Longterm outcomes and damage accrual in patients with childhood systemic lupus erythematosus with psychosis and severe cognitive dysfunction.

OBJECTIVE: (1) To describe the clinical course and response to treatment; and (2) to evaluate and compare damage accrual of distinct phenotypic subgroups of patients with clinically important psychiatric illness of pediatric systemic lupus erythematosus (pSLE). METHODS: A single-center cohort study of patients with pSLE followed at a pediatric lupus clinic from 1985 to July 2009. Clinical course and response to treatment were studied. Remission was defined by absence of psychiatric/cognitive symptoms while receiving minimal doses of prednisone. Disease activity and damage were measured using SLE Disease Activity Index and SLE Damage Index. RESULTS: Fifty-three children were included: 40 with psychosis and cognitive dysfunction (PSYC group) and 13 with isolated cognitive dysfunction (COG group). All received immunosuppressive treatment. Eighteen of 32 treated with azathioprine required a change to cyclophosphamide for poor response but none on cyclophosphamide required a change. The median times to remission were 72 weeks (PSYC) and 70 weeks (COG). Eight patients (7 PSYC, 1 COG) experienced flare following response/remission. New damage was noted in 50% of children at a median of 11 months: 57% of PSYC group, 31% of COG group. Persistent cognitive dysfunction was seen in 16% of PSYC patients and 15% of COG patients. CONCLUSION: Most patients responded to immunosuppressive treatment, although median time to remission was > 1 year. Roughly half the patients acquired a new damage item, most of which did not interfere with functional abilities. Fewer than 20% of patients developed neuropsychiatric damage. Both phenotypes of psychiatric pSLE responded equally well to current treatment.

Psychiatric illness of systemic lupus erythematosus in childhood: spectrum of clinically important manifestations.

OBJECTIVE: To determine the spectrum of manifestations in clinically important (i.e., requiring alterations of immunosuppressive therapy) psychiatric illness of pediatric systemic lupus erythematosus (pSLE) and to describe the laboratory and imaging features associated with psychiatric illness of pSLE (psySLE). METHODS: This was a single-center cohort study of patients with pSLE followed at a pediatric SLE clinic from August 1985 to July 2009. Patients with organic psychiatric disease due to SLE were included. Data regarding psychiatric features at initial presentation and during followup were obtained from psychiatry and rheumatology visits. Data regarding concomitant SLE disease activity and laboratory results were obtained from the institutional SLE database. Information from imaging studies was abstracted from patients' charts. RESULTS: Our cohort consisted of 53 pediatric patients (87% female) diagnosed with psySLE, representing 12% of the total pSLE cohort of 447 in the same time period. The median age at diagnosis of pSLE was 15.0 years and 16.1 years for psySLE. All patients reported symptoms of cognitive dysfunction and 75% of patients had additional psychotic features. Insight was preserved in 64% of patients with psychosis at diagnosis of psySLE. Visual distortion was observed among 32% of children with psySLE. Eighty-two percent of patients demonstrated clinical response to the institutional protocol of immunosuppression. CONCLUSION: Cognitive dysfunction was present in all and additional psychosis present in 75% of pediatric patients with psySLE. Visual distortion and early preservation of insight were unique features of psychosis observed in this cohort of children/adolescents with psySLE.

Feasibility and effectiveness of an aerobic exercise program in children with fibromyalgia: results of a randomized controlled pilot trial.

OBJECTIVE: To determine the feasibility of conducting a randomized controlled trial of a 12-week exercise intervention in children with fibromyalgia (FM) and to explore the effectiveness of aerobic exercise on physical fitness, function, pain, FM symptoms, and quality of life (QOL). METHODS: FM patients ages 8-18 years were randomized to a 12-week exercise intervention of either aerobics or qigong. Both groups participated in 3 weekly training sessions. Program adherence and safety were monitored at each session. Data were collected at 3 testing sessions, 2 prior to and 1 after the intervention, and included FM symptoms, function, pain, QOL, and fitness measures. RESULTS: Thirty patients participated in the trial. Twenty-four patients completed the program; 4 patients dropped out prior to training and 2 dropped out of the aerobics program. Better adherence was reported in the aerobics group than in the qigong group (67% versus 61%). Significant improvements in physical function, functional capacity, QOL, and fatigue were observed in the aerobics group. Anaerobic function, tender point count, pain, and symptom severity improved similarly in both groups. CONCLUSION: It is feasible to conduct an exercise intervention trial in children with FM. Children with FM tolerate moderate-intensity exercise without exacerbation of their disease. Significant improvements in physical function, FM symptoms, QOL, and pain were demonstrated in both exercise groups; the aerobics group performed better in several measures compared with the qigong group. Future studies may need larger sample sizes to confirm clinical improvement and to detect differences in fitness in childhood FM.