The Rheumatologist
COVID-19 News
  • 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
      • Systemic Lupus Erythematosus Resource Center
      • Rheumatoid Arthritis Resource Center
      • Gout Resource Center
      • 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 / Rheumatology Case Report: Concomitant Lupus with Features of Scleroderma, Castleman Disease

Rheumatology Case Report: Concomitant Lupus with Features of Scleroderma, Castleman Disease

July 11, 2016 • By Kwabna Parker, MBBS, Sireesha Datla, MD, & Nancy Soloman, MD

  • Tweet
  • Email
Print-Friendly Version / Save PDF

Seven months later, she was readmitted for cellulitis of the right arm and found to have a neck mass and a new systolic murmur, and was noted to have pitted scars on her fingertips; at that point, she fulfilled the 2013 ACR/EULAR classification criteria for systemic sclerosis. A CT of her neck revealed persistent lymphadenopathy, and a 2D echo showed persistently elevated pulmonary arterial pressure of 44 mmHg.

You Might Also Like
  • Rheumatology Case Report: TINU Syndrome with Concomitant Synovitis
  • Rheumatology Case Report: Bullous Lesions in Patient with Lupus
  • Rheumatology Case Report: Chondromyxoid Fibroma of the Sternum
Explore This Issue
July 2016

Discussion

We present the case of a patient with newly diagnosed SLE and MCD and who also has evidence of EBV reactivation and features of systemic sclerosis.

ad goes here:advert-1
ADVERTISEMENT
SCROLL TO CONTINUE

Castleman disease is a heterogeneous group of disorders with three histological types (i.e., hyaline vascular, plasma cell and mixed) and two clinical subtypes (i.e., localized and multicentric).1 Most cases of MCD occur in middle-aged men, and there is an association between MCD and HIV and human herpes virus 8.2,3 SLE, however, occurs mainly in women of childbearing age. The most common lymph node lesions in SLE are follicular hyperplasia and coagulative necrosis, with the latter being more specific.4

In one review of 33 SLE patients, lymph node pathology consistent with Castleman disease was found in 10% of these patients.4 The reported cases of concomitant MCD and SLE were mainly young to middle-aged women who were HIV negative, like our patient.5-10 Our patient was also noted to have evidence of EBV infection with IgM and IgG antibodies, as well as EBV DNA detected on PCR. These findings suggest that either her EBV infection was subacute or she was reinfected with EBV.11

ad goes here:advert-2
ADVERTISEMENT
SCROLL TO CONTINUE

EBV occurs worldwide, with over 80% of individuals older than 30 being infected. The infection persists for life and is associated with the development of malignancies, such as Burkitt’s lymphoma, Hodgkin’s disease and lymphoproliferative disorders.12 EBV has been reported in association with Castleman disease in variable frequencies, ranging from 2 out of 20 to 19 out of 20 cases.13,14 In one instance, EBV was reported to occur in MCD, with fatal results.15

IL-6 may be produced in an autocrine fashion from EBV-transformed B cells.16 Excessive IL-6 production in mice produces a syndrome characterized by anemia, transient granulocytosis, splenomegaly, hypoalbuminemia and polyclonal hypergammaglobulinemia, which closely resembles the systemic features of Castleman disease.17 The level of IL-6 produced by hyperplastic lymph nodes in patients with Castleman disease is directly related to the severity of clinical features and worsening of the lab results, which resolved with the removal of the lymph nodes.18

Pages: 1 2 3 4 5 | Single Page

Filed Under: Conditions, Scleroderma Tagged With: castleman disease, Clinical, Lupus, outcome, patient care, rheumatology, Scleroderma, SLEIssue: July 2016

You Might Also Like:
  • Rheumatology Case Report: TINU Syndrome with Concomitant Synovitis
  • Rheumatology Case Report: Bullous Lesions in Patient with Lupus
  • Rheumatology Case Report: Chondromyxoid Fibroma of the Sternum
  • Systemic Lupus Erythematosus Without Kidney Involvement: A Case Report

ACR Convergence

Don’t miss rheumatology’s premier scientific meeting for anyone involved in research or the delivery of rheumatologic care or services.

Visit the ACR Convergence site »

American College of Rheumatology

Visit the official website for the American College of Rheumatology.

Visit the ACR »

Meeting Abstracts

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

Visit the Abstracts site »

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

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

Copyright © 2006–2022 American College of Rheumatology. All rights reserved.

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