Relapsing polychondritis: a diagnosis not to be missed

Relapsing polychondritis: a diagnosis not to be missed Rheumatology 2018;0:1 Clinical Vignette doi:10.1093/rheumatology/key124 Relapsing polychondritis: a diagnosis not to be FIG.1 Patient with relapsing polychondritis: at presenta- missed tion inflammation and deformation (cauliflower ear) of the right ear pinna was evident (A), while the second and A 33-year-old healthy woman developed, 5 months prior fourth toes showed a diffuse erythroviolaceous swelling to our evaluation, inflammation of the right ear pinna, with- with a dactylitis-like picture (B). After two months of out preceding ear trauma/piercing/insect bite. Despite combined methylprednisolone and azathioprine treatment treatment with different broad-spectrum antibiotics, auricular inflammation regressed yet pinna deformity pinna abscesses developed, which were drained through remained (C) and arthritis was no longer evident (D). 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 erythrovio- laceous 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; nonethe- less, 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 Correspondence to: Evangelia Zampeli, Institute for Systemic external ear involvement. The latter subgroup, as in the Autoimmune and Neurological Diseases, M. Asias 75, 11527, case herein, often presents with joint involvement [2]. Athens, Greece. E-mail: zampelieva@gmail.com Early, accurate diagnosis of relapsing polychondritis and application of proper treatment is essential to prevent dis- ease-related complications. References Funding: No specific funding was received from any 1 Ferrada MA, Grayson PC, Banerjee S et al. Patient-perception bodies in the public, commercial or not-for-profit of disease-related symptoms and complications in relapsing sectors to carry out the work described in this polychondritis. Arthritis Care Res 2017; Advance Access manuscript. published 15 December 2017, doi: 10.1002/acr.23492. 2 Shimizu J, Yamano Y, Yudoh K, Suzuki N. Organ in- Disclosure statement: The authors have declared no con- volvement pattern suggests subgroups within relapsing flict of interest. polychondritis: comment on the article by Dion et al. Arthritis Rheumatol 2018;70:1489. Evangelia Zampeli and Haralampos 1,2 M. Moutsopoulos Institute for Autoimmune Systemic and Neurological Diseases and Academy of Athens, Athens, Greece The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com Downloaded from https://academic.oup.com/rheumatology/advance-article-abstract/doi/10.1093/rheumatology/key124/4990367 by Ed 'DeepDyve' Gillespie user on 12 July 2018 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Rheumatology Oxford University Press

Relapsing polychondritis: a diagnosis not to be missed

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© The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com
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1462-0324
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1462-0332
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10.1093/rheumatology/key124
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Abstract

Rheumatology 2018;0:1 Clinical Vignette doi:10.1093/rheumatology/key124 Relapsing polychondritis: a diagnosis not to be FIG.1 Patient with relapsing polychondritis: at presenta- missed tion inflammation and deformation (cauliflower ear) of the right ear pinna was evident (A), while the second and A 33-year-old healthy woman developed, 5 months prior fourth toes showed a diffuse erythroviolaceous swelling to our evaluation, inflammation of the right ear pinna, with- with a dactylitis-like picture (B). After two months of out preceding ear trauma/piercing/insect bite. Despite combined methylprednisolone and azathioprine treatment treatment with different broad-spectrum antibiotics, auricular inflammation regressed yet pinna deformity pinna abscesses developed, which were drained through remained (C) and arthritis was no longer evident (D). 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 erythrovio- laceous 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; nonethe- less, 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 Correspondence to: Evangelia Zampeli, Institute for Systemic external ear involvement. The latter subgroup, as in the Autoimmune and Neurological Diseases, M. Asias 75, 11527, case herein, often presents with joint involvement [2]. Athens, Greece. E-mail: zampelieva@gmail.com Early, accurate diagnosis of relapsing polychondritis and application of proper treatment is essential to prevent dis- ease-related complications. References Funding: No specific funding was received from any 1 Ferrada MA, Grayson PC, Banerjee S et al. Patient-perception bodies in the public, commercial or not-for-profit of disease-related symptoms and complications in relapsing sectors to carry out the work described in this polychondritis. Arthritis Care Res 2017; Advance Access manuscript. published 15 December 2017, doi: 10.1002/acr.23492. 2 Shimizu J, Yamano Y, Yudoh K, Suzuki N. Organ in- Disclosure statement: The authors have declared no con- volvement pattern suggests subgroups within relapsing flict of interest. polychondritis: comment on the article by Dion et al. Arthritis Rheumatol 2018;70:1489. Evangelia Zampeli and Haralampos 1,2 M. Moutsopoulos Institute for Autoimmune Systemic and Neurological Diseases and Academy of Athens, Athens, Greece The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com Downloaded from https://academic.oup.com/rheumatology/advance-article-abstract/doi/10.1093/rheumatology/key124/4990367 by Ed 'DeepDyve' Gillespie user on 12 July 2018

Journal

RheumatologyOxford University Press

Published: Apr 30, 2018

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