Infection
Bacterial osteomyelitis± septic arthritis
Mycobacterium
Postviral (rubella, EBV, hepatitis B etc)
Lyme
arthritis
Malignancy
Leukemia
Neuroblastoma
Osteoid
osteoma
Juvenile arthritis
Systemic JA
Pauciarticular JA
Polyarticular JA
Juvenile spondylitis
Juvenile rheumatoid arthritis
Arthritis of other rheumatic diseases
Juvenile psoriatic arthritis
Dermatomyositis
Systemic lupus erythematosus (SLE)
Scleroderma
Mixed
connective tissue disease (MCTD)
necrotizing vasculitis
Reflex
dystrophy
'Reactive' arthritis
Reiter's syndrome
Rheumatic fever
Meningitis with H. Influenza: meningococcus
Henoch Schonlien Purpura
Kawasaki's disease
Arthritis associated with chronic diseases
Arthritis of inflammatory bowel disease
Cystic
fibrosis
Sickle
cell disease
Hemophilia
Thalassemia
Orthopedic causes
Hip
slipped
capital femoral epiphysis
Legg-Calve-Perthes'
disease
Knee
synovial
hemangioma
discoid
meniscus
Terminology / classification
of juvenile arthritis
Classic American
classification
(refers to juvenile rheumatoid arthritis JRA)
Systemic JRA
Pauciarticular JRA
Type
I - ANA positive (young girls)
Type
II- HLA B27- positive (older boys)
Polyarticular JRA
Rheumatoid
factor-negative (younger children)
Rheumatoid
factor-positive (older children)
Criteria for
Classification of Juvenile Arthritis
|
1. |
Age of onset<16 years |
|
2. |
Arthritis in one or more joints defined as swelling or effusion, or presence of two or more of the following signs: limitation of range of motion, tenderness or pain on motion, and increased heat |
|
3. |
Duration of disease ≥ 6 weeks |
|
4. |
Type of onset of disease during the first 6 months
classified as: a. Polyarthritis:
5 or more joints b. Pauciarticular
(oligoarthritis): 4 or fewer joints c. Systemic
disease: arthritis with intermittent fever |
|
5. |
Exclusion of other forms of juvenile arthritis |
Cassidy
& Petty 1990
Features of JA
|
|
Systemic JA |
Pauciarticular JA |
Polyarticular JA |
Juvenile spondylitis |
Seropositive JA |
|
Median age at onset (years) |
5 |
2 |
3 |
10 |
12 |
|
% of
patients |
10 |
40 |
20 |
20 |
10 |
|
sex ratio |
M=F |
F>M |
F>M |
M>F |
F>M |
|
ANA |
No |
60% |
25% |
No |
60% |
|
Rheumatoid factor |
No |
No |
No |
No |
100% |
|
HLA association |
_ |
DR5 |
- |
B27 |
DR4 |
|
Chronic uveitis |
No |
30% |
10% |
No |
No |
|
Prognosis of
arthritis |
30%
sever |
10%
sever |
20%
sever |
mild
but spondylitis later |
>75%
sever |
Non JA causes of MSK pain in Children
Hypermobility Syndrome
Pes Planus
Genu recurvatum
Patellar dislocation
Chondromalacia Patella
Osgood Schlatter disease
Tenosynovitis
Frostbite arthritis
Reflex Sympathetic Dystrophy (RSD)
Growing pain
fibromyalgia
HLA Relashionship in Children with JA
|
Type of Onset |
Class I |
Class II |
|
Oligoarthritis
(early onset ≤6 years) |
A2, B44,35,16, Cw4 |
Dw7, DR4 |
|
With uveitis |
|
Dw/DR5 Dw/DRw8 Dw6 DRw52,w62 DPw2 |
|
Without
uveitis |
|
DR5,w8 |
|
ANA+ |
A2 |
DR5,w6,w8 DQw1 |
|
Polyarthritis RF+ |
B8,15 |
Dw14 Dw/DR4 |
|
Systemic
disease |
B8,35 |
DR4 Dw7 |
Etiology
HLA
Environmental
factors
infection
mycoplasma
parvo
V
Rubella
V
Klebsiela
Chlamydia
Reoviruses
Barometric
pressure
humidity
(high)
pressure
(low)
Diet
UV irradiation (juvenile dermatomyositis, systemic
lupus erythematosus SLE)
Immune complex
disease (rheumatic
fever, SLE, Henoch Schonlein purpura)
systemic
Onset juvenile arthritis
fever 100%
arthritis 100%
rash 95%
hepatosplenomegaly 85%
lyphadenopathy 70%
pericarditis 35%
pleuritis 20%
abdominal pain 10%
muscle pain 5%
uveitis <1%
fatigue
anemia
anorexia
leukocytosis
high acute phase reactants
Comparison of juvenile
spondyloarthropathies
|
|
JRA |
JAS |
SEA |
|
M:F |
1:4 |
7:1 |
9:1 |
|
Average age of onset |
5 |
>10 |
10 |
|
Average # joints |
9 |
6 |
5 |
|
Fx Hx |
30% |
65% |
65% |
|
Back pain |
2% |
100% |
45% |
|
ANA +ve |
30-80% |
0% |
0% |
|
RF +ve |
