musculoskeletal disorders in children · post-infectious arthropathies • acute rheumatic fever,...
TRANSCRIPT
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Musculoskeletal disorders in
children
Pavla Doležalová
Klinika dětského a dorostového lékařství 1.LFUK
Ke Karlovu 2, Praha 2
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•Pain or limb dysfunction - various
organ system involvement
•Musculoskeletal pain - common in
chidren (4-30% otherwise healthy
kids)
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• Arthritis - small proportion:
108,5/100000 children under 16
(transitory conditions in most cases,
duration shorter than 6 weeks)
• Chronic (idiopathic) arthritis (over
6 weeks duration):
5,3 - 19,6/100000 dětí
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History
• Pain characteristics:
– type of the pain (dull, sharp, colicky…)
– Intenzity, duration, localisation, radiance
– Predisposing and relieving factors (relation to activity)
– Time location of the most intensive pain (morning,
evening, night)
• Presence of stiffness? (eg early morning)
• Limb dysfunction?
• Age-appropriate activities?
• Presence of visible changes? (skin colour, oedema)
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Joint symptoms
Příznaky Souvislosti Preferenčnílokalizace
Specifické příznaky
Mechanické Po tělesnéaktivitěČastěji ustarších dětí
KolenoKotník
Blokáda,instabilitaBolest:ostrá,náhlá,přechodná,úlevapo odpočinkuPřechodná synovitida
Zánětlivé Po klidu Jakýkolikloub
Časná ranní ztuhlostBolest: palčivá nebo tupá,přetrvávající kolísavě, nezmizí zcelapři odpočinku, může probudit zespaní. Otok, kožní teplota zvýšená
Idiopatické PředcházejícítraumaStres
KončetinaGeneralizované potíže
Bolest:trvalá, zneschopňujícíUnavitelnost, školní absence, výraznádysfunkce, poruchy spánku
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Basic joint exam
• Color
• Bone structures
• Muscle bulk
• Length and width discrepancy
• Limb position
• Appearance of the painful site
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Joint exam: Palpation
• Skin temperature
• Oedema
– soft tissues, effusion, bone overgrowth
• Site of maximum pain
– joint space, soft tissues, bone
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Joint exam: ROM
• ROM
– passive
– active
• Compensatory movements
• Pain with motion
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Differencial diagnosis of
musculoskeletal painAvascular necrosis and degenerative conditions:
Perthes,osteochondritis,chondromalacia patellae,hypermobility
Reactive arthritis: post:-streptococcal,-enteritic, -viral
Trauma, non-accidental injury
Hematological diseases: hemoblastosis,hemofília,lymphoma
Rachitis, other metabolic disorders and endocrinopathies
Infections: septic arthritis, osteomyelitis
Tumors: cartilage, bone, muscle, synovium, blood vessels
Idiopathic pain: localised, generalised
Systemic connective tissue diseases: SLE, vasculitis,
dermatomyositis, scleroderma
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Mechanical arthropaties
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Osteochondritis and similar disorders
• „Avascular necrosis“
– Etiology unclear
• Sites
– Femoral head (Legg-Calvé-Perthes)
– Tuberositas tibiae (Osgood-Schlatter)
– Os naviculare (Köhler)
– Vertebral bodies (Scheuermann)
• Patelo-femoral syndrome (anterior knee painsyndrome, chondromalacia)
• Hypermobility-related pain
– Overuse injury
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Trauma
• Osteochondritis dissecans (transchondral
fracture with fragment separation)
• Traumatic arthropathy (following haemarthros)
• Non-accidental trauma
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Inflammatory disorders
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Infection
• Lyme disease
• Viral arthritis (rubella, parvovirus, HB, herpesvirus)
• Septic arthritis
– Staph, HIB, Pneumococcus, Salmonella
– Haematogeneous
– Acte - systemic signs, subacute, chronic – difficult dg (TB!!!)
• Osteomyelitis acute, subacute, chronic
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Post-infectious arthropathies
• Acute rheumatic fever, poststreptococcal reactive
arthritis
• Postenteritic arthritis: gut infections symptomatic
and asymptomatic (Salmonella, Shigella, Yersinia,
Helicobacter), acute anterior uveitis, mucous
membrane ulcerations, often HLA B-27+
(+urethritis=Reiter´s syndrome; Chlamydia)
• Postviral arthritis (EBV, CMV,rubella,
ADV,VZV,parvoB19)
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Hemato-oncological diseases
• Generalised
– Haemofilia
– Hemoblastsis
– Lymphoma
– Neuroblastoma
• Localised tumors
– Osteosarkoma
– Ewing sarkoma
– Synovial tumors (villonodular synovitis?)
