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Respiratory subtype of relapsing polychondritis frequently presents as difficult asthma: a descriptive study of respiratory involvement in relapsing polychondritis with 13 patients from a single UK centre

Shirish Dubey, Colin Gelder, Grace Pink, Asad Ali, Christopher Taylor, Joanna Shakespeare, Susan Townsend, Patrick Murphy, Nicholas Hart, David D'Cruz
ERJ Open Research 2021 7: 00170-2020; DOI: 10.1183/23120541.00170-2020
Shirish Dubey
1Nuffield Orthopaedic Centre, Oxford University Hospitals NHS Trust, Oxford, UK
2University Hospitals Coventry and Warwickshire NHS Trust, Coventry, UK
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  • ORCID record for Shirish Dubey
  • For correspondence: s.dubey@nhs.net
Colin Gelder
2University Hospitals Coventry and Warwickshire NHS Trust, Coventry, UK
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Grace Pink
2University Hospitals Coventry and Warwickshire NHS Trust, Coventry, UK
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Asad Ali
2University Hospitals Coventry and Warwickshire NHS Trust, Coventry, UK
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Christopher Taylor
3Heart of England NHS Foundation Trust, Birmingham, UK
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Joanna Shakespeare
4Dept of Respiratory and Sleep Sciences, University Hospitals Coventry and Warwickshire NHS Trust, Coventry, UK
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Susan Townsend
4Dept of Respiratory and Sleep Sciences, University Hospitals Coventry and Warwickshire NHS Trust, Coventry, UK
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Patrick Murphy
5The Lane Fox Unit, St Thomas’ Hospital, London, UK
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Nicholas Hart
6Guy's and St Thomas’ NHS Foundation Trust, London, UK
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David D'Cruz
7Louise Coote Lupus Unit, Guy's Hospital, London, UK
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  • FIGURE 1
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    FIGURE 1

    a) Admission computed tomography (CT) scan showing near complete collapse of trachea in a patient that was subsequently diagnosed with relapsing polychondritis. b) Repeat CT after intravenous corticosteroids with inspiratory and expiratory films showing significant improvement of tracheal narrowing (expiratory phase CT).

  • FIGURE 2
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    FIGURE 2

    a) Patient with admission computed tomography (CT) chest showing near complete collapse of trachea and pleural effusions. b) Repeat CT after treatment with high-dose corticosteroids with improvement in trachea and resolution of pleural effusions.

  • FIGURE 3
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    FIGURE 3

    Patient with collapse of trachea.

  • FIGURE 4
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    FIGURE 4

    a) Patient with presentation computed tomography showing significant narrowing of trachea. b) Post-treatment imaging showing improvement in dimensions of trachea.

  • FIGURE 5
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    FIGURE 5

    Pre-treatment tracheal collapse in a patient.

  • FIGURE 6
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    FIGURE 6

    Patient images demonstrating auricular chondritis with inflammation of the external ear with sparing of non-cartilaginous part.

  • FIGURE 7
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    FIGURE 7

    Flow–volume loop of patient 4 showing flattening of the expiratory limb and inspiratory limb to a lesser extent.

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    FIGURE 8

    Flow–volume curve of patient 6 showing flattening of the expiratory limb and inspiratory limb to a lesser extent.

Tables

  • Figures
  • TABLE 1

    Clinical features of patients with relapsing polychondritis

    PatientSexAge yearsComorbiditiesBACNCRTCSPOIADResponse to corticosteroidsTBM proven
    1M65T2DM, hypothyroid, psoriasisYYYNYNYY
    2F70Memory lossNYYYNNYY
    3F50T2DMYYYYNYYY
    4F53Hypothyroid, fibromyalgia, HTN, Behçet's, obesityYYYYNNYY
    5F76Previous TB, immunodeficiency, HTN, T2DM, OANNYYNYYY
    6F74HTN, angina, AF, T2DM, antiphospholipid antibodiesYYYYNNYY
    7F76EmphysemaNNYNNNYY
    8F70HTN, obesity, acoustic neuroma, hyperlipidaemiaNNYYNNYY
    9F78T2DM, obesity, MI, AF, CKD, dementia, asthmaYYYYNYYY
    10M31Hypoadrenalism, bronchiectasisYYYYNNYY
    11F52Obesity, COPD, ankylosing spondylitis, psoriasisYYNYNYYN
    12M79Myelodysplasia, follicular lymphoma, osteoporosisYNYNYNYN
    13M78T2DM, IHD, CKD, myositisNNYYNYYY

    BAC: bilateral auricular chondritis; NC: nasal chondritis; RTC: respiratory tract chondritis; SP: seronegative polyarthritis; OI: ocular inflammation; AD: audiovestibular damage; TBM: tracheobronchomalacia; T2DM: type 2 diabetes mellitus; HTN: hypertension; TB: tuberculosis; OA: osteoarthritis; AF: atrial fibrillation; MI: myocardial infarction; CKD: chronic kidney disease; IHD: ischaemic heart disease.

    • TABLE 2

      Pharmacological and non-pharmacological treatment for patients with relapsing polychondritis

      PatientSexAge yearsCPAPStentIS drugsCorticosteroid dosePrevious drugsBaseline RPDAI
      1M65NNMTXPred37
      2F70NNMMF, infliximabPred 5 mgSSZ27
      3F50DNTNMTXPred45
      4F53YYMMF, MTXPred 10 mgAZA, cyclophosphamide, ADA and ETN47
      5F76YNSSZ, HCQ, ABT, IVIGPred 7.5 mgMTX, ETN, leflunomide, AZA44
      6F74YYMTX, AZAPred 10 mgCyclophosphamide43
      7F76YNCyclophosphamidePred 10 mgMTX27
      8F70NNAZAPred 5 mg15
      9F78DNTNPred 5 mgMTX, AZA, HCQ24
      10M31NNHCT 20/10/1033
      11F52NNSecukinumabPred 10 mgMTX, ETN, HCQ, cyclophosphamide, ADA38
      12M79NNPred 5 mg35
      13M78YYMTXPred 5 mgAZA40

      CPAP: continuous positive airway pressure; IS: immunosuppressant; RPDAI: Relapsing Polychondritis Activity Index; MTX: methotrexate; Pred: prednisolone; MMF: mycophenolate mofetil; SSZ: sulfasalazine; DNT: did not tolerate; AZA: azathioprine; ETN: etanercept; HCQ: hydroxychloroquine; ABT: abatacept; IVIG: intravenous immunoglobulin; HCT: hydrocortisone; ADA: adalimumab.

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      Respiratory subtype of relapsing polychondritis frequently presents as difficult asthma: a descriptive study of respiratory involvement in relapsing polychondritis with 13 patients from a single UK centre
      Shirish Dubey, Colin Gelder, Grace Pink, Asad Ali, Christopher Taylor, Joanna Shakespeare, Susan Townsend, Patrick Murphy, Nicholas Hart, David D'Cruz
      ERJ Open Research Jan 2021, 7 (1) 00170-2020; DOI: 10.1183/23120541.00170-2020

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      Respiratory subtype of relapsing polychondritis frequently presents as difficult asthma: a descriptive study of respiratory involvement in relapsing polychondritis with 13 patients from a single UK centre
      Shirish Dubey, Colin Gelder, Grace Pink, Asad Ali, Christopher Taylor, Joanna Shakespeare, Susan Townsend, Patrick Murphy, Nicholas Hart, David D'Cruz
      ERJ Open Research Jan 2021, 7 (1) 00170-2020; DOI: 10.1183/23120541.00170-2020
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