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Global Consensus on Definition, Classification, Diagnosis, and Staging of Limbal Stem Cell Deficiency

Deng, Sophie X., MD, PhD*; Borderie, Vincent, MD, PhD; Chan, Clara C., MD; Dana, Reza, MD, MSc, MPH§; Figueiredo, Francisco C., MD, PhD, FRCOphth; Gomes, José A. P., MD, PhD; Pellegrini, Graziella, PhD**; Shimmura, Shigeto, MD††; Kruse, Friedrich E., MD‡‡ and The International Limbal Stem Cell Deficiency Working Group

doi: 10.1097/ICO.0000000000001820
Special Article

Purpose: Despite extensive knowledge gained over the last 3 decades regarding limbal stem cell deficiency (LSCD), the disease is not clearly defined, and there is lack of agreement on the diagnostic criteria, staging, and classification system among treating physicians and research scientists working on this field. There is therefore an unmet need to obtain global consensus on the definition, classification, diagnosis, and staging of LSCD.

Methods: A Limbal Stem Cell Working Group was first established by The Cornea Society in 2012. The Working Group was divided into subcommittees. Four face-to-face meetings, frequent email discussions, and teleconferences were conducted since then to obtain agreement on a strategic plan and methodology from all participants after a comprehensive literature search, and final agreement was reached on the definition, classification, diagnosis, and staging of LSCD. A writing group was formed to draft the current manuscript, which has been extensively revised to reflect the consensus of the Working Group.

Results: A consensus was reached on the definition, classification, diagnosis, and staging of LSCD. The clinical presentation and diagnostic criteria of LSCD were clarified, and a staging system of LSCD based on clinical presentation was established.

Conclusions: This global consensus provides a comprehensive framework for the definition, classification, diagnosis, and staging of LSCD. The newly established criteria will aid in the correct diagnosis and formulation of an appropriate treatment for different stages of LSCD, which will facilitate a better understanding of the condition and help with clinical management, research, and clinical trials in this area.

*Cornea Division, Stein Eye Institute, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA;

Quinze-Vingts National Eye Hospital, Faculté de Médecine Sorbonne Université, Paris, France;

Department of Ophthalmology & Vision Sciences Toronto, University of Toronto, ON, Canada;

§Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA;

Department of Ophthalmology, Royal Victoria Infirmary and Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom;

Department of Ophthalmology and Visual Sciences, Escola Paulista de Medicina, Universidade Federal de São Paulo (EPM/UNIFESP), Brazil;

**Holostem Terapie Avanzate, Centre for Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy;

††Department of Ophthalmology, Keio University School of Medicine, Tokyo, Japan; and

‡‡Department of Ophthalmology, University Hospital Erlangen and Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), Erlangen, Germany.

Correspondence: Sophie X. Deng, MD, PhD, Stein Eye Institute, University of California, Los Angeles, 100 Stein Plaza, Los Angeles, CA 90095 (e-mail: deng@jsei.ucla.edu).

Supported by the Cornea Society.

C. C. Chan is a consultant or receives grants from Alcon Labs Inc, Allergan, Bausch & Lomb, Santen, Shire, Johnson & Johnson, Tearlab; V. Borderie is a consultant for Chiesi Farmaceutici S.P.A and Dompe; R. Dana receives grant support from the National Eye Institute (EY-12963) and Allergan, and is a consultant for Shire, Dompe, Santen; S. X. Deng receives grants from the National Eye Institute (R01EY021797) and California Institute for Regenerative Medicine (CLIN1-08686) and is a consultant for Chiesi Farmaceutici S.P.A; F. C. Figueiredo is a consultant or receives research grants from Chiesi Farmaceutici S.P.A, Dompe, Santen, Shire and Théa; J. A. P. Gomes is a consultant or receives grant from Allergan, Alcon, MSD, Bausch & Lomb/Valeant, Mundipharma, Ofta Vision Health, Genon, Grin, Shire, FAPESP, Capes, Cnpq; P. Pellegrini is a shareholder of Holostem Terapie Avanzate and a consultant for J-TEC; S. Shimmura receives research funds from Santen Pharmaceutical Co, Rohto Pharmaceuticals, Sumitomo Dainippon Pharma; F. E. Kruse receives travel support from Santen Pharmaceutical Co, Ltd, and Kowa Company, Ltd.

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Web site (www.corneajrnl.com).

Received August 16, 2018

Accepted September 30, 2018

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