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Idiopathic/chronic pain
syndromes
• „Pain amplification“ syndromes
• Localised
– CRPS (Complex Regional Pain Syndrome,
RSD Reflex Sympathetic Dystrophy, Sudeck´satrophy)
• Generalised
– Fibromyalgia
• „Growing pains“
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• http://www.pmmonline.org/
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Chronic inflammatory diseases
• Vasculitides
– Henoch-Schönlein purpura
– Kawasaki disease
• Juvenile Idiopathic Arthritis
• Rare connective tissue diseases
– SLE, JDM, scleroderma, MCTD…
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Rheumatic diseases in children-
epidemiology
Incidence Prevalence
JIA 5,3 - 19,6 minim. 1/1000
JSLE 0,5 - 0,6 10% new dg.
JDM/JPM 0,2 - 0,5 (0,15)
SS 0,45 - 1,2 total. ?
1,0-9,0% do 20 let
HSP 13,5 ?
KD 5,95 - 7,6 do 5 let ?
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JUVENILE IDIOPATHIC
ARTHRITIS:
Diagnosis per exclusionem
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Classification of Juvenile Idiopathic
Arthritis
2nd revision-Edmonton 2001
Definition: Arthritis of unknown origin affecting a
person younger than 16 years of age of minimum 6
weeks duration in at least one joint
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JIA classification
• Clinical features during the first 6 months
– „onset“ types
• Re-classification later on
• Each group
– Criteria, descriptors, exclusions
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JIA classification
1. Systemic arthritis
2. Polyarthritis IgM RF-
3. Polyarthritis IgM RF+
4. Oligoarthritis: persistent, extended
5. Arthritis with enthesitis
6. Psoriatic arthritis
7. Other arthritis
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Disease assessment – disease
activity and damage
• Core set of outcome measures
– Global assessments physician and patient
– Active and limited joint counts
– Disability score
– ESR
• ACRpedi, JADAS – composite disease
activity score
• Definitions of improvement and relapse
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Systemic JIA
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Still’s disease: epidemiology
• sJIA: 10-15% of all JIA
• annual incidence 2-20/100.000 Cassidy and Petty, 2000
– any age, peak onset 2 years
– boys and girls equally affected
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Definition and classification: sJIAPetty et al, 1998
• Arthritis with or preceded by daily fever of at least 2 weeks’ duration
(documented as quotidian for minimum 3 days) accompanied with
one or more of:
-Evanescent, non-fixed erythematous rash
– Generalised LNpathy
– Hepato or splenomegaly
– Serositis
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Clinical features of sJIA
• Fever often preceding arthritis, systemic unwellness
• Arthritis – variable, often destructive polyarthritis
• Pericarditis
• High non-specific inflammation, activation of coagulation pathways
• Acute complication: MAS
• Chronic complications
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Complication: Macrophage
activation syndrome - MAS
• Persistent fever and malaise, neurological abnormalities, rash
• Acute hepatopathy, multi-organ failure
• Cytopenia with haemophagocytosis in marrow aspirate
• Consumptive coagulopathy with DIC →
fibrinogen and ESR, d-dimer, FDP, APTT
• Drop in ESR and fibrinogen
• Raised ferritin and IL-18
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Complications: osteoporosis
Ca and Vitamin D3 supplementation
• calcitonin, bisphosphonates (oral alendronate, i.v.
