Rheumatoid Disease in
Children
小儿风湿性疾病
Children’s Hospital of
Fudan University
Xu Hong
hxu@shmu.edu.cn
Rheumatoid Disease
Connective tissue diseases
（包含有 120种与关节、骨骼、肌肉等相关的疾病）
过敏性紫癜 （ anaphylactoid purpura）
类风湿性关节炎 （ JRA）
SLE（ systemic lupus erythematosis）
皮肌炎 （ dermatomyositis）
硬皮病 （ scleroderma）
风湿热 （ rheumatic fever）
川崎病 (Kawasaki disease)
Rheumatoid Disease in Children
The most common diseases：
? Juvenile Rheumatiod Arthritis
? Henoch-Schonlein purpura
? Kawasaki Disease
(Mucocutaneous Lymph Node Syndrome,MCLS)
? Rheumatic Fever
冠状动脉造影 Coronary angiogram
Henoch-Schonlein purpura
Alternative names:
?Anaphylactoid purpura
? Vascular purpura
Definition
Henoch-Schonlein purpura is a disease that
has the symptoms
? purple spots on the skin
? joint pain
? gastrointestinal symptoms
? glomerulonephritis
? (a type of kidney disorder)
History story
? Schonlein's Purpura
( In 1837,Johan Schonlein )
the skin and joints are
affected
but the gastrointestinal
tract is not
? Henoch's Purpura
(Later,his student,Eduard
Henoch )
purpura spots on the skin
and acute abdominal
problems
not affected by joint
disease
General conditions
? a type of hypersensitivity vasculitis and
inflammatory response within the blood vessel
? It is caused by an abnormal response of the
immune system
? The exact cause for this disorder is unknown.
? The syndrome is usually seen in children,but
people of any age may be affected
? It is more common in boys than in girls
? Many people with Henoch-Schonlein purpura
had an upper respiratory illness in the previous
weeks
Causes and pathogenesis
? An inflammatory disease of the small
blood vessels (capillaries)
? usually a self-limited disease
? It is the most common form of childhood
vascular inflammation (vasculitis) and
results in inflammatory changes in small
blood vessels
Common allergens include
? Medications
(such as certain antibiotics,antihistamines
and thiazide diuretics )
? Pathogens
( bacterium/virus/parasite,such as group A
strep,varicella,parvovirus and hepatitis B )
? An acute respiratory infection
Common allergens include
? foods ( such as berries,shellfish,fish,
nuts,eggs,milk,and others )
? pollen
? animal dander ( scales of shed skin )
? insect bites
? Certain vaccinations,such as for measles,
yellow fever and cholera
Pathophysiology
? a small-vessel vasculitis characterized by
immunoglobulin A (IgA),C3,and immune
complex deposition in arterioles,
capillaries,and venules
? HSP and IgA nephropathy are related disorders,
Both illnesses have elevated serum IgA levels
and identical findings on renal biopsy
Pathophysiology
? Lower Leg Direct
immunofluoresence
(DIF) showed
deposits of IgA about
dermal vessels.
Renal biopsy showed deposits of IgA in the mensangium
Frequency:
? In the US,Approximately 14 cases occur per 100,000
school-aged children,HSP also occurs in adults,although
less commonly than in children.
Mortality/Morbidity:
In general,HSP is a benign self-limited disorder.
? Fewer than 5% of cases cause chronic symptoms.
? Fewer than 1% of cases progress to end-stage renal
failure.
Sex,
Male-to-female ratio is about 2:1.
Age:
75% of patients affected are aged 2-11 years,In some series,
as many as 27% of the patients are adults.
Manifestations
? History,The most common symptoms of HSP
include the following,
? Rash (95-100%),especially involving the legs,
may not be present on initial presentation
? Subcutaneous edema (20-50%)
? Abdominal pain and vomiting (85%)
? Joint pain (60-80%),especially involving knees
and ankles
? Scrotal edema (2-35%)
? Bloody stools ("hematochezia","melena" )
Manifestations
Physical:
? Palpable purpura,particularly on the buttocks
and legs
? Edema of the hands,feet,scalp,and ears
? Arthritis,most commonly involving the knees
and ankles
? Abdominal tenderness
? Gastrointestinal bleeding
? Acute scrotal edema that may mimic testicular
torsion
With
Angioneurotic edema
Swelling – eyes,lip,hand
On the buttocks
臀部
in rare cases
? the brain and spinal cord (central
nervous system) involved
Arthritis,the knee
Lab Studies
? Routine lab test results are usually within
reference ranges.
