{
  "abstract": "Background Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is heterogeneous. Forced vital capacity (FVC) decline and high-resolution CT (HRCT) pattern are widely used as prognostic markers, but the natural history of RA-ILD identified in rheumatology clinics, particularly in mild disease, remains insufficiently described.Methods We conducted a prospective, multicentre cohort study across three Brazilian centres. Adults with RA-ILD were followed every 6 months for 24 months. Pulmonary function tests, HRCT, 6 min walk test and King’s Brief Interstitial Lung Disease health-related quality-of-life questionnaire were performed at baseline, 12 and 24 months. Blood biomarkers were assessed at baseline, 6 and 18 months. HRCT scans were independently reviewed by two chest radiologists and analysed using densitometry-based quantitative CT (QCT). Pulmonary fibrosis was qualitatively defined by unequivocal traction bronchiectasis and/or honeycombing on HRCT.Results Ninety-five subjects were included (mean age 63±10 years; 81% female). Pulmonary fibrosis was present in 80%, although only 20% were classified as definite or probable usual interstitial pneumonia (κ=0.52). Individuals with fibrosis had lower FVC (75%±17 vs 86%±15, p=0.02) and worse QCT measures (%high-attenuation areas −600 to −250 HU 12.1±6.6 vs 5.5±0.8, p<0.001; lung volume 3407±958 mL vs 4077±923 mL, p=0.015). In 24 months, FVC and QCT measures remained stable. Eight subjects (8%) died. Deaths were not preceded by measurable functional or radiological decline. Health-related quality-of-life scores and blood biomarkers remained unchanged.Conclusions In this predominantly mild RA-ILD cohort, pulmonary function and QCT metrics remained stable over 2 years despite an 8% mortality rate. The absence of preceding decline in conventional prognostic markers suggests potential limitations of current monitoring strategies, although our findings should be interpreted as hypothesis-generating.Trial registration number NCT04136223.",
  "authors": [
    {
      "affiliations": [
        "HCor Research Institute, Hospital do Coracao, Sao Paulo, Brazil",
        "Pulmonary Division, Heart Institute (InCor), Universidade de Sao Paulo, Sao Paulo, Brazil"
      ],
      "name": "Leticia Kawano-Dourado"
    },
    {
      "affiliations": [
        "Rheumatology Division, Hospital das Clinicas HCFMUSP, Universidade de São Paulo, Sao Paulo, Brazil"
      ],
      "name": "Karina Bonfiglioli"
    },
    {
      "affiliations": [
        "Rheumatology Division, Hospital das Clinicas HCFMUSP, Universidade de São Paulo, Sao Paulo, Brazil"
      ],
      "name": "Ana C M Ribeiro"
    },
    {
      "affiliations": [
        "Hospital Universitário de Brasília da Universidade de Brasília, EBSERH (HUB-UnB), Brasília, Brazil"
      ],
      "name": "Licia Maria Henrique da Mota"
    },
    {
      "affiliations": [
        "Department of Orthopedics, Rheumatology and Traumatology, Campinas State University (UNICAMP), Campinas, UK"
      ],
      "name": "Alisson Pugliesi"
    },
    {
      "affiliations": [
        "Rheumatology Division, Hospital Sírio-Libanês, Brasilia, Brazil"
      ],
      "name": "Luciana Muniz"
    },
    {
      "affiliations": [
        "Institute of Radiology (InRad), Hospital das Clinicas HCFMUSP, Universidade de São Paulo, Sao Paulo, Brazil"
      ],
      "name": "Daniel G Strabelli"
    },
    {
      "affiliations": [
        "Rheumatology Division, Hospital das Clinicas HCFMUSP, Universidade de São Paulo, Sao Paulo, Brazil"
      ],
      "name": "Andrea Y Shimabuco"
    },
    {
      "affiliations": [
        "Hospital Universitário de Brasília da Universidade de Brasília, EBSERH (HUB-UnB), Brasília, Brazil"
      ],
      "name": "Luciana Lourençoni"
    },
    {
