[Macrophage activation syndrome in children with rheumatic disorders: a retrospective study on 6 patients].

OBJECTIVE: To study the clinical manifestations of rheumatic disorders with macrophage activation syndrome (MAS) in children. METHODS: The authors characterized MAS by carrying out a retrospective study on patients who were identified during the past 12 years in Tianjin Children's Hospital. RESULTS: Six cases (4 females, 2 males) were studied. Four had typical systemic onset juvenile idiopathic arthritis (SOJIA), two had systemic lupus erythematosus (SLE) with lupus nephritis. Clinical manifestations at diagnosis, which occurred in the lower activity state of these primary diseases, included high spiking fever (in 5 cases) or high fever (in 1), hepatosplenomegaly (in 6), lymphadenopathy (in 6), profound decrease of all 3 blood cell lines (in 6), significant injury of liver (in 6), diseminated intravascular coagulation (DIC)-like picture (in 2), and central nervous system dysfunction (in 3). Hypofibrinogenemia, elevated liver enzymes and hypertriglyceridemia were found consistently. The phagocytic histiocytes with plasmacytosis were found in 3 bone marrow smears (not done in others). MAS was presumed to have been precipitated by viral infections in 3 patients, two had evidences for herpes simplex virus infection and one for hepatitis A virus infection. The treatment regimen was tailored to each patient, as the clinical course was variable. CONCLUSIONS: MAS may not only be most frequently seen in children with SOJIA, but also in those with other rheumatic diseases, and may be a syndrome that is more common than previously thought. Infection may be main trigger factor for MAS. The immunoapheresis combined with immunochemotherapy may be optimal for severe injury of the liver in patients with MAS.

[Methylprednisolone and cyclophosphamide pulse therapy of severe systemic lupus erythematosus in children].

OBJECTIVE: To study the effect of methylprednisolone (MP) and cyclophosphamide (CPA) intermittent intravenous pulse therapy and the clinical prognosis in children with severe juvenile onset systemic lupus erythematosus (JOSLE). METHODS: Thirty patients with JOSLE, diagnosed by clinical, laboratory or renal histological examinations, were enrolled in this study. Of the 30 patients, 27 were females and 3 were males, the mean age was (12 +/- 3) years, and 20 of the 22 patients who had undergone initial therapy had LN, and the clinical courses before being involved in the study were 3 to 12 months in nine patients. Twenty-three of the 30 patients had clinical manifestations of renal damages, of whom 4 patients were proven by initial renal biopsy to have WHO type IV, 2 had type II,1 had type V and 1 had type III, and 7 patients had one or more manifestations of central nervous system, including chorea, seizures, cerebrovascular accident (CVA) and organic brain syndrome (OBS), simultaneously, 9 patients had nervous system symptoms without the clinical manifestations of renal damages, 3 patients had lupus crisis, 7 patients did not have any manifestations of renal or neurological damages. According to the protocol of the therapy, the patients were divided into 3 groups: group A (n = 18) patients were treated with MP plus CPA intermittent intravenous pulse for children with lupus nephritis, and with or without neuropsychiatric lupus erythematosus (NPLE), group B (n = 7) with pulsed doses of MP, followed by prednisone and tripterygium wilfordii hook f(T(whf)) for patients without renal or central nerves system damage, and group C (n = 5) with prednisone alone for patients with LN determined by clinical and laboratory features. The effects of those regimes and the clinical prognosis were observed. RESULTS: On short-term follow-up, the SLEDAI-2K (by weight of the renal damage) showed significant difference between group A and group B, but there was no significant difference at the 9th months of the therapy. The long-term follow-up lasted in average for (37.2 +/- 24.8) months. Nineteen patients were followed up for more than 18 months. At the end of follow-up, the mean age was 14 to 19 years. There was no difference on the effect of both group A and group B, and no frequent infections were seen, ANAs were negative and SLEDAI-2K = 0-point in two patients of each group 12 months after discontinuation of the therapy. Four patients in group C died within 18 months. CONCLUSION: The immunosuppressive regimen MP + CPA in patients with severe JOSLE and MP + prednisone + T(whf) in patients without major organs damage were superior to the regimen of prednisone alone.