{
  "abstract": "Background Interstitial lung diseases (ILDs) comprise a group of more than 200 different subtypes. They vary widely in terms of incidence, prognosis and treatment, yet real-life data from Germany are sparse.Methods The prospective Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases (EXCITING)-ILD registry included patients with all different ILD subtypes from different healthcare settings. Follow-up ranged from 36 months to 5 years. Data were analysed descriptively. Baseline characteristics, diagnostic and treatment information are presented as absolute numbers and percentages. The Wilcoxon signed-rank sum test was used to quantify differences between groups. Line plots and bar plots were used for graphical presentation.Results A total of 601 patients (60.7% men, mean age 64.3 years) from 32 centres were included in the EXCITING-ILD registry. The most common subtypes were sarcoidosis with 26.6% (n=160) and idiopathic pulmonary fibrosis (IPF) with 25.3% (n=152). Pulmonary hypertension was present in 8.7% of patients (n=52), with high incidences in connective tissue disease-associated ILD (16.3%) and pneumoconiosis (27.3%). The mean forced vital capacity was 76.4% predicted, and the mean DLCO-SB (diffusing capacity for carbon monoxide) was 54.1% predicted. The mean time to diagnosis was 38.8 months (SD 64.4) and was significantly shorter when the diagnosis was made after multidisciplinary discussion (31.6 vs 49.2 months, p<0.001). The frequency of surgical lung biopsies decreased over time in the registry, whereas the proportion of cryobiopsies showed a notable increase. In IPF, the number of patients treated with antifibrotics increased from 35.2% before 2015 to 48.4% in 2019.Conclusion The EXCITING-ILD registry describes the frequency of ILD subtypes, ILD-related impairments, selected comorbidities and diagnostic and treatment patterns in a representative German population.",
  "authors": [
    {
      "affiliations": [
        "Center for Interstitial and Rare Lung Diseases, Pneumology and Respiratory Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany",
        "German Center for Lung Research (DZL), Heidelberg, Germany"
      ],
      "name": "Katharina Buschulte"
    },
    {
      "affiliations": [
        "Department of Pulmonary Medicine, Kantonsspital Aarau, Aarau, Switzerland"
      ],
      "name": "Hans-Joachim Kabitz"
    },
    {
      "affiliations": [
        "Clinic of Pneumology and Allergology, Center of Sleep Medicine and Respiratory Care, Hospital Bethanien Solingen, Solingen, Germany"
      ],
      "name": "Lars Hagmeyer"
    },
    {
      "affiliations": [
        "Chest Clinic Immenhausen, Immenhausen, Germany"
      ],
      "name": "Peter Hammerl"
    },
    {
      "affiliations": [
        "Outpatient Center for Pulmonology, Warendorf, Germany"
      ],
      "name": "Albert Esselmann"
    },
    {
      "affiliations": [
        "Outpatient Center for Pulmonology, Fulda, Germany"
      ],
      "name": "Conrad Wiederhold"
    },
    {
      "affiliations": [
        "Department of Internal Medicine II, University Hospital Bonn, Bonn, Germany"
      ],
      "name": "Dirk Skowasch"
    },
    {
      "affiliations": [
        "Outpatient Center for Pulmonology, Ibbenbüren, Germany"
      ],
      "name": "Christoph Stolpe"
    },
    {
      "affiliations": [
        "Outpatient Center for Pulmonology and Allergology, Bonn, Germany"
      ],
      "name": "Marcus Joest"
    },
    {
      "affiliations": [
        "Outpatient Center for Pulmonology, Böblingen, Germany"
      ],
      "name": "Stefan Veitshans"
    },
    {
      "affiliations": [
        "Outpatient Center for Pulmonology, Rheine, Germany"
      ],
      "name": "Marc Höffgen"
    },
    {
      "affiliations": [
        "Institute of Health Economics and Healthcare Management, Helmholtz Center Munich GmbH, Munich, Germany",
        "German Center for Lung Research (DZL), Munich, Germany",
        "Comprehensive Pneumology Center Munich (CPCM), Munich, Germany"
      ],
      "name": "Phillen Maqhuzu"
    },
    {
      "affiliations": [
        "Institute of Health Economics and Healthcare Management, Helmholtz Center Munich GmbH, Munich, Germany",
        "German Center for Lung Research (DZL), Munich, Germany",
        "Center for Mental Health and Addiction Research, IFT Institut für Therapieforschung, Munich, Germany",
        "Institute for Medical Information Processing, Biometry and Epidemiology, Ludwig Maximilian University of Munich, Munich, Bayern, Germany"
      ],
      "name": "Larissa Schwarzkopf"
    },
    {
      "affiliations": [
        "Outpatient Center for Pulmonology, Augsburg, Germany"
      ],
      "name": "Andreas Hellmann"
    },
    {
      "affiliations": [
        "Medical Clinic II, University of Regensburg and Klinikum Donaustauf, Donaustauf, Germany"
      ],
      "name": "Michael Pfeifer"
    },
    {
      "affiliations": [
        "German Center for Lung Research (DZL), Munich, Germany",
        "Department of Medicine V, LMU University Hospital, Munich, Germany"
      ],
      "name": "Juergen Behr"
    },
    {
      "affiliations": [
        "Patient Support Group Lungenfibrose e.V, Essen, Germany"
      ],
      "name": "Rainer Karpavicius"
    },
    {
      "affiliations": [
        "Medical Clinic II, University Hospital Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), Giessen, Germany",
        "Pulmonary and Critical Care Medicine, Agaplesion Evangelisches Krankenhaus Mittelhessen, Giessen, Germany",
        "German Center for Lung Research (DZL), Giessen, Germany"
      ],
      "name": "Andreas Guenther"
    },
    {
      "affiliations": [
        "Center for Interstitial and Rare Lung Diseases, Pneumology and Respiratory Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany",
        "German Center for Lung Research (DZL), Heidelberg, Germany"
      ],
      "name": "Markus Polke"
    },
    {
      "affiliations": [
        "Center for Interstitial and Rare Lung Diseases, Pneumology and Respiratory Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany",
        "German Center for Lung Research (DZL), Heidelberg, Germany"
      ],
      "name": "Philipp Höger"
    },
    {
      "affiliations": [
        "Mainz Center for Pulmonary Medicine, Departments of Pneumology, Mainz University Medical Center and of Pulmonary, Critical Care & Sleep Medicine, Marienhaus Clinic Mainz, Mainz, Germany"
      ],
      "name": "Vivien Somogyi"
    },
    {
      "affiliations": [
        "Center for Interstitial and Rare Lung Diseases, Pneumology and Respiratory Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany",
        "German Center for Lung Research (DZL), Heidelberg, Germany"
      ],
      "name": "Christoph Lederer"
    },
    {
      "affiliations": [
        "Pulmonary and Critical Care Medicine, Agaplesion Evangelisches Krankenhaus Mittelhessen, Giessen, Germany",
        "Medical Clinic V (Pneumology), Cardiothoracic Center, Universitätsmedizin Marburg Campus Fulda, Fulda, Germany"
      ],
      "name": "Philipp Markart"
    },
    {
      "affiliations": [
        "Mainz Center for Pulmonary Medicine, Departments of Pneumology, Mainz University Medical Center and of Pulmonary, Critical Care & Sleep Medicine, Marienhaus Clinic Mainz, Mainz, Germany"
      ],
      "name": "Michael Kreuter"
    }
  ],
  "title": "Baseline characteristics from the EXCITING-ILD registry",
  "uid": "d486805e-b469-57c9-870a-e6403c97669d"
}