pamidronate) Noguera et al 2003, Steelman and Zeitler 2003
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Complications: growth retardation
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sJIA disease course and outcome
• 3 clinical subtypes
– Monocyclic (11%)
– Intermittent (34%)
– Persistent (55%) Lomater et al 2000
• Active disease after >10 years follow-up in 23-58 % of sJIA patients
• Therapy
– Corticosteroids, cytokine inhibitors – IL-1 and IL-6 blockade (anakinra, tocilizumab)
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Oligoarticular JIA
• No affected joints < 5
• Frequency: 60% of all JIA
• Subtypes:
– Persistent
– Extended
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Clinical picture• Onset 2-3 years, girls predominate
• Knee, ankle
• Limp, stiffness, swelling, flexion deformity
• Lab often normal
• Fever and general symptoms – careful differential dg
– Malignancies (leukemia, neuroblastoma)
– Infections, other systemic diseases
• CAVE: ANA
• eyes
• psoriasis
• local growth disturbancies
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Prognosis
• Excellent if treated early and appropriately
• Chronic uveitis – limits favourable prognosis
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Oligoarthritis extended
• Initial 6 months less than 5 joints affected,
polyarticular course later on
• Often elevated inflammatory activity
• Prognosis varies, similar to poly-JIA
• Therapy similar to poly-JIA
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Polyarthritis RF negative
• More than 4 affected joints
during initial 6 mo
• ↑ nonspecific inflammation
• Often foot, wrist, hip
involvement – negative
prognostic factor
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Polyarthitis RF positive
• 1-2% JIA
• Often adolescent girls
• Similar to adult RA
• Rapidly progressive
destruction, symmetrical
small joint disease
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Psoriatic arthritis
• Artritida + psoriasis or arthritis + nail
pitting/psoriasis in 1st degree
relative/dactylitis (2 of 3)
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Arthritis with enthesitis
• „Enthesis“ = insertion of tendon or ligament into
bone
• Plantar, Achilles tendon, knee, pelvis
• Lower extremities, SI
• Often HLA B-27, enteropathogen-triggered
• Exclude IBD
• Therapy: local CS, Salazopyrin, (MTX, Enbrel)
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Acute anterior uveitis (iridocyclitis)
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JIA – therapy principles
• Multidisciplinary approach
• Drug treatment
– NSAID
– Corticosteroids – intraarticular, systemic
– Methotrexate, Sulphasalazine
– Biologics – TNFα, IL-1, CTLA blockade
– Osteoporosis prevention, topical eye treatment
• Physical and occupational therapy
• Education, supportive/social care
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Juvenile idiopathic
inflammatory myopathies
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Epidemiology
Incidence Prevalence
JIA 5,3 - 19,6 minim. 1/1000
JSLE 0,5 - 0,6 10% nových dg.
JDM/JPM 0,2 - 0,5 (0,15)
SS 0,45 - 1,2 celk. ?
1,0-9,0% do 20 let
HSP 13,5 ?
KD 5,95 - 7,6 do 5 let ?
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Classification(Rider a Miller, 1997)
IIM = Idiopathic Inflammatory Myopathies:
Chronic striated muscle inflammation of
unknown origin
JDM: 85% JPM: 8%
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DG criteria JDM/JPM(Bohan a Peter, 1975)
• Typical skin manifestations
• Symmetrical proximal muscle
weakness
• Muscle enzymes
• EMG changes
• Histopathology
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Boy, 8 y
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Boy, 5 let
„Kissing“ ulcers –
necrotising skin vasculitis
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Boy, 12 y
• Angiopathy
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Angiopathy
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Angiopathy: nailfolds
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Nailfold capillaroscopy
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JDM investigations
Labs
• Inflammatory markers
• Muscle enzymes (all)
• Immunology: Ig,C, ANA, ENA, immunoblot
(MAS, MSA) B-ly (CD 19), CD3+CD8+ T-ly
• Endothelial damage (vWF, neopterin, adhesion
molecules)
• Metabolic screening
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JDM investigations
• Muscle MRI - T2W, STIR
• EMG - directed
• Muscle biopsy - directed
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19.9.2000 – before therapy
20.5.2001 – after 8 months
→
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HE 10x, intermysial and perivasculůar infiltration (mo/ma CD68+, Tly
CD3+CD4+, CD3+CD8+), fiber size variation, degener/regener.
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Late complications of JDM
• Lipoatrophy Calcinosis
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Early signs of JDM
• Most common: Muscle weakness +
characteristic skin rash (100%,Pachman, J
Rheumatol. 1998)
• Less common: Myopathic syndrome plus
other vasculitis or „dermatomyositis sine
myositis“
• Warning signs: dyslalia, voice change,
dysphagia, fluid regurge
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CAVE:
• Muscle enzymes normal in up to 10% (Pachman 1998)
• Inflammatory parameters can be normal, ANA
neg
• Typical skin changes may be initially absent
• Non-targeted biopsy or EMG may be
noninformative in up to 20% (Pachman 1998)
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JDM /JPM - DIF. DG.