? Some lab studies help in excluding other
diagnoses and in evaluating renal function:
urinalysis / CBC with platelet count and
differential / BUN,creatinine / prothrombin
time (PT),activated partial thromboplastin
time (aPTT) / lipase
Lab Studies
? Hematuria and/or proteinuria are present in 10-
20% of patients
? Platelet count is usually in the reference range but
may be elevated
? A normal lipase makes acute pancreatitis very
unlikely
? WBC count may be in the reference range or
elevated,Eosinophilia is sometimes present
? ESR in the reference range or elevated
? BUN and creatinine levels may be elevated from
renal involvement of HSP or from dehydration
Imaging Studies:
? Abdominal ultrasound may be better than
barium enema to diagnose intussusception,
since HSP-related intussusception is more often
ileoileal instead of ileocolic as is typical in
idiopathic intussusception.
? Doppler or radionuclide testicular scan results
show normal or increased blood flow in HSP,in
contrast to the decreased blood flow seen in
testicular torsion.
Diagnosis and differentiation
Diagnosis is clinical and not based on
laboratory evaluation
? A physical examination reveals skin
lesions and joint tenderness
? A urinalysis shows microscopic blood in
the urine
? A skin biopsy shows vasculitis
Diagnosis and differentiation
? A normal platelet count rules out
idiopathic thrombocytopenic purpura
(ITP)
? A normal platelet count and normal
coagulation studies (ie,PT,aPTT,fibrin
split products) rule out thrombotic
thrombocytopenic purpura (TTP)
Other Problems to be Considered
? Essential mixed cryoglobulinemia
? Waldenstr?m macroglobulinemia
? Systemic lupus erythematosus
? Rickettsial diseases
? Bowel infarction/perforation
Treatment
? Anti-inflammatory medications,such as aspirin
or ibuprofen,to relieve joint pain and swelling
? Stopping medication that may be the
underlying cause
? Corticosteroids
If symptoms persist,therapy with corticosteroids
such as prednisone,tried to treat abdominal pain,
subcutaneous edema,and nephritis
Treatment
Patients with severe abdominal pain,
significant gastrointestinal bleeding,
or marked renal insufficiency may
require hospitalization.
Treatment in HSPN (HSP nephritis)
many different modalities in severe cases
IV or oral steroids with or without:
? Azathioprine （ 硫唑嘌呤 ）
? Cyclophosphamide （ CTX）
? Cyclosporine （ 环孢素 A）
? Dipyridamole (潘生丁 )
? Plasmapheresis （ 血浆置换 ）
? high-dose IV immunoglobulin G (IVIg)
Prognosis
? HSP is generally a benign disease with an
excellent prognosis
? More than 80% of patients have a single isolated
episode lasting a few weeks
? Approximately 10-20% of patients have
recurrences
? Fewer than 5% of patients develop chronic HSP
? Abdominal pain resolves spontaneously within
72 hours in most patients
General conditions
? A chronic systemic disease
? Primarily of the joints
? Marked by inflammatory changes in the
synovial membranes（ 滑膜） and articular
structures
? Widespread fibrinoid degeneration of the
collagen fibers in mesenchymal tissues,and by
atrophy and rarefaction of bony structures
（ 纤维化和骨质疏松）
? Etiology is unknown
? Autoimmune mechanisms have been implicated
General conditions
? Arthritis can be short-term - lasting for
just a few weeks or months,then going
away forever
? or it can be chronic and last for months
or years,In rare cases,it can last a
lifetime
Causes and pathogenesis
? Causes unknown exactly
? Research indicates that it is an autoimmune
disease
? White blood cells lose the ability to tell the
difference between the body's own healthy cells
and harmful invaders like bacteria and viruses
? The immune system,which is supposed to
protect the body from these harmful invaders,
instead releases chemicals that can damage
healthy tissues and cause inflammation and pain
Clinical manifestations
Signs and Symptoms
