Undifferentiated connective tissue disease and interstitial lung disease: Trying to define patterns

Reumatol Clin (Engl Ed). 2018 Mar-Apr;14(2):75-80. doi: 10.1016/j.reuma.2016.10.007. Epub 2016 Dec 13.
[Article in English, Spanish]

Abstract

Objectives: To identify clinical or immunological features in patients with undifferentiated connective tissue disease (UCTD) associated interstitial lung disease (ILD), in order to group them and recognize different functional and high resolution computed tomography (HRCT) behavior.

Methods: Retrospective cohort study. Patients meeting Kinder criteria for UCTD were included. We defined the following predictive variables: 'highly specific' connective tissue disease (CTD) manifestations (Raynaud's phenomenon, dry eyes or arthritis), high antinuclear antibody (ANA) titer (above 1: 320), and 'specific' ANA staining patterns (centromere, cytoplasmic and nucleolar patterns). We evaluated the following outcomes: change in the percentage of the predicted forced vital capacity (FVC%) during the follow-up period, and HRCT pattern.

Results: Sixty-six patients were included. Twenty-nine (43.94%) showed at least one 'highly specific' CTD manifestation, 16 (28.57%) had a 'specific' ANA staining pattern and 29 (43.94%) high ANA titer. Patients with 'highly specific' CTD manifestations were younger (mean [SD] 52 years [14.58] vs 62.08 years [9.46], P<.001), were more likely men (10.34% vs 48.65%, P<.001) and showed a smaller decline of the FVC% (median [interquartile range] 1% [-1 to 10] vs -6% [-16 to -4], P<.006). In the multivariate analysis, the presence of highly specific manifestations was associated with improvement in the FVC% (B coefficient of 13.25 [95% confidence interval, 2.41 to 24.09]). No association was observed in relation to the HRCT pattern.

Conclusion: The presence of 'highly specific' CTD manifestations was associated with female sex, younger age and better functional behavior. These findings highlight the impact of the clinical features in the outcome of patients with UCTD ILD.

Keywords: Anticuerpos antinucleares; Antinuclear antibodies; Autoimmune diseases; Connective tissue diseases; Enfermedad del tejido conectivo; Enfermedad pulmonar intersticial; Enfermedades autoinmunes; Fenómeno de Raynaud; Interstitial lung diseases; Raynaud phenomenon.

Publication types

  • Observational Study

MeSH terms

  • Adult
  • Aged
  • Female
  • Follow-Up Studies
  • Humans
  • Linear Models
  • Logistic Models
  • Lung Diseases, Interstitial / diagnostic imaging
  • Lung Diseases, Interstitial / etiology*
  • Lung Diseases, Interstitial / immunology
  • Male
  • Middle Aged
  • Odds Ratio
  • Retrospective Studies
  • Undifferentiated Connective Tissue Diseases / complications
  • Undifferentiated Connective Tissue Diseases / diagnosis*
  • Undifferentiated Connective Tissue Diseases / immunology