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 / Anti-Interleukin-6 Therapy for Erdheim-Chester Disease Warrants Study

Anti-Interleukin-6 Therapy for Erdheim-Chester Disease Warrants Study

February 16, 2017 • By Stefanie D. Wade, MD, Michael A. Seidman, MD, Edward C. Jones, MD, Arnold Radu, MD, Ryan Paterson, MD, Vikram Deshpande, MD, John H. Stone, MD, & Mollie N. Carruthers, MD

  • Tweet
  • Email
Print-Friendly Version / Save PDF

Erdheim-Chester disease (ECD) is a rare, non-Langerhan’s cell histiocytosis characterized by tissue infiltration of CD68-positive and CD1a-negative foamy histiocytes.1 ECD was discovered as a lipid granulomatosis in 1930 by Jakob Erdheim and his pupil, William Chester, and approximately 500 cases have been described to date.1

You Might Also Like
  • Skin Complications of Anti-TNF Therapy Common in IBD
  • Interleukin 1-alpha Is Critical for Establishment of Inflammatory Lung Disease
  • Long-Term Benefits, Risks of Biologic Disease-Modifying Anti-Rheumatic Drugs in Patients with RA
Explore This Issue
February 2017

ECD has a heterogeneous course and prognosis ranging from an asymptomatic process to a multi-systemic disease that can be life threatening. The most common ECD manifestations include symmetric osteosclerosis of long bones, followed by retroperitoneal infiltration.1 ECD can involve several organ systems, including cardiovascular, pulmonary, neurologic, ophthalmologic, skin and musculoskeletal systems.1 ECD has a male predominance and is typically diagnosed in adults between ages 40 and 70.2 Diagnosis is often delayed due to its rarity, multiple disease presentations and lack of awareness.

ad goes here:advert-1
ADVERTISEMENT
SCROLL TO CONTINUE

The etiology and pathogenesis of ECD remain only partially understood. Studies have shown elevation in chemokines and cytokines, including interleukin (IL-) 1, IL-6 and interferon alpha (IFN‑α), which point toward a Th-1 mediated immune response.3 Recent advances have linked aberrant BRAFV600E to patients with ECD and demonstrated an activation of downstream kinases.4

In recent years, IFN‑α has become a first-line therapy for ECD; however, limited efficacy and a poor side effect profile have highlighted a need for pursuing better therapies.2 IL-1 inhibition with anakinra is now also considered to be first-line treatment based on case reports and small case series.2 Early observations have shown that anti BRAFV600 may be a potential treatment, but further study is needed.

ad goes here:advert-2
ADVERTISEMENT
SCROLL TO CONTINUE

IL-6 levels were markedly elevated in patients with ECD who underwent analysis of their cytokine profile.5 It was further noted that patients treated with IFN-α normalized their serum levels of IL-6.5 Tocilizumab is a humanized monoclonal antibody that inhibits the IL-6 receptor. Therefore, anti-IL-6 therapy with tocilizumab (TCZ) was used to treat two cases of multisystem ECD.

In our cases, we show dramatic improvement with anti-interleukin-6 therapy and think it warrants more study. Current therapy is suboptimal, & there is a role for alternative treatments in ECD.

Case Series

Two patients referred to the rheumatology service met criteria for diagnosis of Erdheim-Chester based on a combination of clinical and radiographic features. Findings from standard clinical, radiologic and histopathologic techniques are described.

TABLE 1: Baseline Characteristics of ECD Patients

(click for larger image)
TABLE 1: Baseline Characteristics of ECD Patients

The clinical history of Patients 1 and 2 is described below. The baseline characteristics of Patients 1 and 2 are described in Table 1, and the immunohistochemical features of the two patients are shown in Table 2.

Pages: 1 2 3 4 5 6 7 8 9 | Single Page

Filed Under: Conditions, Systemic Inflammatory Syndromes Tagged With: anti-interleukin, case report, Clinical, Diagnosis, Disease, Erdheim-Chester, inflammatory, interferon, Pathogenesis, therapy, TreatmentIssue: February 2017

You Might Also Like:
  • Skin Complications of Anti-TNF Therapy Common in IBD
  • Interleukin 1-alpha Is Critical for Establishment of Inflammatory Lung Disease
  • Long-Term Benefits, Risks of Biologic Disease-Modifying Anti-Rheumatic Drugs in Patients with RA
  • Mesenchymal Stem Cell Therapy May Help Slow, Repair Degenerative Signs of Osteoarthritis, Musculoskeletal Disease

Meeting Abstracts

Browse and search abstracts from the ACR Convergence and ACR/ARP Annual Meetings going back to 2012.

Visit the Abstracts site »

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 »

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)