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Paeds BPT Written Exam > Rheumatology > Flashcards

Rheumatology Flashcards

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?

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

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

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)?

A

Behcet disease

24
Q

Cause of neonatal lupus?

A

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

25
Q

Common presentation of neonatal lupus

A

Congenital heart block
Cytopenias
Hepatitis
Annular or discoid rash, commonly on face and scalp

26
Q

Common presentations of Paediatric SLE?

A

Arthritis (80-90%)
Rash (70-80%)
Nephritis (50-60%)

27
Q

Autoantibodies found in lupus

A

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
Q

Which autoantibody in SLE fluctuates with disease activity and strongly correlates with renal involvement?

A

Anti-dsDNA

29
Q

Most common cardiac abnormality in SLE?

A

Pericarditis - occurs in 25-35%

30
Q

Benefits of hydroxychloroquine in SLE?

A

Reduces flares (3x reduction)
Reduces mortality
Lowers serum cholesterol levels

31
Q

Antibody positive in mixed connective tissue disease?

A

Anti-U1 RNP antibody

(also usually high titre speckled ANA, RF and have hypergammaglobulinemia)

32
Q

Antibodies in Sjogren Syndrome

A

Anti-Ro / Anti-SSA (70%)
Anti-La/ Anti-SSB (50%)

33
Q

Differences between PFAPA and familial Mediterranean fever?

A
  • 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
Q

Which period fever syndrome: attack lasting 7-21 days of fever, conjunctivitis, periorbital oedema, migratory myalgia and rash?

A

TNF Receptor 1 Associated Period Fever Syndrome (TRAPS)

35
Q

What condition is anti-histone antibody associated with?

A

Drug induced lupus

36
Q

High serum ACE suggests which rheumatological condition?

A

Sarcoidosis
- elevated during active disease

37
Q

Prognosis of linear scleroderma (most common form of scleroderma in children)?

A

Typically resolves within 3-5yrs
Need physic to prevent contractures and may need systemic steroids or MTX if there is extensive subdermal involvement

38
Q

Features of limited cutenaoues scleroderma (CREST)?

A

Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactyly
Telangiectasia

39
Q

Anti-SCL 70 is associated with which condition?

A

Diffuse cutaneous scleroderma

40
Q

Anti-centromere ab is associated with which condition?

A

Limited cutaneous scleroderma
(CREST) and
Primary biliary cirrhosis

41
Q

Management of Raynauds phenomenom?

A

Calcium channel blockers eg nifedipine
Alpha blockers

42
Q

Clinical features of dermatomyositis

A
  • proximal muscle weakness
  • gottrons papules
  • heliotrope rash
  • periungal changes
  • GIT muscle involvement -> difficulty swallowing, dysphonia
  • arthralgia (70%)
43
Q

Complications of dermatomyositis

A
  • Ulcerative skin disease
  • Lipodystrophy (insulin resistance, hyperlipidemia)
44
Q

In which conditions do you see abnormalities on nail fold capillaroscopy?

A

Juvenile dermatomyositis
Mixed connective tissue disease
Systemic sclerosis

45
Q

Grades of lupus nephritis

A

1: minimal mesangial
2: mesangial
3: focal
4: diffuse
5: membranous

46
Q

Management of lupus nephritis

A

Class 2-4: steroids
Class 4-5 2nd line: cyclophosphamide, azathioprine, MMF

47
Q

Diagnostic criteria for dermatomyositis

A

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
Q

Management of juvenile dermatomyositis

A
  • Steroids (high dose then taper over 2yrs)
  • MTX + folic acid
  • Hydroxychloroquine for skin manifestations
  • 2n line: IVIG, cyclosporin, azathioprine
49
Q

How to remember microscopic polyangiitis

A

Microscopic angiitis
P-anca
nOn granulomatous, nOn upper respiratory tract

(letters make MPO)

Paeds BPT Written Exam (18 decks)

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