PURPOSE: To describe the ocular findings of 12 subjects with paraproteinemic keratopathy associated with monoclonal gammopathy of undetermined significance (MGUS). METHODS: Ocular examination included corneal spectral domain optical coherence tomography. In three individuals with an initial diagnosis of a lattice or Thiel-Behnke corneal dystrophy, the TGFBI gene was screened by conventional Sanger sequencing. RESULTS: We confirmed a diagnosis of MGUS by systemic examination and serum protein electrophoresis in 12 individuals (9 males and 3 females), with a mean age at presentation of 52.2 years (range 24-63 years) and mean follow-up 6.4 years (range 0-17 years). The best-corrected 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 years of age, we documented the progression of corneal disease over the subsequent 17 years. In another individual, despite systemic treatment for MGUS, recurrence of deposits was noted following bilateral penetrating keratoplasties. The three individuals initially diagnosed with inherited corneal dystrophy were negative for TGFBI mutations by direct sequencing. CONCLUSION: A diagnosis of MGUS should be considered in patients with bilateral corneal opacities. The appearance can mimic corneal dystrophies or cystinosis. In our experience, systemic treatment of MGUS did not prevent recurrence of paraproteinemic keratopathy following keratoplasty.
- MeSH
- dospělí MeSH
- keratoplastika perforující škodlivé účinky MeSH
- konfokální mikroskopie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladý dospělý MeSH
- monoklonální gamapatie nejasného významu komplikace MeSH
- následné studie MeSH
- optická koherentní tomografie MeSH
- paraproteinemie komplikace diagnóza MeSH
- předpověď * MeSH
- recidiva MeSH
- rohovka patologie MeSH
- senioři MeSH
- vyšetření štěrbinovou lampou MeSH
- zákal rohovky diagnóza etiologie MeSH
- zraková ostrost MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- MeSH
- extracelulární matrix účinky léků MeSH
- intravenózní podání MeSH
- keratitida farmakoterapie komplikace MeSH
- klinické zkoušky jako téma MeSH
- kyselina askorbová * terapeutické užití MeSH
- lidé MeSH
- ortomolekulární terapie * MeSH
- výsledek terapie MeSH
- zákal rohovky * diagnóza etiologie farmakoterapie MeSH
- Check Tag
- lidé MeSH