? The first signs of arthritis can be subtle or
obvious,
? Signs may include limping (跛行 ) or a sore wrist,
finger,or knee
? Joints may suddenly swell and remain enlarged,
? Stiffness in the neck,hips,or other joints
? Rashes may suddenly appear and disappear
developing in 1 area and then another,
? High fevers that tend to spike in the evenings
and suddenly disappear are characteristic of
systemic juvenile rheumatoid arthritis
High fevers in
Systemic JRA
Clinical manifestations
? Typically appears between the ages of 6 months
and 16 years
? Not a single disease,but a group of diseases
? What they all have in common is chronic joint
inflammation,the first signs are often joint pain
or swelling and reddened or warm joints
? The diseases are very different in their
symptoms,their treatments,and their outcomes
? There are 3 major types of juvenile rheumatoid
arthritis (according to onset within the first 6
months)
Clinical manifestations
Polyarticular arthritis (多关节型 )
affects more girls than boys
Symptoms include swelling or pain in 5 or more
joints
The small joints of the hands are affected as well
as the weight-bearing joints such as the knees,
hips,ankles,feet,and neck
In addition,a low-grade fever may appear,as well
as bumps or nodules on the body on areas
subjected to pressure from sitting or leaning
swelling of small joints
of the hands
a lot of stiffness when they first wake up in
the morning,Once their joints warm up,
they can usually move more easily,
Clinical manifestations
Pauciarticular JRA (少关节型 )
? affects 4 or fewer joints
? Symptoms include pain,stiffness,or swelling in
the joints
? The knee and wrist joints are the most
commonly affected
? iridocyclitis or iritis or uveitis may occur with
or without active joint symptoms
( can be detected early by an ophthalmologist )
Clinical manifestations
Systemic JRA （全身型）
? affects the whole body
? Symptoms include high fevers that often increase in the
evenings and then may suddenly drop to normal
? During the onset of fever,the child may feel very ill,
appear pale,or develop a rash
? The rash may suddenly disappear and then quickly
appear again
? The spleen and lymph nodes may also become enlarged
? Eventually many of the body's joints are affected by
swelling,pain,and stiffness
Lab Studies
? Abnormalities in the numbers and
appearances of CBC can be useful in the
diagnosis of many medical conditions
? A blood culture may be taken to rule out
infections
? Bone marrow examination is a test to
rule out conditions such as leukemia
Lab Studies
? Erythrocyte sedimentation rate often increases
in people when inflammation is occurring in
the body
? Rheumatoid factor（ RA 类风湿因子） is an
antibody,common in rheumatoid arthritis
o Rare in persons with systemic JRA
o a marker for persistence of polyarticular
JRA into adulthood
Lab Studies
? ANA (antinuclear antibody) is a test performed
on the blood to detect autoimmunity,It is also
useful in predicting which children are likely to
have eye disease with JRA
? Test for certain viral infections such as Lyme
disease that may cause similar symptoms or
occur along with the arthritis.
Imaging Studies
Radiography of affected joints
? When only a single joint is affected,
radiography is important to exclude other
diseases,such as osteomyelitis 骨髓炎 or
septic arthritis
? Bone scanning,When physical findings do
not document definite arthritis,consider
bone scanning as a means of identifying a
potential focus of osteomyelitis or other
abnormality
Imaging Studies
o MRI is helpful when considering trauma
in the differential diagnosis
o CT scanning of long bones,Perform
when considering osteoid osteoma in a
child with lower extremity pain (often at
night) and unremarkable findings on
physical examination.
Imaging Studies
? Echocardiography
o This is performed in a child with possible
systemic JRA and with fevers.
