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Paeds BPT Written Exam > Rheumatology > Flashcards

Rheumatology Flashcards

(49 cards)

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1
Q

What are the 3 types of ANCA-associated vasculitis

A
  1. Granulomatosis with polyangiitis (GPA/ Wegeners)
  2. Microscopic polyangiitis (MPA)
  3. Eosinophilic granulomatosis with polyangiitis (EGPA/Churg-Strauss)

GPA >MPA >EGPA

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2
Q

Which vasculitis can present with asthma, allergic rhinitis and eosinophilia?

A

Eosinophilic Granulomatosis with polyangiitis (EGPA/ Churg-Strauss)

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3
Q

C-ANCA and PR3 are associated with which vasculitis

A

Granulomatosis with polyangiitis (Wegeners)

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4
Q

P-ANCA and MPO are associated with which vasculitis?

A

Microscopic polyangiitis

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5
Q

Management of ANCA vasculitis?

A
  • Steroids + cyclophosphamide (or steroids +MTX for non-life threatening disease)
  • Rituximab becoming increasingly used
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6
Q

Manifestations of Granulomatosis with Polyangiitis (GPA) AKA Wegeners

A

Renal
- haematuria, proteinuria, cast
- necrotising pauci-immune GN
Upper Resp Tract
- Chronic/ recurrent nasal discharge
- Epistaxis
- Saddle nose/ septum deformity
Lower Resp Tract
- Haemopysis
- Nodules/cavities
Other
- vasculitis rash
- arthralgia, myalgia
- malaise, weight loss

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7
Q

Types of JIA

A

Systemic
RF+ Polyarticular
RF- Polyarticular
Oligoarticular
Enthesitis-related arthritis
Psoriatic arthritis

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8
Q

Systemic JIA diagnostic criteria?

A

Arthritis with or preceded by 2 weeks of fever and 1 or more of the following:
- evanescent rash
- serositis
- hepatosplenomegaly
- generalised LN enlargement

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9
Q

Definition of Polyarthritis - RF positive and RF negative?

A

5 + joints affected during first 6 months of disease

If RF+, need 2x RF to be positive >3mo apart

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10
Q

Definition of Oligoarthritis JIA?

A

1-4 joints affected during first 6mo of the disease

“Extended oligoarticular JIA” is where a total of >4 joints are affected after 6mo

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11
Q

Risk factors for uveitis in JIA?

A

Oligoarthritis
Onset <7yrs
Disease <4yrs and
ANA + HLA-DR5 positive

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12
Q

Risks of uveitis

A
  • Glaucoma
  • Keratopathy
  • Blindness
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13
Q

Side effects of methotrexate?

A

GI upset
Oral ulcers
Hepatotoxicity - transaminitis
Myelosuppression - macrocytosis, neutropenia, anaemia, thrombocytopenia

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14
Q

Side effects of biologics?

A

Reactivation of TB
Infections esp VZV
Anaphylactic and infusion reactions
Risk of manipulating immune system long term (..malignancies)

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15
Q

MOA of anakinra?

A

IL-1 Receptor antagonist
(daily injection)

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16
Q

MOA of Infliximab and Adalimumab?

A

Anti-TNF antibody

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17
Q

MOA of etanercept?

A

Competitively inhibits TNF alpha and beta, preventing them from binding to TNF receptors

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18
Q

Which biologic used in sJIA can CAUSE uveitis?

A

Etanercept

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19
Q

Management of uveitis?

A

Steroid eye drops
MTX
Biologics (eg adalimumab, infliximab, rituximab, abatacept)

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20
Q

What are the most common manifestations in Kawasaki Disease?

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A

Fever >5 days (100%)
Polymorphous exanthema (90%)
Mucosal changes (80-90%)
Nonexudative conjuncitivitis (80-90%)
Extremity changes (80%)
Cervical LN >1.5CM (50%)

21
Q

‘Other’ manifestations of Kawasaki Disease

Study These Flashcards
A
  • Sterile pyuria
  • Aseptic meningitis
  • Acute uveitis
  • Diarrhoea
  • Hydrops of the gallbladder
  • Mild obstructive jaundice with elevated transaminases
  • Pericardial effusions and myocarditis (up to 30%)
22
Q

Triad of Behcet’s disease

Study These Flashcards
A
  • Oral ulcers (painful, recurrent)
  • Genital ulcers (painful, recurrent)
  • Inflammatory eye disease
23
Q

In which condition do you commonly see a positive pathergy test (prick skin with needle, development of pustule/papule 48hrs later)?

