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Paediatrics RACP written exam > Rheumatology/dermatology > Flashcards

Rheumatology/dermatology Flashcards

1
Q

Causes of ANCA+ve vasculitis

A

Wegener’s granulomatosis (cANCA, PR3)
Churg-Strauss granulomatosis
Microscopic polyangiitis (pANCA, MPO)

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2
Q

Pyoderma gangrenosum is associated with..?

A

Underlying systemic disorders such as inflammatory bowel disease, arthritis, and lymphoproliferative disorders.

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3
Q

Risk factors for JIA associated uveitis?

A

Young females, ANA+ve, oligoarticular (<5 joints)

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4
Q

Methotrexate side effects

A

GI upset, nausea, oral ulcers, hepatitis, haematological. Monitor LFTs + FBC 3-monthly.

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5
Q

Macrophage activation syndrome

A

Fever, cytopenia, inc ferritin ++, inc LFTs, hepatosplenomegly, deranged coags, decr fibrin.
High mortality up to 30% due to cytokine storm

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6
Q

One-three days of fever, serositis, arthritis and an erysipeloid rash (ankle/dorsum foot)

A

Familial mediterranean fever. Mutation MEFV. Give colchicine to protect from amyloidosis (presents with proteinuria).

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7
Q

PFAPA feature

A

Periodic fever, apthous stomatitis, pharyngitis, cervical adenitis

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8
Q

Behcet’s disease features

A

Recurrent oral and genital ulcers, pathergy (skin reaction 24-48hrs post needle prick), ant + post uveitis, vasculitis, erythema nodosum

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9
Q

Specific autoantibody for lupus nephritis?

A

Anti dsDNA is the most specific autoantibody for Lupus Nephritis

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10
Q

Which autoantibody is associated with HSP?

A
  • pANCA

- IgA vasculitis

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11
Q

HLA-B27 associated conditions?

A 
Ankylosing spondylitis
Reiter's syndrome
Reactive arthritis
Arthritis associated with IBD + psoriasis
Acute iridocyclitis
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12
Q

Sjogren syndrome features?

A

Dry eyes, dry mouth, carious teeth, parotitis. May occur alone or with other rheumatic disease. Can lead to neonatal lupus

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13
Q

Autoantibodies associated with neonatal lupus?

A

Anti-Ro, anti-La

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14
Q

Wegener’s granulomatosis features…

A

Necrotising granulomatous vasculitis affecting resp (lungs, ears, nose, sinuses) and glomerulonephiritis. ANCA +ve.

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15
Q

Causes of Wormian bones?

A 
Osteogenesis imperfecta
Rickets
"Kinky-hair" Menke's syndrome
Hypothyroidism and hypophosphatasia
Down syndrome
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16
Q

ANA is positive in…?

A

SLE (esp. dsDNA), 40-85% of JIA (esp. oligo/polyarthritis), increased risk uveitis, some normal population

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17
Q

ENA is positive in…?

A

SLE

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18
Q

Rheumatoid factors (RF) is positive in…?

A 
JIA, adult rheumatoid arthritis
SLE
HIV, hepatitis, TB
Leukaemia, lymphoid malignancy
Pulmonary fibrosis
General population
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19
Q

Describe Achenbach Syndrome

A
  • Acute idiopathic blue finger or paroxysmal finger haematoma
  • Sudden onset of painful swelling of a single finger (but can be more than one). The distal segment is usually spared
  • It is a rare benign syndrome and resolves spontaneously - Raynaud phenomenon usually involves more than one digit and does not spare the distal segment
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20
Q

Describe Sjogrens syndrome

A
  • Recurrent parotitis, xerostomia (dry mouth), dry eyes, polyarthritis. vulvovaginitis
  • F>M, mean age 9-10 yrs
  • Anti-SSA/B, high titre ANA, RF+, leucopenia
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21
Q

Describe sarcoidosis

A
  • Multisystem non caseating epithelioid granulomatous disease
  • Systemic features (fever, weight loss, malaise), pulmonary involvement and lymphadenopathy
  • Age <4: triad of rash, uveitis, and polyarthritis
  • Inc CRP/ESR, anaemia, leucopenia, eosinophilia
  • Tx: steroids
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22
Q

List the major criteria of rheumatic fever from most to least common

A 
Arthritis 75%
Carditis 50-60%
Chorea 10-15%
Erythema marginatum 1%
SC nodules <1%
23
Q

IL-6 inhibitor?

A
  • Tocilizumab

- Polyarticular JIA

24
Q

Daily SC IL-1 inhibitor?

A
  • Anakinra
  • Systemic JIA
  • Note: canakinumab = monthly, rilonacept = weekly
25
Q

Discuss dermatomyositis?

