Learn About An Autoimmune Disease That Affects More Than A Million People In The United States
Kids with Lupus Need Better Tx
Study finds that female, non-Caucasian patients with juvenile onset SLE are at greater risk for the disease.
By Nancy Walsh, MedPage Today
Medically Reviewed byZalman S Agus, MD
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MONDAY, July 2, 2012 (MedPage Today) —Major organ involvement can occur rapidly in juvenile-onset systemic lupus erythematosus (SLE) even with near universal corticosteroid use, data from a U.K. national cohort showed.
After only 4.5 years of follow-up, 91 percent of young lupus patients already had hematologic manifestations of the disease, 82 percent had musculoskeletal involvement, 80 percent had developed kidney problems, and 26 percent had neurologic abnormalities, according to Michael W. Beresford, MBChB, PhD, of the University of Liverpool, and colleagues.
A total of 93 percent had received oral prednisone, despite the potential for serious long-term adverse effects with this treatment.
"Alternative therapies to relieve the burden of corticosteroids in this population are urgently needed," the researchers wrote in the July issue ofArthritis & Rheumatism.
The overall prognosis in juvenile SLE has improved in recent decades, with 10-year survival now reaching 90 percent, but much less is known about the disease manifestations and its treatment compared with adult-onset lupus.
To more fully characterize these concerns, Beresford and colleagues analyzed data from a collaborative network that has enrolled children with the disease from across the U.K. since 2006.
Of the 198 children included in the study, 85 percent were female, with a median age at disease onset of 12.6 years.
Among the slightly more than half who were white, the standardized incidence rate was 0.1 per 100,000, while incidence rates were 2 and 2.5 per 100,000 for blacks and Asians.
A notable 38 percent of the children had a family history of autoimmune disease, including 16 percent with rheumatoid arthritis, 15 percent with lupus, and 13 percent with thyroid disease, suggesting a strong genetic influence, according to the researchers.
Among the hematologic manifestations were lymphopenia in 73 percent of patients, leukopenia in 32 percent, hemolytic anemia in 27 percent, and thrombocytopenia in 20 percent.
A total of 69 percent also had anti-double-stranded DNA antibodies and 36 percent had antiphospholipid antibodies.
To assess disease activity, the researchers used the pediatric version of the British Isles Lupus Assessment Group index as modified in 2004, which they found to be more sensitive than the American College of Rheumatology criteria.
For instance, the ACR criteria identified neurologic involvement in only 10 percent of the cohort, compared with 26 percent identified by pBILAG-2004.
Disease activity did respond to treatment, with mean scores falling from 8.5 to 2, but within less than 5 years disease damage had already begun to accrue.
Of particular concern was the neurologic damage, which was already present in 8 percent of children, and included psychosis, seizures, and cerebrovascular accidents.
Renal damage had developed in 4 percent, including one case of end-stage renal failure, vascular damage in 3 percent, and musculoskeletal changes in 6 percent.
The severe scarring alopecia afflicting 10 percent of the patients also posed particular problems, "and may have devastating effects on patients who are already adapting to taking medications, attending hospital, trying to gain peer acceptance, and trying to attain educational qualifications," the researchers wrote.
An important difference they found in this young cohort compared with adults was that the damage was primarily caused by the disease itself, whereas in patients with later-onset disease, the damage tends to be associated with steroid use.
Approximately half of the patients were diagnosed as teenagers, "a critical time for adolescent growth, puberty, education, and emotional maturation," Beresford and colleagues observed.
"This emphasizes the importance of access to specialist, multidisciplinary pediatric and adolescent holistic care for patients with juvenile SLE," they stated.
They also stressed the need for rapid diagnosis, which can be challenging because of the typical gradual and nonspecific onset of lupus in children, and for safer, more effective treatments.
The use of rituximab (Rituxan), while not formally evaluated in this population, may be a key steroid-sparing agent, they noted.
The research group is continuing to enroll patients with both definite and probable juvenile SLE, with the goal of further defining the disease characteristics and ultimately improving overall care for these vulnerable patients.
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