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The search was conducted in the PubMed, SciELO, Scopus, and Web of Science databases. This review article focuses on the common molecular and genetic bases of renal and ocular involvement in both systemic diseases and rare syndromes. The interdependence between renal and ocular morphogenesis is mediated by conserved molecular mechanisms, notably the\n                    <jats:italic>Bone Morphogenetic Protein-7 (BMP-7)<\/jats:italic>\n                    pathway, which regulates nephrogenesis and lens development, and the transcription factor\n                    <jats:italic>Paired Box 2 (PAX2),<\/jats:italic>\n                    which is essential for the formation of the genitourinary tract and the optic nerve. Shared expression of molecular components and dependency on their expression for the integrity of both the eye and the kidney lie in the stability of extracellular matrix components, specifically through laminin \u03b22 (LAMB2) and type IV collagen, whose pathogenic variants underlie syndromic phenotypes such as Pierson and Alport syndromes. In addition, ciliopathies, developmental disorders, immune-mediated diseases, and inborn errors of metabolism, including Fabry disease, cystinosis, and primary hyperoxaluria, promote parallel injury to renal and ocular tissues through mechanisms involving abnormal cellular signaling, metabolite accumulation, complement activation and systemic inflammation. These mutual pathways affect diverse ocular structures, including the cornea, lens, retina, optic nerve, and basement membranes. Furthermore, the presence of the kidney\u2013retina axis enables the use of the retina as a sentinel organ, allowing for non-invasive evaluation of glomerular microvasculature by means of high-resolution imaging technologies of retinal vessels. The kidneys and eyes share several genetic, developmental, structural, metabolic, and immunological mechanisms that explain the frequent association of congenital anomalies and acquired lesions in these organs and the associated diagnostic and prognostic implications.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Graphical Abstract<\/jats:bold>\n                  <\/jats:p>\n                  <jats:p>A higher resolution version of the\u00a0Graphical abstract is available as Supplementary information<\/jats:p>","DOI":"10.1007\/s00467-026-07456-7","type":"journal-article","created":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T07:51:57Z","timestamp":1783497117000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Reno-ocular syndromes: pathophysiological mechanisms linking kidney and ocular disorders"],"prefix":"10.1007","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8065-1959","authenticated-orcid":false,"given":"Ana Fl\u00e1via","family":"Conegundes","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-8530-211X","authenticated-orcid":false,"given":"Luiza","family":"Haikal de Paula","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1530-6523","authenticated-orcid":false,"given":"Rafael","family":"dos Santos Borges","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9222-3882","authenticated-orcid":false,"given":"Ana Cristina","family":"Sim\u00f5es e Silva","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,7,8]]},"reference":[{"key":"7456_CR1","doi-asserted-by":"publisher","first-page":"2795","DOI":"10.1101\/gad.9.22.2795","volume":"9","author":"AT Dudley","year":"1995","unstructured":"Dudley AT, Lyons KM, Robertson EJ (1995) A requirement for bone morphogenetic protein-7 during development of the mammalian kidney and eye. 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