The Rheumatologist
COVID-19 NewsACR Convergence
  • Connect with us:
  • Facebook
  • Twitter
  • LinkedIn
  • YouTube
  • Feed
  • Home
  • Conditions
    • Rheumatoid Arthritis
    • SLE (Lupus)
    • Crystal Arthritis
      • Gout Resource Center
    • Spondyloarthritis
    • Osteoarthritis
    • Soft Tissue Pain
    • Scleroderma
    • Vasculitis
    • Systemic Inflammatory Syndromes
    • Guidelines
  • Resource Centers
    • Axial Spondyloarthritis Resource Center
    • Gout Resource Center
    • Psoriatic Arthritis Resource Center
    • Rheumatoid Arthritis Resource Center
    • Systemic Lupus Erythematosus Resource Center
  • Drug Updates
    • Biologics & Biosimilars
    • DMARDs & Immunosuppressives
    • Topical Drugs
    • Analgesics
    • Safety
    • Pharma Co. News
  • Professional Topics
    • Ethics
    • Legal
    • Legislation & Advocacy
    • Career Development
      • Certification
      • Education & Training
    • Awards
    • Profiles
    • President’s Perspective
    • Rheuminations
    • Interprofessional Perspective
  • Practice Management
    • Billing/Coding
    • Quality Assurance/Improvement
    • Workforce
    • Facility
    • Patient Perspective
    • Electronic Health Records
    • Apps
    • Information Technology
    • From the College
    • Multimedia
      • Audio
      • Video
  • Resources
    • Issue Archives
    • ACR Convergence
      • Gout Resource Center
      • Axial Spondyloarthritis Resource Center
      • Psoriatic Arthritis
      • Abstracts
      • Meeting Reports
      • ACR Convergence Home
    • American College of Rheumatology
    • ACR ExamRheum
    • Research Reviews
    • ACR Journals
      • Arthritis & Rheumatology
      • Arthritis Care & Research
      • ACR Open Rheumatology
    • Rheumatology Image Library
    • Treatment Guidelines
    • Rheumatology Research Foundation
    • Events
  • About Us
    • Mission/Vision
    • Meet the Authors
    • Meet the Editors
    • Contribute to The Rheumatologist
    • Subscription
    • Contact
  • Advertise
  • Search
You are here: Home / Articles / What Rheumatologists Should Know About Childhood-Onset SLE & Vasculitis

What Rheumatologists Should Know About Childhood-Onset SLE & Vasculitis

December 2, 2021 • By Mary Beth Nierengarten

  • Tweet
  • Email
Print-Friendly Version / Save PDF

ACR CONVERGENCE 2021—Many of the effects of childhood-onset systemic lupus erythematosus (cSLE) and vasculitis carry into adulthood and present adult rheumatologists with key differences in managing these patients after their transition from a pediatric to an adult provider.

You Might Also Like
  • How Hospitals Rank in Treating Childhood-Onset SLE
  • REF-Funded Research Provides Prognostic Information about Childhood-onset SLE
  • Familial Patterns in Childhood- & Adult-Onset SLE
Also By This Author
  • What Orthopedists Want Rheumatologists to Know: Surgical Treatment for Foot & Ankle Arthritis

“The young adult with childhood-onset lupus is similar in many ways to adults with lupus, but there are important differences, and it is important to be aware of those,” said Stacy Ardoin, MD, professor of pediatric and adult rheumatology at Nationwide Children’s Hospital, Ohio State University, Columbus. During ACR Convergence 2021, she opened a session titled Not Just Little Adults: What Rheumatologists Should Know About Childhood-Onset SLE & Vasculitis by discussing these differences and management issues in patients with cSLE.

ad goes here:advert-1
ADVERTISEMENT
SCROLL TO CONTINUE

Paul Brogan, MD, professor of vasculitis and section head for infection, inflammation and rheumatology, University College London, Institute of Child Health at Great Ormond Street Hospital, spoke about the differences in vasculitis in children and adults. He dispelled myths for adult rheumatologists and provided evidence to support the treatment of children with vasculitis transitioning into adulthood.

