Back to Table of Contents

A 10-year-old girl presents with abdominal cramps, fever, weight loss and chest pain.

A teen is seen by a psychiatrist – then a rheumatologist – for headaches, mood swings and declining grades.

A 12-year-old girl goes to the emergency room with a vasculitic rash and returns two years later with fatigue and another year later with arthritis.

SLE: The Differential to Consider Every Time
Rarely a straightforward diagnosis, SLE often leads frustrated pediatricians and specialists on a circuitous wild goose chase as the disease “slowly declares itself,” says Patricia Irigoyen, MD, attending physician, Pediatric Rheumatology, Children’s Hospital at Montefiore (CHAM). While patients may experience a dizzying “constellation of symptoms,” explains Dr. Irigoyen, complaints are often vague, frequently manifest slowly, come and go – sometimes disappearing for years, sometimes forever – and maddeningly mimic other diseases.

Teen Girls at Higher Risk
An autoimmune disorder that causes multisystem microvascular inflammation and autoantibody generation, SLE’s genesis is unclear, but genetic, environmental, racial and hormonal factors play a role in the development of the disease.

Teenage girls make up the vast majority of the 0.36 to 0.9 out of 100,000 U.S. children who develop SLE each year: 50 to 60 percent of all pediatric lupus patients are teens and the disease strikes nine times more girls than boys in adolescence. Children who are African-American, Asian or Latino are at higher risk than Caucasians. All SLE patients test positive for ANA.

Renal Involvement Can Be Fatal
The disease’s protean presentation can “pretty much involve any organ that’s involved in any disease,” says Norman Ilowite, MD, chief, Pediatric Rheumatology, CHAM. “And it can involve that organ in any way,” he says.

Patients with SLE may have cardiac, hematologic, cutaneous and pulmonary complications, and two-thirds of patients with SLE will have kidney involvement that ranges from mild to severe, according to Frederick Kaskel, MD, chief, Pediatric Nephrology, CHAM.

Symptoms of renal involvement include proteinuria, hematuria, edema, headache and hypertension, but “more often than not,” notes Dr. Kaskel, “the diagnosis is mixed: A little bizarre behavior. Decreased school performance. People aren’t thinking that it could be a collagen vascular disease.”

Lupus patients with kidney involvement “should be referred immediately,” says Dr. Kaskel, who warns that “renal prognoses can fall from fair to fatal in hours. You either catch it on time, or patients can die.”

CHAM’s pediatric rheumatologists are committed to treating the whole patient. They are skilled in identifying and managing all physical, psychosocial and developmental issues related to childhood and adolescent SLE.

SLE Survival Rates Rise from 50 percent to 95 percent
Today, 95 percent of all SLE patients will survive – an enormous gain from the 50 percent mortality rate of the 1950s – reflecting the efficaciousness of corticosteroids and targeted cytotoxic agents as well as advances in kidney dialysis and transplant technologies.

At CHAM, the pediatric rheumatologic team customizes therapies to reduce flares, maximize therapeutic benefits and minimize toxicities. Creative alternate-day dosing of corticosteroids “reduces side effects as we taper,” says Dr. Irigoyen, and “pulse dosing with a large up-front IV medication catches disease and lowers cumulative dosing later on,” she explains.

Stem Cell Transplant Reboots Immune System
When life-threatening SLE is unresponsive to other therapies, CHAM’s pediatric hematologic experts can use advanced stem cell transplant that – in essence – restarts the patient’s immune system. “It’s like a reset button,” explains E. Anders Kolb, MD, director, Pediatric Stem Cell Transplantation, CHAM. Dr. Kolb removes the patient’s bone marrow and extracts “the T cells – the cells most likely responsible for causing the lupus,” he says. “The marrow is then reintroduced to the patient so the immune system can reconstitute itself and hopefully prevent the lupus from returning.”

Laundry List of Disease- and Therapy-associated Disorders
As therapeutic advances lengthen SLE patients’ lives, CHAM specialists manage an increasingly long list of disease- and therapy-associated complications. “Infection,” says Dr. Irigoyen, “is the main cause of death for lupus patients today.” Other associated disorders include osteoporosis, growth failure, infertility, hypertension and artherosclerosis.

Pioneering Investigator of Lupus-Associated Artheroscleros
Dr. Ilowite was one of the first pediatric rheumatologists to note lupus-associated cholesterol and investigate artherosclerosis prevention and treatment. An early architect of the landmark NIH-funded Artherosclerosis Prevention in Pediatric Lupus Erythematosus (APPLE) trial and one of its principal investigators, Dr. Ilowite today chairs APPLE’s Writing Committee to determine post-trial access to data.

As therapeutic advances lengthen SLE patients’ lives, CHAM specialists manage an increasingly long list of disease- and therapy-associated complications. Infection is one of the most serious challenges.

Strategies That Boost Teen Compliance
CHAM’s pediatric rheumatologists are committed to treating the whole patient. They are skilled in identifying and managing all physical, psychosocial and developmental issues related to childhood and adolescent SLE. Therapeutic compliance, for example, is “a huge problem,” says Dr. Irigoyen, who successfully handles compliance issues by “really engaging the patient,” she says. “I treat the complaints that are most important to her – like acne – even if they’re not important to me.”

With the support of a diverse treatment team that includes pediatric sub-specialists, practice nurses, physical therapists, nutritionists, child-life experts, social workers and psychotherapists – and an SLE support group made up of patients and parents – CHAM’s rheumatologic team is finding new strategies to meet SLE’s multiple challenges.

Back to Table of Contents