Paediatric RheumatologyPaediatric Rheumatology
Phil RileyPhil Riley
Consultant Paediatric Consultant Paediatric RheumatologistRheumatologist
TeachingTeaching
IntroductionIntroduction
Musculoskeletal pain common in Musculoskeletal pain common in childhoodchildhood
Wide range of diagnoses -benign to Wide range of diagnoses -benign to malignantmalignant
JIA- early recognition and JIA- early recognition and appropriate treatment prevents appropriate treatment prevents damage damage
JIA- Multidisciplinary teamJIA- Multidisciplinary team
Differential DiagnosisDifferential Diagnosis
InflammatoryInflammatory MechanicalMechanical PsychologicalPsychological
Differential Diagnosis-Differential Diagnosis-InflammatoryInflammatory
ReactiveReactive InfectionInfection Inflammatory diseaseInflammatory disease Systemic diseaseSystemic disease MalignancyMalignancy Irritable hipIrritable hip
Differential Diagnosis-Differential Diagnosis-MechanicalMechanical
Trauma-accidental and NAITrauma-accidental and NAI HypermobilityHypermobility OsteochondritidesOsteochondritides Degenerative disordersDegenerative disorders HaematologicalHaematological MetabolicMetabolic Tumours- benign and malignantTumours- benign and malignant
Differential-Differential-PsychogenicPsychogenic
FibromyalgiaFibromyalgia Functional illnessFunctional illness Reflex Sympathetic DystrophyReflex Sympathetic Dystrophy
Juvenile Idiopathic ArthritisJuvenile Idiopathic Arthritis
Unknown aetiologyUnknown aetiology Genetic predispositionGenetic predisposition incidence 1 per 10,000incidence 1 per 10,000 prevalence 1 per 1,000prevalence 1 per 1,000
Disease CourseDisease Course
50% still active after 25 years50% still active after 25 years X-ray Joint damage by 2.6 yearsX-ray Joint damage by 2.6 years Increased mortalityIncreased mortality Increased morbidity-PhysicalIncreased morbidity-Physical -Growth-Growth -Psychological-Psychological -Social-Social
Classification of Classification of Juvenile ArthritisJuvenile Arthritis
<16 years<16 years >6 weeks arthritis>6 weeks arthritis Subtypes classified for first 6 Subtypes classified for first 6
monthsmonths
SubtypesSubtypes
OligoarthritisOligoarthritis 1-41-4
-persistent-persistent
-extended-extended Polyarthritis (RF negative)Polyarthritis (RF negative) >=5>=5 Polyarthritis (RF positive)Polyarthritis (RF positive) >=5>=5 systemicsystemic
Investigations-JIAInvestigations-JIA
ANA- Antinuclear antibodyANA- Antinuclear antibody Inflammatory markers- CRP,ESRInflammatory markers- CRP,ESR FBC- Anaemia of chronic diseaseFBC- Anaemia of chronic disease x-ray -loss of joint space, erosions x-ray -loss of joint space, erosions
and carpal bone overcrowdingand carpal bone overcrowding MRI- synovitis (gadolinium MRI- synovitis (gadolinium
enhanced)enhanced)
UveitisUveitis
Chronic anterior uveitisChronic anterior uveitis Often AsymptomaticOften Asymptomatic Young,female, oligoarthritis, Young,female, oligoarthritis,
positive ANA (30%)positive ANA (30%) Polyarthritis (5%)Polyarthritis (5%) systemic(rare)systemic(rare) Slit lamp 3-6 monthly for 7 yearsSlit lamp 3-6 monthly for 7 years
SubtypesSubtypes
Systemic ArthritisSystemic Arthritis
-Rash-Rash
-temp over 2 weeks-temp over 2 weeks
-with/without arthritis-with/without arthritis
-with/without serositis-with/without serositis
-hepatosplenomegaly, -hepatosplenomegaly, lymphadenopathylymphadenopathy
JIAJIA
systemic rashsystemic rash
Systemic JIASystemic JIA
Quotidian feverQuotidian fever
Systemic JIASystemic JIA
Macrophage Activation Syndrome(MAS)Macrophage Activation Syndrome(MAS)
-Bleeding, purpura, bruising-Bleeding, purpura, bruising
--Nodes,liver,spleenNodes,liver,spleen
--FBC,ESRFBC,ESR
--PT,APTT, FDPPT,APTT, FDP
- - Fibrinogen,clotting factorsFibrinogen,clotting factors Bone MarrowBone Marrow IV steroids,CyclosporinIV steroids,Cyclosporin
SubtypesSubtypes
Enthesitis-related arthritisEnthesitis-related arthritis
-HLA B27-HLA B27 Psoriatic ArthritisPsoriatic Arthritis OtherOther
Treatment OptionsTreatment Options
NSAIDsNSAIDs SteroidsSteroids Joint injectionsJoint injections MethotrexateMethotrexate Sulphasalazine Sulphasalazine CiclosporinCiclosporin TNF drugsTNF drugs Autologous stem cell transplantation Autologous stem cell transplantation
QuestionsQuestions
OligoarthritisOligoarthritis ANA posANA pos normal slightly raised ESR,CRPnormal slightly raised ESR,CRP NSAIDSNSAIDS Joint injectionJoint injection Ophthalmology referralOphthalmology referral Rehab/MDTRehab/MDT
QuestionsQuestions
PolyarthritisPolyarthritis ANA positive or negativeANA positive or negative Rh factor positive or negativeRh factor positive or negative very raised ESR,CRPvery raised ESR,CRP SteroidsSteroids MethotrexateMethotrexate EyesEyes Rehab/MDTRehab/MDT
QuestionsQuestions
