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Non-specific interstitial pneumonia and features of connective tissue disease: What are the consequences of a different point of view?

Alice Biffi, Giulia Dei, Federica De Giacomi, Anna Stainer, Lorenzo Olmo Parma, Maria Rosa Pozzi, Paola Faverio, Alberto Pesci
  • Alice Biffi
    University of Milan Bicocca, Dipartimento Cardio-Toraco-Vascolare, Italy
  • Giulia Dei
    University of Milan Bicocca, Dipartimento Cardio-Toraco-Vascolare, Italy
  • Federica De Giacomi
    University of Milan Bicocca, Dipartimento Cardio-Toraco-Vascolare, Italy | i.fede@live.it
  • Anna Stainer
    University of Milan Bicocca, Dipartimento Cardio-Toraco-Vascolare, Italy
  • Lorenzo Olmo Parma
    Dipartimento di Medicina Interna, UOSD Reumatologica, San Gerardo Hospital, ASST di Monza, Italy
  • Maria Rosa Pozzi
    Dipartimento di Medicina Interna, UOSD Reumatologica, San Gerardo Hospital, ASST di Monza, Italy
  • Paola Faverio
    University of Milan Bicocca, Dipartimento Cardio-Toraco-Vascolare, Italy
  • Alberto Pesci
    University of Milan Bicocca, Dipartimento Cardio-Toraco-Vascolare, Italy

Abstract

Patients with Interstitial Lung Disease (ILD) without a definitive diagnosis of connective tissue diseases (CTD) were historically described as Undifferentiated Connective Tissue Disease (UCTD-ILD). Recently a new classification, Interstitial Pneumonia with Autoimmune Features (IPAF), has been proposed. Aim of this study was to describe the prevalence, clinical characteristics and prognostic factors of UCTD and IPAF subjects in a cohort of Non-Specific Interstitial Pneumonia (NSIP) patients. This retrospective, observational study enrolled 102 adult patients characterized by NSIP pattern on High Resolution Computed Tomography, without a specific diagnosis of CTD. Three groups were identified according to patients’ characteristics: IPAF, UCTD or idiopathic NSIP (iNSIP). Forty percent, 27% and 55% of patients showed diagnostic criteria for IPAF, UCTD and iNSIP, respectively. No significant differences in age, gender, smoking habit, pulmonary function tests and three-year survival rate were observed among study groups. IPAF patients with antisynthetase antibodies positivity, in comparison to IPAF without antisynthetase antibodies positivity, showed more frequently an acute onset (44% vs 9%, p<0.012). The presence of autoimmune features seems not to be associated with better outcomes in NSIP patients. IPAF criteria seem to be more representative than UCTD criteria in identifying patients with autoimmune features. Further studies are needed to verify if IPAF should include patients with positive antisynthetase serology.

Keywords

Interstitial lung diseases; connective tissue diseases; non-specific interstitial pneumonia; interstitial pneumonia with autoimmune features; antisynthetase syndrome.

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Submitted: 2018-05-27 12:01:49
Published: 2018-09-04 16:04:31
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Copyright (c) 2018 Alice Biffi, Giulia Dei, Federica De Giacomi, Anna Stainer, Lorenzo Olmo Parma, Maria Rosa Pozzi, Paola Faverio, Alberto Pesci

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