Skip to main content

Main menu

  • Home
  • Current issue
  • Past issues
  • For authors
    • Instructions for authors
    • Submit a manuscript
    • ERS author centre
  • Journal club
  • Alerts
  • Subscriptions
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

User menu

  • Log in
  • Subscribe
  • Contact Us
  • My Cart

Search

  • Advanced search
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

Login

European Respiratory Society

Advanced Search

  • Home
  • Current issue
  • Past issues
  • For authors
    • Instructions for authors
    • Submit a manuscript
    • ERS author centre
  • Journal club
  • Alerts
  • Subscriptions

Why is a paediatric respiratory specialist integral to the paediatric rheumatology clinic?

Manisha Ramphul, Kathy Gallagher, Kishore Warrier, Sumit Jagani, Jayesh Mahendra Bhatt
Breathe 2020 16: 200212; DOI: 10.1183/20734735.0212-2020
Manisha Ramphul
1Dept of Paediatric Respiratory Medicine, Nottingham Children's Hospital, Nottingham University Hospitals, Nottingham, UK
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Manisha Ramphul
Kathy Gallagher
1Dept of Paediatric Respiratory Medicine, Nottingham Children's Hospital, Nottingham University Hospitals, Nottingham, UK
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Kishore Warrier
1Dept of Paediatric Respiratory Medicine, Nottingham Children's Hospital, Nottingham University Hospitals, Nottingham, UK
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Sumit Jagani
2Dept of Radiology, Nottingham University Hospitals, Nottingham, UK
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Jayesh Mahendra Bhatt
1Dept of Paediatric Respiratory Medicine, Nottingham Children's Hospital, Nottingham University Hospitals, Nottingham, UK
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Jayesh Mahendra Bhatt
  • For correspondence: jayesh.bhatt@nuh.nhs.uk
  • Article
  • Figures & Data
  • Info & Metrics
  • PDF
Loading

Figures

  • Tables
  • Figure 1
    • Download figure
    • Open in new tab
    • Download powerpoint
    Figure 1

    a) Chest radiograph of child with JSLE: the PA radiograph demonstrates normal lung volumes with bilateral pleural effusions, left to greater extent than right with subjacent compressive atelectasis. b) Chest computed tomography of another child with JSLE: coronal reconstruction from contrast-enhanced CT demonstrates pericardial and pleural effusion a feature of serositis. The pulmonary trunk is dilated, a feature seen in pulmonary hypertension. There is also evidence of left axillary lymphadenopathy.

  • Figure 2
    • Download figure
    • Open in new tab
    • Download powerpoint
    Figure 2

    a) Chest radiograph of child with MCTD. This PA chest radiograph demonstrates retrocardiac left lower lobe consolidation; note increased retrocardiac opacity, indistinct bronchovascular markings and loss of cardiophrenic silhouette. Patent ductus arteriosus occluder device is in situ. b) Chest radiograph of a child with JDM with aspiration pneumonia. There is near total white out of the left hemithorax as a result of dense collapse consolidation of the left lung. There is wedge shaped consolidation in the right mid to lower zone.

  • Figure 3
    • Download figure
    • Open in new tab
    • Download powerpoint
    Figure 3

    Pulmonary function testing in a patient with JSSc showing a restrictive pattern and reduced DLCO. VC: vital capacity; PEF: peak expiratory flow; FRC: functional residual capacity; TLC: total lung capacity; KCO: transfer coefficient of the lung for carbon monoxide; VA: alveolar volume; Hb: haemoglobin. For standardised residuals (SR): a value of >1.64 is the upper limit of normal, <−1.64 is below the lower limit of normal. Severity scale: mild = −1.64 to −2.5; moderate = −2.5 to −3.5; severe= <−3.5.

  • Figure 4
    • Download figure
    • Open in new tab
    • Download powerpoint
    Figure 4

    Chest CT of a teenager with JSSc. a) Axial and b) coronal CT reconstructions: There is widespread intralobular septal thickening, predominating in the subpleural region. The apical segment of the left lower lobe demonstrates honeycombing indicating fibrosis. The fine subpleural ground-glass opacities in the right lower lobe indicates the presence of interstitial inflammation.

  • Figure 5
    • Download figure
    • Open in new tab
    • Download powerpoint
    Figure 5

    Skin rash in a child with ILD.

Tables

  • Figures
  • Table 1

    Acute and chronic pulmonary complications of JSLE

    ComplicationsPercentage affectedInvestigationsPrognosis
    Acute
     Infective pneumoniaUp to 90%CXR: consolidation
    BAL: culture may isolate pathogen
    Variable
     Pleuritis50–80%CXR: may show pleural effusionGood
     Thromboembolic diseaseVariableCXR: pulmonary oligaemia, peripheral wedge-shaped consolidation, may show pleural effusion
    CTPA: may show pulmonary embolus
    Doppler: may show venous embolus
    Variable
    Can progress to pulmonary hypertension
     Alveolar haemorrhage (AH)<2%Full blood count: drop in haemoglobin
    CXR: patchy infiltrates at bases
    BAL: blood stained, haemosiderin-laden macrophages
    Mortality >50%
     Acute lupus pneumonitis (ALP)<10%CXR: patchy infiltrates at bases70–90%
    Chronic
     Chronic ILD3%CXR/CT: interstitial infiltrates, ground-glass shadowing, honeycombing
    PFT:↓FVC, ↓DLCO
    Variable, can be slowly progressive
     Pulmonary hypertension5–14%Echocardiography: ↑right ventricular pressures, PAP >20 mmHg
    PFT: stable FVC, ↓DLCO
    Up to 50%
     Shrinking lung syndrome<1%CXR: ↓ lung volume, raised hemi diaphragm
    PFT: ↓FVC, ↓DLCO
    Good

    CXR: chest radiography; BAL: bronchoalveolar lavage; CTPA: computed tomography pulmonary angiogram; PFT: pulmonary function test.

