Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease without proven effective therapy.A multicentre, double-blind, placebo-controlled, randomised phase III clinical trial was conducted in Japanese patients with well-defined IPF to determine the efficacy and safety of pirfenidone, a novel antifibrotic oral agent, over 52 weeks. Of 275 patients randomised (high-dose, 1,800 mg?day ; or placebo groups in the ratio 2:1:2), 267 patients were evaluated for the efficacy of pirfenidone. Prior to unblinding, the primary end-point was revised; the change in vital capacity (VC) was assessed at week 52. Secondary end-points included the progression-free survival (PFS) time.Significant differences were observed in VC decline (primary end-point) between the placebo group (-0.16 L) and the high-dose group (-0.09 L) (p50.0416); differences between the two groups (p50.0280) were also observed in the PFS (the secondary end-point). Although photosensitivity, a well-established side-effect of pirfenidone, was the major adverse event in this study, it was mild in severity in most of the patients.Pirfenidone was relatively well tolerated in patients with IPF. Treatment with pirfenidone may decrease the rate of decline in VC and may increase the PFS time over 52 weeks. Additional studies are needed to confirm these findings.

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It is considered controversial whether superficial temporal artery (STA)-middle cerebral artery (MCA) bypass affects the outcome of patients with ischemic stroke. This prospective study was undertaken to demonstrate the effect of STA-MCA bypass on the cerebral blood flow and neurological status of the patients with ischemic stroke. Seventy-five patients underwent unilateral or bilateral STA-MCA bypass surgery. The selection of the patients closely adhered to the criteria of the Japan EC-IC Bypass Trial (JET). Cerebral blood flow (CBF) before and after Diamox administration was measured by single photon emission computed tomography (SPECT) using iodine-123-N-isopropyl-p-iodoamphetamine (IMP). MRI, contrast-enhanced 3D CT scans, and angiography were performed on each patient pre- and postoperatively. Bypass surgery was successfully done in all patients. CBF was significantly increased after STA-MCA bypass (P < 0.05). In addition, reservation of CBF was significantly improved after STA-MCA bypass (P < 0.05). Patients with transient ischemic attack (TIA) did not experience recurrence of such episodes after STA-MCA bypass. The neurological deficit was unchanged in patients with complete stroke after bypass surgery. However, the NIH stroke scale was significantly improved after bypass surgery (P < 0.01). In addition, the satisfaction rate of treatment as assessed by the patients themselves was very high after STA-MCA bypass (>90%) compared to the conservative treatment group (<50%). STA-MCA bypass still plays a limited role in the treatment of ischemic stroke, but may become a bright hope in depressed patients after cerebral ischemia.

Common variable immunodeficiency (CVID) is a heterogeneous subset of immunodeficiency disorders. Recurrent bacterial infection is the main feature of CVID, but various non-infectious complications can occur. A 42-year-old woman presented with cough and abnormal chest X-ray shadows. Laboratory tests showed remarkable hypogammaglobulinemia. Computed tomography revealed multiple consolidation and nodules on the bilateral lung fields, systemic lymphadenopathy, and splenomegaly. A surgical lung biopsy specimen provided the final diagnosis of lymphoproliferative disease in CVID, which was grouped under the term granulomatous lymphocytic interstitial lung disease. Interestingly, the lung lesions of this case resolved immediately after the initiation of intravenous immunoglobulin monotherapy.