P130 A vision in peril: the silent march of relapsing polychondritis

Abstract Introduction Relapsing polychondritis (RP) is a rare, multisystem autoimmune disorder characterised by recurrent inflammation of cartilaginous structures. Ocular involvement, particularly posterior scleritis, is uncommon but can lead to significant morbidity if not promptly diagnosed and treated. This case report highlights the diagnostic challenges and management strategies for a patient with RP presenting with posterior scleritis, emphasizing the importance of a multidisciplinary approach. Case description A 73-year-old male presented to the eye casualty department with left upper eyelid swelling, exophthalmos, and new-onset visual loss in the right eye. He reported progressive bilateral ocular discomfort and blurred vision, despite multiple prior attendances. Initially, he was managed for orbital cellulitis and scleritis with intravenous antibiotics, without significant improvement. While admitted on an ophthalmology ward, he started noticing sore throat and hoarseness of voice with reduction in vision of the left eye. Subsequently, he developed bilateral posterior scleritis. His left eye vision deteriorated to no perception of light, and his right eye vision was reduced to light and shape perception only. Over the preceding months, the patient had experienced bilateral auricular swelling raising suspicion of chondritis. He also reported nasal bridge deformity and found to have a nasal bridge collapse on assessment by the ENT team. He had a history of bilateral angle-closure glaucoma and was already under ophthalmology care. Systemic examination and clinical correlation of past and present signs and symptoms revealed bilateral auricular chondritis, nasal saddle deformity, and reduced extra-ocular movements (left more than right), with no features of systemic vasculitis. The case was managed with a multidisciplinary team (MDT) approach and discussed in the rheumatology clinical meeting, where a diagnosis of relapsing polychondritis (RP) was established. Initially, management was carried out with IV methylprednisolone for three days; this provided mild relief and clinical and biochemical profile improvement. A decision to provide the patient with early treatment with cyclophosphamide was made; this was administered while the patient was still an inpatient. The patient had good response to cyclophosphamide and moderately gained back his vision in the right eye from barely identifying light to managing to count fingers and appreciating shapes and faces. Post-initial treatment, oral steroids in form of prednisolone with gradual weaning were introduced and methotrexate was commenced as DMARD of choice. With the aforementioned treatment modalities, the patient has shown respectable improvement, gaining about 70% of his right eye vision back with no recurrences of auricular, laryngeal or nasal symptoms. Discussion This case underscores the sight-threatening potential of relapsing polychondritis (RP), particularly when it presents with posterior scleritis, a rare and often misdiagnosed condition. The rarity of posterior scleritis in RP patients poses significant diagnostic challenges, leading to delays in appropriate treatment and, consequently, irreversible visual loss. In this case, despite earlier loss of vision in the left eye and significant damping in the right eye, timely identification and diagnosis from rheumatology and commencement of aggressive immunosuppressive therapy resulted in the prevention of permanent visual impairment in the right eye. The multidisciplinary approach was crucial in managing this complex case. Collaboration between rheumatology, ENT and ophthalmology ensured comprehensive care, addressing the various manifestations of the disease. For instance, the involvement of ophthalmologists was vital in diagnosing posterior scleritis, while rheumatologists managed the treatment and management aspects of RP. Early and accurate diagnosis, followed by prompt treatment, is critical in preventing irreversible damage in RP patients. This case highlights the need for heightened awareness and consideration of RP in patients presenting with ocular symptoms and chondritis. Comparing this case with existing literature reveals that timely intervention can significantly improve outcomes, emphasizing the importance of early multidisciplinary review. Key learning points This case report emphasises the critical importance of early diagnosis and treatment in preventing irreversible visual loss in patients with RP presenting with posterior scleritis. The multidisciplinary approach is essential in managing such complex cases, ensuring comprehensive care and addressing the various manifestations of the disease. We also emphasise the importance of committing to appropriate immunosuppressive therapy and promptly providing and delivering treatment. This case also highlights the uncommon presentation of RP and the importance of approaching a complex presentation with an open mind, yet focusing on thorough history taking, examination and clinical correlation. Finally, raising awareness among clinicians about the diverse presentations of RP can aid in earlier diagnosis and better patient outcomes.