Rheumatology

Question 1

What effect does allopurinol have on azathioprine?

Answer 1

Enhances the effects of azathioprine and hence the dose of azathioprine should be reduced by 75%

Question 2

In a pt with SLE who is ANA negative, what antibody are the usually + for?

Answer 2

Anti Ro

Question 3

What is the first treatment for oligoarticular JIA

Answer 3

1st NSAIDs + intra articular steroids

Question 4

In osteogenesis imperfecta alk phos levels are usually elevated T/F

Answer 4

F - usually blood tests in OI show no particular abnormality

Question 5

Osteogenesis imperfecta can be associated with hearing impairment T/F

Answer 5

T
Other extra skeletal features: blue/grey sclera, discoloured teeth that break easily (dentinogenesis imprefecta), ligamental laxity, increased skin laxity

Question 6

Bisphosphonate infusions are used in the treatment of osteogenesis imperfecta T/F

Answer 6

T

Question 7

Myasthenia gravis is associated with what antibodies?

Answer 7

Antibodies against acetylcholine receptors

Question 8

Criteria of systemic onset juvenile idiopathic arthritis excluding the characteristic rash (SOJIA)

Answer 8

At least 2 weeks of fever, followed by arthritis in one or more joints PLUS generalised lymphadenopathy or hepato or splenomegaly or serositis

Question 9

5 features of Kawasaki disease in addition to fever

Answer 9

1. Lymphadenopathy in neck at least 15mm
2. Lip or oral cavity erythema or cracked lips
3. Conjunctival injection
4. Rash on trunk
5. Swelling/erythema of hands or feet

Need 4 of these + 5 days of fever for kawasaki diagnosis

Question 10

Criteria for the diagnosis of juvenile dermatomyositis?

Answer 10

Pathognomonic rash + 3 of

1. Proximal muscle weakness
2. Elevated serum levels of muscle enzymes
3. Typical electromyographic changes
4. Histopath changes on muscle biopsy

Note: if option between biopsy and electromyography do electromyography first as it is less invasive

Question 11

In juvenile dermatomyositis calcinosis occurs in the majority of children T/F

Answer 11

F - in 40%

Note: JDM is not associated with malignancy

Question 12

How many joints need to be involved to diagnosis polyarticular JIA?

Answer 12

5 or more joints involved in the first 6 months.

Question 13

JIA is assoc with what eye pathology?

Answer 13

Chronic anterior uveitis

Note: often clinically silent

Question 14

Behcet’s disease is assoc with what eye pathology?

Answer 14

Anterior, posterior or pan uveitis

Note: often severe recurrent attacks, usually presents about 3-4 yrs after oral and genital ulcers

Question 15

What is Still’s disease AKA?

Answer 15

Systemic onset juvenile idiopathic arthritis

Question 16

What would be expected for coags and plt count in HSP?

Answer 16

Normal

Question 17

In a pt with active SLE serum complement levels are invariably reduced T/F

Answer 17

T - this is due to the formation of immune complexes

Question 18

What is the most common sub type of JIA?

Answer 18

Oligoarticular

Note: 50-60% of ALL cases of chronic arthritis of childhood

Question 19

Polyarteritis nodosa is assoc with what antibody and other disease?

Answer 19

ANCA (positive in 50% of cases)

Hepatitis B

Note: medium vessel vasculitis

Question 20

Which ANCA is positive in granulomatosis with polyangitis and microscopic polyangitis?

Answer 20

Granulomatosis = cANCA

Micro polyangitis = pANCA

Note: granulomatosis with polyangitis affects nasal/sinuses but micropolyangitis does not. Both affect lung and kidneys. Micro polyangitis may also have non specific abdominal and joint pain

Question 21

Neonatal lupus may be present at birth but can also develop months later T/F

Answer 21

T

Question 22

Is the facial rash in neonatal lupus a malar distribution?

Answer 22

No, it is not the typical malar distribution that is seen in older children/adults with lupus

Note: rash may be precipitated by phototherapy for hyperbili

Question 23

In a patient with polyarticular JIA what is the next medication after the have failed NSAIDS?

Answer 23

Methotrexate

Question 24

Children with ank spond may experience inflammatory back pain. Common clinical features of this type of pain?

Answer 24

Pain at night with morning stiffness and improvement throughout the day with exercise (No improvement with rest)
Insidious onset
Good response to NSAIDs

Question 25

How does ank spond typically present in children?

Answer 25

Enthesis and peripheral arthritis of lower limbs | Axial disease and inflammatory back pain are less common at disease ONSET than in the adult pop

Question 26

What cardiac abnormality is often present in ank spond?

Answer 26

Aortic regurgitation

Question 27

In patients with SLE who have a non photosensitive rash what antibody is most specific and what antibody tends to be negative

Answer 27

Anti Ro is most specific ANA tends to be negative

Question 28

What is the difference in eye disease in those with JIA vs enthesitis related arthritis?

