, high ESR

Question 31

common extrapulmonary manifestations

Answer 31

lymphadenopathy, arthrotis, HSM, leucopenia, renal calculi, meningitis, bilateral parotid enlargement.