o In a person who has nonspecific rash,
adenopathy,and possible mucocutaneous
changes,to exclude coronary arterial dilation
resulting from (possibly atypical) Kawasaki
disease,
? In an individual who has findings suggestive of
SLE (eg,nephritis,pleuritic chest pain,
thrombocytopenia)
Others
Synovial biopsy
? This procedure may be helpful to exclude
other diagnoses
? particularly when the knee is affected (eg,
villonodular synovitis 绒毛膜结节样滑膜炎
granulomatous arthritis)
Diagnosis and differentiation
Diagnosis
? To effectively manage and minimize the effects
of arthritis,an early and accurate diagnosis is
essential
? A detailed physical examination is a critical
tool in diagnosing JRA
? Physical findings are important to detect
abnormalities suggestive of other possible
diagnoses
Diagnosis and differentiation
? The diagnosis of JRA is based on the physical
finding of arthritis (or synovitis) in at least one
joint that persists for at least 6 weeks
? With other causes being excluded and with
onset when the individual is younger than 16
years
? Arthritis on examination is defined as either
joint swelling or the combination of limited
motion with pain (on motion or to palpation)
Diagnosis and differentiation
DIFFERENTIALS
? Acute Lymphoblastic Leukemia
? Autoimmune Chronic Active Hepatitis
? Crohn Disease
? Endocarditis,Bacterial
? Fever in the Toddler
? Kawasaki Disease
? Systemic Lupus Erythematosus
Treatment
The goals of treatment:
? To relieve pain and inflammation
? To slow down or prevent the destruction of joints
? To restore use and function of the joints to
promote optimal growth,physical activity,and
social and emotional development
Combination of medication,physical therapy,and
exercise
(physician,rheumatologist,and physical therapist)
Treatment
? Initial treatment limited to the use of salicylates
? then other nonsteroidal anti-inflammatory drugs
(NSAIDs) resulted in many patients becoming
wheelchair bound
? Other patients underwent synovectomies to
remove excess tissue resulting from uncontrolled
arthritis
? The addition of second-line drugs,starting with
gold salt injections and then replaced by the
much more effective methotrexate
(MTX),improved the outlook for these patients
Treatment
? In specific situations,injection of
corticosteroids into the joint
? The approval of the biologic antagonist to
tumor necrosis factor (TNF) ---- a new
era of treatment more closely tailored to
the pathophysiology of the disease
Treatment
Physical Therapy
? An appropriate physical therapy
program is essential in the management
of any type of arthritis
? To recommend range-of-motion exercises
to restore flexibility in stiff,sore joints
and other exercises to help build strength
and endurance
Treatment
Regular Exercise
? When pain strikes,it's natural to want to sit
still
? It's important to maintain a regular exercise
program,especially for those with arthritis
? Muscles must be kept strong and healthy so
they can help support and protect joints
? Regular exercise also helps to maintain range
of motion of joints
?Patients with JRA may have a gradually
responsive disease (particularly those with
pauciarticular JRA)
?A small number of patients with pauciarticular
JRA develop aggressive arthritis confined to a
single joint,requiring more intensive medical
treatment and physical therapy.
?Some patients with polyarticular JRA
demonstrate rapid response to treatment; however,
most have prolonged courses,requiring frequent
adjustments in medical and nonmedical therapy
Treatment
Children
小儿风湿性疾病
Children’s Hospital of
Fudan University
Xu Hong
hxu@shmu.edu.cn
Rheumatoid Disease
Connective tissue diseases
（包含有 120种与关节、骨骼、肌肉等相关的疾病）
过敏性紫癜 （ anaphylactoid purpura）
类风湿性关节炎 （ JRA）
SLE（ systemic lupus erythematosis）
皮肌炎 （ dermatomyositis）
硬皮病 （ scleroderma）
风湿热 （ rheumatic fever）
川崎病 (Kawasaki disease)
Rheumatoid Disease in Children
The most common diseases：
? Juvenile Rheumatiod Arthritis
? Henoch-Schonlein purpura
? Kawasaki Disease
(Mucocutaneous Lymph Node Syndrome,MCLS)
? Rheumatic Fever
冠状动脉造影 Coronary angiogram
Henoch-Schonlein purpura
Alternative names:
?Anaphylactoid purpura
? Vascular purpura
Definition
Henoch-Schonlein purpura is a disease that
has the symptoms
? purple spots on the skin
? joint pain
? gastrointestinal symptoms
? glomerulonephritis
? (a type of kidney disorder)
History story
? Schonlein's Purpura
( In 1837,Johan Schonlein )
the skin and joints are
affected
but the gastrointestinal
tract is not
? Henoch's Purpura
(Later,his student,Eduard
Henoch )
purpura spots on the skin
and acute abdominal
problems
not affected by joint
disease
General conditions
? a type of hypersensitivity vasculitis and
inflammatory response within the blood vessel
? It is caused by an abnormal response of the
immune system
? The exact cause for this disorder is unknown.
