No full-text available
To read the full-text of this research,
you can request a copy directly from the authors.
Visual loss in relapsing polychondritis: Rapid progression of orbital inflammation
... Although it is uncommon, idiopathic orbital inflammation and inflammatory pseudotumor have been observed in RPC, leading to proptosis, periorbital lid edema, eye pain, and restriction of extraocular movements [10,24,25,[64][65][66][67][68][69][70][71][72][73]. Proptosis with chemosis due to orbital inflammation may be the first manifestation of RPC [25]. ...
... Proptosis with chemosis due to orbital inflammation may be the first manifestation of RPC [25]. Orbital inflammation sometimes affects the cranial nerve and induces oculomotor nerve palsy, optic perineuritis, and tumor invasion of the optic nerve [65,67,68]. Although a biopsy may sometimes be difficult due to the lesion location, biopsy of the orbital mass may be helpful to confirm the type of inflammation and exclude malignancy. ...
Relapsing polychondritis (RPC) is a rare systemic immune-mediated disease characterized by recurrent inflammation of cartilaginous and proteoglycan-rich tissues throughout the body. Auricular, nasal, tracheal, and articular chondritis and arthritis are common systemic symptoms in patients with RPC. Ocular tissues are also targets of inflammation in RPC, and a variety of ocular symptoms are observed in approximately half of the patients with RPC. Scleritis/episcleritis, uveitis, and conjunctivitis are common symptoms associated with RPC. Less frequently, keratitis, retinopathy, optic neuropathy, muscle palsy, and orbital inflammation are also observed. Ocular inflammation could also be the first manifestation of RPC. Although RPC is a potentially fatal and sight-threatening disease, the rarity of the disease and its protean clinical presentation may lead to delayed diagnosis or misdiagnosis. Given the high prevalence of ocular involvement in RPC, to avoid misdiagnosis, physicians should be suspicious of RPC when they see patients with recurrent ocular inflammatory conditions and various systemic symptoms. In this article, we provide a comprehensive review of ocular manifestations associated with RPC.
Relapsing polychondritis is a rare disease characterized by cartilage inflammation. Our aim was to estimate the incidence, prevalence and mortality of relapsing polychondritis and describe the clinical features of relapsing polychondritis in a large population.
All participants diagnosed with relapsing polychondritis were sampled from the Clinical Practice Research Datalink. Prevalence and incidence rates for 1990-2012 were estimated. Relative mortality rates were estimated in a time-to-event framework using reference UK life tables. A questionnaire validation study assessed diagnostic accuracy.
There were 117 participants with relapsing polychondritis ever recorded. Fifty (82%) of 61 cases were validated by a physician and unconfirmed cases were excluded. The analysis included 106 participants (42 men, 64 women) diagnosed with relapsing polychondritis. The mean age (range) at diagnosis in men was 55 (range 17-81) years and in women 51 (range 11-79) years. The median interval from first symptom to diagnosis was 1.9 years. The incidence of relapsing polychondritis between 1990 and 2012 was 0.71 (95% CI 0.55, 0.91) per million population per year. There were 19 deaths from any cause. There were 16 observed deaths eligible for survival analysis and 7.4 deaths expected for the UK population of the same age, sex and period. The standardized mortality ratio was 2.16 (95% CI 1.24, 3.51), P < 0.01. Respiratory disease, cardiac conditions and cancer were the most frequent causes of death.
The incidence of relapsing polychondritis may be lower than previously estimated, and diagnostic misclassification and delay are common. Mortality in relapsing polychondritis is more than twice that of the general population.
© The Author 2015. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.
Relapsing polychondritis (RP) is a rare systemic autoimmune disease characterized by episodic inflammation of cartilaginous tissues throughout the body. It is distinguished by recurrent bouts of inflammation, which lead to the permanent destruction of the involved structures. It can be a life-threatening, debilitating, and frightening disease and is often difficult to diagnose in its early stages. Ocular manifestations have been reported to occur in up to 65% of cases and include proptosis, eyelid edema, extraocular muscles palsy, episcleritis, scleritis, conjunctivitis, corneal infiltrate, peripheral ulcerative keratitis, corneal thinning or perforation, iridocyclitis, cataract, retinopathy, exudative retinal detachment, and optic neuritis. Corticosteroids remain the mainstay of treatment for RP; however, other treatment modalities include nonsteroidal anti-inflammatory drugs, colchicine, dapsone, and immunomodulatory drugs. This article reviews the literature and summarizes the epidemiology, pathogenesis, clinical features, treatment, and prognosis of the systemic and ocular manifestations of RP.
The purpose of this study was to report a case of bilateral blindness, bilateral necrotizing scleritis, and bilateral deafness in a patient with myelodysplastic syndrome (MDS). In such a patient, the possibility of relapsing polychondritis (RPC) associated with MDS must be considered. CASE REPORT/METHODS: A 66-year-old patient suffered from myelodysplastic syndrome (MDS). Shortly before his death, he became bilaterally blind and deaf. A biopsy was taken from the conjunctiva and the bone marrow, and both eyes were obtained after death for further investigation. Findings of the clinical and laboratory work-up for the patient's hematologic disorder as well as an examination of the eyes by light microscopy and immunohistochemistry are presented.
Ocular sections showed a diffuse necrotizing scleritis with moderate uveitis and no identifiable infectious agent. Neither was there any evidence of a leukemic infiltration. The deafness had been due to inner ear failure, and the patient died of a cardiac failure.
Non-infectious scleritis associated with inner ear deafness is a strong indication of relapsing polychondritis (RPC). Furthermore, RPC can be associated with MDS. Thus, in addition to leukemic infiltration and infection involving ocular structures, ophthalmologists and otolargyngologists should be aware of the association between MDS and RPC and the potential complications.
Optic neuropathy is an uncommon manifestation of relapsing polychondritis (RPC), a rare systemic disease affecting cartilaginous and proteoglycan-rich structures. The optic neuropathy has been attributed to ischemia, intrinsic inflammation of the optic nerve, or spread of inflammation to the nerve from adjacent intraconal orbital tissues. We report a case of recurrent corticosteroid-responsive optic neuropathy in which MRI did not show ocular, optic nerve, or intraconal orbital abnormalities but did show periosteal thickening and enhancement in the apical orbit and adjacent intracranial space consistent with periostitis. The periostitis, which is a manifestation of a systemic vasculitis or an autoimmune reaction to progenitors of cartilage, probably caused the optic neuropathy by compression or inflammation. It is important to recognize this mechanism of optic neuropathy as its imaging features may be a subtle yet critical clue to an underlying systemic condition that can be life-threatening if not properly managed.
Relapsing polychondritis is an autoimmune disease that primarily manifests as cartilaginous tissue destruction. However, the immune impairment may also involve noncartilaginous tissues such as kidneys, blood vessels, etc. The disease may occur as a primary disorder or in association with other diseases. The case of a female patient with concurrent manifestation of myelodysplastic syndrome-refractory anemia with excess blasts, and relapsing polychondritis, is presented. The diagnosis of relapsing polychondritis was established ten months after the diagnosis of myelodysplastic syndrome, when the criteria for the former were met. Total hearing loss and blindness developed soon thereafter.