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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References
Cervera R (2017) Antiphospholipid syndrome. Thromb Res 151(Suppl 1):S43–S47. https://doi.org/10.1016/S0049-3848(17)30066-X
Sahin M, Oguz A, Tuzun D, Boysan SN, Mese B, Sahin H, Gul K (2015) Primary adrenal failure due to antiphospholipid syndrome. Case Rep Endocrinol 2015:161497. https://doi.org/10.1155/2015/161497
Cervera R, Boffa MC, Khamashta MA, Hughes GR (2009) The Euro-Phospholipid Project: epidemiology of the antiphospholipid syndrome in Europe. Lupus 18:889–893. https://doi.org/10.1177/0961203309106832
Espinosa G, Cervera R, Font J, Asherson RA (2003) Adrenal involvement in the antiphospholipid syndrome. Lupus 12:569–572. https://doi.org/10.1191/0961203303lu404oa
Espinosa G, Santos E, Cervera R, Piette JC, de la Red G, Gil V, Font J, Couch R, Ingelmo M, Asherson RA (2003) Adrenal involvement in the antiphospholipid syndrome: clinical and immunologic characteristics of 86 patients. Medicine (Baltimore) 82:106–118. https://doi.org/10.1097/00005792-200303000-00005
Oliveira D, Ventura M, Melo M, Paiva S, Carrilho F (2018, 2018) Addison’s disease in antiphospholipid syndrome: a rare complication. Endocrinol Diabetes Metab Case Rep. https://doi.org/10.1530/EDM-18-0118
Uthman I, Salti I, Khamashta M (2006) Endocrinologic manifestations of the antiphospholipid syndrome. Lupus 15:485–489. https://doi.org/10.1191/0961203306lu2318rr
Behera KK, Kapoor N, Seshadri MS, Rajaratnam S (2013) Acute adrenal insufficiency due to primary antiphospholipid antibody syndrome. Indian J Endocrinol Metab 17(Suppl 1):S240–S242. https://doi.org/10.4103/2230-8210.119584
Pinto A, Barletta JA (2015) Adrenal tumors in adults. Surg Pathol Clin 8:725–749. https://doi.org/10.1016/j.path.2015.07.005
Khan IN, Adlan MA, Stechman MJ, Premawardhana LD (2015) A retroperitoneal leiomyosarcoma presenting as an adrenal incidentaloma in a subject on warfarin. Case Rep Endocrinol 2015:830814. https://doi.org/10.1155/2015/830814
Kannan S, Satra A, Hamrahian A (2013) Incidental lipid poor adrenal mass in a patient with antiphospholipid syndrome. Case Rep Endocrinol 2013:379852. https://doi.org/10.1155/2013/379852
Marinelli C, Petramala L, Iannucci G et al (2015) Resistant arterial hypertension in a patient with adrenal incidentaloma multiple steno-obstructive vascular lesions and antiphospholipid syndrome. Ann Clin Exp Hypertens 3:1020
Mackay I, Aspinall S (2016) Advances in our understanding of the prognosis of adrenal incidentaloma. Expert Rev Endocrinol Metab 11:529–541. https://doi.org/10.1080/17446651.2016.1233055
Funder JW, Carey RM, Fardella C, Gómez-Sánchez CE, Mantero F, Stowasser M, Young WF Jr, Montori VM, Endocrine Society (2008) Case detection, diagnosis, and treatment of patients with primary aldosteronism: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab 93:3266–3281. https://doi.org/10.1210/jc.2008-0104
Tektonidou MG, Andreoli L, Limper M, Amoura Z, Cervera R, Costedoat-Chalumeau N, Cuadrado MJ, Dörner T, Ferrer-Oliveras R, Hambly K, Khamashta MA, King J, Marchiori F, Meroni PL, Mosca M, Pengo V, Raio L, Ruiz-Irastorza G, Shoenfeld Y, Stojanovich L, Svenungsson E, Wahl D, Tincani A, Ward MM (2019) EULAR recommendations for the management of antiphospholipid syndrome in adults. Ann Rheum Dis 78:1296–1304. https://doi.org/10.1136/annrheumdis-2019-215213
Cuadrado MJ, Buendía P, Velasco F, Aguirre MA, Barbarroja N, Torres LA, Khamashta M, López-Pedrera C (2006) Vascular endothelial growth factor expression in monocytes from patients with primary antiphospholipid syndrome. J Thromb Haemost 4:2461–2469. https://doi.org/10.1111/j.1538-7836.2006.02193.x
Motoki Y, Nojima J, Yanagihara M, Tsuneoka H, Matsui T, Yamamoto M, Ichihara K (2012) Anti-phospholipid antibodies contribute to arteriosclerosis in patients with systemic lupus erythematosus through induction of tissue factor expression and cytokine production from peripheral blood mononuclear cells. Thromb Res 130:667–673. https://doi.org/10.1016/j.thromres.2011.11.048
Nakagawa H, Yasuda S, Matsuura E, Kobayashi K, Ieko M, Kataoka H, Horita T, Atsumi T, Koike T (2009) Nicked β2-glycoprotein I binds angiostatin 4.5 (plasminogen kringle 1-5) and attenuates its antiangiogenic property. Blood 114:2553–2559. https://doi.org/10.1182/blood-2008-12-190629
Yu P, Passam FH, Yu DM, Denyer G, Krilis SA (2008) Beta2-glycoprotein I inhibits vascular endothelial growth factor and basic fibroblast growth factor induced angiogenesis through its amino terminal domain. J Thromb Haemost 6:1215–1223. https://doi.org/10.1111/j.1538-7836.2008.03000.x
Passam FH, Qi JC, Tanaka K, Matthaei KI, Krilis SA (2010) In vivo modulation of angiogenesis by beta 2 glycoprotein I. J Autoimmun 35(3):232–240. https://doi.org/10.1016/j.jaut.2010.06.013
Wu YY, Nguyen A, Wu XX et al (2014) Antiphospholipid antibodies promote tissue factor-dependent angiogenic switch and tumor progression. Am J Pathol 184:3359–3375. https://doi.org/10.1016/j.ajpath.2014.07.027
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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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DOI: https://doi.org/10.1007/s10067-020-04978-9