AICTD

Question 1

SLE autoab

Answer 1

Antismith
Anti dsDNA (lupus nephritis)
Anti histone (drug induced)
C1q (severe SLE)
rRNP (neuropsychiatric lupus)

Question 2

SCLE

Answer 2

Anti SSA Ro

Question 3

SSc

Answer 3

RNA polymerase III: acute SSc, renal crisis, breast cancer, rapidly progressive SSc
U3RNP: severe SSc
U1RNP: scleroderma renal crisis
Anti-Ku: overlap SSc and DM
Anti-topoisomerase 1 (SCL70): diffuse SSc, more ILD
Anti-centromere: limited SSc, more PAH

Question 4

MCTD

Answer 4

U1RNP

Question 5

Anti SSA Ro and La

Answer 5

SCLE
Sjogren

Question 6

Anti Smith

Answer 6

SLE

Question 7

Anti U1RNP

Answer 7

MCTD
SSc

Question 8

Anti SCL70 / topoisomerase 1

Answer 8

dcSSC

Question 9

Anti Jo1

Answer 9

Dermatomyositis - antisynthetase syndrome

Question 10

Anti C1q

Answer 10

Severe SLE

Question 11

RNA polymerase III

Answer 11

SSc - acute SSc, renal crisis, breast cancer. rapidly progressive SSc

Question 12

U3RNP (fibrillarin)

Answer 12

Severe SSc - GIT, PAH, cardiac, myositis

Question 13

Anti centromere Ab

Answer 13

Limited SSc. More PAH, calcinosis

Question 14

Anti Mi2

Answer 14

Dermatomyositis - classic

Question 15

Anti Ku

Answer 15

Overlap SSc and DM

Question 16

SLE Diagnostic criteria

Answer 16

SLE EULAR/ACR 2019 Diagnostic criteria - Clinical - MONARCHS - like butterfly
MSK - joint involvement
Oral/cutaneous - nonscarring alopecia, oral ulcers, SCLE/DLE/ACLE.
Neuropsychiatric - delirium, psychosis, seizure
ANA positive
Renal - proteinuria, renal Bx showing lupus nephritis
Constitutional - fever
Haematologic - leukopaenia, thrombocytopaenia, AI haemolysis
Serosal - pleural or pericardial effusion, acute pericarditis

Immunology -
Antiphospholipid antibodies, complement (low C3 and/or C4) or SLE specific Ab (anti-sm, anti-dsDNA)

Question 17

Familial mediterranean fever
1. Inheritance
2. Gene
3. Clinical
4. Associations
5. Mgmt

Answer 17

1. AR
2. MEFV
3. Recurrent fevers a/w serositis (pleuritis, peritonitis, pericarditis). Erysipelas like erythema
   Last 1-3 days
4. AA amyloidosis
5. Colchicine, anakinra

Question 18

TRAPS
1. Stands for?
2. Clinical
3. Ax
4. Mgmt

Answer 18

1. TNF receptor associated periodic syndrome
2. Long attacks >7/7
   Migratory myalgia with erythematous patches, plaques
3. AA amyloidosis
4. Anti TNFs. Pred

Question 19

CAPS
1. Stands for?
2. Includes? and what they stand for
3. Features MWS
4. General Sx for them all

Answer 19

1. Cryopyrin associated periodic syndromes
2. MWS: Muckle Wells
   FCAS: familial cold autoinflammatory syndrome
   NOMID: neonatal onset multisystem inflammatory disease
3. SNHL, polyarthritis
4. Urticarial papules and plaques, myalgia, arthralgia. Conjunctivitis

Question 20

Schnitzler
1. Clinical
2. Associations
3. Ix
4. Key DDx, how to differentiate

Answer 20

1. Fever, arthralgias, bone pain
   Recurrent nonpruritic wheals
2. IgM monoclonal gammopathy
3. SPEP
   Bx
   Raised ESR
   Neutrophilia
4. Adult onset Still’s. High ferritin. No MGUS