Relapsing polychondritis in an asthma clinic: a pulmonologist perspective

Objectives: To report a case of profound bilateral sensorineural hearing and vestibular loss from relapsing polychondritis and hearing outcomes after cochlear implantation. Methods: Case report and literature review. Results: A 43 year-old woman developed sudden loss of hearing and balance that progressed over several weeks to bilateral, profound hearing and vestibular loss. Steroid treatments were ineffective. She underwent vestibular physical therapy and left cochlear implantation. About 10 months after her initial presentation, she developed erythema, warmth, swelling, and pain of the left auricle sparing the lobule, flattening of the bridge of her nose, and right ankle swelling, warmth, and skin erythema. A biopsy of the left auricle revealed histopathologic findings consistent with relapsing polychondritis. She was treated with high dose prednisolone. The ear inflammation resolved, however, despite excellent auditory response to pure tone thresholds, the patient reported no improvement in speech perception after cochlear implantation. Conclusions: Relapsing polychondritis can present with rapidly progressive, profound loss of hearing and vestibular function. Hearing outcomes after cochlear implantation can include poor speech discrimination despite good pure tone detection thresholds.

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G304(P) A case of relapsing polychondritis in a paediatric patient with severe airway involvement – the challenges of long term airway & respiratory management

10.1136/archdischild-2020-rcpch.265 ◽

2020 ◽

Author(s):

T Kothari ◽

E Magnussen ◽

P Dawson ◽

E Al Abadi ◽

M Elloy ◽

...

Keyword(s):

Paediatric Patient ◽

Relapsing Polychondritis ◽

Respiratory Management

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Clinical patterns and the evolution of relapsing polychondritis based on organ involvement: a Chinese retrospective cohort study

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-021-01861-x ◽

2021 ◽

Vol 16 (1) ◽

Author(s):

Lei Zhang ◽

Shuang Yun ◽

Tiange Wu ◽

Yujie He ◽

Jinyan Guo ◽

...

Keyword(s):

Early Recognition ◽

Age At Onset ◽

Relapsing Polychondritis ◽

Organ Involvement ◽

Strong Negative Correlation ◽

Negative Pattern ◽

Inflammatory Status ◽

Clinical Patterns ◽

Systemic Form

Abstract Background Relapsing polychondritis (RPC) is a rare autoimmune disease and its early diagnosis remains challenging. Defining the clinical patterns and disease course may help early recognition of RPC. Results Sixty-six males and 60 females were included in this study. The average age at onset were 47.1 ± 13.8 years and the median follow-up period was 18 months. Correlation analysis revealed a strong negative correlation between airway involvement and auricular chondritis (r = − 0.75, P < 0.001). Four distinct clinical patterns were identified: Ear pattern (50.8%), Airway pattern (38.9%), Overlap pattern (4.8%) and Airway-Ear negative pattern (5.6%), and patients with Ear pattern and Airway pattern were further divided into limited and systemic form of RPC (27.8% with limited form of Ear pattern and 24.6% with limited form of Airway pattern initially). During follow-up, a minority of patients with Ear pattern and Airway pattern progressed into Overlap pattern, and some Airway-Ear negative pattern patients progressed into Ear pattern. While a large majority of limited RPC patients remained limited form during follow-up, a minority of limited RPC patients progressed into systemic form. Patients with Ear pattern had the highest survival rate and relatively lower inflammatory status. Conclusions RPC patients can be categorized as 4 different clinical patterns and 2 distinct presenting forms (limited and systemic) based on organ involvement. The clinical patterns and presenting forms may evolve during follow-up. Our findings may facilitate early recognition of this rare disease.

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