Masato Kono1, Yutaro Nakamura2, Katsuhiro Yoshimura3, Yasunori Enomoto3, Yoshiyuki Oyama3, Hironao Hozumi3, Noriyuki Enomoto3, Tomoyuki Fujisawa3, Naoki Inui3, Etsuko Hamada4, Thomas V Colby5, Masato Maekawa4, Takafumi Suda3. 1. Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan; Department of Laboratory Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan. 2. Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan. Electronic address: nakayuta@hama-med.ac.jp. 3. Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan. 4. Department of Laboratory Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan. 5. Department of Laboratory Medicine and Pathology, Mayo Clinic, Scottsdale, AZ, USA.
Abstract
BACKGROUND: 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). METHODS: 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. RESULTS: 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. CONCLUSIONS: It is difficult to predict CVD occurrence and careful attention is needed to detect the development of CVD in patients with idiopathic NSIP.
BACKGROUND: 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). METHODS: 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. RESULTS: 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. CONCLUSIONS: It is difficult to predict CVD occurrence and careful attention is needed to detect the development of CVD in patients with idiopathic NSIP.
Authors: Elisabeth Bendstrup; Janne Møller; Sissel Kronborg-White; Thomas Skovhus Prior; Charlotte Hyldgaard Journal: J Clin Med Date: 2019-11-21 Impact factor: 4.241