• Postinfectious myositis (influenza A,B, coxackievirus B, toxoplasmsis, trichinosis, staph pyomyositis)
• Neuromuscular disorders (spinal atrophy, myastenia)
• Metabolic and heritable myopathies (glykogen storage dis, lipidoses, carnitin def, -oxidation disorders, dystrophinopathies…)
• Secondary myopathies (endocrinopthies, steroid induced )
• Other CTD (MCTD, SLE, vasculitis..)
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THERAPY
• Drug treatment:
- CS (+ osteoporosis prevention, K suppl)
- immunosuppressive (hydroxychloroquine, MTX,
CFM, CyA, IVIG)
• Multi-discipliplinary: PT/OT, supportive,
alimentation, hydration (myoglobinuria)
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PROGNOSIS
Disease course:
• monocyclic : limited, steroid responsive (27%)
• persistent (chronic ulcerative (33%)
• polycyclic (chronic nonulcerative): (40%)
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Vasculitides
• Primary systemic vasculitis
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Chapel Hill Consensus vasculitis
nomenclature
Jennette JC et al, A&R 2013
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Distributopn of vessel involvement
Jennette JC et al, A&R 2013
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IgA Vasculitis (Henoch-Schönlein)
• Most common paediatric vasculitis
Definition :
- vasculitis with IgA1 dominant imune deposits affecting small blood
vessels
- typically skin, GIT, joints, kidneys
- glomerulonephritis indistinguishable from IgA nephropathy
Jennette JC…:Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.
Arthritis Rheum.2012: 20143 (65):1-11
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IgAV
Classification criteria:
- palpable purpura with predominant distribution overlower extremities
+ at least 1 of: - abdominal pain
- histopathology
- arthritis/arthralgia
- renal involvement
Ozen S et al.: EULAR/PRINTO/PRES criteria for HSP, childhood PN, WG a TA: Ankara 2008: Part II: Final classificationcriteria. Ann Rheum Dis 2010: 69: 798-806
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IgAV – course and prognosis
• Variable, often relapsing– 1/3 – acute disease, ovet wwithin 2 wks
– 1/3 – within 1 month
– 1/3 – recurrent course for several months
• IgAV nephritis– 1,6-3% terminal renal disease
Eleftheriou and Brogan : Therapeutic advances in the treatment of vasculitis, Pediatric Rheumatology (2016) 14:26
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Therapy
• Analgesia– NSA
• Corticosteroids– Indicated in case of: Orchitis, CNS vasculitis or other major organ disease.
– Severe abdominal pain, enterorrhagia
– 1-2mg/kg/day
• IgAV– Pediatric nephrology consult
– ACE inhibitor when persistent proteinuria
– Renal biopsy result informs therapeutic decision
Nienke de Graeff et al:Evidence Based Recommendations for Diagnosis anf Treatment of KD and HSP, SHARE – Complete
list of recommendations (publikace v přípravě)
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Kawasaki disease (KD)
• 2nd most common primary paediatric vasculitis
• Asian ethnicity prevails
• Systemic vasculitis affecting medium-sized arteries
• 85% of patients are < 5 yrs (max. incidence 18-24 months)
• Most frequent cause of acquired heart disease in developed countries
• 15-25% untreated patients develop coronary aneurysms (CAA)
• 2-3% untreated patients die
Elephteriou D..: Management of KD, Arch Dis Child 2014 , 99:74-83
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Kawasaki disease (KD)
Diagnostic criteria (AHA) :
Fever at least 5 days + at least 4 of :
1. erythema and induration of palms and soles→ desquamation duringconvalescent phase
2. rash - polymorphous
3. conjunctivitis
4. mucous membrane changes
5. cervical lymphadenopathy often asymetrical >1,5 cm
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Klasifikační kriteria KD Ozen et al 2006
Horečka alespoň 5 dnů (mandatorní) + 4 z:
• Změny na periferii končetin nebo perineálně
• Polymorfní exantém
• Oboustranná injekce spojivek
• Změny rtů a dutiny ústní: erytém orální a
faryngeální sliznice
• Krční lymfadenopatie
V přítomnosti postižení CA (dle ECHO) a horečky stačí
méně než 4 kritéria (přesný počet vyžaduje validační
studii).
