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Severe panuveitis with relapsing polychondritis

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Naonori Masuda
Naonori Masuda
Naonori Masuda
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Ryoko Nishikawa
Ryoko Nishikawa
Ryoko Nishikawa
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Tetsuo Ueda
Tetsuo Ueda
Tetsuo Ueda
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Nahoko Ogata
Nahoko Ogata
Nahoko Ogata
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Abstract

Purpose Relapsing polychondritis is a rare multiorgan disease characterized by repeated episodes of inflammation and deterioration of cartilages. We report a case of relapsing polychondritis that presented with severe panuveitis. Observations A 53-year-old man visited our hospital because of ocular pain in both eyes of 2 weeks' duration. His best-corrected visual acuity was 20/20 in both eyes but he had severe hyperemia of the conjunctiva bilaterally. Inflammation in the anterior segment and vitreous opacity had been getting worse in his right eye. Systemic and topical treatments were not effective, and the visual acuity of the right eye was reduced to hand motion. Thus, pars plana vitrectomy with silicone oil tamponade was performed. After the operation, the vitreous opacities and white lesions in the retina were completely resolved. His visual acuity was improved to 20/20. Three years later, he developed dizziness and swelling of both auricles of his ears, and he was found to have sensorineural deafness. He was diagnosed with relapsing polychondritis after a laryngoscopic examination. Twelve months after the diagnosis, scleritis and panuveitis developed in his left eye, and his visual acuity fell to 20/2000. We performed pars plana vitrectomy with silicone oil tamponade on his left eye. After the vitrectomy, the inflammation of the left eye was resolved. Conclusion and importance: Ophthalmologist should be aware that severe panuveitis with vitreous opacities may be the initial signs of relapsing polychondritis. In addition, vitrectomy was effective for the treatment of the ocular complications.
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American Journal of Ophthalmology Case Reports
journal homepage: www.elsevier.com/locate/ajoc
Case report
Severe panuveitis with relapsing polychondritis
Naonori Masuda, Ryoko Nishikawa, Tetsuo Ueda, Nahoko Ogata
∗
Department of Ophthalmology, Nara Medical University, Kashihara, 634-8522 Nara, Japan
ARTICLE INFO
Keywords:
Relapsing polychondritis
Panuveitis
Type II collagen
Pars plana vitrectomy
ABSTRACT
Purpose: Relapsing polychondritis is a rare multiorgan disease characterized by repeated episodes of in-
flammation and deterioration of cartilages. We report a case of relapsing polychondritis that presented with
severe panuveitis.
Observations: A 53-year-old man visited our hospital because of ocular pain in both eyes of 2 weeks' duration. His
best-corrected visual acuity was 20/20 in both eyes but he had severe hyperemia of the conjunctiva bilaterally.
Inflammation in the anterior segment and vitreous opacity had been getting worse in his right eye. Systemic and
topical treatments were not effective, and the visual acuity of the right eye was reduced to hand motion. Thus,
pars plana vitrectomy with silicone oil tamponade was performed. After the operation, the vitreous opacities and
white lesions in the retina were completely resolved. His visual acuity was improved to 20/20.
Three years later, he developed dizziness and swelling of both auricles of his ears, and he was found to have
sensorineural deafness. He was diagnosed with relapsing polychondritis after a laryngoscopic examination.
Twelve months after the diagnosis, scleritis and panuveitis developed in his left eye, and his visual acuity fell
to 20/2000. We performed pars plana vitrectomy with silicone oil tamponade on his left eye. After the vi-
trectomy, the inflammation of the left eye was resolved.
Conclusion: and importance: Ophthalmologist should be aware that severe panuveitis with vitreous opacities
may be the initial signs of relapsing polychondritis. In addition, vitrectomy was effective for the treatment of the
ocular complications.
1. Introduction
Relapsing polychondritis is a rare multiorgan disease characterized
by repeated episodes of inflammation and deterioration of cartilage.
