Rheumatology

Question 1

RF for hydralazine drug induced lupus

Answer 1

• high dose >200mg
• slow acetylator
• HLA-DRw4
• > 3month of therapy
• Female
• FHx of autoimmune

Question 2

Antibodies associated with anti synthatase sx

Answer 2

Anti-Jo1
Anti PL-7
Anti PL 12

Question 3

scleromalacia perforans

Answer 3

Anterior necrotizing scleritis without inflammation, so called scleromalacia perforans, is a rare, severe eye disorder developing an autoimmune damage of episcleral and scleral performing vessels (hypersensitivity type III)

Associated with GPA and also RA

Question 4

RA

Answer 4

Rheumatoid nodules are commonly seen in seropositive patients treated with Methotrexate alone.

Question 5

Which clinical features of GCA is associated with positive biopsy?

Answer 5

Jaw claudication

Question 6

GCA

Answer 6

Genetic association:
- IL17A, IL33, HLA DRB104

Question 7

Which group of agents should we not precribe colchicine with due to increased risk of SE?

Answer 7

Strong CYP3A4 and P-gp inhibitors
amiodarone, cyclosporin, digoxin
diltiazem, antifungals such as itraconazole, macrolide antibiotics, statins and fibrates

Question 8

What shoulder patholgy exhibits the painful arc?

Answer 8

Supraspinatus tendonitis

Question 9

Secukinumab

Answer 9

IL17a inhibitor used in the treatment of ankylosing spondylitis and associated arthropathy. It has effect on both peripheral and axial arthritis and appears to slow radiographic progression. It is unclear whether this drug can worsen inflammatory bowel disease and therefore the current recommendation is to avoid using this drug during IBD flares. Secukinumab has some benefit in improving psoriasis

Question 10

What kind of ILD is RA more like?

Answer 10

UIP

Question 11

Belimumab

Answer 11

Belimumab is a human monoclonal antibody that inhibits B-cell activating factor, also known as B-lymphocyte stimulator. It causes reduction in B cell survival instead of depletion (which is what Rituximab causes)

Question 12

Histological features of GCA

Answer 12

Panarteritis (CD4 Lt, histiocytes, plasma cells)
Giant cell granuloma
Distruption to the internal lamina (not specific)
Patchy and skin lesions
Thrombosed and stenosed vessels

Disruption of the internal elastic lamina is a hallmark feature and tends to persist even after steroid treatment.

Question 13

If you have GCA and your vision is threatened, what is the treatment?

Answer 13

Methylpred

Question 14

Does the use of OCP increase or decrease the risk of RA?

Answer 14

Decrease (esp those who are CCP positive)

Question 15

Factors that increase the risk of lymphoma in those with sjogren’s disease

Answer 15

• Recurrent swelling of the parotids
• Presence of RF
• Presence of purpura
• Cryoglobulinemia
• Splenomegaly, Lymphadenopathy
• Low CD4+
• Low C4
• Presence of ectopic germinal centres
• Germinal mutation TNFAIP3

Question 16

Felty syndrome is associated with HLA DR4

Answer 16

True

Bone marrow examination shows normal myelopoiesis but with maturation arrest. There is increase susceptibility to infection as well as an increased risk of developing a lymphoproliferative disorder. Treatment involves rheumatoid arthritis treatment as well as granulocyte colony stimulating Factor to address the neutropenia.

Question 17

What is the gender predominance for reactive arthritis?

Answer 17

M:F
3:1

Question 18

Features consistent with secondary Raynaud’s

Answer 18

Male
Age >40
Abnormal capillaries in nailfold
Known precipitant
Ulceration
Asymmetric

Question 19

RF for developing SRC in SSc

Answer 19

DIFFUSE SKIN INVOLVEMENT

OThers:
- steroid use
- cyclosporin
- presence of Ab

Question 20

HLAb27 and inflmmatory conditions

Answer 20

Anks. Spond 90%
Reactive 70%
Psoriatic 50%
IBD 30%

Question 21

Febuxostat; there is concerns re. increased mortality in which patietns?