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Labs
•ESR, CRP, FBC, LFTs (AST/ALT), renální function,
albumin.
•Dif. Dg. From other (infection, HLH…):
–Feritin, lumbar tap
•Risk stratification:–Na, ALT, PLT, CRP, albumin.
•Monitoring:–ESR (prior to IVIG), CRP, FBC
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Other investigations
•Echo in ALL suspects immediately
•F/U ECHO 2 wks post 1st IVIG –When initially normal
–Ijn ALL another F/U 6-8 wks later
•Persistent inflammation–ECG + ECHO weekly
•Abnormal 1st echo–F/U weekly until stable
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Therapy
•Start as soon as possible
IVIG
•IVIG 2 g/kg
ASA
•30-50 mg/kg/day until–Afebrile for 48h
–Clinically improving
–Dropping CRP
•Reduction to 3-5 mg/kg o.d.
•When CAA–longterm
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Corticosteroids
•Indications: – IVIG Resistence
– Kobayashi score ≥5 [Sleeper et al.]
– HLH
– KSS
•Consider to add to IVIG immediately:– Infants
– Aneurysms identified at onset
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Additional therapies
•Anti TNF-alpha (infliximab, adalimumab)
•Anticoagulation
•Angioplasty…
•Adjustment of vaccination scheme
–No vaccines for 6 months
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Juvenile polyarteritis - cPAN
• Necrotising inflammation of medium and/or small arteries
• Epidemiology
– worldwide, rare
– equal sex distribution
– peak age at onset 10 years
• Histopathology
– fibrinoid necrosis
– pleiomorphic infiltration
– disarranged wall architecture
– healing with fibrosis.
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PAN: Deep skin biopsy
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• Types of vessel wall involvement:
Focal Segmental
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cPAN – clinical characteristics(Cassidy and Petty 2001)
• Fever (84%)
• Arthritis/arthralgia(74%)
• Abdominal pain (68%)
• Myalgia (67%)
• Skin:
– rash (58%)
– edema (20%)
– petechiae (17%)
• Renal (25%)
• Cardiac (21%)
• Nervous system
– seizures (16%)
– other (10%)
• Mucous memb (17%)
• Respiratory (7%)
• Cervical LN(
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cPAN – cutaneous findings
• Livedo reticularis Purpura
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cPAN: Evolution of skin ulcers
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Granulomatous vasculitis
• GPA – granulomatosis with polyangiitis
• EGPA
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Classification criteria GPA Ozen et al 2006
3 of the following:
• Abnormal urinanalysis
• Granulomatous inflammation on biopsy
• Nasal sinus inflammation
• Subglottic, tracheal or endobronchial stenosis
• Abnormal CXR or CT
• PR3 ANCA or C-ANCA staining
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TAKAYASU ARTERITIS
• Segmental arteritis causing stenoses of large
muscular arteries, prediminantly aorta and
its main branches
• Abdominal aorta more often involved in
children, often systemic hypertension
• Non-specific symptoms common
– Fevers, elevation of ESR/CRP, malaise, weight
loss, palpitations, headaches
– Vascular bruits, pulse and BP assymetry
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Classification criteria for
Takayasu arteritis
• Angiographic abnormalities (conventional,
CT, MR) of the aorta or its main branches +
at least one of:
– Decreased peripheral artery pulse(s) and/or
claudication of extremities
– Blood pressure difference >10 mm Hg
– Bruits over aorta and/or its major branches
– Hypertension (related to childhood normative
data)
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Pulmonary angiography in 10-years old girl
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Isolated CNS vasculitis/vasculopthy
• GANS, PACNS, BACNS (Calabrese 2001, Gallagher et al 2001, Stone et al 2001)
• Acquired focal CNS symptoms– Acute severe headache, focal neurologic deficit, gross motor
deficit, hemiparesis, cranial nerve involvement, cognitive dysfunction, seizures (Benseller and Schneider 2004)
• Angiographic or histopathologic features of CNS angiitis
• Absence of systemic disease (clinical and laboratory)
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PACNS / GANS
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Systemic lupus erythematosus
Definition: ..“episodic, multisystem
autoimmune disease characterised by
inflammation of blood vessels and
