Rheum Cram Flashcards

1
Q

Best test to screen for SLE?

A

ANA (almost 100% positive)

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2
Q

Most sensitive test for SLE?

A

Anti-Smith (ENA)

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3
Q

Most specific test for lupus nephritis?

A

Anti-dsDNA

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4
Q

ENA associated w neonatal congenital heart block?

A

Anti-Ro

Begins crossing placenta at 16weeks

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5
Q

cANCA assoc w

A

Wegners (granulomoatosis polyangitis)

CF

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6
Q

pANCA assoc w

A
Microscopic polyangitis
Polyarteritis nodosa
PSC/IBD
HSP
KD
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7
Q

RF assoc with

A

Rheum arthritis
Sjogrens
SLE

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8
Q

Histone assoc w

A

Drug induced lupus/SLE

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9
Q

HLA-B27 assoc w

A

Enthesitis related arthritis
Ankylosing spondylitis 90% pos
Uveitis

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10
Q

MTX mechanism and SEs

A
Blocks purine synthesis
Bone marrow suppression (not at rheum doses)
Teratogenic
Accumulates in 3rd spaces
AVOID bactrim/penicillins
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11
Q

Leflunomide mechanism and SEs?

A

Blocks pyrimidine synthesis
Liver dysfunction
Parasthesia
Teratogenic

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12
Q

Hydroxychloroquine mechanism and SEs?

A

Anti-inflammatory and immunosuppressive effects
Ototoxic - colour vision
Ophthal review 6-12mthly

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13
Q

Mycophenalate mechanism and SEs?

A

Suppressive lymphoproliferation and antibody formation
Bone marrow suppression
Increased risk of neoplasia

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14
Q

Infliximab mechanism and SEs?

A

Anti-TNFalpa
Cytopenia
Demyelination
Lupus like syndrome

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15
Q

Rituximab mechanism and SEs?

A

AntiCD20 - depletes B lymphocytes
MSK pain/weakness
Infection
Progressive multifocal leukoencephalopathy

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16
Q

Anakinra mechanism and SEs?

A

IL-1 receptor antagonist
Injection site reaction
Headache
neutropenia

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17
Q

Tocilizumab mechanism and SEs?

A

IL-6 receptor antagonist
Deranged LFTs
Injection site reaction
GI perforations

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18
Q
>2/52 hx of:
arthritis
daily fever 
lymphadenopathy
evanescent erythematous rash
serositis
A

Systemic JIA

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19
Q

Most common form of JIA?

A

Oligoarthritis

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20
Q

DIP arthritis and psoriasis with nail/finger changes and Fhx of psoriasis?

A

Psoriatic arthritis

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21
Q

HLA-B27 positive and hip/low back pain

A

Enthesitis related arthritis

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22
Q

First line mx for JIA?

A

NSAIDs
Steroids
2nd line DMARDs - MTX, antiTNF, anakinra

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23
Q

When not to use etanercept?

A

Uveitis

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24
Q

Complications of JIA?

A
Uveitis
Limb length discrepancy/growth retardation
Interstitial lung disease/Pulm HTN
TMJ (50%)
Decreased BMD from steroids
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25
Q

JIA type most assoc w chronic uveitis?

A

Oligoarticular

Particularly ANA pos, RF neg

26
Q

JIA type most assoc w acute uveitis?

A

Enthesitis JIA

Particularly if HLA-B27 pos

27
Q

Unremitting fever, rash, arthritis
Splenomegaly
Cytopenia, High trigs, low ESR, very high ferritin
Elevated soluble CD24

A

Macrophage Activation Syndrome

AKA HLH assoc w rheumatic disease

28
Q

Mx of choice for MAS?

A

High dose steroids

Anakinra

29
Q

Teenage girl, not-caucasian. Prolonged hx.
Photosensitive rash, nasal ulcers, hard palate macules
Fever, arthritis/arthralgia, hepatosplenomegaly, nephritis

A

SLE

30
Q

Marker of disease activity in SLE? Role of C3/C4 in SLE?

A

Anti-dsDNA marker of disease activity

low C3 marker of disease activity but low C4 indicative of genetic pre-disposition for SLE

31
Q

Mx of choice for SLE?

A

All pts should be on hydroxychloroquine - flare reduction, improves lipid profiles also
Steroids for acute flares (topical to skin)
Aspirin/warfarin if procoagulant

32
Q

Most common inflammatory myopathy of childhood?

