{
  "abstract": "Introduction Airway disease affects the bronchi resulting in air trapping, ventilation inhomogeneity and increased airway-resistance. Airway disease is prevalent in rheumatic diseases like rheumatoid arthritis and Sjogren’s syndrome, affecting 20-40% of patients irrespective of the presence of interstitial lung disease (ILD). Pulmonary complications are frequent in systemic sclerosis (SSc), but the prevalence of airway disease (AD) is unknown as the primary focus lies on ILD and pulmonary hypertension. Nevertheless, airway disease might explain a significant part of pulmonary symptoms. Insight in the prevalence of AD in SSc can improve our understanding of pulmonary manifestations of in SSc and might reveal a new treatment target for pulmonary burden of disease. This review summarizes hitherto data on AD in SSc as well as methods to assess AD.Material and Methods A systemic literature review was conducted using PubMed, Embase, Cochrane and Scopus. Of the 326 articles screened, 44 studies reported on AD in SSc of which 40 clinical studies and 4 reviews. Methodologic quality assessment was carried out using the Newcastle-Ottawa score.Results Pulmonary function tests (PFTs) and/or high-resolution chest tomography (HRCTs) were used to assess AD. Based on PFTs, AD-prevalence ranged between 12-67%, in which the mid-expiratory flow (FEF25-75% ) was the indice mostly used to define AD. AD on HRCT, defined as the presence of air trapping, was present in 11-34% of patients. The latter demonstrating a decreased sensitivity of HRCT for detecting AD compared to PFT. Interestingly, only in 33-52% of SSc patients AD occurred concomitantly with ILD. Data on patient or disease-specific characteristics associated with AD was lacking, but a history of smoking was not associated with AD in SSc. The use of bronchodilators or inhalation-steroids were used in 5-24% of SSc patients in contrast to an AD prevalence up to 60%, suggesting undertreatment of AD.Conclusions AD is a prevalent pulmonary manifestation of SSc of which the true incidence remains unknown. Pulmonary function tests and HRCTs are already frequently performed in the follow-up of SSc and can also provide information on AD. A decreased FEF25-75% and air trapping on HRCT should alarm clinicians that AD might be present. More awareness of AD can improve understanding of pulmonary manifestations of SSc and will lead to better treatment options for pulmonary symptoms.",
  "authors": [
    {
      "affiliations": [
        "Department of Rheumatology & Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands"
      ],
      "name": "Marloes Verstappen"
    },
    {
      "affiliations": [
        "Division of Heart and Lungs, University Medical Center Utrecht, Utrecht, The Netherlands"
      ],
      "name": "M Voortman"
    },
    {
      "affiliations": [
        "Division of Heart and Lungs, University Medical Center Utrecht, Utrecht, The Netherlands"
      ],
      "name": "ADM Vorselaars"
    },
    {
      "affiliations": [
        "Department of Rheumatology & Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands"
      ],
      "name": "J Spierings"
    }
  ],
  "title": "P.081 Airway disease; an underrecognized pulmonary manifestation of systemic sclerosis",
  "uid": "af6600d9-16fe-5d8b-8515-fa9896871638"
}