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RACP Written > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

Treatment for FMF?

A

Colchicine
Can try IL-1 blocker

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2
Q

What are 3 examples of polygenic inflammatory disorders

A

PFAPA
JIA
CRMO

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3
Q

What does PFAPA stand for

A

Periodic fevers with apthous ulcers, pharyngitis and adenitis
No genes identified yet.

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4
Q

Age range for PFAPA

A

Starts around 2-5yo and gone by 10yo

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5
Q

Is neutropenia a hallmark of PFAPA

A

NO! Neutropenia before or during the attack is an exclusion criteria

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6
Q

Typical area for CRMO

A

Mandible or jaw

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7
Q

Definition of CRMO

A

Chronic, recurrent multifocal osteomyelitis. Sterile, autoinflammatory bone disorder.

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8
Q

Clinical presentaion for PFAPA

A

abrupt fevers for 3-6 days, attacks regularly every 28 days.
Symptoms SHOULD resolve between episodes

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9
Q

What is the most common inflammatory disorder associated with amyloidosis

A

FMF
FMF-AM

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10
Q

What is associated with neonatal lupus? Think investigations

A

Anti-Ro or La

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11
Q

What is the use of ANA with JIA

A

Predicts uveitis

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12
Q

What test is a good prognostic indicator for polyarticular JIA

A

RF
RF positive less common but wourse course

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13
Q

What type of JIA is the most common? How many joints are involved

A

Oligo articular, < 4 joints

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14
Q

What are the second and third most common JIA

A
  1. Polyarticular RF negative
  2. Systemic onset
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15
Q

Diagnostic criteria for systemic JIA

A

High fevers with either:
Hepatosplenogamly
lymphadenopathy
serositis- inflammation of peritoneum, pleura or pericardium

Fever 2 weeks and arthralgia 6 weeks

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16
Q

What kind of rash is common in systemic JIA

A

salmon pink rash

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17
Q

What are some indicators for worse prognosis in patients with systemic JIA

A

polyarthritis, hip involvement, thrombocytosis, systemic disease at 6 monhts

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18
Q

Number of joints in polyarticular vs oligoarticular JIA?

A

Oli has a family of 4- so <4
Poly for Pentagon so >5

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19
Q

How long do fevers have to be present for in systemic JIA

A

2 weeks

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20
Q

What condition is commonly associated with systemic JIA

A

MAS

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21
Q

Treatment for sJIA post failure of NSAID/steroid/MTX

A

Tociluzimab or Anakinra (IL-1 agonist)

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22
Q

What are the 2 anti-TNF agents that can be used in JIA

A

Etanercept
Adalumimab

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23
Q

What is the name of the steroid used in joint injections

A

Triamcinoclone Hexacetenide

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24
Q

Is enthesitis related arthritis male or female predominant

A

male
HLA B27

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25
Q

What is the DMARD of choice in enthesitis related arthrits

A

Sulfasalazine

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26
Q

What does juvenile sarcoidosis normally present with?

A

Painless swelling of joints and marked tenosynovitis.
Also multisystem involvement

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27
Q

What is triad for Reiter syndrome

A

mouth ulceration, fever and rashes
Associated with HLA B27

Wouldnt Reite having a fever.

28
Q

What are the 5 major manifestations for ARF? What is the crtieria called?

A

JONES criteria.
Carditis
Polyarthritis
Subcutaneous nodules
Chorea
Erythema marginatum

29
Q

What are the minor criteria for ARF

A

Raised ESR and CRP
Fever
Arthralgia
Prolonged PR interval
Positive GAS or positive ASOT/AntiDNAse
Prev RF/RHD

30
Q

What are the 4 main diagnostic features of dermatomyositis?

A

Proximal weakness, dialted nail fold capillary loops, heliotrope rash and Gottren’s papules (rough, scaly red patches on knuckles and elbows)
Calcinosis= delayed feature

31
Q

What is the test used to look at muscle myositis

A

CMAS

32
Q

What antibodies are associated with worse prognosis in dermatomyositis/

A

Anti-Jo
‘Jo has dermatomyositis and its not good’

33
Q

What is pathagnomic of JDM

A

Gottron’s papules

34
Q

What rash is associated with lupus?