Answer 21

IHD, CHF, hepatic failure

Question 22

Large granular lymphocyte syndrome (LGL)

Answer 22

is a condition that is associated with rheumatoid arthritis. 1 and 3 patients with rheumatoid arthritis who have neutropenia have this condition. It is also associated with lymphocytosis, anaemia, and thrombocytopenia. Bone marrow assessment shows evidence of clonal lymphocyte proliferation. This condition is often managed with immunosuppressive therapy

Question 23

NOD-like receptor protein 3 (NLRP3) inflammasome; which disease is it involved in?

Answer 23

gout

Question 24

Behcet’s

Answer 24

AU, genital ulcers and oral ulcers
HLA-B51

Question 25

Antibodies in antisynthatase sx

Answer 25

triad of interstitial lung disease, inflammatory arthritis, and inflammatory myositis Antibodies against tRNA synthetase are present Anti Jo1, PL7 PL12

Question 26

Mixed connective tissue disease

Answer 26

female 30-40 Rayneud's initial 90% Anti U1RNP MUSTbe postive ## Footnote Anti-U1RNP can be seen in other disorders such as systemic sclerosis and systemic lupus erythematosus, and so antibodies for these conditions, anti-centromere and anti-Scl70 along with anti-dsDNA, respectively, must be negative.

Question 27

FMF recessive or dominant

Answer 27

recessive

Question 27

FMF recessive or dominant

Answer 27

recessive

Question 28

for GCA, when does sensitivity of imaging drop after steriod tx?

Answer 28

2-4 days

Question 29

What is a biopsy requirement for the diagnosis of inclusion body myositis

Answer 29

biopsy must show in the endomysial inflammation rimmed vacuoles with protein inclusion is not required

Question 30

what does HLA B27 suggest in reactive arthritis?

Answer 30

More severe and chronic disease

Question 31

IBD associated spondyloarthritis

Answer 31

1-20% of IBD patients - more common in those with extraintestinal features, complications of bowel disease, large bowel in involvement in crohn's 2 subtypes: - axial - similar to AS - peripheral: usually acute, oligoarticular, LL deformitis and erosions are rare self limiting - related to flares of IBD Tx: NSAID DMARDs if peripheral dx Anti TNF for joints and bowel Axial dx- treat as per AS (**but avoid IL 17 blocker - secukinumab)**

Question 32

what is the ANA pattern in dSSc

Answer 32

Nucleolar

Question 33

CCB are not effective in CTD related PAH

Answer 33

TRUE even the usual treatments are less effective ## Footnote interesting fact: iron deficiency correlated with worse prognosis in SSc-PAH

Question 34

SRC

Answer 34

Associated with RNA polymerase 3 ab occurs within 5 years of diagnosis Characterised by: -abrupt onset of moderate to severe HTN - normal urine sediment or only mild proteinuria with few cells or casts - progressive renal failure - does **not** cause glomerulonephritis - Occurs in upto 10-20% of patients with diffuse SSc and mucgh less frequently in those with limited SSc RF: **Tendon frictuon rub,** RNA polymerase 3 ab **triggered by steroids** Signs of severe SRC: - MAHA and thrombocytopenia - HF and falsh pulm. oedema - Blurred vision due to retinopathy - HA, fever, malaise - Encephalopathy +/- seizures - Pericardial effusion Treatment: - if untreated can lead to ESRD within 1-2 months - prompt BP control increases 1 year survival by 80% OPTIONS: - ACEi - captopril (first line) - non first line: ARB, IV prostacyclin , ACEi and PLEX (better survival), ACEi and bosentan (prseved renal funcion) CNS involvement: ACEi and IV nitroprusside **treatment with above even if normotensive**

Question 35

High risk for SSc-SRC

Answer 35

dc SSc older age MALE STEROIDS RNAP3 ANA - speckeld Pericardial effusion

Question 36

SSC-SRC

Answer 36

renal outcomes: - 20-50% still progress to ESKD despite ACEi - Dialysis is more difficult - Improvment in RF can continue for upto 18mo - Mortality is still high - limited experience with renal transplant in SSc HIGH BP at presentation = **better outcome** if on ACEi prior to SRC = worse outcome Temporary dialysis and no dialysis > permenant dialysis MEN = poor prognosis

