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Rheumatology Flashcards

1
Q

What effect does allopurinol have on azathioprine?

A

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

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2
Q

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

A

Anti Ro

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3
Q

What is the first treatment for oligoarticular JIA

A

1st NSAIDs + intra articular steroids

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4
Q

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

A

F - usually blood tests in OI show no particular abnormality

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5
Q

Osteogenesis imperfecta can be associated with hearing impairment T/F

A

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

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6
Q

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

A

T

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7
Q

Myasthenia gravis is associated with what antibodies?

A

Antibodies against acetylcholine receptors

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8
Q

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

A

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

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9
Q

5 features of Kawasaki disease in addition to fever

A
  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

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10
Q

Criteria for the diagnosis of juvenile dermatomyositis?

A

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

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11
Q

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

A

F - in 40%

Note: JDM is not associated with malignancy

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12
Q

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

A

5 or more joints involved in the first 6 months.

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13
Q

JIA is assoc with what eye pathology?

A

Chronic anterior uveitis

Note: often clinically silent

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14
Q

Behcet’s disease is assoc with what eye pathology?

A

Anterior, posterior or pan uveitis

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

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15
Q

What is Still’s disease AKA?

A

Systemic onset juvenile idiopathic arthritis

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16
Q

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

A

Normal

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17
Q

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

A

T - this is due to the formation of immune complexes

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18
Q

What is the most common sub type of JIA?

A

Oligoarticular

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

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19
Q

Polyarteritis nodosa is assoc with what antibody and other disease?

A

ANCA (positive in 50% of cases)

Hepatitis B

Note: medium vessel vasculitis

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20
Q

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

A

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

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21
Q

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

A

T

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22
Q

Is the facial rash in neonatal lupus a malar distribution?

A

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

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23
Q

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

A

Methotrexate

24
Q

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

A

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

25
Q

How does ank spond typically present in children?

A

Enthesis and peripheral arthritis of lower limbs

Axial disease and inflammatory back pain are less common at disease ONSET than in the adult pop

26
Q

What cardiac abnormality is often present in ank spond?

A

Aortic regurgitation

27
Q

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

A

Anti Ro is most specific

ANA tends to be negative

28
Q

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

A

JIA - 70% of patients will have bilateral

Enthesitis - more likely unilateral

29
Q

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

A

T

Note: HSP is a small vessel vasculitis

30
Q

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

A

F - it is a seronegative arthritis

Note: patients with IBD have increased risk of arthritis when disease is active

31
Q

What is the remission rate of polyarticular JIA

A

About 30%

32
Q

The low dose OCP is CI in those with SLE

A

False - if they have low disease activity and a documented negative anti phospholipid ab

Note: avoid OCP with high dose oestrogen

33
Q

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

A

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

34
Q

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

A

T

35
Q

What is the most common form of JIA?

A

Persistent oligoarthritis

Usually girls, around 3 yrs old

36
Q

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

A

F - they are both usually negative

37
Q

Is systemic JIA more common in girls or boys?

A

Equal

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

38
Q

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

A

T

Note: this is known as Koebner phenomenon

39
Q

Side effects of cyclophosphamide?

A

Myelosuppression
Infertility
Alopecia

40
Q

HLA B27 is often positive in which arthritis

A

Psoriatic arthritis

Enthesitis-related arthropathy

41
Q

Features of hyper-IgD

A

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

42
Q

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

A

NLRP3

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

43
Q

Autosomal recessive mutation in MEFV, what pathology?

A

Familial Mediterranean fever

44
Q

Treatment of familial Mediterranean fever?

A

If persistent treat with colchicine

45
Q

Criteria for diagnosis of familial Mediterranean fever?

A 
3 or more episodes
Lasting up to 96 hours
2 or more of: 
fever
abdo pain
chest pain
arthritis 
family hx of the disorder
46
Q

Skin findings in reactive arthritis

A

Keratoderma blennorrhagicum on palms and soles

Oral and genital ulcer and balanitis

47
Q

Pathergy is a manifestation on Behcet disease T/F

A

T

48
Q

What is the most common long term complication of HSP?

A

Chronic renal disease

49
Q

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

A 
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

50
Q

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

A

F - CRP usually normal. ESR may be elevated

Note: other lab abnormality sterile pyuria

51
Q

Antihistone antibody is associated with what pathology?

A

Drug induced lupus

52
Q

What to antibodies are specific for SLE?

A

Anti double stranded DNA

Anti Smith

53
Q

What complications of Behcets cause the most morbidity and mortality?

A

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

54
Q

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

A

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

55
Q

Involvement of what organs are poor prognostic indicators in SLE?

A

Renal

Cerebritis

56
Q

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

A

HHV 6

57
Q

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

A

Mixed connective tissue disease

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