Clinical efficacy of mycophenolate mofetil in the treatment of systemic-onset juvenile idiopathic arthritis / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To evaluate the clinical efficacy of mycophenolate mofetil (MMF) in the treatment of systemic-onset juvenile idiopathic arthritis (SoJIA).</p><p><b>METHODS</b>Thirty-five patients with a confirmed diagnosis of SoJIA who had received initial treatment were randomly divided into control (n=15), MMF1 (n=7) and MMF2 groups (n=13). The control group received conventional treatment, the MMF1 group received MMF after 2 weeks of conventional treatment that had not led to remission, and the MMF2 group received combination therapy with non-steroidal anti-inflammatory drugs, prednisone and MMF. Symptoms, signs, laboratory indices, and adverse events were observed after 2, 4, and 12 weeks of treatment, and follow-up was performed for 3-6 months.</p><p><b>RESULTS</b>Before treatment, the MMF2 group had a significantly longer disease course than the control group (P<0.05). After 2 weeks of treatment, the MMF1 and MMF2 groups had a significantly lower prednisone dose and erythrocyte sedimentation rate (ESR) than the control group (P<0.05). The MMF1 group had significantly higher body temperature than the other two groups (P<0.05). After 4 weeks of treatment, the MMF1 group had a significantly lower prednisone dose and ESR than the control group (P<0.05). The MMF2 group had a significantly lower prednisone dose, body temperature (recovery to normal), white blood cell count, ESR and serum ferritin concentration than the control group (P<0.05). Body temperature was significantly lower in the MMF2 group than in the MMF1 group (P<0.05). No adverse events were observed in either the MMF1 or MMF2 groups during treatment.</p><p><b>CONCLUSIONS</b>Combination therapy with MMF can lead to better control of the patient's condition, more rapid relief of clinical symptoms and reduced glucocorticoid dose. The therapy with MMF is safe in children.</p>

Efficacy of thalidomide for treatment of juvenile idiopathic arthritis / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To evaluate the efficacy of thalidomide in the treatment of juvenile idiopathic arthritis (JIA).</p><p><b>METHODS</b>Twelve children with JIA who did not respond to conventional treatment were administered with thalidomide (2 mg/kg daily). The symptoms, signs, and laboratory test results were compared before and after treatment. The thalidomide-related side effects were observed.</p><p><b>RESULTS</b>The average dosage of prednisone was reduced from 1.92 ± 0.16 mg/kg•d to 0.49 ± 0.42 mg/kg•d in the 12 patients 6 months after thalidomide treatment (P<0.01). Four patients did not need prednisone treatment any more. White blood cell count, erythrocyte sedimentation rate (ESR), C reactive protein (CRP) and serum ferritin (SF) significantly decreased after treatment in all of 12 patients (P<0.01). Hemoglobin level increased to normal in 8 patients after treatment (P<0.01). The number of affected joints decreased from 5 before treatment to zero to 2 after treatment in patients with polyarticular JIA (P<0.01). Signs of hip involvement and Schober's sign turned negative in enthesitis-related cases. No thalidomide-related side effects were observed.</p><p><b>CONCLUSIONS</b>Thalidomide is effective in the treatment of JIA in children who do not respond to conventional treatment.</p>

Macrophage activation syndrome in Chinese children with systemic onset juvenile idiopathic arthritis / 中华儿科杂志

<p><b>OBJECTIVE</b>To review and analyze the clinical features, treatment, and outcome of macrophage activation syndrome (MAS) in children with systemic onset juvennil rheumatoid arthritis (SOJRA).</p><p><b>METHOD</b>Retrospective review and analysis were performed on cases with MAS from a prospectively collected database of children with SOJRA from the year of 2003 to 2006 in the Hospital.</p><p><b>RESULTS</b>Twenty four patients (21 boys, 3 girls) were diagnosed as having MAS with SOJRA. Mean age of the patients with MAS at diagnosis was 7 years, and the duration prior to diagnosis of MAS was 12 months. No trigger factors were found except in one case whose MAS was triggered by use of methotrexate and in another by parvovirus B19 infection. High grade fever, new onset hepatosplenomegaly and lymphadenopathy, pancytopenia, liver dysfunction were common clinical features in all the 24 cases (100%). Bleeding from skin, mucous membrane and gastrointestinal tract were noted in 9 cases (38%). Twelve (50%) cases had CNS dysfunction (high intracranial pressure, seizure and coma). Six cases (25%) developed ARDS. One patient suffered from renal damage. The laboratory test revealed elevated live enzymes and ferritin, decreased value of ESR, albumin, complete blood count and fibrinogen in all the 24 cases. Bone marrow examination supported the diagnosis of definite hemophagocytosis in the 24 cases. Lymph node biopsy was done for one case and histopathological examination showed that the node was full of activated macrophage. As to treatment, five cases only received high dose steroids (three of them died), 14 cases were treated with high dose steroids plus cyclosporine (one died), two were treated with steroids plus cyclosporine and etoposide (none died). The causes of deaths were ARDS and CNS involvement. In three of the cases who died, treatment was given up by their parents.</p><p><b>CONCLUSIONS</b>MAS is a rare and potentially fatal complication of SOJRA. Most of our patients were male. Bone marrow studies support the diagnosis. CNS involvement and ARDS were poor prognostic signs. Early diagnosis and aggressive therapy are essential.</p>