幼年性类风湿性关节炎 (JRA)
Juvenile Rheumatoid Arthritis
儿科医院 邬惊雷
是一种少年儿童常见的结缔组织病
One of the most common connective tissue diseases of
children
以慢性关节炎 (滑膜炎 )为主要特点
Characterized by chronic arthritis (synovitis of the joints)
多脏器受累，如肝脾肿大、淋巴结肿大、浆膜
炎 (心包积液 )等
Visceral involvement,such as hepatosplenomegaly,lym-
phadenopathy and serositis (pericardial effusion)
分类 Classification
全身型
systemic-onset disease
多关节型,,5个或以上关节受累
Polyarthritis:5 or more inflamed joints
少关节型,5个以下关节受累
Oligoarthritis,< 5 inflamed joints
流行病学 Epidemiology
发病率约 113/10万
Overall prevalence approximately 113/100,000
约占住院 0.1%-0.15%
Accounts for about 0.1%-0.15% hospitalizations
女性发病较多
Females affected more frequently than males
病因 Etiology
病因尚不清楚,可能与下列因素有关,
Etiology is unknown,maybe related to the follows:
免疫遗传易感性
Immunogenetic suseptibility
外源性触发因素 (病毒感染）
External triggers （ virus infection)
易感儿童在感染（环境因素）作用下出现免疫
异常的结果
Exaggerated immune reactivity in predisposed
children in response to exposure to certain
infections (environmental factors)
病理变化 Pathologic changes
关节：绒毛状增厚为特征的滑膜炎
Synovitis characterized by villous hypertrophy
滑膜下组织充血、水肿
Hyperemia and edema of the subsynovial tissues
血管翳形成
Pannus formation
关节软骨和邻近骨组织的破坏
Erosion of articular cartilage and contiguous bone
皮下结节,Subcutaneous (Rheumatoid) nodules
见于肘关节伸侧面
Found on extensor surfaces of elbows
眼部病变
虹膜睫状体炎，尤见于少关节型病例
Iridocyclitis,mainly in the cases of
oligoarthritis
临床表现 Clinical manifestations
1.全身型,占 10％ -20％ JRA病例
Systemic-onset disease,10％ -20％ cases
发热：呈弛张热型是此型特点，体温高峰达 40oC,
热退时一般情况好
Fever,Characterized by a remmitent fever with
temperature spikes to 40oC,Present well when
fever going down
肛
门
体
温
oC
3 岁 JRA女孩：
－发烧多天
－经反复应用退烧药无效
皮疹：淡红色斑丘疹，以躯干和肢体近端为多
热高时明显
Rash,Erythematous,macular rash,distributed
most commonly over the trunk and proximal
extremities,Becoming conspicuous on each
febrile episode
肝脾、淋巴结肿大，可伴轻度肝功能异常
Hepatosplenomegaly and lymphadenopathy,
may be accompanied by mild abnormality of
liver function.
胸膜炎及心包炎（心包积液 )
Pleuritis and pericarditis(pericardial effusion)
3 岁女孩患 JRA：
胸部 X 线检查所见为：
－心脏明显增大，尤其是左侧，右侧稍增大
－心腰几乎消失
－左侧肺充血
3 岁女孩患 JRA：
胸部 X 线检查（病程随访）所见为：
－心脏仍增大
－右侧胸腔积液 8 mm 宽（箭头）
－疑及左侧心角积液
3 岁女孩患全身性 JRA：
超声波检查（显示肋下）所见为：
－环形心包积液，宽 10 mm （ 箭头）
2,多关节炎类型, Polyarthritis
约占 30％－ 40％ JRA病例
Accounts for 30％－ 40％ cases of JRA
大、小 关节均 受累，肿痛、活动受限
Both large and small joints involved,pain and
swelling,lack of full range of motion
晨僵是本型特点
Characterized by morning stiffness
9 岁女孩患 JRA：
所见为双外踝上方呈球形波动性肿胀
此型类风湿因子 (RF)阳性者
Positive rheumatoid factor in patients
with polyarticilitis may
多见于年长儿
be often associated with older children
常有类风湿皮下结节
have rheumatoid nodules
预后差,(关节 )功能受损
have poor prognosis in association with functional
disability (of joints )
