Ocular Manifestations of Relapsing Polychondritis
All content on Eyewiki is protected by copyright law and the Terms of Service. This content may not be reproduced, copied, or put into any artificial intelligence program, including large language and generative AI models, without permission from the Academy.
Relapsing polychondritis (RPC) is a recurrent immune-mediated disease causing recurrent inflammation of cartilaginous and proteoglycan rich tissues, including the ear, nose and trachea but also commonly affecting ocular structures and adnexa. [1]
Disease and Epidemiology
RPC was first described in 1923 by Jaksch-Wartenhorst and is characterized by episodic inflammation of cartilage-rich tissues (external ears, nose, joints, upper and lower respiratory tract) and proteoglycan-rich tissues (eyes, inner ear, heart and blood vessels). [1]This disease can lead to irreversible damage of involved structures and can even be fetal. RPC has an estimated incidence of 3.5 million per year with a slight female preponderance with peak age of onset between the fourth or fifth decade of life.[1] Ocular manifestations can occur in up to 65% of cases. [1]The arthritis of RPC often presents concurrent with eye findings. [1]
Auricular chondritis is the most common systemic manifestation of RPC, found in 60-90% of patients, though this is in initial presenting finding in only 40% of patients. Ear pain and redness is commonly reported. [2]
Pathophysiology and Etiology
The pathogenesis is not fully understood and remains unclear if vasculitis is an commonly associated condition with RPC or if it is the cause of RPC. RPC is understood as an autoimmune disease given the fact that around 30-40% of patients in active phase of disease have circulating anti-Type II collagen antibodies, patients have been shown to have cartilage inflammation on biopsy, and clinical response is seen with systemic anti-inflammatory agents such as steroids and steroid sparing agents. RPC is hypothesized to be a TNF-alpha mediated disease as T-cell clones reactive to type-II collagen has been found inciting a Th1-type autoimmune response producing TNF-alpha. Furthermore, TNF-alpha has been shown to be associated with the induction and release of matrix-degrading proteases from chondrocytes. [1]
Ocular Signs and Findings[1][3]
| Orbit | Proptosis, orbital inflammation, vasculitis of extraocular muscles (particularly lateral rectus) or cranial nerves (CN 3 and 6) |
| Eyelid | Unilateral or bilateral edema, ptosis or lid retraction |
| Conjunctiva | Nonspecific bilateral conjunctival erythema, itching, keratoconjunctivitis sicca, chronic follicular conjunctivitis, subconjunctival hemorrhages |
| Sclera/Cornea | Episcleritis and scleritis (most common occurring in 47 % of patients during their clinical course), PUK, epithelial and stromal corneal infiltrates, ulceration, perforation |
| Iris/Uvea | Uveitis (25% of patients, usually anterior or sclerouveitis), hypopyon uveitis |
| Lens | PSC |
| Retina | CWS, IRH, retinal vasculitis, CRVO, BRVO, retinal artery occlusions, exudative retinal detachments, choroidal detachments, chorioretinitis, CME |
| Optic nerve | Ischemic optic neuropathy, optic neuritis, optic perineuritis, papilledema |
| Other | Dacryocystitis, panophthalmitis, glaucoma, visual field defects (infarction of occipital lobe or optic radiation fibers) |
Diagnosis
RPC is diagnosed clinically with no specific laboratory test available to establish the diagnosis, although patients can have elevated ESR, CRP, anemia, and leukocytosis with this disease. There are a variety of proposed criteria for diagnosis. Commonly used are the McAdam and Damiani and Levine criteria.[1][3]
| Criteria | Definition |
| McAdam | At least 3 of the following 6:
1. Bilateral auricular chondritis 2. Nonerosive seronegative inflammatory polyarthritis 3. Nasal chondritis 4. Ocular inflammation 5. Respiratory tract chondritis 6. Audiovestibular damage |
| Daimani and Levine | At least 1 of the following 3
1. Three McAdam Criteria 2. One McAdam criterion and positive histologic confirmation 3. Two McAdam criteria and a response to corticosteroids or dapsone |
Differential diagnosis
- Polyarteritis nodosa
- Granulomatosis with polyangiitis
- Rheumatoid arthritis
- Cogan syndrome
- Posterior circulation stroke
- Sarcoidosis
- Takayasu Arteritis
General treatment
There has been no randomized clinical trial or guidelines for the treatment for RPC given the rarity of this condition. In general, it involves a step-wise approach of anti-inflammatory modalities with a rheumatologist as topical treatment alone is usually not enough for treatment. Initially for mild inflammation, NSAIDs, dapsone, and colchicine are used, then systemic corticosteroids, followed by immunosuppressants (cyclophosphamide, methotrexate, azathioprine, and cyclosporine), and biologics (TNF-alpha inhibitors).[1][3] Surgical interventions may also be required for ocular complications, including cataract, secondary glaucoma, or corneal perforation.[2]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 Yoo JH, Chodosh J, Dana R. Relapsing polychondritis: systemic and ocular manifestations, differential diagnosis, management, and prognosis. Semin Ophthalmol. 2011 Jul-Sep;26(4-5):261-9. doi: 10.3109/08820538.2011.588653. PMID: 21958172.
- ↑ 2.0 2.1 Fukuda K, Mizobuchi T, Nakajima I, Kishimoto T, Miura Y, Taniguchi Y. Ocular Involvement in Relapsing Polychondritis. J Clin Med. 2021 Oct 26;10(21):4970. doi: 10.3390/jcm10214970. PMID: 34768492; PMCID: PMC8584789.
- ↑ 3.0 3.1 3.2 Fukuda K, Mizobuchi T, Nakajima I, Kishimoto T, Miura Y, Taniguchi Y. Ocular Involvement in Relapsing Polychondritis. J Clin Med. 2021 Oct 26;10(21):4970. doi: 10.3390/jcm10214970. PMID: 34768492; PMCID: PMC8584789.
