Idiopathic Sclerosing Orbital Inflammation in a Relapsing Polychondritis Patient with Impaired Vision

Chrong Reen Wang, Sheng Min Hsu, Ying Chen Chen, Chung Liang Ho, Sheen Yie Fang

Research output: Contribution to journalComment/debate

Abstract

A 55-year-old woman with relapsing polychondritis had progressively enlarged right retro-orbital tumor invading the optic nerve, followed by left retrobulbar infiltrating lesions despite prescription of high-dose corticosteroids and pulse methylprednisolone. Repeated histopathologic analyses showed dense collagen fibers with scanty inflammatory cells, consistent with the diagnosis of idiopathic sclerosing orbital inflammation. This disorder has been recognized as a distinct entity with unique clinical features and coexisting rheumatologic disorders, requiring more focused diagnostic strategies and therapeutic regimens. In summary, we demonstrate a rare ocular manifestation in relapsing polychondritis and emphasize the importance of serial radiological and pathological evaluations in such patients presenting with exophthalmos.

Original languageEnglish
Pages (from-to)E82-E83
JournalJournal of Clinical Rheumatology
Volume25
Issue number6
DOIs
Publication statusPublished - 2019 Sep 1

Fingerprint

Relapsing Polychondritis
Eye Manifestations
Inflammation
Exophthalmos
Methylprednisolone
Optic Nerve
Prescriptions
Adrenal Cortex Hormones
Collagen
Neoplasms
Therapeutics

All Science Journal Classification (ASJC) codes

  • Rheumatology

Cite this

Wang, Chrong Reen ; Hsu, Sheng Min ; Chen, Ying Chen ; Ho, Chung Liang ; Fang, Sheen Yie. / Idiopathic Sclerosing Orbital Inflammation in a Relapsing Polychondritis Patient with Impaired Vision. In: Journal of Clinical Rheumatology. 2019 ; Vol. 25, No. 6. pp. E82-E83.
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Idiopathic Sclerosing Orbital Inflammation in a Relapsing Polychondritis Patient with Impaired Vision. / Wang, Chrong Reen; Hsu, Sheng Min; Chen, Ying Chen; Ho, Chung Liang; Fang, Sheen Yie.

In: Journal of Clinical Rheumatology, Vol. 25, No. 6, 01.09.2019, p. E82-E83.

Research output: Contribution to journalComment/debate

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AU - Hsu, Sheng Min

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AB - A 55-year-old woman with relapsing polychondritis had progressively enlarged right retro-orbital tumor invading the optic nerve, followed by left retrobulbar infiltrating lesions despite prescription of high-dose corticosteroids and pulse methylprednisolone. Repeated histopathologic analyses showed dense collagen fibers with scanty inflammatory cells, consistent with the diagnosis of idiopathic sclerosing orbital inflammation. This disorder has been recognized as a distinct entity with unique clinical features and coexisting rheumatologic disorders, requiring more focused diagnostic strategies and therapeutic regimens. In summary, we demonstrate a rare ocular manifestation in relapsing polychondritis and emphasize the importance of serial radiological and pathological evaluations in such patients presenting with exophthalmos.

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