title: Nonspecific interstitial pneumonia preceding diagnosis of collagen vascular disease.
abstract: The aim of this study was to evaluate the incidence and clinical features of patients who developed collagen vascular disease (CVD) after an initial diagnosis of idiopathic nonspecific interstitial pneumonia (NSIP).
  
  
  
  We conducted a retrospective review of 72 consecutive patients with NSIP who were diagnosed by surgical lung biopsy in our institution (idiopathic NSIP, n = 35; CVD-NSIP, n = 37 at initial diagnosis). No patients fulfilled the American College of Rheumatology criteria for a diagnosis with CVD within six months after the diagnosis of idiopathic NSIP.
  
  
  
  Of 35 patients initially diagnosed with idiopathic NSIP, six patients (17.1%) developed CVD during the follow-up period (5.5 ± 5.0 years); three patients were diagnosed with dermatomyositis (DM), two patients with overlap syndrome (DM and Sjogren's syndrome), and one patient with rheumatoid arthritis. The mean time until CVD diagnosis was 2.0 years (six months - 3.5 years), and the one-, two- and three-year incidences of CVD development were 3.6%, 15.2% and 20.0%, respectively. There was no significant difference in clinical characteristics and survival among patients with NSIP preceding CVD diagnosis, those with idiopathic NSIP, or those with CVD-NSIP. In addition, at the time of initial diagnosis, there was no significant difference for the fulfillment of previous criteria such as interstitial pneumonia with autoimmune feature (IPAF) between patients with NSIP preceding CVD diagnosis and those with idiopathic NSIP.
  
  
  
  It is difficult to predict CVD occurrence and careful attention is needed to detect the development of CVD in patients with idiopathic NSIP.
authors: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan; Department of Laboratory Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors: Masato
authors: M
authors: Kono
authors: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan. Electronic address: nakayuta@hama-med.ac.jp.
authors: Yutaro
authors: Y
authors: Nakamura
authors: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors: Katsuhiro
authors: K
authors: Yoshimura
authors: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors: Yasunori
authors: Y
authors: Enomoto
authors: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors: Yoshiyuki
authors: Y
authors: Oyama
authors: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors: Hironao
authors: H
authors: Hozumi
authors: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors: Noriyuki
authors: N
authors: Enomoto
authors: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors: Tomoyuki
authors: T
authors: Fujisawa
authors: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors: Naoki
authors: N
authors: Inui
authors: Department of Laboratory Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors: Etsuko
authors: E
authors: Hamada
authors: Department of Laboratory Medicine and Pathology, Mayo Clinic, Scottsdale, AZ, USA.
authors: Thomas V
authors: TV
authors: Colby
authors: Department of Laboratory Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors: Masato
authors: M
authors: Maekawa
authors: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors: Takafumi
authors: T
authors: Suda
mesh_terms: D000328:Adult; D000368:Aged; D001172:Arthritis, Rheumatoid / Q000473:pathology; D001327:Autoimmune Diseases / Q000453:epidemiology / Q000473:pathology; D003094:Collagen / Q000378:metabolism*; D003240:Connective Tissue Diseases / Q000150:complications* / Q000453:epidemiology* / Q000473:pathology; D003882:Dermatomyositis / Q000473:pathology; D005260:Female; D006801:Humans; D054988:Idiopathic Interstitial Pneumonias / Q000150:complications* / Q000000981:diagnostic imaging* / Q000473:pathology; D015994:Incidence; D008168:Lung / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000150:complications / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000473:pathology; D012907:Smoking / Q000453:epidemiology; D015898:Tomography Scanners, X-Ray Computed; D014652:Vascular Diseases / Q000150:complications* / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000473:pathology
chemical_list: D003094:Collagen
keywords: Collagen vascular disease (CVD); Connective tissue disease (CTD); Idiopathic interstitial pneumonias (IIPs); Interstitial pneumonia with autoimmune features (IPAF); Nonspecific interstitial pneumonia (NSIP)