PAEDIATRIC RHEUMATOLOGY
Juvenile Idiopathic Ehler-Danlos HSP
Rheumatic Fever Kawasaki
Arthritis (JIA) Syndrome (EDS) (IgA vasculitis)
Type 2 hypersensitive autoimmune Autoimmune inflammation in joints Umbrella term for genetic IgA deposits in blood • Mucocutaneous LN
condition triggered by antibodies (i.e. arthritis without any cause) collagen defects causing vessels affecting: syndrome
against GAS hypermobility in CT Ø Skin • systemic medium sized
Ø 2-4 weeks after initial infection Types Ø Autosomal dominant Ø Kidneys vessel vasculitis
(e.g. tonsillitis, pharyngitis) Ø GIT
Ø Systemic JIA (Still’s disease)
PP Must exclude
Ø Polyarticular JIA Types
Multi-system disorder affecting Ø Oligoarticular JIA Ø Hypermobile EDS Ø Meningococcal
Ø Heart Ø Enthesitis -related arthritis Ø Classical EDS Ø Sepsis
Ø Juvenile psoriatic arthritis Ø Vascular EDS Ø Acute Leukemia
Ø Joints, skin
Ø Kyphoscoliotic EDS Ø HUS
Ø ITP
• ATSI FHx of autoimmune • Children < 5yo
• Female • Boys
RF
• Low SES or overcrowded DDx: septic, osteomyelitis,
osteosarcoma, transient, Perthes
• Ages 5-14
JONES criteria More than > 6 weeks Sx in patients Hypermobile EDS • Non-blanching • Persistent high fever > 5
(2 major OR 1 major + 2 minor) under age of 16 purpura rash on days
Ø Hypermobility of joints
Ø Joint pain buttocks and CREAM
Ø Swelling Ø Soft stretchy skin lower limbs (100%)
Major • Bilateral Conjunctivitis
Ø Stiffness - inflamed and
• Joints – Migratory painful leaky small BVs • Widespread red
Classical EDS Blanching maculo-
inflammatory polyarthritis • Abdo pain (50%)
Systemic JIA Ø Velvet smooth skin papular rash
• Organ inflamed (endocarditis • Arthralgia (75%)
Ø Salmon-pink rash Ø Abnormal wound healing • Oedema
- ↑HR, pericardial rub, HF)
Ø Swinging fevers (> 5day) • Haematuria - IgA • Adenopathy
• N - SC nodules (firm painless Ø High risk of prolapse,
Ø Joint pain vasculitis (50%)
nodulues) hernias, MR and aortic • Muco-cutaneous -
Ø Enlarged LN
root dilatation cracked lips and
• E – erythema marginatum Ø UWL
strawberry tongue -red
(pink rings on torso and Polyarticular JIA (> 5 joints) tongue w/ large papillae
proximal limb) Ø Idiopathic symmetrical Vascular EDS skin desquamation
• S – Sydenham chorea (XS Ø Mild fever, anaemia, and Ø Thin, translucent skin
Sx dopamine releasee – invol. reduced growth
Ø Fragile BVs – Disease course
Jerky movements) Oligoarticular JIA (≤ 4 large joints) spontaneous bleed
Ø Anterior uveitis Ø Acute phase – unwell
Minor
Ø ANA +ve + rash, fever, LN (for 1-2
• Fever Kyphoscoliotic EDS wks)
Enthesitis-Related Arthritis Ø Subacute phase –
• ECG changes (prolonged PR) Ø Hypotonia when neonate
Ø Seronegative – AS, RA, IBD, arthralgia, skin
• Arthralgia w/o arthritis psoriatic Ø Kyphoscoliosis when desquamation (2-4
• Raised CRP/ESR Ø Enthesitis ® IP joints, wrist, growing older wks)
ASIS, Achilles, patellar Ø Convalescent
Ø Rupture of medium sized
insertion arteries (GIT, coronary) (recovery) period –
Juvenile Psoriatic Arthritis bloods return normal
and aneurysms may
Ø Symmetrical polyarthritis
regress (2-4 weeks)
affecting small joints OR
asymmetrical large joints
Ø Psoriasis, pitting, onycholysis,
dactylitis, psoriasis plaques
• Recurrent rheumatic fever Macrophage activation system Early onset OA • ISS Coronary artery aneurysm
• Structural Heart – mitral stenosis (MAS) – severe immune response POTS – due to ANS dysfunction
presenting as • Bowel infraction
(>10yo) and IE, Ø Inappropriate tachycardia
Comp. Ø DIC • Gi haemorrhage
• Arrythmias – AF on sitting or standing up
Ø Low Hb, Low PLTS Ø Pre-sycope, headache, • IgA nephritis
• Chronic HF Ø Non-blanching rash nausea
• PSGN (weeks after) Ø Low ESR
• Throat swab Clinical Dx Beighton score (/9) for • BP - ?HTN • FBC – anemia, ↑WCC, ↑