SystemicSystemic RashRash Quotidian feverQuotidian fever NSAIDSNSAIDS SteroidsSteroids MethotrexateMethotrexate Macrophage Activation SyndromeMacrophage Activation Syndrome
Treatment ConceptsTreatment Concepts
Early Early MonitoringMonitoring MultidisciplinaryMultidisciplinary
Treatment continuedTreatment continued
PhysiotherapyPhysiotherapy restore functionrestore function improve muscle strengthimprove muscle strength splints/serial castssplints/serial casts
Occupational therapyOccupational therapy PsychologyPsychology
SpotterSpotter
Butterfly RashButterfly Rash
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ButtterflyButtterfly
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Raynaud’s Raynaud’s phenomenonphenomenon
SLE- Diagnostic/ WHO SLE- Diagnostic/ WHO classification criteria(4 of classification criteria(4 of
11)11)
Malar rash(butterfly)Malar rash(butterfly) Discoid lupus rashDiscoid lupus rash PhotosensitivityPhotosensitivity Oral/nasal mucosal ulcerationOral/nasal mucosal ulceration Non-erosive arthritisNon-erosive arthritis nephritisnephritis
-proteinuria/cellular casts-proteinuria/cellular casts
SLE-Diagnostic/WHO SLE-Diagnostic/WHO classification(4 of 11)classification(4 of 11)
Encephalopathy-seizures/psychosisEncephalopathy-seizures/psychosis Pleuritis/pericarditisPleuritis/pericarditis HaematologicalHaematological
-lymphopaemia-lymphopaemia-thrombocytopaenia-thrombocytopaenia
positive immunoserologypositive immunoserology-anti ds-DNA-anti ds-DNA
ANA positiveANA positive
SLE- clinical featuresSLE- clinical features
Constitutional - fever/malaise/weight Constitutional - fever/malaise/weight lossloss
Cutaneous Cutaneous rash/photosensitivity/alopecia/mouth ulcersrash/photosensitivity/alopecia/mouth ulcers
MusculoskeletalMusculoskeletal poly-arthritis/arthralgiapoly-arthritis/arthralgia tenosynovitistenosynovitis myopathymyopathy avascular necrosisavascular necrosis
SLE-Clinical featuresSLE-Clinical features
VascularVascular lupus crisis/Raynaud’s/livedolupus crisis/Raynaud’s/livedo
CardiacCardiac pericarditis/myocarditis/endocarditispericarditis/myocarditis/endocarditis
PulmonaryPulmonary pleuritis/pneumonitis/haemorrhagepleuritis/pneumonitis/haemorrhage
SLE-lab featuresSLE-lab features
FBC- low plateletsFBC- low platelets
- low lymphocytes- low lymphocytes Inflammatory markers- high ESR, Inflammatory markers- high ESR,
normal CRPnormal CRP ANA- very high ie >1:2560ANA- very high ie >1:2560 DsDNA- highDsDNA- high C3,C4- lowC3,C4- low
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Neonatal lupusNeonatal lupus
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Neonatal LupusNeonatal Lupus
Neonatal lupusNeonatal lupus
Maternal autoantibody transmissionMaternal autoantibody transmission Cong heart block (Ro/La) Cong heart block (Ro/La) - 50%- 50% Cutaneous neonatal lupusCutaneous neonatal lupus - 37%- 37% Hepatic/GI tract Hepatic/GI tract - 8%- 8% HaematologicHaematologic -6%-6% Neurologic and pulmonaryNeurologic and pulmonary -1%-1%
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Heliotrope RashHeliotrope Rash
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Gottrons papulesGottrons papules
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CalcinosisCalcinosis
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CapillaroscopyCapillaroscopy Abnormal “bushy”Abnormal “bushy” Thickening and Thickening and
dropoutdropout
Definition of Juvenile Definition of Juvenile Dermatomyositis(JDM)Dermatomyositis(JDM)
limb-girdle and anterior neck flexor limb-girdle and anterior neck flexor weaknessweakness
Muscle biopsyMuscle biopsy Muscle enzyme increaseMuscle enzyme increase EMG EMG Dermatological featuresDermatological features
JDM - clinical featuresJDM - clinical features
Proximal weaknessProximal weakness VasculopathyVasculopathy
Heliotrope facial rashHeliotrope facial rash Gottrons papulesGottrons papules
Raised CK/LDH/AST/ALTRaised CK/LDH/AST/ALT MR scan/muscle biopsyMR scan/muscle biopsy Multi-organ occasionallyMulti-organ occasionally
JDM - treatmentJDM - treatment
SteroidsSteroids pulse ivpulse iv oral taperingoral tapering
MethotrexateMethotrexate Cyclosporin/Immunoglobulin/Cyclosporin/Immunoglobulin/
Cyclophosphamide/Anti TNFCyclophosphamide/Anti TNF Physio/RehabPhysio/Rehab
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en coup de sabreen coup de sabre
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SclerodactlySclerodactly
Scleroderma in childrenScleroderma in children
Systemic sclerosisSystemic sclerosis limited cutaneous/CRESTlimited cutaneous/CREST diffusediffuse
LocalisedLocalised linear scleroderma (en coup de linear scleroderma (en coup de
sabre,morphoea)sabre,morphoea)
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