title: Undifferentiated connective tissue disease and interstitial lung disease: Trying to define patterns.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Hospital María Ferrer, Ciudad Autónoma de Buenos Aires, Argentina. Electronic address: albertimlaura@gmail.com.
authors.forename: María Laura
authors.initials: ML
authors.lastname: Alberti
authors.affiliation: Hospital María Ferrer, Ciudad Autónoma de Buenos Aires, Argentina.
authors.forename: Francisco
authors.initials: F
authors.lastname: Paulin
authors.affiliation: Instituto Nacional de Enfermedades Respiratorias (INER), Distrito Federal, México.
authors.forename: Heidegger Mateos
authors.initials: HM
authors.lastname: Toledo
authors.affiliation: Hospital María Ferrer, Ciudad Autónoma de Buenos Aires, Argentina.
authors.forename: Martín Eduardo
authors.initials: ME
authors.lastname: Fernández
authors.affiliation: Hospital María Ferrer, Ciudad Autónoma de Buenos Aires, Argentina.
authors.forename: Fabián Matías
authors.initials: FM
authors.lastname: Caro
authors.affiliation: Instituto Nacional de Enfermedades Respiratorias (INER), Distrito Federal, México.
authors.forename: Jorge
authors.initials: J
authors.lastname: Rojas-Serrano
authors.affiliation: Instituto Nacional de Enfermedades Respiratorias (INER), Distrito Federal, México.
authors.forename: Mayra Edith
authors.initials: ME
authors.lastname: Mejía
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016014:Linear Models; D016015:Logistic Models; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging / Q000209:etiology* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D016017:Odds Ratio; D012189:Retrospective Studies; D000074079:Undifferentiated Connective Tissue Diseases / Q000150:complications / Q000175:diagnosis* / Q000276:immunology
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