hypermobility to Dx • FBC, +blood film - plt,
• ASOT +ve Ø Raised CRP, ESR
Ø Elbows/knee hypertEXT ?sepsis, leukemia
• ECHO – carditis, IE Ø Raised serum ferritin • LFT – low albumin, ↑LFT
• ECG – AF? Ø ANA
Ø
Ø
Thumb bend to forearm
Pinky extends > 90 degs • EUC ® • ESR - raised
Ix ?nephrotic
• CXR Ø RF, anti-CCP • Urinalysis – pyuria with no
• UA
DDx: Marfan’s – higharched • CRP ® ?sepsis infection
palette, archnodactyly, arm • Blood cultures • ECHO
span • Urine dipstick +
ACR
Main Rx: Referral NO CURE!! Supportive Mx Urgent paeds referral
Penicillin V (phenoxy- • Paed rheumatology • Analgesia Ø High dose aspirin to
methylpenicillin) for 10 days • MDT involvement • Rest reduce risk of
Aim to maintain healthy joints thrombosis
• Ophthal referral if • Hydration
and stop complications Ø IVIg to reduce risk of
Supportive Rx: oligoarticular JIA • Abdo resolves
Ø F/U – rheum within few days coronary artery
• Hand hygiene aneurysms
Supportive Rx to reduce joint Ø PT -stabilise joints
• Analgesia (NSAID) – athralgia Ø F/U ECHO after
Mx inflammation and minimise Sx: Ø OT -max function + Follow up on: recovery
• Aspirin + steroids – for carditis maintain good joint
Ø NSAID (ibuprofen) • Urine dipstick for
• Prophylactic ABx – PO or IM Ø IM/IA steroids (oligoarthritis) posture proteinuria and
(continued into adulthood) Ø DMARDs (MTX, SSZ, Beware of Reye’s syndrome
Ø Psychology - QoL haematuria
= LE swelling of liver and
• Valproate – Sydenham chorea leflunomide) • Regular BP – HTN
Ø Biologic (TNFa inhibitor – brain ® confusion, seizures,
• CHADS-VASC ≥ 1 = DOAC • Warn recurrence in LOC
infliximab, adalimumab, 6/12 for 33% of
etanercpt) patients *ONLY condition where
aspirin is used in children
*Nb: Fevers for more than 5 days, the key non-infective differentials to remember are Kawasaki disease, Still’s disease, rheumatic fever and
leukaemia.
Juvenile Idiopathic Ehler-Danlos HSP
Rheumatic Fever Kawasaki
Arthritis (JIA) Syndrome (EDS) (IgA vasculitis)
Type 2 hypersensitive autoimmune Autoimmune inflammation in joints Umbrella term for genetic IgA deposits in blood • Mucocutaneous LN
condition triggered by antibodies (i.e. arthritis without any cause) collagen defects causing vessels affecting: syndrome
against GAS hypermobility in CT Ø Skin • systemic medium sized
Ø 2-4 weeks after initial infection Types Ø Autosomal dominant Ø Kidneys vessel vasculitis
(e.g. tonsillitis, pharyngitis) Ø GIT
Ø Systemic JIA (Still’s disease)
PP Must exclude
Ø Polyarticular JIA Types
Multi-system disorder affecting Ø Oligoarticular JIA Ø Hypermobile EDS Ø Meningococcal
Ø Heart Ø Enthesitis -related arthritis Ø Classical EDS Ø Sepsis
Ø Juvenile psoriatic arthritis Ø Vascular EDS Ø Acute Leukemia
Ø Joints, skin
Ø Kyphoscoliotic EDS Ø HUS
Ø ITP
• ATSI FHx of autoimmune • Children < 5yo
• Female • Boys
RF
• Low SES or overcrowded DDx: septic, osteomyelitis,
osteosarcoma, transient, Perthes
• Ages 5-14
JONES criteria More than > 6 weeks Sx in patients Hypermobile EDS • Non-blanching • Persistent high fever > 5
(2 major OR 1 major + 2 minor) under age of 16 purpura rash on days
Ø Hypermobility of joints
Ø Joint pain buttocks and CREAM
Ø Swelling Ø Soft stretchy skin lower limbs (100%)
Major • Bilateral Conjunctivitis
Ø Stiffness - inflamed and
• Joints – Migratory painful leaky small BVs • Widespread red
Classical EDS Blanching maculo-
inflammatory polyarthritis • Abdo pain (50%)
Systemic JIA Ø Velvet smooth skin papular rash
• Organ inflamed (endocarditis • Arthralgia (75%)
Ø Salmon-pink rash Ø Abnormal wound healing • Oedema
- ↑HR, pericardial rub, HF)
Ø Swinging fevers (> 5day) • Haematuria - IgA • Adenopathy
• N - SC nodules (firm painless Ø High risk of prolapse,
Ø Joint pain vasculitis (50%)
