Scleritis in a Patient with Castleman Disease

Jae Jung Lee; In Ho Lee; Kang Yeun Park; Sung Who Park; Ji Eun Lee

doi:10.3341/jkos.2018.59.8.785

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Lee, Lee, Park, Park, and Lee: Scleritis in a Patient with Castleman Disease

Case Report

J Korean Ophthalmol Soc 2018;59(8):785-789.

Published online: 25 September 2018

DOI: https://doi.org/10.3341/jkos.2018.59.8.785

Scleritis in a Patient with Castleman Disease

Jae Jung Lee, MD^1,², In Ho Lee, MD^1,², Kang Yeun Park, MD³, Sung Who Park, MD^1,², Ji Eun Lee, MD, PhD^1,^2,

¹Department of Ophthalmology, Pusan National University School of Medicine, Yangsan, Korea

²Medical Research Institute, Pusan National University Hospital, Busan, Korea

³Department of Ophthalmology, Haeundae Paik Hospital, Inje University College of Medicine, Busan, Korea

Address reprint requests to Ji Eun Lee, MD, PhD Department of Ophthalmology, Pusan National University Hospital, #179 Gudeok-ro, Seo-gu, Busan 49241, Korea Tel: 82-51-240-7957, Fax: 82-51-240-7341 E-mail: jlee@pusan.ac.kr

Received 10 May 2018 Revised 30 May 2018 Accepted 31 July 2018

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

To report a case of multicentric Castleman disease that presented with scleritis.

Case summary

A 42-year-old male presented with decreased visual acuity in the left eye. Castleman disease had been diagnosed 21 months before and treated with systemic steroids and combined chemotherapy. Best-corrected visual acuity (BCVA) of the left eye was 0.02 and the intraocular pressure was 42 mmHg. Scleral edema and corneal edema were noted using a slit lamp examination. The anterior chamber cell was 2+ according to Standardization of Uveitis Nomenclature criteria. The fundus was invisible due to the anterior segment lesion. After one month, scleritis developed in the right eye and the patient complained of ocular pain. Topical steroids and non-steroidal anti-inflammatory drugs were prescribed. Due to recurrent scleritis and anterior uveitis, cataract extraction and laser iridectomy were performed on the left eye, and systemic steroids and the antimetabolite methotrexate were started. After 9 years of follow-up, all medications were stopped and there was no recurrence of inflammation, with a BCVA of 1.0 in both eyes.

Conclusions

Treatment of a patient with scleritis accompanied with Castleman disease using systemic steroids and methotrexate resulted in a good prognosis.

Keywords: Castleman disease, Scleritis

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Figure 1.

Clinical and pathological findings of the patient. (A) Bihilar lymphadenopahy was noted in chest X-ray. (B) Computed tomography abdomen revealed multiple low density mass in both kidneys. (C) Histopathologic finding of inguinal lymph node revealed atypically proliferative follicular hyperplasia and increased interfollicular vascularity. Hyalinized germinal center was surrounded by lymphocytes, producing the “onion-skin appearance” (hematoxylin and eosin [H&E] stain, ×100).

Figure 2.

Slit lamp examination of the affected eye at presentation. (A, B) After phacoemulsification and iridectomy, diffuse scleritis persisted with scleral thickening and episcleral vessel engorgement.

Figure 3.

Slit lamp examination at 9 years of follow up. Best corrected visual acuity was 1.0 in both eyes. (A) The patent iridectomy is noted with no inflammatory signs in the right eye. (B) There was no sign of inflammation in the anterior chamber and sclera in the left eye.

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