CT disease

Question 1

HLA-DR3
Afro-Caribbean
anti-dsDNA in all (anti-ENA in some - Sm highly specific, RNP if drug-induced)
Classical complement deficiency
Type III hypersensitivity
Malar rash

Answer 1

SLE (3 letters in SLE)

Question 2

anti-Ro, anti-La
Big parotid glands
Schirmer’s test
Dry mouth, dry nose, dry eyes

Answer 2

Sjogren’s

Question 3

anti-SCL70

Answer 3

Diffuse systemic sclerosis

Question 4

anti-centromere

Answer 4

Limited systemic sclerosis (CREST)

Question 5

anti-dsDNA

Answer 5

SLE

Question 6

anti-Ro, anti-La

Answer 6

Sjogren’s

Question 7

anti-Jo1

Answer 7

Dermatomyositis

Question 8

anti-SRP

Answer 8

Polymyositis

Question 9

anti-SCL70
Tight skin over trunk
No CREST
Pulmonary fibrosis

Answer 9

Diffuse systemic sclerosis

Question 10

anti-centromere
No tight skin over trunk
CREST (calcinosis painful fingers, Raynaud’s, esophageal dysmotility, sclerodactyly, telangiectasia)
Pulmonary hypertension

Answer 10

Limited systemic sclerosis (CREST)

Question 11

anti-Jo1
Muscles + skin
Heliotrope Rash
Gottren’s papules

Answer 11

Dermatomyositis

Question 12

anti-SRP
Muscles only
Proximal muscles weakness
Raised CK, abnormal EMG

Answer 12

Polymyositis

Question 13

ANA antibody types

Answer 13

Anti-nuclear antibody:

• anti-dsDNA (SLE)
• anti-ENA (anti-Ro, anti-La Sjogren’s; anti-SCL70 diffuse SS; anti-centromere limited SS)
• anti-cytoplasmic (anti-Jo1 dermatomyositis, anti-SRP polymyositis)

Question 14

ANCA antibody types

Answer 14

pANCA (aka anti-MPO - MPA, CS)

cANCA (aka anti-PR3 - WG) ‘PRIC’

Question 15

pANCA
Granulomas + eosinophils
Saddle nose, pulmonary haemorrhage, glomerulonephritis

Answer 15

Eosinophilic granulomatosis with polyangitis

Question 16

pANCA

Answer 16

Eosinophilic granulomatosis with polyangitis (CS)

Microscopic polyangitis

Question 17

cANCA

Answer 17

Granulomatosis with polyangitis (WG)

Question 18

Palpable purpuric rash

Answer 18

Vasculitides

Question 19

Categories of vasculitides

Answer 19

Small vessel (ANCA vasculitis, IgA vasculitis (e.g. HSP), Goodpasture's, cryoglobulinaemic)
Medium vessel (Polyarteritis nodosa, Kawasakim Buerger's)
Large vessel (Takayasu arteritis, GCA)

Question 20

Pulseless disease
Japanese women
Absent pulse, bruits, claudication

Answer 20

Takayasu’s arteritis

Question 21

Elderly women
Scalp tenderness when combing hair, jaw claudication, blurred vision
Raised ESR
Ass. with Polymyalgia Rheumatica
Histo: Giant cells, granulomatous transmural inflammation, skip lesions

Answer 21

Temporal arteritis (‘Giant cell arteritis’)

Question 22

Bead like aneurysms
Renal + mesenteric vessels
Histo: Necrotising arteritis

Answer 22

Polyarteritis nodosa

Question 23

Prolonged fever

CRASH + BURN

Answer 23

Kawasaki’s

Question 24

Elderly male smoker

Corkscrew appearance on angiography

Answer 24

Buerger’s

Question 25

pANCA | Pulmonary + renal

Answer 25

Microscopic angiitis

Question 26

pANCA Granulomas + eosinophils Asthma

Answer 26

Eosinophilic granulomatosis with polyangiitis

Question 27

cANCA Granulomas Kidney (Rapidly progressive (crescentic) GN), LRTI (pulmonary haemorrhage), URTI (saddle nose, nosebleeds, sinusitis)

Answer 27

Granulomatosis with polyangitis

Question 28

TETRAD preceded by 1-2d URTI IgA deposits and activates alternative complement - Nephritic syndrome (frank haematuria w coke-coloured urine) - Rash (palpable purpura on extensors + buttocks) - Abdo pain - Arthritis

Answer 28

IgA Vasculitis (aka HSP)

Question 29

Circulting Ig due to SLE, MM, post infection (HepC) deposits in vessels

Answer 29

Cryoglobulinaemic vasculitis

Question 30

``` Anti-GBM against collagen type IV Rapidly progressive (crescentic) GN, pulmonary haemorrhage ```

Answer 30

Goodpasture's disease

Question 31

Non-caseating granulomas Afro-Carribean Bilateral hilar lymphadenopathy w/ fine nodular shadowing in mid zones

Answer 31

Sarcoid

Question 32

Bilateral hilar lymphadenopathy differentials

Answer 32

Sarcoid TB Lymphoma Bronchial Ca

Question 33

Abnormal protein folding -> Aggregation into B-pleated sheets -> Organ deposition Apple green birefringence with Congo red stain under polarised light

Answer 33

Amyloidosis

Question 34

Light chains of Ig misfolded | MM

Answer 34

AL Amyloidosis

Question 35

``` Acute phase protein serum amyloid A misfolded Chronic infection (RA, IBD, Ca) ```

Answer 35

AA Amyloidosis

Question 36

Beta-2 microglobulin misfolded | CKD

Answer 36

Haemodialysis associated amyloidosis

Question 37

Familial Amyloidosis

Answer 37

AA amyloidosis in Familial Mediterranean Fever