3.少关节型 Oligoarthritis
约占 JRA的 30％ -50%
Accounts for 30％－ 50％ cases of JRA
下肢大关节受累为主 (膝、踝 )
Predominantly affects the joints of the lower
extremities (such as the knees and ankles)
可并发虹膜睫状体炎而致视力障碍
May be complicated with iridocyclitis causing
visual disturbance
9 岁女孩患 JRA:
所见为右膝关节肿胀
关于强直性脊柱炎 about ankylosing spondylitis
男孩，起病年龄 >8岁
Male,age at onset >8 yr
关节病变限于下肢大关节
Joints involvement limited to the large joints
in the lower extremilities
HLA-B27 阳性及阳性家族史
Positive HLA-B27 and family history
可有自限性急性虹膜睫状体炎
May have self-limited acute iridocyclitis
实验室检查 Laboratory findings(1)
无特异的实验室诊断指标
No specific diagnostic tests
轻、中度贫血，白细胞计数升高
Mild to moderate anemia,elevated white blood cell count
血沉加快,C-反应蛋白增高
Elevated ESR and C-reactive protein concentration
血培养阴性
Negative blood culture
实验室检查 Laboratory findings(2)
免疫球蛋白增高，细胞因子增多
Elevated serum immunoglobulin and cytokines
RF 阳性率不如成人高
Positive rate of RF not as high as that of adults
X线检查：心影增大、胸腔积液
X-ray,cardiomegaly and pleural effusion
实验室检查 Laboratory findings(3)
骨质疏松、破坏
Osteoporosis and erosions of the bone
关节间隙变窄
Narrowing of the joint space (cartilage space)
关节半脱位
Subluxcation
诊断标准 Criteria for diagnosis
1.发病年龄 16岁以下
Age at onset <16yr
2.1个或多个关节炎 (关节肿胀或积液，并具备下
列两种以上体征, 关节活动受限、活动时疼痛
或触痛及关节局部温度升高
Arthritis in one or more joints (swelling or effusion,or pre-
sence of two or more of the following signs,limitation of
range of motion,tenderness or pain on motion,and increased
heat)
3,病程 6周以上
Duration of disease 6 wk or longer
4,根据起病初 6个月的临床表现确定临床类型
Onset type defined by type of disease in first 6 mo
a,多关节型：受累关节 5个或以上
Polyarthritis,5 or more inflamed joints
b,少关节型：受累关节不超过 4个
Oligoarthritis,<< 4 inflamed joints
c,全身型：关节炎伴特征性发热、皮疹等
Systemic,arthritis with characteristic fever,rash.
5,除外其它疾病 Exclusion of other diseases
鉴别诊断 Differential diagnosis
败血症 Septicemia
风湿热 Rheumatic fever
川崎病 Kawasaki disease
白血病 /淋巴瘤 Leukemia/Lymphoma
系统性红斑狼疮 SLE
结核性 /化脓性关节炎
Suppurative/Tuberculous arthritis
治疗 Treatment
主要原则：控制临床症状
To control symptoms
维持关节功能
To maintain function of joint
预防关节畸形
To prevent joint deformity
一,药物治疗
1.非甾体类抗炎药物
Nonsteroidal anti-inflammatory drugs (NSAIDs)
萘普生 (Naproxen)：每天 10-15mg/kg
布洛芬 (Ibuprofen)：每天 30-40mg/kg
消炎痛 (Indomethacin)：每天 0.5～ 1mg
扶他林 (Sodium Diclofenac)：每天 2～ 3mg/kg
此类药物不能合用
2,病情缓解药
羟氯喹 (Hydroxychloroquine)
青霉胺 (Penicillamine)
柳氮磺胺吡啶 （ Sulfasalazine)
甲氨喋呤 （ Methotrexate,MTX)
3,免疫抑制剂 Immunosuppressive drugs
环磷酰胺 ( Cyclophosphamide,CTX)
硫唑嘌呤 ( Azathioprine)
皮质激素 ( Corticosteroids)
金字塔方案 Pyramid therapeutic approach
NASIDs
羟氯喹、柳氮磺胺吡啶
甲氨喋呤 (MTX)
硫唑嘌呤
环磷酰胺 (CTX)
皮质激素
降阶方案 Step down bridge
使用于顽固病例、危及生命及关节并发症严重者
或激素撤减困难
先联合用药
NSAIDs+SARD(慢作用药物 )+MTX
NSAIDs+steroids 或 steroids+MTX
撤药顺序：激素,NSAIDs
SARD 和 MTX 可作为长期维持药
二, 理疗
