title: Abnormalities in lung volumes and airflow in children with newly diagnosed connective tissue disease.
abstract: BACKGROUND
  Connective tissue diseases (CTDs) of childhood are rare inflammatory disorders, involving various organs and tissues including respiratory system. Pulmonary involvement in patients with CTDs is uncommon but may cause functional impairment. Data on prevalence and type of lung function abnormalities in children with CTDs are scarce. Thus, the aim of this study was to asses pulmonary functional status in children with newly diagnosed CTD and follow the results after two years of the disease course.
  
  
  METHODS
  There were 98 children (mean age: 13 ± 3; 76 girls), treated in Department of Pediatric Rheumatology, Institute of Rheumatology, Warsaw and 80 aged-matched, healthy controls (mean age 12.7 ± 2.4; 50 girls) included into the study. Study procedures included medical history, physical examination, chest radiograph and PFT (spirometry and whole body-plethysmography). Then, the assessment of PFT was performed after 24 months.
  
  
  RESULTS
  FEV₁, FEV₁/FVC and MEF50 were significantly lower in CTD as compared to control group, there was no difference in FVC and TLC. The proportion of patients with abnormal lung function was significantly higher in the study group, 41 (42%) vs 9 (11%). 24-months observation didn't reveal progression in lung function impairment.
  
  
  CONCLUSIONS
  Lung function impairment is relatively common in children with CTDs. Although restrictive ventilatory pattern is considered typical feature of lung involvement in CTDs, airflow limitation could also be an initial abnormality.
authors.forename: Joanna
authors.initials: J
authors.lastname: Peradzyńska
authors.affiliation: Department of Pediatric Pneumonology and Allergy, Medical University of Warsaw. katarzynakrenke@gmail.com.
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Krenke
authors.forename: Anna
authors.initials: A
authors.lastname: Szylling
authors.forename: Beata
authors.initials: B
authors.lastname: Kołodziejczyk
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Gazda
authors.forename: Lidia
authors.initials: L
authors.lastname: Rutkowska-Sak
authors.forename: Marek
authors.initials: M
authors.lastname: Kulus
mesh_terms: D000293:Adolescent; D002648:Child; D003240:Connective Tissue Diseases / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology*; D018450:Disease Progression; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D008176:Lung Volume Measurements; D015995:Prevalence; D012129:Respiratory Function Tests; D013147:Spirometry; D014797:Vital Capacity
keywords: lung function tests; pulmonary involvement; rheumatic diseases of childhood