connective tissue and presence of
antinuclear antibodies“
Clinical course - variable, unpredictable,
potentially life threatening
Epidemiology: adolescence, 4-5x more
frequent in girls
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SLE - DG CRITERIA (ACR)• Malar rash
• Discoid lupus
• Mucocutaneous ulceration
• Non-erosive arthritis
• Nephritis (proteinuria >0,5g/d, cel.casts)
• Encefalopathy (seizures, psychosis)
• Pleuritis or pericarditis
• Cytopenia
• Positive immune serology
• Positive ANA
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SLE - clinical I
• Constitutional: fever, fatigue, weight loss
• Mucocutaneous: malar, discoid lupus, periungual erythema, photosensitivity, maculopap.exant., alopecia, ulcerations
• Musculoskeletal: arthritis/arthralgia, myopathy, asept.necrosis
• Vascular: arterioles, venules. Lupus crisis - acute generalised vasculitis, RS, thromboflebitis, livedo, purpura
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SLE
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SLE - clinical II
• Heart: pericarditis, myocarditis, veruccous
endocarditis (Libmann-Sachs)
• Lungs: pleuritis, pneumonitis,
pulm.haemorrhage, thromboembolism
• GIT: dysmotility, malabsorption, colitis,
peritonitis, ascites, pancreatitis, thrombosis
• RES: Hepatosplenomegaly, lymphadenopathy
• Autoimunne endocrinopathy
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SLE - clinical III
• NS: 20-35% children. Psychosis (organic or
functional), seizures, chorea, vascular accident,
neuropathy, pseudotumor cerebri
• Eyes: retinal vasculitis (cytoid bodies),
papilloedema, retinopathy, episcleritis
• Kidneys: 75% children. Main long-term
prognostic factor. Often asymptomatic, rarely
nephrotic syndrome, haematuria, hypertension,
renal failure
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SLE - investigations
• LABORATORY: non-specific
inflammatory activity, anemia, cytopenia,
autoantibodies (ANA, dsDNA, ENA,
ACLA), C3,C4, renal and hepatic
• IMAGING: organ involvement (XR, US,
MRI, EEG, SPECT)
• BIOPSY: skin, renal
• Disease activity and damage evaluation:
SLEDAI, BILAG..
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SLE - therapy
• Multidisciplinary approach
• Drug: NSAID, glucocorticoid, antimalarials,
immunosupressives (azathioprine, CYC,
MTX, CyA, MMF, rituximab),
anticoagulation, Ca and vitD supplementation
• Prevention and management of infectious
complications
• Regime (stress, diet, sun exposure)
• Education and councelling
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SLE - prognosis
• Protracted course with remissions and
exacerbations
• Mortality - approx. 15%
• Bad prognostic factors: diffuse proliferative
GNF, persistent CNS disease
• Main causes of death: 1) sepsis 2) renal
insufficiency
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NEONATAL LUPUS
• Maternal autoantibodies (mainly anti-Ro) in
foetal circulation from 12th-16th wk gestation,
bind to skin and myocardial structures (AV
node mainly)
• Clinical picture: CCHB, skin manifestations
(erythema annulare, discoid lupus), cytopenia
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• Antiphospholipid syndrome (APLS)
• Drug-induced lupus (anticonvulsants, infliximab) - often associated with anti-histone ANA
• Overlap syndromes: MCTD(SLE,JIA,JDM,SS), posit. ENA (anti U1RNP)
• Sjögren syndrome primary and secondary
(xerostomy, keratoconjunctivitis sicca, antiRo,La)
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Scleroderma I
• Systemic sclerosis: diffuse (proximal skin,
multiorgan involvement), limited ( CREST:
Calsinosis, RS, Esophageal dysmotility,
Sclerodaktyly, Teleangiectasia)
• Clinical features: oedema-induration-sclerosis-
skin atrophy, calcinosis, RS (90%), ischemic
fingertip ulceration, contractures, weakness,
arthralgia, abd pain, GI dysmotility and
malabsorption, dysphagia, pericarditis, pulm
fibrosis, PH, renal vasculitis with hypertension
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Scleroderma II• Localised:
- morphea-oval shaped, circumscripted induration
variable in size, depth and number
- linear scleroderma-often deep tissues involved
incl muscle and bone, epilepsy, organ involvement
• Eosinophilic fasciitis: skin induration with
flexion contractures, eosinophilia,
hypergamaglobulinemia, usually no organ
involvement
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Linear scleroderma