{
  "abstract": "Introduction Interstitial lung disease (ILD) associated with systemic sclerosis (SSc-ILD) is one of the leading causes of death in most clinical series. Our objective was to compare the fulfillment of different progression criteria in the first year after SSc-ILD diagnosis from baseline and the long-term prediction survival of each criteria.Material and Methods From the cohort of patients with SSc in Hospital Universitari Vall d’Hebron (n=383), 168 patients had SSc-ILD diagnosed by HRCT (43.8%). 131 SSc-ILD patients with PFT at 12 months were selected. We analyzed the different progression criteria based on PFT in the first year after SSc-ILD diagnosis: 1) Goh (relative decline in FVC &#8805; 10% or FVC 5-9% + diffusing capacity of the lung for carbon monoxide (DLCO) &#8805; 15%), 2) INBUILD (relative decline in FVC &#8805; 10% or FVC 5-9%), 3) ATS 2022 (absolute decline FVC &#8805; 5% or DLCO &#8805; 10%) and 4) an absolute decline of absolute FVC &#8805; 5%. The statistical study was carried out using the SPSS 20.0 package (Chicago, IL).Results Following the Goh criteria, 25.2% (33/131) were identified as progressors, 35.9% (47/131) with INBUILD criteria, 39.7% (52/131) with ATS 2022 criteria, and finally 29% (38/131) with an absolute decline of FVC &#8805; 5%. Thus, the INBUILD and ATS 2022 criteria identified the progression more frequently.Comparing to the ATS 2022 progressors, Goh progressors had greater statistically significant decline in both absolute in mL and relative in FVC % predicted, INBUILD progressors presented greater relative decline in FVC%, and patients with absolute decline of FVC &#8805; 5% had higher relative and absolute decline in FVC%. The progression at 12 months was associated with long-term mortality in Goh criteria (HR 3.65, p<0.001), INBUILD (HR 2.09, p=0.017) and after an absolute decline of FVC &#8805; 5% (HR 2.22, p= 0.015), but not when the ATS 2022 criteria were met.A higher long-term mortality was predicted fulfilling one or more of the Goh, INBUILD or an absolute decline of FVC&#8805; 5% criteria. When the combination of the Goh, INBUILD and ATS 2022 criteria was evaluated, the long-term mortality only was higher when combining two or more criteria.Conclusions Progression was identified in a quarter of patients with SSc-ILD in the first 12 months. The most exigent criteria such as Goh, INBUILD and an absolute decline of FVC &#8805; 5% are less fulfilled but have a stronger long-mortality effect.Abstract P.116 Figure 1",
  "authors": [
    {
      "affiliations": [
        "Vall Hebron University Hospital, Systemic and Autoimmune Diseases Unit, Internal Medicine Department, Barcelona, Spain"
      ],
      "name": "Clàudia Codina Clavaguera"
    },
    {
      "affiliations": [
        "Vall Hebron University Hospital, Systemic and Autoimmune Diseases Unit, Internal Medicine Department, Barcelona, Spain"
      ],
      "name": "Laura Triginer"
    },
    {
      "affiliations": [
        "Vall Hebron University Hospital, Systemic and Autoimmune Diseases Unit, Internal Medicine Department, Barcelona, Spain"
      ],
      "name": "Alejandra Fernández-Luque"
    },
    {
      "affiliations": [
        "Vall Hebron University Hospital, Respiratory Department, Barcelona, Barcelona, Spain"
      ],
      "name": "Ana Villar"
    },
    {
      "affiliations": [
        "Vall Hebron University Hospital, Respiratory Department, Barcelona, Barcelona, Spain"
      ],
      "name": "íñigo Ojanguren"
    },
    {
      "affiliations": [
        "CSIC, Institute of Parasitology and Biomedicine López-Neyra, Granada, Spain"
      ],
      "name": "Marialbert Acosta"
    },
    {
      "affiliations": [
        "CSIC, Institute of Parasitology and Biomedicine López-Neyra, Granada, Spain"
      ],
      "name": "Javier Martín"
    },
    {
      "affiliations": [
        "Vall Hebron University Hospital, Radiology Department, Barcelona, Spain"
      ],
      "name": "Ivan Vollmer"
    },
    {
      "affiliations": [
        "Vall Hebron University Hospital, Radiology Department, Barcelona, Spain"
      ],
      "name": "Luis Del Carpio"
    },
    {
      "affiliations": [
        "Vall Hebron University Hospital, Gastroenterology Department, Barcelona, Spain"
      ],
      "name": "Luis Alcalá-González"
    },
    {
      "affiliations": [
        "Vall Hebron University Hospital, Immunology Department, Barcelona, Spain"
      ],
      "name": "Janire Perurena"
    },
    {
      "affiliations": [
        "Vall Hebron University Hospital, Immunology Department, Barcelona, Spain"
      ],
      "name": "Maria Teresa Sanz-Martínez"
    },
    {
      "affiliations": [
        "Vall Hebron University Hospital, Immunology Department, Barcelona, Spain"
      ],
      "name": "Laura Viñas-Giménez"
    },
    {
      "affiliations": [
        "Vall Hebron University Hospital, Systemic and Autoimmune Diseases Unit, Internal Medicine Department, Barcelona, Spain"
      ],
      "name": "Carmen Pilar Simeón Aznar"
    },
    {
      "affiliations": [
        "Vall Hebron University Hospital, Systemic and Autoimmune Diseases Unit, Internal Medicine Department, Barcelona, Spain"
      ],
      "name": "Alfredo Guillén-Del-Castillo"
    }
  ],
  "title": "P.116 Analysis and validation of the different progressive pulmonary fibrosis (PPF) criteria in a cohort of patients with interstitial lung disease associated to systemic sclerosis (SSc-ILD",
  "uid": "cb58c9bf-a17e-51f6-a3ea-1bdf258606ff"
}