A

Malar butterlfy rash, worse with sun
Photosensitivity

35
Q

Which 2 markers are the best are predicting disease activity in lupus?

A

anti-dsDNA and C3/C4
(remember C3/C4 LOW- think of lupus nephritis)

36
Q

What are some side effects of hydroxyclhloroquine?

A

nausea. GI, headaches.
Retinotoxicity rare.

37
Q

What is a standard medication used for all patients with lupus and why

A

Plaquinil/hydroxuchloroquine.
Improvement in all cause morbidity and mortality

38
Q

Presentation of neonatal lupus

A

Rash- lesions of discoid lupus
Cogenital heart block
Thrombocytopenia

39
Q

How long does neonatal lupus last

A

only 6-12 months, babies tend to be healthy post
CHB is forever

40
Q

What is antiphospholipid syndrome

A

Autoimmune hypercoagulable disorder
(look for this in patients with repeated miscarraiges)

41
Q

Triad for Behcet’s disease

A

Apthous ulcers, recurrent genital ulcers and uveitis
Also has arhtirits, rash etc

42
Q

Common clinical presentation for Sjogren’s disease
Think of SjoG- RUNNING DRY

A

Sjogren is THIRSTY- DRY EVERYTHING
Dry mouth
Dry eyes
Parotitis

43
Q

Similarities between Scleroderma and systemic sclerosis

A

Both skin conditions, cause tightening of skin

44
Q

What are the two most common forms of childhood vasculitis

A

HSP and Kawasaki

45
Q

Clinical presentation for HSP

A

Rash- urticarial lesions over feet and buttocks
Arthritis- involving large joints
GI involvement- colicky abnormal pain
Renal- nephritis

46
Q

Pathophys for HSP

A

IgA nephropathy and from immune complexes in glommeruli and skin

47
Q

Diagnostic criteria for Kawasaki

A

Fever >38 for 5 days AND
- Oedema of hands and feet and erythema
- mucositis
- lymphadenopathy
-conjunctival injection
- polymorphous rash

48
Q

Treatment for Kawasaki and in what time frame?

A

IVIG
within 10 days of symptom onset

49
Q

What condition is commonly assocaited with erythema and induration at BCG site

A

Kawasaki disease

50
Q

How long is aspirin continued for in Kawasaki disease if heart involvement suspected

A

6-8 weeks

51
Q

Cardiovascular complication of Kawasaki

A

Coronary artery aneurysm

52
Q

How common is coronary artery aneurysmm in Kawasaki

A

20%

53
Q

What size vessels does polyarteritis nordosa affect

A

small to medium

54
Q

What is a large vessel arteritis

A

Takayasu (giant cell arteritis)

55
Q

What is Granulomatosis with polyangiitis

A

Necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract, and necrotizing vasculitis affecting predominantly small- to medium-sized vessels (e.g., capillaries, venules, arterioles, arteries, and veins). Necrotizing glomerulonephritis is common.

56
Q

What is Churg-Strauss or Eosinophilic granulomatosis with polyangiitis

A

Eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small- to medium-sized vessels, and associated with asthma and eosinophilia. ANCA is more frequent when glomerulonephritis is present.

57
Q

Which osteogenesis inperfecta is lethal

A

type 2

58
Q

Which osteogenesis imperfecta is associated with hearing loss

A

type 1

59
Q

which osteogenesis imperfecta it aut recessive

A

type 3
Also lethal

60
Q

What age is toe walking okay till

A

age 3

61
Q

Time frame within which pims ts occurs

A

4 weeks post proven or suspected infection

62
Q

Risk factors for more severe disease in COVID 19

A

Immunosuppression
Children with severe or complex chronic disease
Obesity
Pregnancy
Age
- Less than 3 months corrected age
- Adolescents

63
Q

What is the time frame for risk of myocarditis/pericarditis post COVID vaccination

A

7-14 days

64
Q

When is the highest risk of myocarditis/pericarditis post infection with COVID

A

after 2nd dose, more in males

65
Q

When should PIMS TS be considered

A

PIMS-TS should be considered in an unwell child who presents with fever (≥3 days), signs of shock, rash and abdominal pain

66
Q

Features of systemic sclerosis

A

CREST
Calcinosis
Raunauds
Esophageal hypomobility
Sclerodactyly
Telengectasia

RACP Written (18 decks)

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