A
  • Affects skin, muscles, GI tract
  • Onset 5-10y, autoimmune disease, HLADR3 and 5
  • Symmetrical proximal muscle weakness and skin lesions
  • Calcinosis - small nodules of calcium which erupt through skin
  • Systemic symptoms: malaise, low-grade fever, irritability, lethargy, muscle pain
  • May have difficulty swallowing (dysphagia)
  • Heliotrope rash, hyperaemic nail folds, Gottron’s papules, photosensitive rash
  • Muscle biopsy: inflamm, necrosis. EMG: fibrillations, decr amplitude. Inc CK and ANA
  • Tx: steroids, methotrexate, ciclosporin. Tx min 2 years to decrease risk relapse. Hydroxychloroquine for skin rash
26
Q

Discuss the features of Behcet disease

A
  • More common in Mediterranean population
  • Usually 8-12 years old
  • Oral ulcers >3/year
    • 2 x: genital ulcers, pathergy, eye lesions (uveitis), skin lesions (erythema nodosum)
  • No clear treatment, steroids/immune modulators
27
Q

What is urticaria pigmentosa?

A
  • Urticaria pigmentosa is the most common form of cutaneous mastocytosis.
  • Infantile form resolves by age 3-4y
  • It is a rare disease caused by excessive numbers of mast cells in the skin that produce hives or lesions on the skin when irritated (dermatographism also seen on normal skin)
28
Q

What antibodies do you see in granulomatosis with polyangiitis?

A
  • Wegner’s

- c-ANCA, PR3-ANCA

29
Q

What antibodies do you see in microscopic polyangiitis?

A
  • p-ANCA, MPO-ANCA
30
Q

What is eosinophilic granulomatosis with polyangiitis (EGPA)?

A
  • Churg-Strauss syndrome is a small vessel necrotizing granulomatous vasculitis associated with a history of refractory asthma and peripheral eosinophilia
  • cANCA, PR3-ANCA positive (same as GPA)
  • Cartilage destruction is rare and renal involvement is
    uncommon
  • GI, skin, nerve, cardiac damage
31
Q

Discuss granulomatosis with polyangiitis

A
  • Formerly Wegner’s
  • Due to c-ANCA, PR3-ANCA (proteinase 3)
  • Affects:
    • URT: OM, chronic sinusitis, mastoiditis, perforation nasal septum, nasal ulcers
    • LRT: haemoptysis (don’t confuse with goodpasture’s which doesn’t have URTI involvement)
    • Kidneys: restricted flow, can develop RPGN, non-caseating granulomas, oliguria, hypertension
  • CXR: nodules, cavities, infiltrates, CT: granulomas in lungs. May have ground glass/interstitial fibrosis appearance
  • Histology: granulomatous inflammation
32
Q

Discuss microscopic polyangiitis

A
  • p-ANCA, MPO-ANCA (myeloperoxidase)
  • Similar to GPA but no upper resp tract involvement
  • No granulomas on histology
  • Affects kidney and lungs
  • Precipitated by meds e.g. penicillin
33
Q

Discuss familial mediterranean fever

A
  • Episodes are characterised by high fevers lasting ~3 days, associated with symptoms of serositis – peritonitis, pleuritis, pericarditis, meningitis, arthritis or orchitis – abdominal or chest pain most common
  • Can represent acute abdomen
  • A vasculitic or erysipelas ‐like rash over the lower limbs is seen in some, as is myalgia and diarrhoea.
  • Persistent splenomegaly, otherwise well between
  • Attacks are irregular and unpredictable (can be triggered by stress, infections, menses)
  • 50% children develop renal/GI/lung AA amyloidosis
  • Tx: daily colchicine prophylaxis (2nd line rilonacept = IL-1 inhibitor)
34
Q

Discuss the glucocorticoid potenticies

A 
Hydrocortisone = 1 = 20mg (least potent)
Prednisone = 4 = 5mg
Fludrocortisone = 10
Betamethasone/dexamethasone = 25 = 0.75mg (most potent)
35
Q

Immunology of eczema?

A
  • Th2 pathway

- Increased lymphocytes, eosinophils, IgE production

36
Q

Filaggrin deficiency is seen in?

A
  • 1/3rd of eczema population
  • Skin barrier defect
  • Reduced stratum corneum hydration, reduced epidermal barrier function, enhanced Th2 immune activation, increased staph colonisation
  • Also seen in asthma, rhinitis, food allergy
37
Q

Systemic treatments for eczema?

A
  • Phototherapy - narrowband UVB
  • Low dose methotrexate
  • Ciclosporin A
  • Dupilumab - not available in NZ, targets Th2 pathway
38
Q

Treatment of psoriasis

A
  • Emollients, calcipotriol, coal tar, salicylic acid, daivobet (calcipotriol + betamethasone)
  • Acitretin (related to isotretinoin)
  • Methotrexate
  • Never use systemic steroids as risk of pustular flare which is life-threatening
39
Q

What is pigmentary mosaicism?

