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A 37-year-old woman with autosomal dominant polycystic kidney disease (ADPKD) and normal kidney function, with well-controlled arterial hypertension, and rupture of an intracranial aneurysm at 27 years of age presented to our institute. Ultrasound led to a suspicion of two splenic artery aneurysms, which was subsequently confirmed by computed tomography (Fig. 1). The patient was referred to a vascular surgeon who recommended observation alone. To date, the aneurysms remain clinically silent.
A Contrast-enhanced computed tomography of the abdominal cavity, coronal plane. Aneurysm of the splenic artery (arrow). Polycystic kidneys. B Contrast-enhanced computed tomography (3D reconstruction). Two aneurysms of the spenic artery (arrows)
ADPKD is the most common monogenic disease of the kidney. It affects approximately 1 in every 1000 people. As well as being a cause of chronic kidney disease, it is accompanied by numerous extra-renal manifestations, including aneurysms. Due to possible dramatic consequences of rupture, intracranial aneurysms, observed in approximately 10% of ADPKD patients [1], attract the most attention. However, aneurysms can also be found in other arteries [2].
Knowledge of the pathogenesis of aneurysms in ADPKD remains piecemeal. ADPKD is caused by a mutation in the PKD1 or PKD2 gene, leading to disturbed structure and function of their protein products, polycystin-1 (PC-1), or polycystin-2 (PC-2), respectively. Mutations in PKD1 or PKD2 lead to dysregulation of various intracellular signaling pathways. Both PKD1 and PKD2 are expressed in the smooth muscle and myofibroblasts of blood vessels, and polycystins play an important role in maintaining the normal structure and function of blood vessels. Additionally, involvement of vascular enothelial growth factor (VEGF), collagen genes and genes of the TGF-β pathway in vascular remodeling was shown; however, exact mechanisms were not elucidated. These changes, together with arterial hypertension, which is common in ADPKD, may result in aneurysm development [S1, S2—see Suppl. file].
Rupture of an intracranial aneurysm in a patient’s medical history is considered a risk factor for the development of new intracranial aneurysms in ADPKD [1]. Our case reminds us that new aneurysms should also be expected in other locations.
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References
Chapman AB, Devuyst O, Eckardt KU et al (2015) Autosomal-dominant polycystic kidney disease (ADPKD): executive summary from a kidney disease: improving global outcomes (KDIGO) controversies conference. Kidney Int 88:17–27
Ruderman I, Menahem S (2014) Generalized aneurysmal disease in association with autosomal dominant polycystic disease. Clin Kidney J 7:416–417
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JB and ZJ, literature search, writing the manuscript; MG, imaging; MN, management of the patient, idea of the paper, writing the manuscript.
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Borowiecka, J., Jankowska, Z., Gradzik, M. et al. Aneurysms of splenic artery in a patient with autosomal dominant polycystic kidney disease. J Nephrol (2024). https://doi.org/10.1007/s40620-024-01946-3
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DOI: https://doi.org/10.1007/s40620-024-01946-3