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Cutaneous manifestations of patients with relapsing polychondritis: an association with extracutaneous complications

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Abstract

The aim of this study was to determine the prognostic characteristics of patients with relapsing polychondritis (RP) accompanying cutaneous manifestations in Japan. We analyzed a cohort of 239 patients with RP in view of cutaneous and extracutaneous complications. Thirty three cases (14%) developed cutaneous manifestations and 23 cases had both cutaneous and extracutaneous manifestations. Five RP patients developed myelodysplastic syndrome (MDS) and all of the five patients had cutaneous manifestations, including Sweet’s syndrome. Only one patient died of MDS among the five patients, suggesting rather better prognosis as compared with ordinary MDS. Five RP patients developed Behcet’s disease and all the five patients had cutaneous manifestations. Death rate of the RP patients with cutaneous manifestations (15%) was slightly higher than that of whole Japanese RP patient cohort (9.2%). RP patients with cutaneous manifestations had a slightly higher death rate, than those without cutaneous manifestations. MDS of RP patients had a rather better prognostic impact in Japan. Further studies are needed to elucidate the pathophysiology of RP, which brings about development of extracutaneous manifestations, especially MDS and Behcet’s disease.

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Authors and Affiliations

  1. Institute of Medical Science and Departments of Immunology and Medicine, St. Marianna University School of Medicine, Kawasaki, 216-8511, Japan

    Jun Shimizu, Yoshihisa Yamano, Kazuo Yudoh & Noboru Suzuki

  2. Rheumatic Disease Center, Tokyo Medical University Hachioji Medical Center, Hachioji, Tokyo, 193-0998, Japan

    Hiroshi Oka

Authors
  1. Jun Shimizu
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  2. Hiroshi Oka
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  3. Yoshihisa Yamano
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  4. Kazuo Yudoh
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  5. Noboru Suzuki
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Corresponding author

Correspondence to Noboru Suzuki.

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Source of funding

This work and the original epidemiological study [4] were supported in part by Grants-in-Aid from the Research Committee of Rare Disease, the Ministry of Health, Labor and Welfare of Japan.

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Shimizu, J., Oka, H., Yamano, Y. et al. Cutaneous manifestations of patients with relapsing polychondritis: an association with extracutaneous complications. Clin Rheumatol 35, 781–783 (2016). https://doi.org/10.1007/s10067-015-3160-2

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  • DOI: https://doi.org/10.1007/s10067-015-3160-2

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