三, 眼科治疗
Juvenile Rheumatoid Arthritis
儿科医院 邬惊雷
是一种少年儿童常见的结缔组织病
One of the most common connective tissue diseases of
children
以慢性关节炎 (滑膜炎 )为主要特点
Characterized by chronic arthritis (synovitis of the joints)
多脏器受累，如肝脾肿大、淋巴结肿大、浆膜
炎 (心包积液 )等
Visceral involvement,such as hepatosplenomegaly,lym-
phadenopathy and serositis (pericardial effusion)
分类 Classification
全身型
systemic-onset disease
多关节型,,5个或以上关节受累
Polyarthritis:5 or more inflamed joints
少关节型,5个以下关节受累
Oligoarthritis,< 5 inflamed joints
流行病学 Epidemiology
发病率约 113/10万
Overall prevalence approximately 113/100,000
约占住院 0.1%-0.15%
Accounts for about 0.1%-0.15% hospitalizations
女性发病较多
Females affected more frequently than males
病因 Etiology
病因尚不清楚,可能与下列因素有关,
Etiology is unknown,maybe related to the follows:
免疫遗传易感性
Immunogenetic suseptibility
外源性触发因素 (病毒感染）
External triggers （ virus infection)
易感儿童在感染（环境因素）作用下出现免疫
异常的结果
Exaggerated immune reactivity in predisposed
children in response to exposure to certain
infections (environmental factors)
病理变化 Pathologic changes
关节：绒毛状增厚为特征的滑膜炎
Synovitis characterized by villous hypertrophy
滑膜下组织充血、水肿
Hyperemia and edema of the subsynovial tissues
血管翳形成
Pannus formation
关节软骨和邻近骨组织的破坏
Erosion of articular cartilage and contiguous bone
皮下结节,Subcutaneous (Rheumatoid) nodules
见于肘关节伸侧面
Found on extensor surfaces of elbows
眼部病变
虹膜睫状体炎，尤见于少关节型病例
Iridocyclitis,mainly in the cases of
oligoarthritis
临床表现 Clinical manifestations
1.全身型,占 10％ -20％ JRA病例
Systemic-onset disease,10％ -20％ cases
发热：呈弛张热型是此型特点，体温高峰达 40oC,
热退时一般情况好
Fever,Characterized by a remmitent fever with
temperature spikes to 40oC,Present well when
fever going down
肛
门
体
温
oC
3 岁 JRA女孩：
－发烧多天
－经反复应用退烧药无效
皮疹：淡红色斑丘疹，以躯干和肢体近端为多
热高时明显
Rash,Erythematous,macular rash,distributed
most commonly over the trunk and proximal
extremities,Becoming conspicuous on each
febrile episode
肝脾、淋巴结肿大，可伴轻度肝功能异常
Hepatosplenomegaly and lymphadenopathy,
may be accompanied by mild abnormality of
liver function.
胸膜炎及心包炎（心包积液 )
Pleuritis and pericarditis(pericardial effusion)
3 岁女孩患 JRA：
胸部 X 线检查所见为：
－心脏明显增大，尤其是左侧，右侧稍增大
－心腰几乎消失
－左侧肺充血
3 岁女孩患 JRA：
胸部 X 线检查（病程随访）所见为：
－心脏仍增大
－右侧胸腔积液 8 mm 宽（箭头）
－疑及左侧心角积液
3 岁女孩患全身性 JRA：
超声波检查（显示肋下）所见为：
－环形心包积液，宽 10 mm （ 箭头）
2,多关节炎类型, Polyarthritis
约占 30％－ 40％ JRA病例
Accounts for 30％－ 40％ cases of JRA
大、小 关节均 受累，肿痛、活动受限
Both large and small joints involved,pain and
swelling,lack of full range of motion
晨僵是本型特点
Characterized by morning stiffness
9 岁女孩患 JRA：
所见为双外踝上方呈球形波动性肿胀
此型类风湿因子 (RF)阳性者
Positive rheumatoid factor in patients
with polyarticilitis may
多见于年长儿
be often associated with older children
常有类风湿皮下结节
have rheumatoid nodules
预后差,(关节 )功能受损
have poor prognosis in association with functional
disability (of joints )
3.少关节型 Oligoarthritis
