https://orcid.org/0000-0002-3668-4454
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Evangelia Zampeli, Haralampos M Moutsopoulos, Relapsing polychondritis: a diagnosis not to be missed, Rheumatology, Volume 57, Issue 10, October 2018, Page 1768, https://doi.org/10.1093/rheumatology/key124
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A 33-year-old healthy woman developed, 5 months prior to our evaluation, inflammation of the right ear pinna, without preceding ear trauma/piercing/insect bite. Despite treatment with different broad-spectrum antibiotics, pinna abscesses developed, which were drained through skin incisions. At presentation in our clinic, the right pinna was inflamed and deformed (cauliflower ear, Fig. 1A) and the left pinna was red and swollen. A diffuse erythroviolaceous swelling of the second and fourth toes with a dactylitis-like picture was evident (Fig. 1B). The ESR (75 mm/h, <20 mm/h) and CRP (69 mg/L, <5 mg/l) were increased, while the autoantibody profile (RFs/ANA/p-and-cANCA) was negative. The diagnosis of relapsing polychondritis was undertaken and methylprednisolone (0.5 mg/kg/day) in combination with AZA (1.5 mg/kg/day) was initiated. Two months later, auricular inflammation (Fig. 1C) and arthritis (Fig. 1D) had regressed; nonetheless, pinna deformity remained.
Relapsing polychondritis is a systemic disease causing inflammation of cartilaginous structures in multiple organs [1]. Clinical phenotypes of relapsing polychondritis follow two patterns: either nasal cartilage/airway involvement or external ear involvement. The latter subgroup, as in the case herein, often presents with joint involvement [2]. Early, accurate diagnosis of relapsing polychondritis and application of proper treatment is essential to prevent disease-related complications.
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