cSLE

The pathogenic mechanism and patterns of disease involvement in childhood-onset SLE and adults are the same, as is the treatment approach. But important differences exist in childhood-onset disease, which is marked by more organs affected, more severe disease (i.e., higher disease activity), faster damage accrual, higher mortality, higher risk of complications due to more severe disease and different pharmacokinetics.

ad goes here:advert-2
ADVERTISEMENT
SCROLL TO CONTINUE

cSLE also has a greater genetic component, with these children at greater risk of developing the rarer, monogenic form of disease. Dr. Ardoin explained when adult rheumatologists should suspect a monogenic form of SLE and how to pursue an evaluation to make the diagnosis. Clues, she said, include onset in a child 6 years or younger with a history of immunodeficiency, family history of immunodeficiency, as well as involvement of the skin, kidneys and central nervous system.

Dr. Ardoin

Walking attendees through treatment strategies for cSLE, Dr. Ardoin emphasized that treatment is similar to the treatment of adults in that it’s tailored to organ involvement—or to mild to severe disease activity. However, for children, the dosage of the same drugs given to adults needs to be based on weight and body surface to avoid either underdosing or overdosing these patients.

“I’ve often found rheumatologists are concerned about giving doses that may approximate adult disease and tend to use a dose lower than is needed by the child,” she said, adding that this can result in undertreating their disease.

ad goes here:advert-3
ADVERTISEMENT
SCROLL TO CONTINUE

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


Mary Beth Nierengarten is a freelance medical journalist based in Minneapolis.

References

  1. Hunder GG. The use and misuse of classification and diagnostic criteria for complex diseases. Ann Intern Med. 1998 Sep 1;129(5):417–418 Annals of Internal Medicine 1998.
  2. Brogan PA, Arch B, Hickey H, et al. Mycophenolate mofetil versus cyclophosphamide for remission induction in childhood polyarteritis nodosa: An open-label, randomized, bayesian noninferiority trial. Arthritis Rheumatol. 2021 Sep;73(9):1673–1682.

Pages: 1 2 3 | Multi-Page

Filed Under: ACR Convergence, Conditions, Meeting Reports, SLE (Lupus), Vasculitis Tagged With: ACR Convergence 2021, ACR Convergence 2021 – SLE, childhood-onset SLE, Pediatric Rheum

You Might Also Like:
  • How Hospitals Rank in Treating Childhood-Onset SLE
  • REF-Funded Research Provides Prognostic Information about Childhood-onset SLE
  • Familial Patterns in Childhood- & Adult-Onset SLE
  • Childhood-onset Lupus Brings Distinct Challenges at Each Phase of Life

Simple Tasks

Learn more about the ACR’s public awareness campaign and how you can get involved. Help increase visibility of rheumatic diseases and decrease the number of people left untreated.

Visit the Simple Tasks site »

American College of Rheumatology

Visit the official website for the American College of Rheumatology.

Visit the ACR »

Rheumatology Research Foundation

The Foundation is the largest private funding source for rheumatology research and training in the U.S.

Learn more »

The Rheumatologist newsmagazine reports on issues and trends in the management and treatment of rheumatic diseases. The Rheumatologist reaches 11,500 rheumatologists, internists, orthopedic surgeons, nurse practitioners, physician assistants, nurses, and other healthcare professionals who practice, research, or teach in the field of rheumatology.

About Us / Contact Us / Advertise / Privacy Policy / Terms of Use / Cookie Preferences

  • Connect with us:
  • Facebook
  • Twitter
  • LinkedIn
  • YouTube
  • Feed

Copyright © 2006–2023 American College of Rheumatology. All rights reserved.

ISSN 1931-3268 (print)
ISSN 1931-3209 (online)