Ocular Manifestations of Relapsing Polychondritis

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 by Michael T Yen, MD on May 13, 2022.


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 effecting 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]

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][2]

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][2]

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.       Audiovestiublar 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

RA

Congan 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][2]

References

  1. 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. 2.0 2.1 2.2 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.
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