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Prevalence and characterization of non-sicca onset primary Sjögren syndrome with interstitial lung involvement

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An Erratum to this article was published on 25 April 2017

Abstract

Primary Sjögren syndrome (pSS)-related interstitial lung disease (ILD) involved about 10–20% of patients. In 20% of cases, ILD can be diagnosed before pSS; anyway, few studies have investigated the frequency of ILD as the first clinically relevant manifestation of pSS, generally referred to retrospective studies. Aim of our prospective study was to describe prevalence, clinical, serological, and instrumental features of non-sicca onset pSS patients with interstitial lung involvement. During a period of 48 months, all consecutive patients diagnosed as pSS were enrolled. For all patients, the reason for the first visit was recorded. When present, ILD was categorized as definite, possible, or inconsistent with usual interstitial pneumonia (UIP) pattern, according to the current criteria. ILD was the main presenting symptom in 13/77 new diagnoses of pSS patients; in particular, 6/13 patients were initially diagnosed as idiopathic ILD, and only later developed clinical manifestations suggestive for pSS; ILD-pSS patients were older than others and showed a higher EULAR primary Sjögren’s syndrome disease activity index. A radiologic definite or possible UIP pattern was detected in 12/13 pSS. For the first time, we prospectively observed a prevalence of 16.8% of non-sicca onset pSS patients with ILD. Interestingly, UIP pattern was the most frequently detected, while typical autoantibodies were often absent. These features stressed the importance of differential diagnosis in the first stage of the disease, considering the possible poorer prognosis in this subgroup of patients. Multidisciplinary approach is crucial for a correct and early diagnosis, at both onset and follow-up.

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Authors and Affiliations

  1. Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Via del Pozzo, 71 41121, Modena, Italy

    Andreina Manfredi, Marco Sebastiani, Giulia Cassone & Clodoveo Ferri

  2. Respiratory Disease Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy

    Stefania Cerri & Fabrizio Luppi

  3. Unit of Oral-Maxillofacial Surgery, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy

    Pietrantonio Bellini

  4. Radiology Unit, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy

    Giovanni Della Casa

Authors
  1. Andreina Manfredi
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  2. Marco Sebastiani
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  3. Stefania Cerri
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  4. Giulia Cassone
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  8. Clodoveo Ferri
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Corresponding author

Correspondence to Andreina Manfredi.

Ethics declarations

The study was approved by the local ethical committee and involved all consecutive patients diagnosed as pSS from January 2013 to December 2106.

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Additional information

The original version of this article was revised: This article originally published with all author names incorrectly listed. All author names have now been transposed and appear correctly above, as in the article.

An erratum to this article is available at http://dx.doi.org/10.1007/s10067-017-3635-4.

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Manfredi, A., Sebastiani, M., Cerri, S. et al. Prevalence and characterization of non-sicca onset primary Sjögren syndrome with interstitial lung involvement. Clin Rheumatol 36, 1261–1268 (2017). https://doi.org/10.1007/s10067-017-3601-1

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  • DOI: https://doi.org/10.1007/s10067-017-3601-1

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