Juvenile rheumatoid arthritis (JRA), also known as Still's disease, refers to the general term for rheumatoid arthritis in children under the age of 16. Unlike adult rheumatoid arthritis, it presents with severe systemic symptoms in addition to joint involvement. The incidence rate in children is 1/1500, with higher occurrence around school age, and girls are more affected than boys.

bubble_chart Etiology

The cause of the disease is unclear, but there are several theories:

1. Autoimmune theory: Some children's serum contains specific antibodies against IgG, namely rheumatoid factors (RF), and a few have antinuclear antibodies (ANT). The total complement in joint synovial fluid is decreased.
2. Infection theory: Infections related to rheumatoid conditions, such as hemolytic streptococcus, mycoplasma, and rubella virus. However, no specific pathogenic microorganism has been identified so far.
3. Genetic theory: As a genetic marker, human leukocyte antigen (HLA), certain family antigen types such as HLA-B27 carriers have a higher incidence of JRA.

bubble_chart Pathological Changes

Its main features are chronic non-suppurative inflammation of the fibrous synovial membrane:

1. The synovial membrane is infiltrated with leukocytes and plasma cells, accompanied by edema, congestion, thickening of the synovial membrane, and proliferation of granulation tissue, which invades cartilage and joint surfaces, leading to adhesions and ultimately joint stiffness or deformity.
2. Subcutaneous nodules, with necrotic phenomena in the center of older lesions, differ from subcutaneous nodules caused by wind-dampness heat.
3. Other manifestations include iridocyclitis; conjunctivitis and keratitis; hepatosplenomegaly caused by amyloidosis; rare involvement of the heart, lungs, or pleura; and nonspecific inflammation characterized by mononuclear cell infiltration around small blood vessels.

bubble_chart Clinical Manifestations

Based on clinical features, it is roughly divided into three types: systemic type, polyarticular type, and oligoarticular type.

(1) Systemic type (Still's type)

Also known as acute fever type, it was previously referred to as subsepsis allergica or acute allergic subsepsis. It is characterized by fever, rash, hepatomegaly and/or splenomegaly, lymphadenopathy, arthritis, and possible involvement of the heart, blood vessels, and nervous system.

1. Fever is often intermittent and remittent, with body temperature ranging between 36–40°C. After the fever subsides, the child's mental state remains good, and activity is normal. It often recurs and persists for several weeks before resolving spontaneously but is prone to relapse.
2. At the onset, joint symptoms are often absent, but joint pain may appear after several weeks, not necessarily accompanied by swelling. It may involve single or multiple joints, with some children experiencing morning stiffness in the limbs. Pain usually correlates with fever, subsiding or disappearing as the fever abates.
3. About 95% of children develop a rash, which appears as light red macules and papules or papules, presenting as polymorphic eruptions. The rash is prone to recurrence and is distributed over the trunk, limbs, face, palms, and soles, varying in size. It typically correlates with fever, disappearing as the fever subsides.
4. A minority may develop pneumonia, pleuritis, pericarditis, or carditis.
5. Hepatosplenomegaly and lymphadenopathy may occur, with transient liver dysfunction (e.g., elevated GOT, GPT). Splenomegaly is more common than hepatomegaly.

This refers to involvement of more than four joints, more common in girls, about twice as frequent as in boys.

1. Systemic symptoms include fever rarely exceeding 39°C, fatigue, poor appetite, grade I anemia, and failure to gain weight.
2. Joint symptoms often first affect large joints such as the knees, ankles, elbows, and wrists, presenting as swelling and pain, usually symmetrical without redness or heat. Later, small joints (in children under 5 years old) may be involved, with fusiform swelling of the finger joints. The first, second, and third cervical vertebrae are frequently affected, often leading to restricted movement.

This involves no more than four joints, more common in girls, three times more frequent than in boys, and typically seen around ages 7–8.

1. Systemic symptoms include low-grade fever, rash, delayed growth, lymphadenopathy, and frequent iridocyclitis.
2. Joint symptoms also primarily affect large joints, commonly the knees, ankles, hips, sacrum, and elbows. Swelling and morning stiffness are common, with recurrent episodes that may become chronic or disabling after many years. Involvement of small joints is rare but often accompanied by tenosynovitis.

bubble_chart Auxiliary Examination

1. Blood Test (1) Mild or grade II anemia. (2) Increased erythrocyte sedimentation rate. (3) Elevated total white blood cell count, with neutrophils reaching up to 70%. A few cases may exhibit a leukemoid reaction, reaching 60–70×10⁹/L (60,000–70,000/mm3).
2. The presence of a positive rheumatoid factor (RF) can serve as a diagnostic basis, with a positivity rate of 10–30%. A few cases may yield false positives, and a negative result does not rule out rheumatoid disease.
3. Others (1) α2-globulin, common bletilla tuber IgA, IgM, and IgG may all be elevated. (2) Antinuclear antibodies may be positive. (3) ASO may be elevated in 30% of cases. (4) C-reactive protein is mostly positive, and mucoprotein (MPT) may also rise above 40mg/L. (5) Urinalysis is mostly normal, but a few patients with kidney involvement may show proteinuria (+), with red and white blood cells present.
4. X-ray Examination: Joint soft tissue swelling appears fusiform, with osteoporosis, narrowed joint space, and blurred articular surfaces. In the advanced stage, bone destruction, fusion, fibrosis, deformity, and joint ankylosis may occur.

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bubble_chart Diagnosis

The diagnosis of this disease is primarily based on clinical manifestations. The main symptoms include chronic joint inflammation (lasting 6 weeks), spindle-shaped joint swelling, and the presence of one of the following symptoms: iridocyclitis, skin rash, recurrent remittent fever, morning stiffness, positive RF, or radiographic changes. These findings are sufficient for diagnosis.

bubble_chart Treatment Measures

The disease manifests differently depending on the type, with varying symptoms and durations, and complex pathological changes. Treatment should be tailored to the specific stage. Although there is no specific cure, the prognosis is generally better than in adults.

1. General treatment: Bed rest, symptomatic treatment, enhanced nutrition, maintaining joint function, and preventing deformities.
2. Drug therapy
   1. Immunosuppressants
   2. Hormones: For systemic cases with high fever and visceral involvement, prednisone 1–2 mg/kg·d is commonly used. After symptoms are controlled, switch to alternate-day therapy and gradually reduce the dosage. The course lasts 3–6 weeks or longer to prevent relapse.
   3. Cyclophosphamide 2 mg/kg·d, chlorambucil 0.2 mg/kg·d, 6-MP, etc. Monitor blood counts; discontinue promptly if leukopenia occurs. Reduce the dosage after symptoms are controlled, and continue for 3–4 months.
   4. Salicylates are often the first choice: Aspirin 80–120 mg/kg·d, divided into four doses. Reduce the dosage after 1–2 weeks of symptom relief and maintain for several months.
   5. Other options like flufenamic acid, clofenamic acid, phenylbutazone, indomethacin, piroxicam, tolmetin, naproxen, ibuprofen, and D-penicillamine show varying efficacy in symptom control. Zinc sulfate occasionally helps, while Chinese medicinals such as Root, Leaf, or Flower of Common Threewingnut have notable effects.
3. Physical therapy: After joint symptoms are controlled, perform passive and active joint exercises, infrared irradiation, and hot compresses to promote functional recovery and reduce sequelae.