Abstract

Relapsing polychondritis (RP), falls into the category of rare diseases. The true incidence and prevalence of this rare disease is unknown. The ocular implications in relapsing polychondritis (RP) are numerous and variable, including mainly inflammation in different structures of the eye. As a complication of this inflammatory condition, a closed secondary angle glaucoma has been described. The purpose of our work is to highlight the diversity of ocular determinations of the same rare disease, including different types of glaucoma that may occur under the same circumstances and to make a detailed analysis of chemical therapies based on drug treatment pathways. The paper includes a report of the cases series admitted to the Clinic of Ophthalmology in St. Andrew Emergency Clinical Hospital, Constanta, between 2007 and 2018, cases analyzed and compared with international literature. One of the cases is a 43-year-old male patient with bilateral open-angle bilateral glaucoma. Other cases with RP, are patients of 41 and 46 years old, respectively, presented with unilateral episcleritis. This case series report aims to show that RP can associate even more protean ocular manifestations than already discussed in the literature, with specific chemical therapies and to emphasize the need for team approach and ophthalmological monitoring in the care of RP patients with chemical therapy (drug treatment) for each patient.

Highlights

  • Relapsing polychondritis (RP) is a severe, episodic, rare, progressive and potentially fatal multisystemic autoimmune granulomatous inflammatory condition involving cartilaginous structures and proteoglycan-rich structures, more often those of the ears, nose, laryngo-tracheo-bronchial system, and those in joints, skin, epiglottis and eye [1]

  • The diagnostic criteria for RP were initially established in 1976 by Mc Adam et al based on the clinical presentation observed in patients [4], but at a later date they were modified by Damiani and Levine [5] and Michet et al [6]

  • The chemical therapy treatment has been applied according to the indicators obtained from the patient's anamnesis

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Summary

Introduction

Relapsing polychondritis (RP) is a severe, episodic, rare, progressive and potentially fatal multisystemic autoimmune granulomatous inflammatory condition involving cartilaginous structures and proteoglycan-rich structures, more often those of the ears, nose, laryngo-tracheo-bronchial system, and those in joints, skin, epiglottis and eye [1]. In 1923 this condition was described by Jaksch-Wartenhorst as "polychondropathy" [2]; in 1960, Pearson et al introduced the term "relapsing polychondrite", to highlight the particular intermittent observing course in 12 patients [3]. The literature studies [11] show that the clinical presentation of RP in children is similar to that of the adult. Neonatal cases are not described [12] This condition (RP) occurs with a similar frequency in both sexes [13], may affect populations from all ethnic groups with very high variability in clinical presentations [14]. Ocular involvement occurs in 14 – 24% of patients at presentation, and eventually the eye is involved in 51-65% of cases. Other studies revealed 50-60% of the cases of RP with ocular manifestations, but rarely as inaugural phenomenon [14]

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