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Okazaki, Japan

Watanabe N.,Tosei General Hospital
Nihon Kokyūki Gakkai zasshi = the journal of the Japanese Respiratory Society | Year: 2011

Therapy with sildenafil has been shown to decrease pulmonary vascular resistance and may improve functional status in patients with interstitial pneumonia (IP) and pulmonary hypertension (PH). Patients with IP and PH defined by a mean pulmonary artery pressure (MPAP) of > or = 25mm Hg on right-heart catheterization were followed up in an open-label study of sildenafil. A multilateral evaluation was conducted before, and after 3 months of therapy. We studied 11 patients [8 men and 3 women, mean age 66.5] 6 of whom had IPF (1 with usual interstitial pneumonia {UIP}), 2 with IIP, and 3 with collagen-vascular disease interstitial pneumonia (CVD-IP). The mean modified Medical Research Council (MRC) score was 3.0 +/- 0.89, baseline dyspnea index (BDI) score was 4.5 +/- 1.9, % VC was 58.7 +/- 15.6%, percentage of carbon monoxide diffusing capacity (%DLco) was 20.0 +/- 10.9%, six-minute walk distance (6MWD) was 269.8 +/- 105.5m, shuttle walking test (SWT) was 179.1 +/- 99.7m, St. George Respiratory Questionnaire (SGRQ) was 70.9 +/- 15.6, mean pulmonary artery pressure (MPAP) was 33.8 +/- 7.61mm Hg, and pulmonary vascular resistance index (PVRI) was 658.9 +/- 236.1 dynes x s x cm(-5) x m2. After 3 months of therapy, improvements in BDI (< or = -1), 6MWD (> or = 20%), SWT (> or = 20%), and SGRQ (< or = -7) were observed in 4, 2, 3, and 6 patients, respectively. Improvements in MPAP (< or = - 20%) and PVRI (< or = -20%) were observed in 2 and 3 patients, respectively. No parameter showed statistically significant differences. We conclude that sildenafil may improve dyspnea, exercise tolerance and health-related quality of life (QOL) in some IP patients with PH. Source


Kondoh Y.,Tosei General Hospital
Japanese Journal of Chest Diseases | Year: 2013

I discribe interpretation and summary of new international IPF guidelines for diagnosis and clinical management. IPF is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of UIR The diagnosis of IPF requires specific combinations of HRCT and surgical lung biopsy pattern in patients subjected to surgical lung biopsy. The accuracy of the diagnosis of IPF increases with multidisciplinary discussion (MDD) among ILD experts. Clinicians are required to spend adequate time with patients to discuss patients' values, preferences, and prognosis. Patients at increased risk of mortality should be considered for lung transplantation. Pharmacological treatment should be limited to a carefully selected minority of patients who are willing to accept possible adverse consequences even if expected benefits are small. In such cases, it is recommended to discuss indications of weak no recommendation therapies with patients based on their individual values and preferences. Oxygen supplementation (if hypoxemic) and pulmonary rehabilitation are recommended treatments. Symptom control (palliative care) focuses on reducing symptoms (e. g., cough and dyspnea) and providing comfort to patients, rather than treating patients' disease, is also important. Source


Sato I.,Tosei General Hospital
Nagoya journal of medical science | Year: 2011

A 58-year-old woman was referred to our hospital because of liver dysfunction. Her serum levels of AST (619 IU/l) and ALT (603 IU/l) had increased. Histological findings in the liver biopsy were compatible to autoimmune hepatitis (AIH), and the diagnosis of AIH was confirmed by the diagnostic criteria. She was admitted to a nearby hospital 3 years ago, and diagnosed with Graves' disease. She received methimazole (MMI) at first, which was discontinued due to liver injury in one month, then propylthiouracil (PTU) was administered. One year later, transaminase increased and was decreased by stopping PTU administration. PTU was restarted after her transaminase decreased, but a recurrence of hepatotoxicity was observed, and she was referred to our hospital. Oral prednisolone decreased liver function immediately. In this case, PTU-induced liver injury was suspected as a possible trigger of AIH. While PTU remains a commonly used drug in the treatment of hyperthyroidism, severe liver injury is reported in some cases. If liver injury is observed in patients treated with PTU, rechallenge is not recommended in order to avoid severe hepatotoxicity. Source


Abe S.,Nippon Medical School | Azuma A.,Nippon Medical School | Mukae H.,University of Occupational and Environmental Health Japan | Ogura T.,Kanagawa Cardiovascular and Respiratory Center | And 3 more authors.
Internal Medicine | Year: 2012

Objective The prognosis of idiopathic pulmonary fibrosis (IPF) patients with acute exacerbation (AE) is reported to be extremely poor. Several clinical studies suggest that direct hemoperfusion with polymyxin B-immobilized fiber (PMX) may have beneficial effects on AE in patients with interstitial pneumonia (IP). The aim of this multi center retrospective analysis was to investigate whether PMX treatment could provide improvement of oxygenation and survival benefits in IPF patients with AE. Methods We conducted a retrospective study of 160 IP patients (including 73 IPF) with AE treated by PMX at 18 institutions in Japan. PMX treatment was carried out twice. The total hemoperfusion time of PMX treatment was, on average, 12 hours. Data concerning oxygenation on PMX treatment and survival after AE were collected and analyzed. Results In IPF patients with AE, arterial oxygen tension (PaO2)/inspiratory oxygen fraction (FiO2), (P/F) ratio was significantly improved at the end of the 2nd treatment with PMX (173.9±105.4 to 195.2±106.8 Torr, p=0.003). White blood cell count was significantly reduced at the end of the 2nd treatment (13,330±7,002 to 9,426±5,188/mm 3, p<0.001). These clinical changes were also observed on analysis of all 160 IP patients with AE. The one- and three-month survival rates of IPF patients after AE were 70.1% and 34.4%, respectively. Conclusion PMX treatment may improve oxygenation and survival in IPF patients with AE. Prospective, controlled trials of PMX treatment for IPF with AE are warranted to verify this potential benefit. © 2012 The Japanese Society of Internal Medicine. Source


Ogura T.,Kanagawa Cardiovascular and Respiratory Center | Taniguchi H.,Tosei General Hospital | Azuma A.,Nippon Medical School | Inoue Y.,Clinical Research Center | And 12 more authors.
European Respiratory Journal | Year: 2015

A randomised, double-blind, phase II, dose escalation trial was conducted to assess the safety, tolerability and pharmacokinetics of the tyrosine kinase inhibitor nintedanib, alone and when added to ongoing pirfenidone therapy, in Japanese patients with idiopathic pulmonary fibrosis. 50 Japanese patients were randomised to receive nintedanib or placebo in one of three cohorts (nintedanib 50 mg twice daily or 100 mg twice daily for 14 days, or 150 mg twice daily for 28 days). Patients receiving pirfenidone at inclusion were stratified to every nintedanib dose group and placebo. Adverse events were reported in nine out of 17 patients receiving nintedanib alone and 10 out of 21 patients receiving nintedanib added to pirfenidone. All adverse events were mild or moderate in intensity. Gastrointestinal disorders were the most common adverse event. Maximum plasma concentration and area under the curve at steady state for nintedanib and its metabolites tended to be lower when nintedanib was added to pirfenidone. Nintedanib had no effect on the pharmacokinetics of pirfenidone. In conclusion, further study is needed to evaluate the safety and tolerability profile of nintedanib when added to pirfenidone in patients with idiopathic pulmonary fibrosis. There was a trend toward lower exposure of nintedanib when it was added to pirfenidone. Copyright © ERS 2015. Source

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