15% |
0% |
0% |
|
HLA B27 |
15% |
90% |
72% |
Spondyloarthropathy
|
low back
pain and stiffness pauciarticular
disease-small and large joints asymmetry in
joint involvement painful
arthritis enthesitis |
dactylitis sacroileitis limitation
of chest expansion tenosynovitis uveitis |
Clinical features in SLE
|
Constitutional |
Fever, malaise, weight loss |
|
Cutaneous |
Butterfly rash, discoid lupus, periungual
erythema,photosensitivity, mucocutaneous ulceration, alopecia |
|
MSK |
Polyarthralgia and arthritis, tenosynovitis,
myopathy, aseptic necrosis |
|
Vascular |
Lupus crisis, Raynaud's phenomenon, erythromelalgia,
thrombophlebitis, livedo reticularis, |
|
CVS |
Pericarditis and effusion, myocarditis, Libman-Sack
endocarditis |
|
Pulmonary |
Pleuritis, basilar pneumonitis, discoid atelectasis |
|
GI |
abdominal crisis, esophageal motor dysfunction,
colitis, |
|
RES |
Hepatomegaly, splenomegaly, lymphadenopathy |
|
CNS |
Organic brain syndrome, convulsions, psychosis,
chorea, CVA, polyneuritis and peripheral neuropathy, cranial nerve palsy,
pseudotumor cerebri |
|
Ocular |
Cotton wool spots, papilledema, retinopathy |
|
Renal |
Glomerulonephritis, nephrotic syndrome, uremia,
hypertension |
Drug induced SLE
Definite association Possible
association
Alpha methyldopa Captopril
Chlorpromazine Diphenylhydantoin
Hydralazine d-penicillamine
Isoniazid Ethosuximide
Nitrofurantoin
Primidone
Propylthiouracil
Trimethadone
WHO classification for SLE nephritis
|
Class I |
Normal |
No detectable disease |
|
Class II IIA |
Mesangial Minimal alteration |
Normal LM; mesangial deposits of Ig and C by IFM;
mesangial deposits by EM |
|
IIB |
Mesangial glomerulitis |
IIA plus mesangial hypercellularity (more than 3
cells per mesangial area or increased mesangial matrix); minimal
tubulo-interstitial disease |
|
Class III |
Focal and segmental proliferative GN |
Focal areas of intra- and extracapillary cellular
proliferation, necrosis, and leukocytes infiltration in <50% of the
glomeruli; subendothelial or mesangial deposits on IFM or EM; focal tubular
and interstitial disease |
|
Class IV |
Diffuse proliferative GN (DPGN) |
Class III involving more glomerular surface area and
>50% of the glomeruli; IFM and EM show abundant subendothelial deposits;
marked interstitial involvement; membranoproliferative variant has prominent
mesangial cell proliferation and capillary wall thickening |
|
Class V |
Membranous GN |
No mesangial, endothelial, or epithelial cellular
proliferation; diffusely and uniformly thickened capillary walls; IFM and EM
show mesangial and subepithelial deposits; minimal interstitial involvement |
Etiology of SLE
Primary B cell abnormality
Polyclonal B-cell activation
Genetic preprogramming (lupus haplotype)
Immunodeficiency (SIgAD, C2, C4)
Primary T-cell abnormality
Loss of negative immunoregulatory T cells
Suppressor tolerance
Impaired immune surveillance
Stem cell defect
Abnormal modulation of the immune response
T-cell and B cell defect, macrophage/dendritic cell
defect
ARA
criteria for SLE
Malar (butterfly) rash
Discoid-lupus rash
Photosensitivity
Oral or nasal mucocutaneous
ulceration
Nonerosive arthritis
Nephritis
Proteinuria>500mg/d
Cellular casts
Encephalopathy
Seizures
Psychosis
Pleuritis or pericarditis
Cytopenia
Positive immunoserology
Antibodies to nDNA
Antibodies to Sm nuclear
antigen
Positive LE cell
preparation
Biologic false positive test
for syphilis
Positive antinuclear antibody
test (ANA)
Principal diagnostic criteria for Kawasaki
syndrome
(5 of 6 criteria required for secure
diagnosis)
Fever
Conjuctival injection
Changes in the mouth
Erythema, fissuring, and
crusting of the lips
Diffuse oropharyngeal
erythema
Strawberry tongue
Changes in the peripheral
extremities
Induration of hands and feet
Erythema of palms and soles
Desquamation of fingertips and toetips, about