Reviews
Rare within rare. Necrotising scleritis and peripheral ulcerative keratitis: eye-threatening complications of relapsing polychondritis
L. Damian1, C. Pamfil2, C. Bucșa3, C. Nicula4, L. Mouthon5, Z. Amoura6, M. Cutolo7, G.R. Burmester8, J.E. Fonseca9, L. Grapini10, L. Arnaud11, S. Rednic12
- Rheumatology Department, County Emergency Clinical Hospital Cluj, Centre for Rare Musculoskeletal Autoimmune and Autoinflammatory Diseases, Cluj-Napoca, Romania. ldamian.reumatologie@gmail.com
- Rheumatology Department, County Emergency Clinical Hospital Cluj, Centre for Rare Musculoskeletal Autoimmune and Autoinflammatory Diseases, Cluj-Napoca, and Iuliu Hațieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
- Drug Information Research Centre, Iuliu Hațieganu University of Medicine and Pharmacy, Cluj-Napoca, and ASPOR Romanian Association of Relapsing Polychondritis Patients, Cluj-Napoca, Romania.
- Ophthalmology Department, Iuliu Hațieganu University of Medicine and Pharmacy, Cluj-Napoca, County Emergency Clinical Hospital Cluj, Romania.
- Service de Médecine Interne, Hôpital Cochin, Centre de Référence Maladies Systémiques Autoimmunes Rares d’Ile de France, Assistance Publique-Hôpitaux de Paris (AP-HP), Université de Paris, France.
- Service de Médecine interne, Hôpital Pitié-Salpêtrière, Centre National de Référence pour le lupus, le syndrome des antiphospholipides et autres maladies auto-immunes rares, Assistance Publique Hôpitaux de Paris, France.
- Laboratory for Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Italy.
- Department of Rheumatology and Clinical Immunology, Charité University Medicine, Berlin, Germany.
- Serviço de Reumatologia e Doenças Ósseas Metabólicas, Centro Hospitalar Universitário Lisboa Norte, Lisboa, and Unidade de Investigação em Reumatologia, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Portugal.
- ASPOR Romanian Association of Relapsing Polychondritis Patients, Cluj-Napoca, Romania.
- Service de rhumatologie, Hôpitaux Universitaires de Strasbourg, Centre National de Référence des Maladies Systémiques et Auto-immunes Rares Grand-Est Sud-Ouest (RESO), Strasbourg, France.
- Rheumatology Department, County Emergency Clinical Hospital Cluj, Centre for Rare Musculoskeletal Autoimmune and Autoinflammatory Diseases, Cluj-Napoca, and Iuliu Hațieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
CER15379
2022 Vol.40, N°5 ,Suppl.134
PI 0086, PF 0092
Reviews
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PMID: 35238768 [PubMed]
Received: 29/11/2021
Accepted : 07/02/2022
In Press: 24/02/2022
Published: 18/05/2022
Abstract
OBJECTIVES:
Relapsing polychondritis (RP) evolves with variable and intermittent involvement of cartilage and proteoglycan-rich structures. Ocular manifestations are present in up to two-thirds of RP patients. Necrotising scleritis (NS) and peripheral ulcerative keratitis (PUK) may be inaugural and may lead to eye perforation and vision loss. We aimed to review NS and PUK in RP, in order to characterise them, to identify successful treatment options and unmet needs.
METHODS:
A systematic review of the currently available evidence in PubMed, EMBASE and Scopus was performed according to PRISMA, including observational studies, single case reports and case series of NS/PUK in RP. Study design, number of patients, age, gender, treatment and outcome, were extracted. Two RP patients also provided their opinion.
RESULTS:
Five case reports and two case series were eligible for inclusion. We identified 10 RP patients with eye-threatening complications (NS and/or PUK), 9 adults (2 males, 7 females, aged 35-72, median age 57.6 years) and one paediatric patient (F, 11 years). Apart from glucocorticoids, cyclophosphamide was effective in 4 patients; infliximab, high-dose immunoglobulins, dapsone, or cyclosporine were also successfully employed in a case each. Surgical repair was reported in 2 cases.
CONCLUSIONS:
Ocular inflammation is often bilateral and recurring in RP; NS/PUK are rare complications. All patients who develop NS/PUK should be specifically questioned for RP signs and symptoms. Early institution of immunosuppressive therapies is mandatory. Increasing awareness, physicians’ and patients’ education and a multidisciplinary approach may help improve the prognosis of these serious complications of RP.
