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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
What is the DMARD of choice in enthesitis related arthrits
Sulfasalazine
26
What does juvenile sarcoidosis normally present with?
Painless swelling of joints and marked tenosynovitis. Also multisystem involvement
27
What is triad for Reiter syndrome
mouth ulceration, fever and rashes Associated with HLA B27 Wouldnt Reite having a fever.
28
What are the 5 major manifestations for ARF? What is the crtieria called?
JONES criteria. Carditis Polyarthritis Subcutaneous nodules Chorea Erythema marginatum
29
What are the minor criteria for ARF
Raised ESR and CRP Fever Arthralgia Prolonged PR interval Positive GAS or positive ASOT/AntiDNAse Prev RF/RHD
30
What are the 4 main diagnostic features of dermatomyositis?
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
What is the test used to look at muscle myositis
CMAS
32
What antibodies are associated with worse prognosis in dermatomyositis/
Anti-Jo 'Jo has dermatomyositis and its not good'
33
What is pathagnomic of JDM
Gottron's papules
34
What rash is associated with lupus?
Malar butterlfy rash, worse with sun Photosensitivity
35
Which 2 markers are the best are predicting disease activity in lupus?
anti-dsDNA and C3/C4 (remember C3/C4 LOW- think of lupus nephritis)
36
What are some side effects of hydroxyclhloroquine?
nausea. GI, headaches. Retinotoxicity rare.
37
What is a standard medication used for all patients with lupus and why
Plaquinil/hydroxuchloroquine. Improvement in all cause morbidity and mortality
38
Presentation of neonatal lupus
Rash- lesions of discoid lupus Cogenital heart block Thrombocytopenia
39
How long does neonatal lupus last
only 6-12 months, babies tend to be healthy post CHB is forever
40
What is antiphospholipid syndrome
Autoimmune hypercoagulable disorder (look for this in patients with repeated miscarraiges)
41
Triad for Behcet's disease
Apthous ulcers, recurrent genital ulcers and uveitis Also has arhtirits, rash etc
42
Common clinical presentation for Sjogren's disease Think of SjoG- RUNNING DRY
Sjogren is THIRSTY- DRY EVERYTHING Dry mouth Dry eyes Parotitis
43
Similarities between Scleroderma and systemic sclerosis
Both skin conditions, cause tightening of skin
44
What are the two most common forms of childhood vasculitis
HSP and Kawasaki
45
Clinical presentation for HSP
Rash- urticarial lesions over feet and buttocks Arthritis- involving large joints GI involvement- colicky abnormal pain Renal- nephritis
46
Pathophys for HSP
IgA nephropathy and from immune complexes in glommeruli and skin
47
Diagnostic criteria for Kawasaki
Fever >38 for 5 days AND - Oedema of hands and feet and erythema - mucositis - lymphadenopathy -conjunctival injection - polymorphous rash
48
Treatment for Kawasaki and in what time frame?
IVIG within 10 days of symptom onset
49
What condition is commonly assocaited with erythema and induration at BCG site
Kawasaki disease
50
How long is aspirin continued for in Kawasaki disease if heart involvement suspected
6-8 weeks
51
Cardiovascular complication of Kawasaki
Coronary artery aneurysm
52
How common is coronary artery aneurysmm in Kawasaki
20%
53
What size vessels does polyarteritis nordosa affect
small to medium
54
What is a large vessel arteritis
Takayasu (giant cell arteritis)
55
What is Granulomatosis with polyangiitis
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
What is Churg-Strauss or Eosinophilic granulomatosis with polyangiitis
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
Which osteogenesis inperfecta is lethal
type 2
58
Which osteogenesis imperfecta is associated with hearing loss
type 1
59
which osteogenesis imperfecta it aut recessive
type 3 Also lethal
60
What age is toe walking okay till
age 3
61
Time frame within which pims ts occurs
4 weeks post proven or suspected infection
62
Risk factors for more severe disease in COVID 19
Immunosuppression Children with severe or complex chronic disease Obesity Pregnancy Age - Less than 3 months corrected age - Adolescents
63
What is the time frame for risk of myocarditis/pericarditis post COVID vaccination
7-14 days
64
When is the highest risk of myocarditis/pericarditis post infection with COVID
after 2nd dose, more in males
65
When should PIMS TS be considered
PIMS-TS should be considered in an unwell child who presents with fever (≥3 days), signs of shock, rash and abdominal pain
66
Features of systemic sclerosis
CREST Calcinosis Raunauds Esophageal hypomobility Sclerodactyly Telengectasia

RACP Written (18 decks)

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