A
  • New term, includes hypomelanosis of Ito
  • Congenital or early onset, widespread non-progressive hyper or hypo pigmentation
  • Sporadic
  • Systemic features - skeletal, renal, cleft palate, seizures, ID, hip dislocation, autism
40
Q

Discuss Incontinentia Pigementi

A
  • X-linked dominant, NEMO gene
  • Lethal in males
  • Follows Blaschko’s lines
  • 4 stages:
    • Vesicular - may occur in utero - 6m, spares face, erythema + blistering. DDx HSV
    • Verrucous - wart-like lesions, thick scabs, inc with fever. Up to 1-2y, distal limbs
    • Hyperpigmented - until 16y, variable pigmentation, axilla, groin, nipples, follows Blaschko’s lines
    • Atrophic - adult females, pale linear lesions trunk and legs, loss of hair
      + delayed dentition, missing teeth, peg-shaped teeth, strabismus, retinopathy, seizures, 15% ID 10%, nail dystrophy, alopecia
41
Q

Features of Takaysau arteritis

A
  • Large vessel vasculitis
  • Hypertension, aortic involvement (dilation), renal artery involvement (narrowed)
  • Histology: giant cells and granulomas
42
Q

Features of polyarteritis nodosa

A
  • Transmural inflammation of endothelium - leads to necrotising vasculitis and then fibrosis (due to healing) of vessel walls
  • Fibrosed wall is weak, prone to aneurysms - “string of beads” along vessel, unique appearance, aneurysms and fibrotic stenosis
  • Associated with strep, Hep B, CMV, parvovirus, FMF
  • Symptoms depend on area of organ ischaemia: kidneys (hypertension), mesenteric artery (abdo pain, bleeding), brain (neuro symptoms), skin (lesions)
  • Histology: necrotising vasculitis
  • Tx: corticosteroids
43
Q

What are examples of small vessel vasculitis?

A
  • Microscopic polyangiitis
  • Granulomatous polyangiitis
  • SLE
  • Juvenile dermatomyositis
44
Q

Types of granulomatous vasculitis

A
  • Takayasu (large vessel)
  • Granulomatosis with polyangiitis (Wegner, small vessel)
  • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss, small vessel)
45
Q

Types of granulomatous vasculitis

A
  • Takayasu (large vessel)
  • Granulomatosis with polyangiitis (Wegner, small vessel)
  • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss, small vessel)
46
Q

Types of necrotising vasculitis

A
  • Polyarteritis nodosa (medium)
  • Kawasaki (medium)
  • Granulomatosis with polyangiitis (Wegner, small vessel)
  • Microscopic polyangiitis (small)
47
Q

Treatment of vasculitis

A
  • Mild: induction with prednisone, maintenance with methotrexate, azathioprine, cotrimoxazole for URT involvement
  • Severe: induction with methylpred or cyclophosphamide, maintenance with prednisone or azathioprine
  • Failed induction or relapse: rituximab (anti CD20), infliximab (anti TNFa), IVIG
  • Stop treatment once stable >12m, and taper >5m
48
Q

Histology showing leukocytoclastic vasculitis?

A
  • Usually HSP (has IgA deposition on immunofluorescence, thickened glomerular loops on biopsy)
  • Can be isolated
49
Q

Is arthritis more common in UC or CD?

A
  • Twice as common in Crohn’s disease

- Mirrors bowel disease and associated with flares

50
Q

Discuss SLE

A
  • T3 hypersensitivity, immune complex deposition throughout body, causing inflammation and activation of complement
  • Decr C3 and C4, ANA +ve
  • Relapsing, remitting, systemic. Need 4/11 criteria
  • Antibodies to blood cells leads to pancytopenia (T2 hypersensitivity)
  • Skin: butterfly rash, discoid rash, photosensitivity
  • Membranes: ulcers in mouth or nose, serositis
  • Extraglandular: >2 joints arthritis, diffuse proliferative GN “wire loop”, neuro (headache, seizures, psychosis, depression)
  • Blood: pancytopenia, ANA+ve, anti-Smith/anti dsDNA/ , anti-phospholipid (anti-cardiolipin, lupus anticoagulant)
  • Tx: hydroxychloroquine, steroids, cyclophos, azathioprine
51
Q

Discuss Sjogrens

A
  • Autoimmune disorder against exocrine glands
  • Mediated by CD4+ T cells, macrophages, plasma cells
  • Most commonly affects lacrimal and salivary glands: keratoconjunctivitis, dry eyes and mouth, xerostomia, ulceration nasal septum
  • Swollen glands, dry skin, inc risk infections
  • Anti-Ro (SSA) and anti-La (SSB) antibodies
  • Tx: steroids, increase secretions
52
Q

Causes of parotitis

A
  • Viral: mumps, parainfluenza, coxsackie, CMV, EBV
  • Bacterial: staph, strep
  • Recurrent parotitis of childhood
  • Sjogrens (from salivary stasis), CF, stones, sarcoidosis
53
Q

Discuss sarcoidosis

A
  • Hilar lymphadenopathy, erythema nodosum, uveitis, cardiac arrhythmias
  • Fever, weight loss, fatigue
  • SOB + cough common
  • Non-caseating granulomas consisting of macrophages and T cells
  • Langhans giant cell - large fused nucleated cell
  • Hypercalcaemia (excess Vit D from macrophages), increased ACE level (T-cells), inc T cells in lungs (lavage)
  • Tx: steroids

Paediatrics RACP written exam (18 decks)

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