Study These Flashcards
A

Behcet disease

24
Q

Cause of neonatal lupus?

Study These Flashcards
A

Transplacental passage of maternal autoantibodies (commonly Anti-SSA / anti-Ro and Anti-SSB/anti-La)

25
Common presentation of neonatal lupus
Congenital heart block Cytopenias Hepatitis Annular or discoid rash, commonly on face and scalp
26
Common presentations of Paediatric SLE?
Arthritis (80-90%) Rash (70-80%) Nephritis (50-60%)
27
Autoantibodies found in lupus
ANA (98-99% sensitivity) Anti-dsDNA (60-70%) Anti-smith (33%) Antiphospholipid abs (lupus anticoagulant, anti-cardiolipin, b2-glycoprotein 1 abs) (50%) Anti-U1 RNP (more often associated with MCTD)
28
Which autoantibody in SLE fluctuates with disease activity and strongly correlates with renal involvement?
Anti-dsDNA
29
Most common cardiac abnormality in SLE?
Pericarditis - occurs in 25-35%
30
Benefits of hydroxychloroquine in SLE?
Reduces flares (3x reduction) Reduces mortality Lowers serum cholesterol levels
31
Antibody positive in mixed connective tissue disease?
Anti-U1 RNP antibody (also usually high titre speckled ANA, RF and have hypergammaglobulinemia)
32
Antibodies in Sjogren Syndrome
Anti-Ro / Anti-SSA (70%) Anti-La/ Anti-SSB (50%)
33
Differences between PFAPA and familial Mediterranean fever?
- PFAPA flares occurs cyclically whilst FMF flares occur at random - May see tonsillitis during episodes of PFAPA which is uncommon with FMF - FMF more likely to present with abdo pain and arthritis - FMF attacks often shorter than PFAPA and cannot be aborted with steroids
34
Which period fever syndrome: attack lasting 7-21 days of fever, conjunctivitis, periorbital oedema, migratory myalgia and rash?
TNF Receptor 1 Associated Period Fever Syndrome (TRAPS)
35
What condition is anti-histone antibody associated with?
Drug induced lupus
36
High serum ACE suggests which rheumatological condition?
Sarcoidosis - elevated during active disease
37
Prognosis of linear scleroderma (most common form of scleroderma in children)?
Typically resolves within 3-5yrs Need physic to prevent contractures and may need systemic steroids or MTX if there is extensive subdermal involvement
38
Features of limited cutenaoues scleroderma (CREST)?
Calcinosis Raynauds Esophageal dysmotility Sclerodactyly Telangiectasia
39
Anti-SCL 70 is associated with which condition?
Diffuse cutaneous scleroderma
40
Anti-centromere ab is associated with which condition?
Limited cutaneous scleroderma (CREST) and Primary biliary cirrhosis
41
Management of Raynauds phenomenom?
Calcium channel blockers eg nifedipine Alpha blockers
42
Clinical features of dermatomyositis
- proximal muscle weakness - gottrons papules - heliotrope rash - periungal changes - GIT muscle involvement -> difficulty swallowing, dysphonia - arthralgia (70%)
43
Complications of dermatomyositis
- Ulcerative skin disease - Lipodystrophy (insulin resistance, hyperlipidemia)
44
In which conditions do you see abnormalities on nail fold capillaroscopy?
Juvenile dermatomyositis Mixed connective tissue disease Systemic sclerosis
45
Grades of lupus nephritis
1: minimal mesangial 2: mesangial 3: focal 4: diffuse 5: membranous
46
Management of lupus nephritis
Class 2-4: steroids Class 4-5 2nd line: cyclophosphamide, azathioprine, MMF
47
Diagnostic criteria for dermatomyositis
Presence of gottron papules or heliotrope plus 3+ (definite) or 2+ (probable) of the following - symmetrical prox muscle weakness - elevated muscle enzymes (CK, LDH, aldolase, transaminases) - EMG abnormalities - Muscle biopsy abnormalities incl degeneration, regeneration, necrosis, phagocytosis, mononuclear cell infiltrate
48
Management of juvenile dermatomyositis
- Steroids (high dose then taper over 2yrs) - MTX + folic acid - Hydroxychloroquine for skin manifestations - 2n line: IVIG, cyclosporin, azathioprine
49
How to remember microscopic polyangiitis
Microscopic angiitis P-anca nOn granulomatous, nOn upper respiratory tract (letters make MPO)

Paeds BPT Written Exam (18 decks)

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