Answer 28

JIA - 70% of patients will have bilateral | Enthesitis - more likely unilateral

Question 29

HSP is associated with IgA deposits in the mesangium T/F

Answer 29

T Note: HSP is a small vessel vasculitis

Question 30

Enteropathic arthritis is associated with positive IgM rheumatoid factor T/F

Answer 30

F - it is a seronegative arthritis Note: patients with IBD have increased risk of arthritis when disease is active

Question 31

What is the remission rate of polyarticular JIA

Answer 31

About 30%

Question 32

The low dose OCP is CI in those with SLE

Answer 32

False - if they have low disease activity and a documented negative anti phospholipid ab Note: avoid OCP with high dose oestrogen

Question 33

In a pt with SLE neutropenia is the most common haem abnormality T/F

Answer 33

F- a mild leukopenia is the most common abnormality usually caused by lymphopenia, neutropenia is rare

Question 34

In a pt with SLE lymphopenia is the most common haem abnormality T/F

Answer 34

T

Question 35

What is the most common form of JIA?

Answer 35

Persistent oligoarthritis Usually girls, around 3 yrs old

Question 36

Rheumatoid factor and ANA are usually positive in systemic JIA T/F

Answer 36

F - they are both usually negative

Question 37

Is systemic JIA more common in girls or boys?

Answer 37

Equal Note: compare to polyarticular or oligoarticular which are more common in females

Question 38

The rash of systemic JIA often reappears with stroking or minor trauma T/F

Answer 38

T Note: this is known as Koebner phenomenon

Question 39

Side effects of cyclophosphamide?

Answer 39

Myelosuppression Infertility Alopecia

Question 40

HLA B27 is often positive in which arthritis

Answer 40

Psoriatic arthritis | Enthesitis-related arthropathy

Question 41

Features of hyper-IgD

Answer 41

Fever, headache, nausea, vomiting, oral and vaginal ulcers. Large joint arthritis, diffuse non-migratory erythematous rash, splenomegaly Usually presents by one year of age

Question 42

Cryopyrin-assoc periodic syndromes are assoc with what gene mutation?

Answer 42

NLRP3 Note: 3 types - familial cold auto inflammatory syndrome, Muckle-Wells syndrome and neonatal onset multi system inflammatory disease

Question 43

Autosomal recessive mutation in MEFV, what pathology?

Answer 43

Familial Mediterranean fever

Question 44

Treatment of familial Mediterranean fever?

Answer 44

If persistent treat with colchicine

Question 45

Criteria for diagnosis of familial Mediterranean fever?

Answer 45

``` 3 or more episodes Lasting up to 96 hours 2 or more of: fever abdo pain chest pain arthritis family hx of the disorder ```

Question 46

Skin findings in reactive arthritis

Answer 46

Keratoderma blennorrhagicum on palms and soles | Oral and genital ulcer and balanitis

Question 47

Pathergy is a manifestation on Behcet disease T/F

Answer 47

T

Question 48

What is the most common long term complication of HSP?

Answer 48

Chronic renal disease

Question 49

Factors that increase the risk of chronic renal disease in HSP?

Answer 49

``` HTN at presentation Females Older age Persistent/recurrent purpura Haematuria and proteinuria (compared to haematuria alone) ``` Note: tx of HSP is with NSAIDs provided renal function is ok

Question 50

Patients with reactive arthritis usually have an elevated CRP and ESR T/F

Answer 50

F - CRP usually normal. ESR may be elevated Note: other lab abnormality sterile pyuria

Question 51

Antihistone antibody is associated with what pathology?

Answer 51

Drug induced lupus

Question 52

What to antibodies are specific for SLE?

Answer 52

Anti double stranded DNA | Anti Smith

Question 53

What complications of Behcets cause the most morbidity and mortality?

Answer 53

CNS complication (stroke, seizures, meningoencephalitis) Blindness Large vessel aneurysm or thrombus

Question 54

Recurrent episodes of fever, anterior cervical lymphadenopathy, exudate pharyngitis, aphthous ulcers, what pathology and what treatment?

Answer 54

PFAPA Tx: steroids, usually respond to a single dose 2mg/kg but can be repeated if symptoms recurrent in 2-3 days. Tx is indicated if children are missing school or adults need to miss work to stay home with child. Tonsillectomy only if refractory to treatment

Question 55

Involvement of what organs are poor prognostic indicators in SLE?

Answer 55

Renal | Cerebritis

Question 56

Drug Rash with Eosinophila and Systemic Symptoms is associated with reactivation of what virus in 80% of cases

Answer 56

HHV 6

Question 57

Anti-U1 RNP is a distinctive autoantibody for what disease?

Answer 57

Mixed connective tissue disease