Downloaded from https://academic.oup.com/rheumatology/article-abstract/57/10/1768/4990367 by Ed 'DeepDyve' Gillespie user on 17 October 2018 Martijn A. H. Oude Voshaar et al. 24 Mokkink LB, Terwee CB, Patrick DL et al.The 25 Terwee CB, Bot SDM, de Boer MR et al. Quality criteria COSMIN checklist for assessing the methodological were proposed for measurement properties of health quality of studies on measurement properties of health status questionnaires. J Clin Epidemiol 2007;60:3442. status measurement instruments: an international 26 Hambleton R, Swaminathan H, Rogers H. Fundamentals Delphi study. Qual Life Res 2010;19:53949. of Item Response Theory. Newbury Park, CA: Sage, 1991. Rheumatology 2018;57:1768 doi:10.1093/rheumatology/key124 Clinical vignette Advance Access publication 30 April 2018 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 erythroviolac- eous swelling of the second and fourth toes with a dacty- litis-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 de- formity remained. Relapsing polychondritis is a systemic disease causing inflammation of cartilaginous structures in multiple organs . 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 . Athens, Greece. E-mail: email@example.com Early, accurate diagnosis of relapsing polychondritis and application of proper treatment is essential to prevent dis- ease-related complications. Funding: No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this References manuscript. 1 Ferrada MA, Grayson PC, Banerjee S et al. Patient-perception Disclosure statement: The authors have declared no con- of disease-related symptoms and complications in relapsing flict of interest. polychondritis. Arthritis Care Res 2017; Advance Access published 15 December 2017, doi: 10.1002/acr.23492. Evangelia Zampeli and Haralampos 2 Shimizu J, Yamano Y, Yudoh K, Suzuki N. Organ involve- 1,2 M. Moutsopoulos ment pattern suggests subgroups within relapsing poly- Institute for Autoimmune Systemic and Neurological chondritis: comment on the article by Dion et al. Arthritis Diseases and Academy of Athens, Athens, Greece Rheumatol 2018;70:1489. The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: firstname.lastname@example.org 1768 https://academic.oup.com/rheumatology
Rheumatology – Oxford University Press
Published: Oct 1, 2018
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