1
The diagnosis of relapsing polychondritis is made on the basis of clin-
ical symptoms. McAdam et al. established 6 clinical criteria: (1) re-
current chondritis of both auricles; (2) nonerosive inflammatory poly-
arthritis; (3) chondritis of the nasal cartilage; (4) inflammation of the
ocular structures; (5) chondritis of the respiratory tract involving lar-
yngeal or tracheal cartilage; and (6) cochlear or vestibular damage
manifested by neurosensory hearing loss, tinnitus, or vertigo.
2
Because
of its rarity, the pathogenesis of this disease is still unclear, and epi-
demiological studies are not conclusive. Auricular chondritis and
polyarthritis are the most common signs.
The eye is also a target organ.
1
We reported a patient who presented
with ocular pain, scleritis, and severe panuveitis as the manifestations
of relapsing polychondritis.
2. Case report
A 53-year-old man consulted our hospital with bilateral ocular pain
of 2 weeks' duration. At the initial examination, his best-corrected vi-
sual acuity was 20/20 in both eyes but he had severe hyperemia of the
conjunctiva and sclera bilaterally (Fig. 1). Ophthalmoscopy was es-
sentially normal in both eyes. He was diagnosed with scleritis in both
eyes and was treated with topical 0.1% betametazon four times a day.
His laboratory results were within the normal limits, and the results for
antineutrophil antibody, rheumatoid factor, toxoplasma serologic, and
syphilis were negative. A general physical examination by an internist
showed no systemic diseases.
Despite the treatments, the inflammation of the anterior segment
worsened and an inflammation of the posterior segment in the right eye
developed. Anterior chamber examination revealed 2 + cells, and
fundus examination showed severe vitreous opacities and ill-defined
white lesions along the retinal veins (Fig. 2A). He was then treated with
systemic antibiotics, antiviral drug, and predonisone intravenously.
However, his visual acuity decreased to 20/2000 within a few days.
Thus, we performed pars plana vitrectomy with silicone oil
https://doi.org/10.1016/j.ajoc.2018.04.024
Received 5 March 2018; Received in revised form 14 April 2018; Accepted 18 April 2018
∗
Corresponding author. Department of Opthalmology, Nara Medical University, 840 Shijo-cho, Kashihara 634-8522, Japan.
E-mail address: ogata@naramed-u.ac.jp (N. Ogata).
American Journal of Ophthalmology Case Reports 11 (2018) 3–5
Available online 20 April 2018
2451-9936/ © 2018 Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
T
tamponade on his right eye. After the operation, the vitreous opacities
and white lesions in the retina were completely resolved ( Fig. 2B). The
visual acuity of the right eye improved to 20/20, and there were no
recurrences for at least 3 years.
Three years after the initial presentation, he developed dizziness,
and we referred him to an otolaryngologist in our hospital. The auricles
of both ears were swollen (Fig. 3) and he had sensorineural deafness.
The patient was diagnosed with relapsing polychondritis by the lar-
yngoscopic examination. His dizziness and swelling of his auricles of
ears were resolved with oral predonisone 10 mg/day.
Twelve months after the diagnosis, scleritis and panuveitis devel-
oped in his left eye, and his visual acuity fell to 20/2000. The fundus in
his left eye was not visible due to vitreous opacities. He was treated
with 0.1% betametazon four times a day but the eye did not respond to
the therapy. We performed pars plana vitrectomy with silicone oil
tamponade on his left eye. After the vitrectomy, the inflammation of the
left eye was resolved, and there have been no recurrences with con-
tinued treatment of topical 0.1% betametazone four times a day.
3. Discussion
As best we know, there have been no reports of severe panuveitis
which required pars plana vitrectomy in cases of relapsing poly-
chondritis. Nicholas et al. reported uveitis with hypopyon in two cases
of relapsing polychondritis.
3
They reported that the inflammation was
localized to the anterior segment, and dilated ophthalmoscopic ex-
aminations showed no vitreous cell, no signs of retinitis, and normal
optic discs. In our case, severe panuveitis was detected at the initial
examination, and pars plana vitretomy was necessary. Paroli et al. re-
ported uveitis with retinal occlusive vasculitis as a first symptom of
relapsing polychondritis.
4
In their case, slit-lamp examination showed
1 + vitreous cells, and fluorescein angiography revealed dye leakages
Fig. 1. Slit-lamp photograph of our patient at the first visit showing severe hyperemia bilaterally. His best-corrected visual acuity was both 20/20 in both eyes.
