{
  "abstract": "In this article, we report a case of idiopathic multicentric Castleman disease (iMCD) with an atypical manifestation in a man in his early 50s, whose first symptoms included recurrent episodes of pancreatitis, retroperitoneal fibrosis and lymphadenopathy. These features are more commonly associated with IgG4-related disease (IgG4-RD). Although clinical findings were suggestive of IgG4-RD, IgG4 levels were within the normal range, along with a histopathological examination, ultimately leading to an iMCD diagnosis, showing characteristic vascular hyperplasia and a polyclonal plasma cell infiltrate showing no signs of IgG4+ plasma cells. In addition, the patient showed improvement with siltuximab, an interleukin-6 inhibitor drug specific for iMCD. This challenging case enhances the importance of combining clinical evaluation, serological testing and histopathological analysis to establish a definitive diagnosis.",
  "authors": [
    {
      "affiliations": [
        "Department of Hematology and Hemotherapy of the Hospital de Móstoles, Hospital Universitario de Móstoles, Móstoles, Spain"
      ],
      "name": "Juan Sastre Ortega"
    },
    {
      "affiliations": [
        "Systemic Autoimmune Diseases Unit of the Hospital de Móstoles, Hospital Universitario de Mostoles, Mostoles, Spain",
        "Department of Internal Medicine of the Hospital de Móstoles, Hospital Universitario de Móstoles, Móstoles, Community of Madrid, Spain"
      ],
      "name": "Carlos Martinez-Caballero"
    },
    {
      "affiliations": [
        "Systemic Autoimmune Diseases Unit of the Hospital de Móstoles, Hospital Universitario de Mostoles, Mostoles, Spain"
      ],
      "name": "Marta Rueda Herrera"
    },
    {
      "affiliations": [
        "Department of Pathological Anatomy of the Hospital de Móstoles, Hospital Universitario de Móstoles, Móstoles, Community of Madrid, Spain"
      ],
      "name": "Marcos José Carrillo Pohl"
    },
    {
      "affiliations": [
        "Department of Hematology and Hemotherapy of the Hospital de Móstoles, Hospital Universitario de Móstoles, Móstoles, Spain"
      ],
      "name": "Mariana Cerrato Salas"
    },
    {
      "affiliations": [
        "Department of Hematology and Hemotherapy of the Hospital de Móstoles, Hospital Universitario de Móstoles, Móstoles, Spain"
      ],
      "name": "Ane Brieva López"
    },
    {
      "affiliations": [
        "Department of Hematology and Hemotherapy of the Hospital de Móstoles, Hospital Universitario de Móstoles, Móstoles, Spain"
      ],
      "name": "Pavel Manuel Acevedo Pérez"
    },
    {
      "affiliations": [
        "Department of Pathological Anatomy of the Hospital de Móstoles, Hospital Universitario de Móstoles, Móstoles, Community of Madrid, Spain"
      ],
      "name": "Cristina Diego Hernández"
    },
    {
      "affiliations": [
        "Department of Internal Medicine of the Hospital de Móstoles, Hospital Universitario de Móstoles, Móstoles, Community of Madrid, Spain"
      ],
      "name": "Sonia Hernandez-Puche"
    },
    {
      "affiliations": [
        "Systemic Autoimmune Diseases Unit of the Hospital de Móstoles, Hospital Universitario de Mostoles, Mostoles, Spain"
      ],
      "name": "Amalia Muñana Fuentes"
    }
  ],
  "title": "Idiopathic multicentric Castleman disease in a patient with an IgG4-related disease phenotype",
  "uid": "fd1cc066-13ed-55d6-a61d-1be29f095f41"
}