What Rheumatologists Should Know About Childhood-Onset SLE & Vasculitis

Dr. Ardoin talked briefly about the only U.S. Food & Drug Administration-approved, targeted therapy for pediatric SLE, belimumab, which is currently available only in an intravenous option for children. But she suspects, it will soon be available for subcutaneous administration, pending evaluation of forthcoming trial data.

A key area Dr. Ardoin focused on is the long-term complications many children with cSLE will carry into adulthood, such as obesity, atherosclerosis, reproductive concerns and osteoporosis, and the need for recognition by adult rheumatologists of the lifelong impact of these complications.

She cited evidence showing, for example, the much higher incidence of atherosclerosis in children with cSLE than other children. “I would say to an adult rheumatologist, when taking care of an adolescent with lupus, be cognizant of these kinds of complications and don’t wait to address them,” she said.

Vasculitis

In talking about childhood vasculitis, Dr. Brogan emphasized important distinctions in the differential diagnosis of pediatric vasculitis. He dispelled a common myth that classification criteria can be used to help diagnose individual children with small vessel vasculitis.

“Rheumatologists have been misusing classification criteria for diagnosis for decades,” he said, citing an editorial written by Gene Hunder, MD, on the use and misuse of classification and diagnostic criteria for complex diseases.¹ Diagnosis based on clinical features is much simpler, he noted.

Dr. Brogan

Another myth Dr. Brogan dispelled is that IgA vasculitis is the only small vessel vasculitis in children. Anti-neutrophil cytoplasmic antibody (ANCA) vasculitides are also seen in children, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). For each of these diseases, the differential diagnosis is based on clinical features and the presence of ANCA, he emphasized.

Other types of vasculitis seen in children are Kawasaki disease and polyarteritis nodosa (PAN)—both medium vessel vasculitides. Dr. Brogan emphasized that treatment for Kawasaki disease should target zero fever and normal C-reactive protein as measures of treatment success. He also said the rate of coronary artery aneurysms is much higher than typically thought in patients with these types of vasculitis, despite intravenous immunoglobulin (IVIG) treatment.

Dr. Brogan described evidence from the first pediatric trial, MYPAN, for the treatment of PAN. The trial showed the noninferiority of mycophenolate mofetil (MMF) vs. cyclophosphamide for remission reduction, indicating that MMF plus glucocorticoids can be used for first-line remission induction, reserving cyclophosphamide for patients with refractory disease.²