[Reiter's syndrome in children: a clinical analysis of 22 cases].

OBJECTIVE: To study the clinical features of Reiter's syndrome (RS) in children. METHOD: Twenty-two patients with RS were referred to our department between August 2002 and September 2008. Their clinical features were analyzed retrospectively. RESULT: Of the 22 patients, 19 were male, only 3 were female. Age ranged from 4 to 14 years, and the average was 10.7 years, most patients (20/22) were older than eight. Among their relatives, 2 had ankylosing spondylitis, 4 had undifferentiated spondyloarthropathy or presented with a history of inflammatory low back pain, and 2 had psoriasis. The season of onset of all patients was summer months from June to September every year. Ten had a history of diarrhea within 1 month preceding the symptoms of arthritis. Twenty-one had fever at the onset. Conjunctivitis occurred in 20 patients, only one was complicated with uveitis. Urethral symptoms occurred in 12 patients, and another 3 patients had abnormal results of urine analysis only. Synovitis occurred in all cases, most of whom had oligoarthritis, predominantly affecting large joints of the lower limbs in an asymmetric pattern with enthesitis occurred in 9. Balanitis circinata was common in male patients (10/19). Elevated inflammatory indicators such as white blood cell, neutrophil, platelet, erythrocyte sedimentation rate, C-reactive protein, immunoglobulins and serum complement C3 were common during the acute illness. All of the 22 cases were negative for rheumatoid factor and 16 (72.7%) were HLA-B27 positive. Nonsteroidal anti-inflammatory drugs and sulfasalazine were the mainstay of treatment. Cyclophosphamide was used in 14 patients (total doses 0.6 - 2.0 g), in 4 cases methotrexate was added. Corticosteroids were added in 4 patients and cyclosporine was given to the patient complicated with uveitis. Most patients achieved full remission within 6 months. CONCLUSION: RS is common in children with clinical features different from those in adults and a relatively good prognosis.

[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.