{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,15]],"date-time":"2026-05-15T00:39:09Z","timestamp":1778805549484,"version":"3.51.4"},"reference-count":15,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2019,5,2]],"date-time":"2019-05-02T00:00:00Z","timestamp":1556755200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100007397","name":"Univerzita Karlova v Praze","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007397","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acta Ophthalmologica"],"published-print":{"date-parts":[[2019,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Purpose<\/jats:title><jats:p>To describe the ocular findings of 12 subjects with paraproteinemic keratopathy associated with monoclonal gammopathy of undetermined significance (<jats:styled-content style=\"fixed-case\">MGUS<\/jats:styled-content>).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Ocular examination included corneal spectral domain optical coherence tomography. In three individuals with an initial diagnosis of a lattice or Thiel\u2013Behnke corneal dystrophy, the <jats:italic><jats:styled-content style=\"fixed-case\">TGFBI<\/jats:styled-content><\/jats:italic> gene was screened by conventional Sanger sequencing.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We confirmed a diagnosis of <jats:styled-content style=\"fixed-case\">MGUS<\/jats:styled-content> by systemic examination and serum protein electrophoresis in 12 individuals (9 males and 3 females), with a mean age at presentation of 52.2\u00a0years (range 24\u201363\u00a0years) and mean follow\u2010up 6.4\u00a0years (range 0\u201317\u00a0years). The best\u2010corrected visual acuity (BCVA) at presentation ranged from 1.25 to 0.32. In all individuals, the corneal opacities were bilateral. The appearances were diverse and included superficial reticular opacities and nummular lesions, diffuse posterior stromal opacity, stromal lattice lines, superficial and stromal crystalline deposits, superficial haze and a superficial ring of hypertrophic tissue. In one individual, with opacities first recorded at 24\u00a0years of age, we documented the progression of corneal disease over the subsequent 17\u00a0years. In another individual, despite systemic treatment for <jats:styled-content style=\"fixed-case\">MGUS<\/jats:styled-content>, recurrence of deposits was noted following bilateral penetrating keratoplasties. The three individuals initially diagnosed with inherited corneal dystrophy were negative for <jats:italic><jats:styled-content style=\"fixed-case\">TGFBI<\/jats:styled-content><\/jats:italic> mutations by direct sequencing.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>A diagnosis of <jats:styled-content style=\"fixed-case\">MGUS<\/jats:styled-content> should be considered in patients with bilateral corneal opacities. The appearance can mimic corneal dystrophies or cystinosis. In our experience, systemic treatment of <jats:styled-content style=\"fixed-case\">MGUS<\/jats:styled-content> did not prevent recurrence of paraproteinemic keratopathy following keratoplasty.<\/jats:p><\/jats:sec>","DOI":"10.1111\/aos.14123","type":"journal-article","created":{"date-parts":[[2019,5,2]],"date-time":"2019-05-02T08:01:50Z","timestamp":1556784110000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Paraproteinemic keratopathy associated with monoclonal gammopathy of undetermined significance (<scp>MGUS<\/scp>): clinical findings in twelve patients including recurrence after keratoplasty"],"prefix":"10.1111","volume":"97","author":[{"given":"Pavlina","family":"Skalicka","sequence":"first","affiliation":[{"name":"Research Unit for Rare Diseases Department of Pediatrics and Adolescent Medicine First Faculty of Medicine Charles University and General University Hospital Prague Czech 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