nodulues) hernias, MR and aortic • Muco-cutaneous -
Ø Enlarged LN
root dilatation cracked lips and
• E – erythema marginatum Ø UWL
strawberry tongue -red
(pink rings on torso and Polyarticular JIA (> 5 joints) tongue w/ large papillae
proximal limb) Ø Idiopathic symmetrical Vascular EDS skin desquamation
• S – Sydenham chorea (XS Ø Mild fever, anaemia, and Ø Thin, translucent skin
Sx dopamine releasee – invol. reduced growth
Ø Fragile BVs – Disease course
Jerky movements) Oligoarticular JIA (≤ 4 large joints) spontaneous bleed
Ø Anterior uveitis Ø Acute phase – unwell
Minor
Ø ANA +ve + rash, fever, LN (for 1-2
• Fever Kyphoscoliotic EDS wks)
Enthesitis-Related Arthritis Ø Subacute phase –
• ECG changes (prolonged PR) Ø Hypotonia when neonate
Ø Seronegative – AS, RA, IBD, arthralgia, skin
• Arthralgia w/o arthritis psoriatic Ø Kyphoscoliosis when desquamation (2-4
• Raised CRP/ESR Ø Enthesitis ® IP joints, wrist, growing older wks)
ASIS, Achilles, patellar Ø Convalescent
Ø Rupture of medium sized
insertion arteries (GIT, coronary) (recovery) period –
Juvenile Psoriatic Arthritis bloods return normal
and aneurysms may
Ø Symmetrical polyarthritis
regress (2-4 weeks)
affecting small joints OR
asymmetrical large joints
Ø Psoriasis, pitting, onycholysis,
dactylitis, psoriasis plaques
• Recurrent rheumatic fever Macrophage activation system Early onset OA • ISS Coronary artery aneurysm
• Structural Heart – mitral stenosis (MAS) – severe immune response POTS – due to ANS dysfunction
presenting as • Bowel infraction
(>10yo) and IE, Ø Inappropriate tachycardia
Comp. Ø DIC • Gi haemorrhage
• Arrythmias – AF on sitting or standing up
Ø Low Hb, Low PLTS Ø Pre-sycope, headache, • IgA nephritis
• Chronic HF Ø Non-blanching rash nausea
• PSGN (weeks after) Ø Low ESR
• Throat swab Clinical Dx Beighton score (/9) for • BP - ?HTN • FBC – anemia, ↑WCC, ↑
hypermobility to Dx • FBC, +blood film - plt,
• ASOT +ve Ø Raised CRP, ESR
Ø Elbows/knee hypertEXT ?sepsis, leukemia
• ECHO – carditis, IE Ø Raised serum ferritin • LFT – low albumin, ↑LFT
• ECG – AF? Ø ANA
Ø
Ø
Thumb bend to forearm
Pinky extends > 90 degs • EUC ® • ESR - raised
Ix ?nephrotic
• CXR Ø RF, anti-CCP • Urinalysis – pyuria with no
• UA
DDx: Marfan’s – higharched • CRP ® ?sepsis infection
palette, archnodactyly, arm • Blood cultures • ECHO
span • Urine dipstick +
ACR
Main Rx: Referral NO CURE!! Supportive Mx Urgent paeds referral
Penicillin V (phenoxy- • Paed rheumatology • Analgesia Ø High dose aspirin to
methylpenicillin) for 10 days • MDT involvement • Rest reduce risk of
Aim to maintain healthy joints thrombosis
• Ophthal referral if • Hydration
and stop complications Ø IVIg to reduce risk of
Supportive Rx: oligoarticular JIA • Abdo resolves
Ø F/U – rheum within few days coronary artery
• Hand hygiene aneurysms
Supportive Rx to reduce joint Ø PT -stabilise joints
• Analgesia (NSAID) – athralgia Ø F/U ECHO after
Mx inflammation and minimise Sx: Ø OT -max function + Follow up on: recovery
• Aspirin + steroids – for carditis maintain good joint
Ø NSAID (ibuprofen) • Urine dipstick for
• Prophylactic ABx – PO or IM Ø IM/IA steroids (oligoarthritis) posture proteinuria and
(continued into adulthood) Ø DMARDs (MTX, SSZ, Beware of Reye’s syndrome
Ø Psychology - QoL haematuria
= LE swelling of liver and
• Valproate – Sydenham chorea leflunomide) • Regular BP – HTN
Ø Biologic (TNFa inhibitor – brain ® confusion, seizures,
• CHADS-VASC ≥ 1 = DOAC • Warn recurrence in LOC
infliximab, adalimumab, 6/12 for 33% of
etanercpt) patients *ONLY condition where
aspirin is used in children
*Nb: Fevers for more than 5 days, the key non-infective differentials to remember are Kawasaki disease, Still’s disease, rheumatic fever and
leukaemia.
