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Primary antiphospholipid syndrome, Addison disease, and adrenal incidentaloma

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Abstract

Primary adrenal failure comprises an insufficient production of mineralocorticoids and glucocorticoids in the adrenal cortex. A rare manifestation of antiphospholipid syndrome (APS) is adrenal failure. The majority of patients with adrenal involvement in APS develop an irreversible cortisol deficiency and atrophy of the adrenal glands. Adrenal incidentalomas are adrenal masses larger than 1 cm that are discovered in the course of diagnostic evaluation or treatment for another medical condition. Its prevalence is calculated in 1.5–9% of individuals. We describe an exceptional case of a 23-year-old male patient with APS with persistent high levels of antiphospholipid antibodies (aPL) from the time of diagnosis, who developed Addison’s disease as a manifestation of APS with atrophy of the adrenal glands, in whom an adrenal incidentaloma was developed later and was corroborated as an aldosterone-producing adenoma. Currently, the patient is asymptomatic and without manifestations of tumor recurrence. The protumoral effect of elevated and persistent aPL is discussed.

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Authors and Affiliations

  1. Translational Research Unit, Hospital de Especialidades, Centro Médico Nacional “La Raza,” IMSS, Mexico City, Mexico

    Gabriela Medina

  2. Universidad Veracruzana, Xalapa, Veracruz, Mexico

    María Pilar Jiménez-Arellano

  3. Endocrinology Department, Hospital de Especialidades, Centro Médico Nacional “La Raza,” IMSS, Mexico City, Mexico

    Andrés Muñoz-Solís

  4. Surgery Department, Hospital de Especialidades, Centro Médico Nacional “La Raza,” IMSS, Mexico City, Mexico

    Erick Servín-Torres

  5. Pathology Department, Hospital de Especialidades, Centro Médico Nacional “La Raza,” IMSS, Mexico City, Mexico

    Pablo Ramírez-Mendoza

  6. Direction of Education and Research, Hospital de Especialidades, Centro Médico Nacional “La Raza,” IMSS, Seris y Zaachila S/N Col. La Raza CP, 02990, Mexico City, Mexico

    Luis J. Jara

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  1. Gabriela Medina
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Correspondence to Luis J. Jara.

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The authors certify that they obtained the patient’s consent for the use of his images and other clinical information reported in the manuscript. The patient understands that his name and initials will not be published, and due efforts will be made to conceal his identity.

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Medina, G., Jiménez-Arellano, M.P., Muñoz-Solís, A. et al. Primary antiphospholipid syndrome, Addison disease, and adrenal incidentaloma. Clin Rheumatol 39, 1997–2001 (2020). https://doi.org/10.1007/s10067-020-04978-9

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