? The syndrome is usually seen in children,but
people of any age may be affected
? It is more common in boys than in girls
? Many people with Henoch-Schonlein purpura
had an upper respiratory illness in the previous
weeks
Causes and pathogenesis
? An inflammatory disease of the small
blood vessels (capillaries)
? usually a self-limited disease
? It is the most common form of childhood
vascular inflammation (vasculitis) and
results in inflammatory changes in small
blood vessels
Common allergens include
? Medications
(such as certain antibiotics,antihistamines
and thiazide diuretics )
? Pathogens
( bacterium/virus/parasite,such as group A
strep,varicella,parvovirus and hepatitis B )
? An acute respiratory infection
Common allergens include
? foods ( such as berries,shellfish,fish,
nuts,eggs,milk,and others )
? pollen
? animal dander ( scales of shed skin )
? insect bites
? Certain vaccinations,such as for measles,
yellow fever and cholera
Pathophysiology
? a small-vessel vasculitis characterized by
immunoglobulin A (IgA),C3,and immune
complex deposition in arterioles,
capillaries,and venules
? HSP and IgA nephropathy are related disorders,
Both illnesses have elevated serum IgA levels
and identical findings on renal biopsy
Pathophysiology
? Lower Leg Direct
immunofluoresence
(DIF) showed
deposits of IgA about
dermal vessels.
Renal biopsy showed deposits of IgA in the mensangium
Frequency:
? In the US,Approximately 14 cases occur per 100,000
school-aged children,HSP also occurs in adults,although
less commonly than in children.
Mortality/Morbidity:
In general,HSP is a benign self-limited disorder.
? Fewer than 5% of cases cause chronic symptoms.
? Fewer than 1% of cases progress to end-stage renal
failure.
Sex,
Male-to-female ratio is about 2:1.
Age:
75% of patients affected are aged 2-11 years,In some series,
as many as 27% of the patients are adults.
Manifestations
? History,The most common symptoms of HSP
include the following,
? Rash (95-100%),especially involving the legs,
may not be present on initial presentation
? Subcutaneous edema (20-50%)
? Abdominal pain and vomiting (85%)
? Joint pain (60-80%),especially involving knees
and ankles
? Scrotal edema (2-35%)
? Bloody stools ("hematochezia","melena" )
Manifestations
Physical:
? Palpable purpura,particularly on the buttocks
and legs
? Edema of the hands,feet,scalp,and ears
? Arthritis,most commonly involving the knees
and ankles
? Abdominal tenderness
? Gastrointestinal bleeding
? Acute scrotal edema that may mimic testicular
torsion
With
Angioneurotic edema
Swelling – eyes,lip,hand
On the buttocks
臀部
in rare cases
? the brain and spinal cord (central
nervous system) involved
Arthritis,the knee
Lab Studies
? Routine lab test results are usually within
reference ranges.
? Some lab studies help in excluding other
diagnoses and in evaluating renal function:
urinalysis / CBC with platelet count and
differential / BUN,creatinine / prothrombin
time (PT),activated partial thromboplastin
time (aPTT) / lipase
Lab Studies
? Hematuria and/or proteinuria are present in 10-
20% of patients
? Platelet count is usually in the reference range but
may be elevated
? A normal lipase makes acute pancreatitis very
unlikely
? WBC count may be in the reference range or
elevated,Eosinophilia is sometimes present
? ESR in the reference range or elevated
? BUN and creatinine levels may be elevated from
renal involvement of HSP or from dehydration
Imaging Studies:
? Abdominal ultrasound may be better than
barium enema to diagnose intussusception,
since HSP-related intussusception is more often
ileoileal instead of ileocolic as is typical in
idiopathic intussusception.
? Doppler or radionuclide testicular scan results
show normal or increased blood flow in HSP,in
contrast to the decreased blood flow seen in
testicular torsion.
Diagnosis and differentiation
Diagnosis is clinical and not based on
laboratory evaluation
? A physical examination reveals skin
lesions and joint tenderness
? A urinalysis shows microscopic blood in
the urine
? A skin biopsy shows vasculitis
Diagnosis and differentiation
? A normal platelet count rules out
idiopathic thrombocytopenic purpura
(ITP)
? A normal platelet count and normal
coagulation studies (ie,PT,aPTT,fibrin
split products) rule out thrombotic
thrombocytopenic purpura (TTP)
Other Problems to be Considered
? Essential mixed cryoglobulinemia
? Waldenstr?m macroglobulinemia
? Systemic lupus erythematosus
? Rickettsial diseases
? Bowel infarction/perforation
Treatment
? Anti-inflammatory medications,such as aspirin
or ibuprofen,to relieve joint pain and swelling
? Stopping medication that may be the
underlying cause
? Corticosteroids
If symptoms persist,therapy with corticosteroids
such as prednisone,tried to treat abdominal pain,
subcutaneous edema,and nephritis
Treatment
Patients with severe abdominal pain,
significant gastrointestinal bleeding,
or marked renal insufficiency may
require hospitalization.
