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good paeds > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

7 types of JIA

A

SUP POPE

Systemic arthritis
Undifferentiated arthritis
Polyarthritis (RF negative) 5+ joints in 6 months need RF tested two occasions at least 3m apart
Polyarthritis (RF positive)
Oligoarthritis (1-4 involved, often ANA positive, most common)
Psoriatic arthritis
Enthesis-related arthritis

ALL have eye exam (uveitis in 20-30%)

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2
Q

Felty syndrome (3)

A

RA, Neutropenia and splenomegaly

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3
Q

Enthesitis related arthritis

A 
HLA B27 80%
Male over 6y
Anterior uveitis
Ank spend
IBD
Dactylitis (also with sickle cell and psoriatic arthritis)
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4
Q

therapy for SLE

A 
Hydroxychloroquine (standard)
Steroids
Azathioprine
Mycophenolate mofetil
Cyclophosphamide 
Rituximab
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5
Q

SLE most deaths related to..

A

Infection

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6
Q

Drugs that cause drug induced lupus

A

Minocycline
Anticonvulsants
Hydralazime
TNF agents

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7
Q

Granulomatosis with polyangitis triad

A

Small vessel

Upper and lower respiratory tract inflammation and renal disease

ANCA positive

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8
Q

HSP other name

A

IgA vasculitis

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9
Q

HSP recurrence rat

and end stage renal disease rate

A

Recurrence in 1/3rd

ESRF in 1-3%

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10
Q

Behcet criteria and mx

A 
Recurrent mouth ulcers (exhibit pathergy)
Recurrent genital ulcers
Eye lesions
Skin lesions
Pathergy

Mx steroid, colchicine etc

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11
Q

Juvenile dermatomyositis criteria

A

Symmetric proximal muscle weakness
Skin changes - gottron papules knuckles and eyelid rash (50% have photosensitivity and can have malar rash)
Elevated CK, AST, LDH, Aldolase
Abnormal EMG and muscle biopsy
Positive anti jo and Ro indicate high risk early death

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12
Q

Limited systemic sclerosis 5 features

A 
CREST previous name
Calcinosis
Raynaud
Eosophageal dysmotiliy
Sclerodactyly
Telangiectasia
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13
Q

Raynauds Tx

A

Nifedipine

Nitroglycerin 2% ointment for digital ulcers

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14
Q

Tx for chorea

A

Carbamazepine
Phenobarbital
Haloperidol
Chlorpromazine

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15
Q

Macrophage activation syndrome

A 
Cytokines storm with 10-30% mortality
Pancytopenia
Fever
Splenomegaly
High TG, High ferritin, high ddimer 
Decreased fibrinogen (DIC)
Deranged LFTs

Haemophagocytosis of bone marrow
Persistent high CRP but decreasing ESR (consumption of fibrinogen - DIC)

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16
Q

Anti-glomerular basement membrane antibodies seen in

A

Goodpasture syndrome (IgG deposits BM with crescenteric changes))

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17
Q

B cell depletion agent

A

Rituximab

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18
Q

IL-1 inhibitors

A

Anakinra
Canakinumab (very impressive SJIA)
Rilonacept

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19
Q

How much normal population HLA B27 positive

A

10%

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20
Q

Criteria for SLE

A

Rash on maids

Renal
Arthritis
Serotitis
Haematological 
Oral uk era
Neurological 
Malar rash
ANA (most sensitive)
Immunological
Discoid rash 
Sunlight sensitivity
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21
Q

Periodic fevers (PFAPA vs FMF)

A

PFAPA well in between and self resolves, 0% risk amyloidosis and FmF 40-75% risks amyloidosis

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22
Q

Livedo reticularis indicates..

