title: Clinical pulmonary involvement in primary Sjogren's syndrome: prevalence, quality of life and mortality--a retrospective study based on registry data.
abstract: OBJECTIVE
  The aim of the present study was to describe the prevalence of clinical pulmonary manifestations in primary SS (pSS), and based on registry data, to assess quality of life (QoL) and mortality in these patients.
  
  
  METHODS
  Patients with pSS consecutively included in the Norwegian systemic CTD and vasculitis registry (NOSVAR) were investigated for pulmonary manifestations when presenting with clinical pulmonary symptoms. Pulmonary involvement was defined as typical abnormalities identified with high-resolution CT (HRCT) and/or pulmonary function tests (PFTs).
  
  
  RESULTS
  Among patients referred from our primary area, Oslo (n = 117), lung involvement was found in 26 patients (22%). In our total cohort (n = 216), 59 patients (27%) were affected. A higher rate of pulmonary complications and trends towards longer disease duration and higher age indicated a selection of more complicated cases referred from outside our primary area. Abnormal HRCTs were found in 50 patients (23%) and PFTs in 34 patients (16%). The Medical Outcomes Study 36-Item Short-Form Health Survey Physical Functioning subscore, was significantly reduced in patients with lung involvement (P = 0.03). Furthermore, a 4-fold increased risk of dying after 10 years of disease among patients with lung involvement (n = 10, 17%) compared with those without lung involvement (n = 7, 4.5%) (P = 0.002) was found.
  
  
  CONCLUSIONS
  We found a high population-based prevalence of clinical pulmonary involvement (22%) among patients with pSS. Moreover, patients with lung involvement had reduced QoL represented by the subscale Physical Functioning, and mortality was increased.
authors.affiliation: Department of Rheumatology, Clinic for Cancer, Surgery and Organ Transplantation, Oslo University Hospital Rikshospitalet, PO Box 4950 Nydalen, 0424 Oslo, Norway. opalm@ous-hf.no
authors.forename: Oyvind
authors.initials: O
authors.lastname: Palm
authors.forename: Torhild
authors.initials: T
authors.lastname: Garen
authors.forename: Tone
authors.initials: T
authors.lastname: Berge Enger
authors.forename: Janicke Liaaen
authors.initials: JL
authors.lastname: Jensen
authors.forename: May-Brit
authors.initials: MB
authors.lastname: Lund
authors.forename: Trond Mogens
authors.initials: TM
authors.lastname: Aaløkken
authors.forename: Jan Tore
authors.initials: JT
authors.lastname: Gran
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015897:Comorbidity; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000453:epidemiology* / Q000401:mortality / Q000523:psychology; D008297:Male; D008875:Middle Aged; D009664:Norway / Q000453:epidemiology; D015995:Prevalence; D011788:Quality of Life; D012042:Registries; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000453:epidemiology* / Q000401:mortality / Q000523:psychology; D016019:Survival Analysis