      "affiliations": [
        "Hospital Universitário de Brasília da Universidade de Brasília, EBSERH (HUB-UnB), Brasília, Brazil"
      ],
      "name": "Fernanda L F B A Riscado"
    },
    {
      "affiliations": [
        "Rheumatology Division, Hospital das Clinicas HCFMUSP, Universidade de Sao Paulo, Sao Paulo, Brazil"
      ],
      "name": "Sandra Gofinet Pasoto"
    },
    {
      "affiliations": [
        "Hospital Universitário de Brasília da Universidade de Brasília, EBSERH (HUB-UnB), Brasília, Brazil"
      ],
      "name": "Cleando P Albuquerque"
    },
    {
      "affiliations": [
        "LABOX, Department of Biochemistry, Federal University of Santa Catarina, Florianopolis, Brazil"
      ],
      "name": "Luis F Marqueze"
    },
    {
      "affiliations": [
        "LABOX, Department of Biochemistry, Federal University of Santa Catarina, Florianopolis, Brazil"
      ],
      "name": "Romuel Barros Costa Silva"
    },
    {
      "affiliations": [
        "Department of Orthopedics, Rheumatology and Traumatology, Campinas State University (UNICAMP), Campinas, UK"
      ],
      "name": "Luciana C Palhares"
    },
    {
      "affiliations": [
        "Department of Orthopedics, Rheumatology and Traumatology, Campinas State University (UNICAMP), Campinas, UK"
      ],
      "name": "Ana C Londe"
    },
    {
      "affiliations": [
        "Laboratory of Genetic Polimorphisms (LAPOGE), Federal University of Santa Catarina, Florianopolis, Brazil"
      ],
      "name": "Juliana Dal-Ri Lindenau"
    },
    {
      "affiliations": [
        "Laboratory of Genetic Polimorphisms (LAPOGE), Federal University of Santa Catarina, Florianopolis, Brazil"
      ],
      "name": "Yara Costa Netto Muniz"
    },
    {
      "affiliations": [
        "Hospital Universitário de Brasília da Universidade de Brasília, EBSERH (HUB-UnB), Brasília, Brazil"
      ],
      "name": "Tassiane R C M Martins"
    },
    {
      "affiliations": [
        "Hospital Universitário de Brasília da Universidade de Brasília, EBSERH (HUB-UnB), Brasília, Brazil"
      ],
      "name": "Ana Carolina Rezende de Freitas"
    },
    {
      "affiliations": [
        "HCor Research Institute, Hospital do Coracao, Sao Paulo, Brazil"
      ],
      "name": "Rachel H V Machado"
    },
    {
      "affiliations": [
        "Hospital do Coracao, Sao Paulo, Brazil"
      ],
      "name": "Thabata Silva Veiga"
    },
    {
      "affiliations": [
        "HCor Research Institute, Hospital do Coracao, Sao Paulo, Brazil"
      ],
      "name": "Leila O Silva"
    },
    {
      "affiliations": [
        "Insper Institute of Education and Research, Sao Paulo, Brazil"
      ],
      "name": "Tiago Mendonça dos Santos"
    },
    {
      "affiliations": [
        "Hcor Hospital, São Paulo, Brazil"
      ],
      "name": "Samira Martins Tokunaga"
    },
    {
      "affiliations": [
        "HCor Research Institute, Hospital do Coracao, Sao Paulo, Brazil"
      ],
      "name": "Débora H K Kodama"
    },
    {
      "affiliations": [
        "Pulmonary Division, Heart Institute (InCor), Universidade de Sao Paulo, Sao Paulo, Brazil"
      ],
      "name": "Bruno Baldi"
    },
    {
      "affiliations": [
        "LABOX, Department of Biochemistry, Federal University of Santa Catarina, Florianopolis, Brazil"
      ],
      "name": "Alexandra Latini"
    },
    {
      "affiliations": [
        "Institute of Radiology (InRad), Hospital das Clinicas HCFMUSP, Universidade de São Paulo, Sao Paulo, Brazil"
      ],
      "name": "Marcio Valente Yamada Sawamura"
    }
  ],
  "title": "Functional, radiological and survival outcomes in rheumatoid arthritis-associated interstitial lung disease: 24-month results from the BERTHA prospective cohort",
  "uid": "4ce142bd-70b8-5582-8f67-1b35a2ec0106"
}