A 
Mottled purple skin can be from:
Anti phospholipid 
Systemic lupus erythematosus
Dermatomyositis
Rheumatoid arthritis
Polyarteritis nodosa
Granulomatosis with polyangiitis
Sjogren syndrome
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23
Q

C-ANCA in

A

Wegeners
Reacts against proteinase 3

(P-ANCA in microscopic polyangitis against myeloperoxidase)

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24
Q

Churg-Strauss

A

Atopic/asthma and multi system vasculitis

P ANCA positive

25
Q

Large vasculitis
Medium vasculitis
Small vasculitis

A

Large vasculitis - GCA, takayasu
Medium vasculitis - PAN, Kawasaki
Small vasculitis -Chung-Strauss, HSP, wegners, microscopic polyangitis

26
Q

JIA arthritis for how long at least

A

6w

27
Q

Intra articulate steroid lasts for at least

A

4 months

Triamcinolone hexacetonide

28
Q

Joints most effected by psoriatic arthritis

A

Polyarticular arthritis involving DIP joints

29
Q

Radiologic changes diagnostic of ank spond

A

Sclerosis of sacroiliac joint margins on X-ray

30
Q

Most specific marker SLE

A

Anti smith

DsDNA

31
Q

Pemphigus biopsy

A

Is a rare autoimmune skin disease causing blisters skin and mucous membranes
Biopsy shows intraepidermal blister with IgG deposits

32
Q

SJS biopsy

A

Full thickness epidermal necrosis

33
Q

Can eight loss improve cutaneous psoriasis

A

Yes

34
Q

Psoriasis treatment

A 
Calcipotriol (vitamin d analogue)
Steroids topically
Dithranol 
Coal tar
UVB
MTX 
Biological agents
35
Q

SLE percent ANA positive

A

ANA- 97% positive

36
Q

What does aspirin do in Kawasaki

A

Aspirin protects from coronary events but not development of aneurysms

37
Q

Biological big risk

A

Infection (deactivation TB)

38
Q

Anti CCP antibody positive in most with…

A

JIA

39
Q

Nikolskys sign is and reflects what

A

Elicit by applying pressure to affected skin and there is extension of blister or removal of epidermis where touched
Positive in pemphigus Vulgaris and negative in bullies pemphigoid
Reflects autoimmune disease

40
Q

Heliotrope in what? Blue violet discolouration of eyelids

A

Dermatomyositis

41
Q

RF positive in

A

Sjogren (also anti ro and la) and JIA

42
Q

Raynauds, tight thick skin, ulcers
Salt and pepper skin
Positive anticentromere

A 
Systemic sclerosis (scleroderma)
Rare in children
43
Q

AntiJo antibodies in dermstomyositis relates to

A

Risk of interstitial lung disease

44
Q

Rash of systemic JIA

A

Salmon colour evanescent discrete borders best den with fever migratory

45
Q

Hunchback child called

A

Sheuermanns disease

46
Q

Back pain and fever in preschool child with narrowing of vertebral disc on X-ray consistent with

A

Discitis

47
Q

Percent with arthritis in IBD

A

15%

48
Q

Reiters/reactive arthritis occurs 1-4 weeks following what infections

A

Salmonella, shigella, campylobacter
TB
Yersinia
Chlamydia

49
Q

Calcinosis in

A

Inherited CT diseases
Hypercalcaemia
Connective tissue disease (dermatomyositis, systemic sclerosis, cutaneous lupus erythematosus)

50
Q

10 year old with 1-3 day fever episodes wth serositis, knee arthritis with rash over joint

A

Familial Mediterranean fever

Colchine

51
Q

Painful rash migrates distally with exercise in 3 year old 2-6 times per year

A

TRAPS
AD
Gene TNFRSF1A
25% get amyloidosis

52
Q

Fever every 21-28 days and ulcers adenitis syndrome

A

PFAPA
Periodic fever, aphthous ulcer, stomatitis, pharyngitis, adenitis

Prednisone
Adenotonsillectomy

53
Q

(cutaneous hypersensitivity to superficial
trauma).

A

Koebner phenomenom

54
Q

Dermatomyositis facial rash

A

Facial erythema crossing nasolabial folds (malar rash in SLE does not
involve nasolabial folds).

55
Q

FMF and PFAPA treatment

A

FMF colchicine and amyloid

PFAPA p for prednisone

56
Q

Keppra/ leveteracitam SE

A

Tiredness

Aggression/behaviour change

57
Q

DIP joints and Nails may show pitting, yellowing, transverse ridges or destruction (onycholysis)

A

Psoriatic arthritis

58
Q

Three causes of photosensitivity

A

SLE
Dermatomyositis
Psoriasis

good paeds (19 decks)

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