hronic thromboembolic pulmonary hypertension (CTEPH) is a relatively rare disease. Its natural history and etiology remain unclear. [1][2][3][4][5] There have been a number of reports that pulmonary thromboendarterectomy (PTE) is an effective modality for treatment in selected patients with CTEPH. [1][2][3][6][7][8][9][10][11] However, the hemodynamic benefit varies according to the location and extent of the thromboembolic occlusion, and Bergin and colleagues reported that the computed tomography (CT) angiographic extent of central disease (ie, CD score) is related to a low pulmonary vascular resistance (PVR) after surgery. 12 Our preliminary data also showed high operative mortality with CD scores of 0 (25%) and 1 (20%), compared with those with a CD score of ≥2 (7.7%), so we classified those patients with a CD score of ≤1 as having relatively peripheral type CTEPH. The cause of operative death was related to residual pulmonary hypertension as a result of failure to remove a distal obstruction. New guidelines have recommended a post-surgical estimated reduction in PVR of >50%. 10 The validity of PTE might be limited to central-type patients, especially in institutions in which the operation is performed infrequently.The prognosis of CTEPH in the medically treated group had been thought to be poor, 13-15 but Ono et al reported that oral beraprost sodium improved their survival. 16 Recently, there have been some reports about improved 6-min walk distance and pulmonary hemodynamics after epoprostenol, 17,18 sildenafil 19 and bosentan [20][21][22] in patients with CTEPH. These new drugs might improve vascular remodeling, and may offer improved survival in patients with relatively peripheral type CTEPH, in whom we predicted a poor reduction in PVR after surgery. We retrospectively tested the validity of PTE from the aspects of survival and quality of life (QOL) at long-term follow up in patients with relatively peripheral type CTEPH. Methods PatientsBetween April 1999 and March 2006, a total of 83 patients admitted consecutively to Chiba University Hospital were diagnosed as having CTEPH and evaluated for surgical indication. CTEPH was defined as having a mean pulmonary arterial pressure (PPA) of ≥25 mmHg with normal wedge pressure in patients who had dyspnea on exertion during a period of more than 6 months. In addition, lung
Antifibrotic agents have been widely used in patients with idiopathic pulmonary fibrosis (IPF). Long-term continuation of antifibrotic therapy is required for IPF treatment to prevent disease progression. However, antifibrotic treatment has considerable adverse events, and the continuation of treatment is uncertain in many cases. Therefore, we examined and compared the continuity of treatment between pirfenidone and nintedanib in patients with IPF. We retrospectively enrolled 261 consecutive IPF patients who received antifibrotic treatment from six core facilities in Gunma Prefecture from 2009 to 2018. Among them, 77 patients were excluded if the antifibrotic agent was switched or if the observation period was less than a year. In this study, 134 patients treated with pirfenidone and 50 treated with nintedanib were analyzed. There was no significant difference in patient background, discontinuation rate of antifibrotic treatment over time, and survival rate between the two groups. However, the discontinuation rate due to adverse events within one year of antifibrotic treatment was significantly higher in the nintedanib group than in the pirfenidone group (76% vs. 37%, p < 0.001). Furthermore, the discontinuation rate due to adverse events in nintedanib was higher than that of pirfenidone treatment throughout the observation period (70.6% vs. 31.2%, p = 0.016). The pirfenidone group tended to be discontinued due to acute exacerbation or transfer to another facility. The results of this study suggest that better management of adverse events with nintedanib leads to more continuous treatment that prevents disease progression and acute exacerbations, thus improving prognosis in patients with IPF.
The efficacy of benralizumab, as well as mepolizumab, to granulomatosis with polyangiitis (EGPA) involved with mononeuritis multiplex remains unclear. We experienced a case of EGPA presenting neuropathy with severe asthma. Muscle weakness due to neuropathy involved with gait disturbance was partly ameliorated by intravenous immunoglobulin therapy. Mepolizumab (100 mg/day) did not promote further improvement of neuropathy. However, the administration of benralizumab instead of mepolizumab improved neuropathy quickly and enabled walking alone. The efficacy of benralizumab for EGPA and its complication has been maintained for over four years. Benralizumab may be a possible treatment for EGPA presenting neuropathy with severe asthma.
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