Treatment in HSPN (HSP nephritis)
many different modalities in severe cases
IV or oral steroids with or without:
? Azathioprine （ 硫唑嘌呤 ）
? Cyclophosphamide （ CTX）
? Cyclosporine （ 环孢素 A）
? Dipyridamole (潘生丁 )
? Plasmapheresis （ 血浆置换 ）
? high-dose IV immunoglobulin G (IVIg)
Prognosis
? HSP is generally a benign disease with an
excellent prognosis
? More than 80% of patients have a single isolated
episode lasting a few weeks
? Approximately 10-20% of patients have
recurrences
? Fewer than 5% of patients develop chronic HSP
? Abdominal pain resolves spontaneously within
72 hours in most patients
General conditions
? A chronic systemic disease
? Primarily of the joints
? Marked by inflammatory changes in the
synovial membranes（ 滑膜） and articular
structures
? Widespread fibrinoid degeneration of the
collagen fibers in mesenchymal tissues,and by
atrophy and rarefaction of bony structures
（ 纤维化和骨质疏松）
? Etiology is unknown
? Autoimmune mechanisms have been implicated
General conditions
? Arthritis can be short-term - lasting for
just a few weeks or months,then going
away forever
? or it can be chronic and last for months
or years,In rare cases,it can last a
lifetime
Causes and pathogenesis
? Causes unknown exactly
? Research indicates that it is an autoimmune
disease
? White blood cells lose the ability to tell the
difference between the body's own healthy cells
and harmful invaders like bacteria and viruses
? The immune system,which is supposed to
protect the body from these harmful invaders,
instead releases chemicals that can damage
healthy tissues and cause inflammation and pain
Clinical manifestations
Signs and Symptoms
? The first signs of arthritis can be subtle or
obvious,
? Signs may include limping (跛行 ) or a sore wrist,
finger,or knee
? Joints may suddenly swell and remain enlarged,
? Stiffness in the neck,hips,or other joints
? Rashes may suddenly appear and disappear
developing in 1 area and then another,
? High fevers that tend to spike in the evenings
and suddenly disappear are characteristic of
systemic juvenile rheumatoid arthritis
High fevers in
Systemic JRA
Clinical manifestations
? Typically appears between the ages of 6 months
and 16 years
? Not a single disease,but a group of diseases
? What they all have in common is chronic joint
inflammation,the first signs are often joint pain
or swelling and reddened or warm joints
? The diseases are very different in their
symptoms,their treatments,and their outcomes
? There are 3 major types of juvenile rheumatoid
arthritis (according to onset within the first 6
months)
Clinical manifestations
Polyarticular arthritis (多关节型 )
affects more girls than boys
Symptoms include swelling or pain in 5 or more
joints
The small joints of the hands are affected as well
as the weight-bearing joints such as the knees,
hips,ankles,feet,and neck
In addition,a low-grade fever may appear,as well
as bumps or nodules on the body on areas
subjected to pressure from sitting or leaning
swelling of small joints
of the hands
a lot of stiffness when they first wake up in
the morning,Once their joints warm up,
they can usually move more easily,
Clinical manifestations
Pauciarticular JRA (少关节型 )
? affects 4 or fewer joints
? Symptoms include pain,stiffness,or swelling in
the joints
? The knee and wrist joints are the most
commonly affected
? iridocyclitis or iritis or uveitis may occur with
or without active joint symptoms
( can be detected early by an ophthalmologist )
Clinical manifestations
Systemic JRA （全身型）
? affects the whole body
? Symptoms include high fevers that often increase in the
evenings and then may suddenly drop to normal
? During the onset of fever,the child may feel very ill,
appear pale,or develop a rash
? The rash may suddenly disappear and then quickly
appear again
? The spleen and lymph nodes may also become enlarged
? Eventually many of the body's joints are affected by
swelling,pain,and stiffness
Lab Studies
? Abnormalities in the numbers and
appearances of CBC can be useful in the
diagnosis of many medical conditions
? A blood culture may be taken to rule out
infections
? Bone marrow examination is a test to
rule out conditions such as leukemia
Lab Studies
? Erythrocyte sedimentation rate often increases
in people when inflammation is occurring in
the body
? Rheumatoid factor（ RA 类风湿因子） is an
antibody,common in rheumatoid arthritis
o Rare in persons with systemic JRA
o a marker for persistence of polyarticular
JRA into adulthood
Lab Studies
? ANA (antinuclear antibody) is a test performed
on the blood to detect autoimmunity,It is also
useful in predicting which children are likely to
have eye disease with JRA
? Test for certain viral infections such as Lyme
disease that may cause similar symptoms or
occur along with the arthritis.