约占 JRA的 30％ -50%
Accounts for 30％－ 50％ cases of JRA
下肢大关节受累为主 (膝、踝 )
Predominantly affects the joints of the lower
extremities (such as the knees and ankles)
可并发虹膜睫状体炎而致视力障碍
May be complicated with iridocyclitis causing
visual disturbance
9 岁女孩患 JRA:
所见为右膝关节肿胀
关于强直性脊柱炎 about ankylosing spondylitis
男孩，起病年龄 >8岁
Male,age at onset >8 yr
关节病变限于下肢大关节
Joints involvement limited to the large joints
in the lower extremilities
HLA-B27 阳性及阳性家族史
Positive HLA-B27 and family history
可有自限性急性虹膜睫状体炎
May have self-limited acute iridocyclitis
实验室检查 Laboratory findings(1)
无特异的实验室诊断指标
No specific diagnostic tests
轻、中度贫血，白细胞计数升高
Mild to moderate anemia,elevated white blood cell count
血沉加快,C-反应蛋白增高
Elevated ESR and C-reactive protein concentration
血培养阴性
Negative blood culture
实验室检查 Laboratory findings(2)
免疫球蛋白增高，细胞因子增多
Elevated serum immunoglobulin and cytokines
RF 阳性率不如成人高
Positive rate of RF not as high as that of adults
X线检查：心影增大、胸腔积液
X-ray,cardiomegaly and pleural effusion
实验室检查 Laboratory findings(3)
骨质疏松、破坏
Osteoporosis and erosions of the bone
关节间隙变窄
Narrowing of the joint space (cartilage space)
关节半脱位
Subluxcation
诊断标准 Criteria for diagnosis
1.发病年龄 16岁以下
Age at onset <16yr
2.1个或多个关节炎 (关节肿胀或积液，并具备下
列两种以上体征, 关节活动受限、活动时疼痛
或触痛及关节局部温度升高
Arthritis in one or more joints (swelling or effusion,or pre-
sence of two or more of the following signs,limitation of
range of motion,tenderness or pain on motion,and increased
heat)
3,病程 6周以上
Duration of disease 6 wk or longer
4,根据起病初 6个月的临床表现确定临床类型
Onset type defined by type of disease in first 6 mo
a,多关节型：受累关节 5个或以上
Polyarthritis,5 or more inflamed joints
b,少关节型：受累关节不超过 4个
Oligoarthritis,<< 4 inflamed joints
c,全身型：关节炎伴特征性发热、皮疹等
Systemic,arthritis with characteristic fever,rash.
5,除外其它疾病 Exclusion of other diseases
鉴别诊断 Differential diagnosis
败血症 Septicemia
风湿热 Rheumatic fever
川崎病 Kawasaki disease
白血病 /淋巴瘤 Leukemia/Lymphoma
系统性红斑狼疮 SLE
结核性 /化脓性关节炎
Suppurative/Tuberculous arthritis
治疗 Treatment
主要原则：控制临床症状
To control symptoms
维持关节功能
To maintain function of joint
预防关节畸形
To prevent joint deformity
一,药物治疗
1.非甾体类抗炎药物
Nonsteroidal anti-inflammatory drugs (NSAIDs)
萘普生 (Naproxen)：每天 10-15mg/kg
布洛芬 (Ibuprofen)：每天 30-40mg/kg
消炎痛 (Indomethacin)：每天 0.5～ 1mg
扶他林 (Sodium Diclofenac)：每天 2～ 3mg/kg
此类药物不能合用
2,病情缓解药
羟氯喹 (Hydroxychloroquine)
青霉胺 (Penicillamine)
柳氮磺胺吡啶 （ Sulfasalazine)
甲氨喋呤 （ Methotrexate,MTX)
3,免疫抑制剂 Immunosuppressive drugs
环磷酰胺 ( Cyclophosphamide,CTX)
硫唑嘌呤 ( Azathioprine)
皮质激素 ( Corticosteroids)
金字塔方案 Pyramid therapeutic approach
NASIDs
羟氯喹、柳氮磺胺吡啶
甲氨喋呤 (MTX)
硫唑嘌呤
环磷酰胺 (CTX)
皮质激素
降阶方案 Step down bridge
使用于顽固病例、危及生命及关节并发症严重者
或激素撤减困难
先联合用药
NSAIDs+SARD(慢作用药物 )+MTX
NSAIDs+steroids 或 steroids+MTX
撤药顺序：激素,NSAIDs
SARD 和 MTX 可作为长期维持药
二, 理疗
三, 眼科治疗
