DOI: 10.12890/2025_005355

Autoantibodies directed against insulin receptor during the course of Castleman disease: a new case reaffirming autoimmune hypoglycemia as a relapse warning signal

Authors

  • Xavier Jannot Department of Clinical Immunology and Internal Medicine, National Reference Center for Systemic Autoimmune Diseases (CNR RESO), Tertiary Center for Primary Immunodeficiency, Strasbourg University Hospital, Strasbourg, France
  • Martine Auclair National Reference Centre for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS), Department of Endocrinology, Diabetology and Reproductive Endocrinology, Assistance Publique–Hôpitaux de Paris, Saint Antoine Hospital, Paris, France
  • Martin Martinot Department of Infectious Diseases, Hôpitaux Civils de Colmar, Colmar, France
  • Amin Maazouzi Department of Clinical Immunology and Internal Medicine, National Reference Center for Systemic Autoimmune Diseases (CNR RESO), Tertiary Center for Primary Immunodeficiency, Strasbourg University Hospital, Strasbourg, France
  • David Boutboul Department of Hematology, Hôpital Cochin, AP-HP, Université Paris Cité; National Reference Center for Castleman disease, Hôpital Saint Louis, AP-HP, Université Paris Cité, Paris, France
  • Corinne Vigouroux National Reference Centre for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS), Department of Endocrinology, Diabetology and Reproductive Endocrinology, Assistance Publique–Hôpitaux de Paris, Saint Antoine Hospital, Paris, France
  • Yannick Dieudonné Department of Clinical Immunology and Internal Medicine, National Reference Center for Systemic Autoimmune Diseases (CNR RESO), Tertiary Center for Primary Immunodeficiency, Strasbourg University Hospital, Strasbourg, France

xavier.jannot@chru-strasbourg.fr

Keywords

Multicentric Castleman disease, hypoglycemia, autoimmune disease, type B insulin resistance syndrome

Abstract

This case study presents the case of a 54-year-old human immunodeficiency virus (HIV)-positive male who developed type B insulin resistance syndrome (TBIRS) in conjunction with a relapse of human herpesvirus 8 (HHV8)-positive multicentric Castleman disease (MCD). This case is only the sixth reported instance of TBIRS associated with HHV8-associated MCD. The diagnosis was confirmed by the presence of anti-insulin receptor autoantibodies, and the patient was treated effectively with rituximab, with no relapse in follow-up.
The cases described are discussed, along with the differences between them and our own case. Additionally, the potential for an autoimmune complication of MCD, even when HIV is well controlled, is addressed, as well as the available therapeutic approaches.

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References

  1. Gérard L, Bérezné A, Galicier L, Meignin V, Obadia M, De Castro N, et al. Prospective Study of Rituximab in Chemotherapy-Dependent Human Immunodeficiency Virus–Associated Multicentric Castleman’s Disease: ANRS 117 CastlemaB Trial. J Clin Oncol 2007;25:3350-3356.
  2. Arioglu E, Andewelt A, Diabo C, Bell M, Taylor SI, Gorden P. Clinical course of the syndrome of autoantibodies to the insulin receptor (type B insulin resistance): a 28-year perspective. Medicine (Baltimore) 2002;81:87-100.
  3. Bourron O, Caron-Debarle M, Hie M, Amoura Z, Andreelli F, Halbron M, et al. Type B Insulin-resistance syndrome: a cause of reversible autoimmune hypoglycaemia. Lancet Lond Engl 2014;384:1548.
  4. Arnautou P, Auclair M, Fellahi S, Bouché C, Fieschi C, Barrak E, et al. Autoimmune hypoglycemia expands the biological spectrum of HHV8+ multicentric Castleman disease. Blood Adv 2021;5:1848-1852.
  5. Minich WB, Abel BS, Schwiebert C, Welsink T, Seemann P, Brown RJ, et al. A Novel In Vitro Assay Correlates Insulin Receptor Autoantibodies With Fasting Insulin in Type B Insulin Resistance. J Clin Endocrinol Metab 2023;108:2324-2329.
  6. Fourman LT, Tsai LL, Brown RJ, O’Rahilly S. Case 10-2024: A 46-Year-Old Woman with Hyperglycemia Refractory to Insulin Therapy. N Engl J Med 2024;390:1219-1229.
  7. Sollier M, Vigouroux C, Duckett K, Auclair M, Lamand V, Nassouri S, et al. Type B insulin resistance syndrome: A case report. Diabetes Metab 2024;50:101549.
  8. Klubo-Gwiezdzinska J, Lange M, Cochran E, Semple RK, Gewert C, Brown RJ, et al. Combined Immunosuppressive Therapy Induces Remission in Patients With Severe Type B Insulin Resistance: A Prospective Cohort Study. Diabetes Care 2018;41:2353-2360.
  9. Dispenzieri A, Fajgenbaum DC. Overview of Castleman disease. Blood 2020;135:1353-1364.
  10. Codish S, Abu-Shakra M, Ariad S, Zirkin HJ, Yermiyahu T, Dupin N, et al. Manifestations of three HHV-8-related diseases in an HIV-negative patient: immunoblastic variant multicentric Castleman’s disease, primary effusion lymphoma, and Kaposi’s sarcoma. Am J Hematol 2000;65:310-314.
  11. London J, Boutboul D, Agbalika F, Coppo P, Veyradier A, Gérard L, et al. Autoimmune thrombotic thrombocytopenic purpura associated with HHV8-related Multicentric Castleman disease. Br J Haematol 2017;178:486-488.
blood glucose levels over time with response to rituximab infusion

Published

2025-04-29

Issue

Vol. 12 No. 5 (2025)

Categories

How to Cite

1.
Jannot X, Auclair M, Martinot M, Maazouzi A, Boutboul D, Vigouroux C, et al. Autoantibodies directed against insulin receptor during the course of Castleman disease: a new case reaffirming autoimmune hypoglycemia as a relapse warning signal. EJCRIM [Internet]. 2025 Apr. 29 [cited 2026 Nov. 3];12(5). Available from: https://ejcrim.com/index.php/ejcrim/article/view/5355

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