A

Dermatomyositis

33
Q

Weakness, lipodystrophy/calcinosis, plaques over fingers (gottron papule) and heliotrope rash?

A

Dermatomyositis

34
Q

Raynaud followed by skin changes distally, nail fold capillary change. ANA positive nucleolar staining?

A

Scleroderma

35
Q

Anti-SCL-70 and anti-RNA polymerase positive?

A

Diffuse systemic sclerosis

36
Q

Systems involvement in scleroderma?

A

GI - oesophagus (dysphagia, aspiration), rectal prolapse, constipation/fecal incontinence
Lungs - interstitial fibrosis, pulmonary HTN
Heart - dilated cardiomyopathy
Renal - HTN

37
Q

Centromere ANA staining pattern?

A

CREST variant scleroderma

38
Q

Mx of scleroderma?

A

UV therapy/topical steroids
Corticosteroids/MTX
Cyclophos & MMF for resp involvement

39
Q
Petechiae/purpura mostly of lower limbs
Diffuse abdo pain (+/- diarrhoea)
Arthritis/arthralgia
Proliferative GN
IgA deposition
A

Henoch Schonlein Purpura

40
Q

Rate of HSP recurrence? Risk of long term ERSD post HSP?

A

30% recurrence rate usually in 6mths

Renal disease in 1-2%, 10% of these will have ESRD

41
Q

Fever, fatigue, LOW
Abdo pain
Haematuria/proteinuria
Purpura and livedo reticularis with painful nodules

A

Polyarteritis nodosa

42
Q

Biopsy findings of polyarteritis nodes?

A

Necrotising vasculitis

Granulocytes and monocytes in small/medium sized arteries

43
Q

Pulm haemorrhage/nasal ulceration/hearing loss
Haematuria/proteinuria
Palpable purpura
cANCA positive

A

Gramulomatosis Polyangitis AKA Wegners

44
Q

Pulm haemorrhage/hearing loss
Haematuria/proteinuria
Palpable purpura
pANCA positive

A

Microscopic polyangitis

45
Q

Refractory asthma
Proteinuria/haematuria
Eosinophilic infiltration

A

Churg Strauss

46
Q

Genital and oral ulcers, scars from old ulcers
Uveitis
Erythema nodosum

A

Behcet’s

47
Q

Neonatal lupus syndrome most associated with?

A

Maternal Sjogrens

48
Q

DIagnostic criteria for fibromyalgia?

A

Diffuse MSK pain in at least 3 areas of the body, persisting for 3 months in the absence of an underlying condition
Must have at least 5 well defined tender points of examination

49
Q

Periodic fevers lasting 1-3 days with arthritis, serositis and erysepiloid rash.
Assoc w Behcets, HSP, polyarteritis nodosa

A

Familial Mediterranean fever

MEFV gene

50
Q

Risk of developing amyloidosis in familial Mediterranean fever?

A

30-50% if untreated

Treat with colchicine

51
Q

Baby with fever lasting up to 1 week rash and abdo pain and lymphadenitis, triggered by immunisation/surgery.

A

Hyper IgD Syndrome

MVK gene

52
Q

Fever with painful rash, conjunctivitis, episodes last weeks at a time and occur multiple times/year.

A

TRAPS
TNF receptor associated periodic syndrome
Receptor 1A gene mutation TNFRSF1A

53
Q

Risk of amyloidosis in TRAPS?

A

25% - kidneys and liver

Colchicine does not treat TRAPS - NSAID/pred

54
Q

Infant in first week of life with fevers, meningitis, rash, arthropathy of knees.

A

NOMID

Mx anakinra

55
Q

Febrile attacked 1-3hrs post cold exposure, resolve within 14hrs?

A

Familial cold auto inflammatory syndrome

56
Q

Intermittent fevers with SNHL?

A

Muckle-Wells

57
Q

Fever, adeninitis, pharyngitis, apthous stomatitis lasting 4-5 days every month.

A

PFAPA/Marshall Syndrome
Periodic Fever, Apthous Stomatitis, Pharyngitis and Adenitis
Mx - prev, colchicine and tonsillectomy

58
Q

Biopsy showing eosinophilic material that stains with Congo red dye and shows apple green biofringence in polarised light?

A

Amyloidosis

59
Q

Rash, uveitis and polyarthritis with large boggy effusions. Non-caeseating epithelia granulomas with hypercalcaemia and hypercalcuria

A

Sarcoidosis

60
Q

Granulomas of eyes, skin and joints?

A

Blau syndrome