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Paeds > Rheumatology/dermatology > Flashcards

Rheumatology/dermatology Flashcards

1
Q

Causes of ANCA+ve vasculitis

A

C-ANCA (PR3) Granulomatosis with polyangitis (Wegener’s) Eosinophilic granulomatosis with polyangitis (Churg-Strauss) pANCA (MPO) 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 (lasts 3-7 days ; every 3-4weeks) Apthous stomatitis Pharyngitis, Cervical adenitis Onset <10, resolves 20s Usually responds to steroids ; if not 80% cured after tonsillectomy

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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 Polyarthritis and GI inflame

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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

GPA (Wegener’s) granulomatosis features…

A

Necrotising granulomatous vasculitis affecting resp (lungs, ears, nose, sinuses) and glomerulonephiritis. ANCA +ve. (c-ANCA, PR3) Upper resp pulmonary Renal - GN

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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%

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23
Q

IL-6 inhibitor?

A
  • Tocilizumab - Polyarticular JIA
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24
Q

Daily SC IL-1 inhibitor?

A
  • Anakinra - Systemic JIA - Note: canakinumab = monthly, rilonacept = weekly
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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
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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
54
Q

Pulmonary renal syndromes

A

Alveolar haemorrhage and GN Goodpastures (Anti GBM Ab) GPA (wegners) Eosnophilic granulomatosis with polyangitis SLE

55
Q

Drug induced lupus

A

Fever, arthralgia/arthritis, serositis ANA +ve, anti-histone +ve Usually resolves within 2 weeks of stopping drug Offending drugs - AEDs - phenytoin, ethosux - Isoniazid - Beta blockers

56
Q

Osteopetrosis

A

disorder of osteoclast function (failure to resorb bone, which leads to increased density of bone but bones are weak and prone to fractures) AR - mutation CA2 gene (carbonic anhydrase) - More severe, growth F, Dental/visual/hearing problems -Hydrocephalus, HSM, bone marrow failure AD - mutation CLCN7, milder phenotype

57
Q

Score to assess hypermobility

A

Beighton score, >7 =hypermobility

58
Q

Reiters

A

Arthritis Conjunctivitis Urethritis

59
Q

Lupus diagnostic criteria

A

Need 4/11 criteria Mnemonic MD SOAP BRAIN Malar rash Discoid Rash Serositis (pericarditis or pleurites) Oral ulcers Arthritis Photosensitivity Blood problems (anaemia, thrombocytopenia, leukopenia) Renal ANA +ve Immune (Anti ds DNA) Neuropsychiatric (seen in 2/3)

60
Q

IBD related arthritis

A

Peripheral arthropathy Type1 - Pauciarticular, asymmetrical, large joint - Mirrors disease activity - HLA-B27, HLA-B35 and HLA-DR related Type2 - Polyarticular, small joint - Independent of gut disease activity - May be associated with uveitis Axial arthropathy - 5-10% of patients - Ankylosing spondylitis or sacroiliac joint arthritis - Independent of gut disease activity

61
Q

Types of vasculitis

Size vessel

Histology

Assoc symptoms

A
62
Q

Causes of elevated AFP

A

Non malignant:

Ataxia telangiectasia, liver disease

Malignant causes

  • Liver: HCC, Hepatoblastoma
  • Germ cell tumours
63
Q

Diagnostic criteria for Juvenile Dermatomyositis

A
64
Q

Complications JDM

Clinical course

Rx

A
  • Calcinosis
  • Interstitial lung disease (Lung function, HRCT)
  • GI and cutaneous vasculopathy
  • Proximal muscle weakness (monitor CMAS score- child myositis assessment score)
  • pharyngeal muscle weakness (SLT monitoring)

Clinical response to Rx - normalisation of muscle enzymes and muscle strenght (CMAS>48)

Remission: Medication cessation with no flare for >6months

Monophasic: Clinical remission without disease flare

Polycyclic: Remission with disease flare

Chronic continous - no remission

1/3 monophasic and do well

1/2 chronic continous

Rx

Steroids to induce remission and then taper over 2 years

MTX (takes 6-8weeks to work)

Cyclosporin - 2nd line rx

Paeds (25 decks)

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