Question 37

SSC-Raynauds

Answer 37

- avoid caffeine, stop smoking 1. CCB (dihydropyridine) 2. ARB, PDE5i, Alphablocker, SSRI 3. IV prostacylin PGI2 in severe

Question 38

SSc-digital ulcers

Answer 38

- avoid cold - pain relief - dressing - tx infection 1. iloprost should be considered 2. PDE5i considered 3. Bosentan for reduction of number of new ulcers

Question 39

SSc - GI

Answer 39

- Oropharyngeal: rigidity of facial musclces and tongue ==> swallowing difficulty - reduced saliva production - inco-ordination of swallow ~25% >oral leakage >retention >aspiration >difficulty swallowing **GORD: 90% of pts with both diffuse and limited ** - absent motility in >50% - Tx: soft food; avoid opioids, smoking, alcohol; PPT - prokinetic (masopride) - treat moniliasis (candidiasis)

Question 40

dSSc-GI | Small bowel

Answer 40

dysfunctional - bloating and distention diverticulae SIBO - diarrhoea, bloating, weight loss Intussuception/volvulus Pneumatosis intestinalis

Question 41

# SSc Management of abdominal pain/distention

Answer 41

Prokinetic: Prucalopride Domperidone/Metoclopramide Erythromycin - *powerful motilin agonist, causes powerful contraction* Oral procholinergic: pyridostigmine bethanacol

Question 42

Treatment options for SIBO

Answer 42

Doxycycline, metronidazole, ciprofloxacin Rifaxamin

Question 43

Auto HSCT in SSc

Answer 43

- conditioning regime is cyclophosphamide or rabbit ATG - Treatment related mortality was all within the first 60 days especially in smokers disease relapse in 37% ## Footnote Cardiac complication with HSCT: - cyclophosphamide haemorrhagic pericarditis - Radiation induced injury \

Question 44

# SSc SSc-cardiac

Answer 44

Microvascular CAD >60% Primary RV dysfunction LV diastolic dysfunction

Question 45

Which DM antibody is associated with higher risk of ILD including rapid progression and mortality?

Answer 45

Anti MDA-5 abs

Question 46

clinically amyopathic DM

Answer 46

10-30% of DM Cutaneous features without muscle invovement **increased risk malignancy More likely to be MDA5 +ve**

Question 47

what are the cancer associated abs in DM>

Answer 47

Anti TIF1g Anti NXP-2

Question 48

myositis specific abs predictive of good and bad prognosis

Answer 48

Worse: Jo-1, SRP Better: Mi-2, overlap (RNP, PMScl, Ku)

Question 49

Treatment for DM,PM, IMNM

Answer 49

High dose GC, taper over year Steroid sparing agents: - AZA - MTX - IVIG - RITUX - PLEX Monitor: CK, PFT, muscle power

Question 50

IBM

Answer 50

Asymm Distal finger flexors Dyspahgia CK elevatd, but not useful for monitoring 15% have AI disease EMG, MRI muscle biopsy Less responsive to immunosuppresive therapy

Question 51

which joint does OA spare in the hand?

Answer 51

MTP joints

Question 52

hook osteophyte in the context of CPPD, indicates?

Answer 52

haemochromotosis

Question 53

# Vasculitis P-anca are associated with more frequent relapses

Question 54

# Vasculitis Hypersensitive vasculitis is also called:

Answer 54

cutaneous or leucocytoclastic ARA criteria: 3/5 Age>16 Temporal relationship to drug Palpable purpura Maculopapular rash Perivascular neutrophils on skin biopsy Common triggers: frusemide, sulfonamides, penicillins, cephalosporins, allopurinols, phenytoin

Question 55

# Vasculitis MPA

Answer 55

Palpable purpura + systemic features Glomerula haematuria, renal impairment +/- abnormal LFTs p-ANCA 50% of haematuria and haemoptysis is due to MPA or GPA Mononeuritis multiplex

Question 55

# Vasculitis MPA

Answer 55

Palpable purpura + systemic features Glomerula haematuria, renal impairment +/- abnormal LFTs p-ANCA 50% of haematuria and haemoptysis is due to MPA or GPA Mononeuritis multiplex