Fig. 2. Fundus photographs showing severe vitreous opacities and ill-defined white lesions located along the retinal veins (A). After pars plana vitrectomy with
silicone oil tamponade, the vitreous opacities and white lesions in the retina are resolved (B).
Fig. 3. Photographs of swelling and reddish auricles in both ears.
N. Masuda et al. American Journal of Ophthalmology Case Reports 11 (2018) 3–5
4
and vascular staining of both retinal arteries and veins. There was in-
flammation of the posterior segment but it was not so severe that pars
plana vitrectmy was required.
The vitreous consist of 98% of water and extracellular matrix. The
major extracellular matrix macromolecules are collagen type II and
hyaluronic acid.
5
Several studies have reported the presence of anti-
bodies for cartilage and collagen type II in the serum of patients with
relapsing polychondritis.
6,7
It is quite possible that these antibodies for
collagen type II caused the inflammation in the vitreous, and thus vi-
trectomy with the removal of the type II collagen was effective.
In conclusion, severe panuveitis accompanying relapsing poly-
chondritis is a rare condition and vitrectomy is an effective treatment.
Patient consent
Consent to publish the case report was obtained. This report does
not contain any personal information that could lead to the identifi-
cation of the patient.
Funding
No funding or grant support.
Authorship
All authors attest that they meet the current ICMJE criteria for
Authorship.
Conflicts of interest
The authors have no financial disclosures relating this topic.
Acknowledgements
None.
References
1. Cantarini L, Vitale A, Brizi MG, et al. Diagnosis and classification of relapsing poly-
chondritis. J Autoimmun. 2014;48–49:53–59.
2. McAdam LP, O'Hanlan MA, Bluestone R, et al. Relapsing polychondritis: prospective
study of 23 patients and a review of the literature. Medicine (Batimore).
1976;55:193–215.
3. Anderson NG, Garcia-Valenzuela E, Martin DF. Hypopyon uveitis and relapsing
polychondritis: a report of 2 patients and review of autoimmune hypopyon uveitis.
Ophthalmology. 2004;111(6):1251–1254.
4. Paroli MP, Priori R, Spinucci G, et al. Uveitis with retinal occlusive vasculitis and
sensorineural hypoacusia as first symptoms of relapsing polychondritis. Clin Exp
Rheumatol. 2012;30(1):S101–S103.
5. Lutty GA. Anti-angiogenic properties of vitreous. Encycl Eye. 2010:112–119.
6. Foidart JM, Abe S, Martin GR, et al. Antibodies to type II collagen in relapsing
polychondritis. N Engl J Med. 1978;299(22):1203–1207.
7. Ebringer R, Rook G, Swana GT, et al. Autoantibodies to cartilage and type II collagen
in relapsing polychondritis and other rheumatic diseases. Ann Rheum Dis.
1981;40(5):473–479.
N. Masuda et al. American Journal of Ophthalmology Case Reports 11 (2018) 3–5
5
... Although there has been one large-scale R. Tanaka (&) Á T. Kaburaki Á H. Nakahara Á K. Komae Department of Ophthalmology, The University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan e-mail: rtanaka-ymn@umin.ac.jp survey of patients with RP from Japan, this report contains only the numbers of patients with ocular inflammation and the types of ocular inflammation found, without the mention of specific details on ocular inflammation-associated RP [3]. Other than this, there are few case reports on this subject in Japan [13][14][15][16]. ...
... Of the 110 patients, 62 had scleritis, 36 had conjunctivitis, and 26 had uveitis; however, they did not report any additional details. To the best of our knowledge, there have been four case reports (five patients) from Japan on RP-associated ocular inflammation that are written in English [13][14][15][16]. These reports identified the following types of ocular inflammation: diffuse scleritis (two cases), posterior scleritis (one case), episcleritis (one case), and panuveitis (one case). ...
... According to previous reports, uveitis occurs less [3,5]. There have been some reported cases of panuveitis [15,21,[23][24][25][26] or hypopyon uveitis [21,[27][28][29] associated with RP. Two patients in the present study had anterior uveitis without hypopyon. ...
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