Imaging Studies
Radiography of affected joints
? When only a single joint is affected,
radiography is important to exclude other
diseases,such as osteomyelitis 骨髓炎 or
septic arthritis
? Bone scanning,When physical findings do
not document definite arthritis,consider
bone scanning as a means of identifying a
potential focus of osteomyelitis or other
abnormality
Imaging Studies
o MRI is helpful when considering trauma
in the differential diagnosis
o CT scanning of long bones,Perform
when considering osteoid osteoma in a
child with lower extremity pain (often at
night) and unremarkable findings on
physical examination.
Imaging Studies
? Echocardiography
o This is performed in a child with possible
systemic JRA and with fevers.
o In a person who has nonspecific rash,
adenopathy,and possible mucocutaneous
changes,to exclude coronary arterial dilation
resulting from (possibly atypical) Kawasaki
disease,
? In an individual who has findings suggestive of
SLE (eg,nephritis,pleuritic chest pain,
thrombocytopenia)
Others
Synovial biopsy
? This procedure may be helpful to exclude
other diagnoses
? particularly when the knee is affected (eg,
villonodular synovitis 绒毛膜结节样滑膜炎
granulomatous arthritis)
Diagnosis and differentiation
Diagnosis
? To effectively manage and minimize the effects
of arthritis,an early and accurate diagnosis is
essential
? A detailed physical examination is a critical
tool in diagnosing JRA
? Physical findings are important to detect
abnormalities suggestive of other possible
diagnoses
Diagnosis and differentiation
? The diagnosis of JRA is based on the physical
finding of arthritis (or synovitis) in at least one
joint that persists for at least 6 weeks
? With other causes being excluded and with
onset when the individual is younger than 16
years
? Arthritis on examination is defined as either
joint swelling or the combination of limited
motion with pain (on motion or to palpation)
Diagnosis and differentiation
DIFFERENTIALS
? Acute Lymphoblastic Leukemia
? Autoimmune Chronic Active Hepatitis
? Crohn Disease
? Endocarditis,Bacterial
? Fever in the Toddler
? Kawasaki Disease
? Systemic Lupus Erythematosus
Treatment
The goals of treatment:
? To relieve pain and inflammation
? To slow down or prevent the destruction of joints
? To restore use and function of the joints to
promote optimal growth,physical activity,and
social and emotional development
Combination of medication,physical therapy,and
exercise
(physician,rheumatologist,and physical therapist)
Treatment
? Initial treatment limited to the use of salicylates
? then other nonsteroidal anti-inflammatory drugs
(NSAIDs) resulted in many patients becoming
wheelchair bound
? Other patients underwent synovectomies to
remove excess tissue resulting from uncontrolled
arthritis
? The addition of second-line drugs,starting with
gold salt injections and then replaced by the
much more effective methotrexate
(MTX),improved the outlook for these patients
Treatment
? In specific situations,injection of
corticosteroids into the joint
? The approval of the biologic antagonist to
tumor necrosis factor (TNF) ---- a new
era of treatment more closely tailored to
the pathophysiology of the disease
Treatment
Physical Therapy
? An appropriate physical therapy
program is essential in the management
of any type of arthritis
? To recommend range-of-motion exercises
to restore flexibility in stiff,sore joints
and other exercises to help build strength
and endurance
Treatment
Regular Exercise
? When pain strikes,it's natural to want to sit
still
? It's important to maintain a regular exercise
program,especially for those with arthritis
? Muscles must be kept strong and healthy so
they can help support and protect joints
? Regular exercise also helps to maintain range
of motion of joints
?Patients with JRA may have a gradually
responsive disease (particularly those with
pauciarticular JRA)
?A small number of patients with pauciarticular
JRA develop aggressive arthritis confined to a
single joint,requiring more intensive medical
treatment and physical therapy.
?Some patients with polyarticular JRA
demonstrate rapid response to treatment; however,
most have prolonged courses,requiring frequent
adjustments in medical and nonmedical therapy
Treatment