    • Table 2

      Medications used in CTD

      MedicationMechanism of action
      Corticosteroids
       PrednisoloneAnti-inflammatory
       MethylprednisoloneAnti-inflammatory
      DMARDs
       MethotrexateDihydrofolate reductase inhibitor
       HydroxchloroquineLysosomal membrane stablisation, reduces IL-1 and TNF synthesis
       Mycophenolate mofetilRestricts T- and B-cell proliferation, acts on purine synthesising enzyme
       AzathioprineMetabolised to 6-mercaptopurine
      Biologic therapy
       EtanerceptSoluble TNF-α receptor
       AdalimumabMonoclonal antibody to TNF-α
       InfliximabMonoclonal antibody to TNF-α
       CanakinumabMonoclonal antibody to IL-1
       RituximabMonoclonal antibody to CD20
       AbataceptCTLA-4 fusion protein
      Cytotoxic agent
       CyclophosphamideActs on all phases of cell cycle; acts on both T- and B-cells

      DMARDs: disease modifying anti-rheumatic drugs; CTLA-4: cytotoxic T-lymphocytes antigen 4; CD: cluster of differentiation.

      • Table 3

        Patterns of pulmonary injury caused by CTD medication

        Pattern of iatrogenic pulmonary complicationOffending drug
        Pulmonary toxicityMethotrexate
        Interstitial pneumonitisCyclophosphamide
        Azathioprine
        Cytokine modulators (etanercept, infliximab, rituximab)
        Organising pneumoniaMethotrexate
        Diffuse alveolar damageAzathioprine
      PreviousNext
      Back to top
      Vol 16 Issue 4 Table of Contents
      Breathe: 16 (4)
      • Table of Contents
      • Index by author
      Email

      Thank you for your interest in spreading the word on European Respiratory Society .

      NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

      Enter multiple addresses on separate lines or separate them with commas.
      Why is a paediatric respiratory specialist integral to the paediatric rheumatology clinic?
      (Your Name) has sent you a message from European Respiratory Society
      (Your Name) thought you would like to see the European Respiratory Society web site.
      CAPTCHA
      This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
      Print
      Alerts
      Sign In to Email Alerts with your Email Address
      Citation Tools
      Why is a paediatric respiratory specialist integral to the paediatric rheumatology clinic?
      Manisha Ramphul, Kathy Gallagher, Kishore Warrier, Sumit Jagani, Jayesh Mahendra Bhatt
      Breathe Dec 2020, 16 (4) 200212; DOI: 10.1183/20734735.0212-2020

      Citation Manager Formats

      • BibTeX
      • Bookends
      • EasyBib
      • EndNote (tagged)
      • EndNote 8 (xml)
      • Medlars
      • Mendeley
      • Papers
      • RefWorks Tagged
      • Ref Manager
      • RIS
      • Zotero

      Share
      Why is a paediatric respiratory specialist integral to the paediatric rheumatology clinic?
      Manisha Ramphul, Kathy Gallagher, Kishore Warrier, Sumit Jagani, Jayesh Mahendra Bhatt
      Breathe Dec 2020, 16 (4) 200212; DOI: 10.1183/20734735.0212-2020
      del.icio.us logo Digg logo Reddit logo Technorati logo Twitter logo CiteULike logo Connotea logo Facebook logo Google logo Mendeley logo
      Full Text (PDF)

      Jump To

      • Article
        • Abstract
        • Abstract
        • Educational aims
        • Incidence of pleuropulmonary complications in CTD
        • Pathophysiology
        • Juvenile systemic lupus erythematosus
        • Juvenile dermatomyositis
        • Juvenile systemic sclerosis
        • Mixed connective tissue disease
        • Sjögren's syndrome
        • Investigations
        • Treatment
        • Iatrogenic complications of CTD treatment
        • Conclusion
        • Footnotes
        • References
      • Figures & Data
      • Info & Metrics
      • PDF

      Subjects

      • Paediatric pulmonology
      • Tweet Widget
      • Facebook Like
      • Google Plus One

      More in this TOC Section

      • Pulmonary manifestations of systemic vasculitis in childhood
      • Sarcoidosis: rarely a single system disorder
      • Pleural effusions in chronic systemic inflammatory diseases
      Show more Reviews

      Related Articles

      Navigate

      • Home
      • Current issue
      • Archive

      About Breathe

      • Journal information
      • Editorial board
      • CME
      • Press
      • Permissions and reprints
      • Advertising

      The European Respiratory Society

      • Society home
      • myERS
      • Privacy policy
      • Accessibility

      ERS publications

      • European Respiratory Journal
      • ERJ Open Research
      • European Respiratory Review
      • Breathe
      • ERS books online
      • ERS Bookshop

      Help

      • Feedback

      For authors

      • Intructions for authors
      • Submit a manuscript
      • ERS author centre

      For readers

      • Alerts
      • Subjects
      • RSS

      Subscriptions

      • Accessing the ERS publications

      Contact us

      European Respiratory Society
      442 Glossop Road
      Sheffield S10 2PX
      United Kingdom
      Tel: +44 114 2672860
      Email: journals@ersnet.org

      ISSN

      Print ISSN: 1810-6838
      Online ISSN: 2073-4735

      Copyright © 2021 by the European Respiratory Society