An 81-year-old man was referred to our hospital with bilateral multiple patchy opacities on chest radiography. His chief complaints were a few months’ history of intermittent mild cough and slightly yellow sputum. Chest computed tomography (CT) showed non-segmental air-space consolidations with ground-glass opacities. Amyloid deposition with organizing pneumonia (OP) was seen in transbronchial lung biopsy (TBLB) specimens from the left S8. Three months later, the infiltration originally seen in the left lower lobe was remarkably diminished, and new infiltrations in the lingual and right lower lobes were detected on chest CT. Amyloid deposition with OP was seen in TBLB specimens from the left S4. Transthyretin was detected following immunohistochemical examination. The presence of wild-type transthyretin (ATTRwt) was proven using genetic analysis. The present report describes a rare case of ATTRwt amyloidosis associated with OP.

Background/Aims: Endoscopic biliary drainage is the gold standard treatment for cholangitis. The two methods of biliary drainage are endoscopic biliary stenting and nasobiliary drainage. A novel integrated outside biliary stent and nasobiliary drainage catheter system (UMIDAS NB stent; Olympus Medical Systems) was recently developed. In this study, we evaluated the efficacy of this stent in the treatment of cholangitis caused by common bile duct stones or distal bile duct strictures. Methods: We conducted a retrospective pilot study by examining the medical records of patients who required endoscopic biliary drainage for cholangitis due to common bile duct stones or distal bile duct strictures, and who were treated with a UMIDAS NB stent, between December 2021 and July 2022. Results: Records of 54 consecutive patients were reviewed. Technical and clinical success rates were 47/54 (87.0%) and 52/54 (96.3%), respectively. Adverse events were observed in 12 patients, with six patients experiencing pancreatitis as an adverse event, following endoscopic retrograde cholangiopancreatography (ERCP). Regarding late adverse events, five cases of biliary stent migration into the bile duct were observed. Disease-related death occurred in one patient. Conclusions The outside-type UMIDAS NB stent is an efficacious new method for biliary drainage and can be applied to many indications.

　　A 56-year-old woman was referred to our hospital because of an abnormal finding in the right pulmonary hilum on chest X-ray. Enhanced chest computed tomography showed hyperplastic bronchial arteries dilating and winding around the trachea and bronchi. A racemose hemangioma of the bronchial artery with multiple bronchial artery aneurysms (diameter <20mm) was seen displacing the trachea and both main bronchi. Bronchoscopy showed submucosal tumor-like lesions at the distal trachea and in both main bronchi, and a dusky-red elevated pulsatile lesion at the orifice of the left B3b＋c. We performed coil embolization of the bronchial artery aneurysm to prevent abrupt rupture of the bronchial aneurysm.

PURPOSE: To evaluate whether hydrodistention with fulguration of Hunner lesions (HD/FUL) plus maintenance dimethyl sulfoxide (DMSO) therapy prolongs the recurrence-free time in patients with Hunner type interstitial cystitis (IC).METHODS: The study enrolled patients with Hunner type IC who required repeat HD/FUL due to recurrence of IC symptoms after the first HD/FUL at our institution. All patients received a second HD/FUL plus maintenance DMSO therapy. The maintenance DMSO therapy was performed every 2 weeks for a total of 8 instillations, and then once every 4 weeks thereafter. The recurrencefree time from HD/FUL to therapeutic failure was estimated using the Kaplan-Meier method. The recurrence-free time between the first HD/FUL and second HD/FUL plus maintenance DMSO therapy was statistically compared using the log-rank test.RESULTS: A total of 21 patients (mean age, 66.3±10.8 years) with Hunner type IC were evaluated. The recurrence-free time for the second HD/FUL plus maintenance DMSO therapy was significantly longer than that for the first HD/FUL (P<0.0001). The median recurrence-free time for the first HD/FUL was 10.1 months, while that for the second HD/FUL plus maintenance DMSO therapy has yet to be reached. The recurrence-free rate for the first HD/FUL was 81.0% at 6 months, 38.1% at 1 year, 9.5% at 2 years, and 4.8% at 3 years. In contrast, the rate for the second HD/FUL plus maintenance DMSO therapy was 100% at 6 months, 94.7% at 1 year, 82.6% at 2 years, and 82.6% at 3 years. There were no significant differences in efficacy between the first and second HD/FUL.CONCLUSIONS: HD/FUL plus maintenance DMSO therapy clearly prolongs the recurrence-free time compared with HD/FUL alone in Hunner type IC.
Cystitis, Interstitial ; Dimethyl Sulfoxide ; Humans ; Methods ; Pilot Projects ; Recurrence

In patients with epidermal growth factor receptor (EGFR) mutation-positive lung cancer (LC) who have acquired resistance to first and/or second-generation EGFR-tyrosine kinase inhibitors (EGFR-TKIs), detection of EGFR T790M (T790M) mutation is essential before administration of osimertinib. Tissue sample is the main specimen used to detect the T790M mutation, and so cell block preparation using pleural or pericardial fluid should be considered. The utility of body cavity effusion cell block methods in T790M mutation detection have not yet been fully evaluated. This study aimed to evaluate the clinical background and treatment course of LC patients harboring the T790M mutation by using body cavity effusion cell block methods at our hospital. All patients were treated with first and/or second-generation EGFR-TKIs and had developed malignant pleural or pericardial fluid as a result of progressive disease. T790M mutation status was evaluated using body cavity effusion cell block method in 9 patients, from April 2016 to August 2017. We retrospectively evaluated the clinical characteristics and treatment course of these 9 patients (3 males and 6 females; median age 76 years). At the first diagnosis of LC, 7 patients had stage IV cancer; 4 patients were diagnosed by bronchial fibroscopy and 3 were diagnosed from pleural fluid examination. Regarding EGFR mutation, 3 and 6 patients carried the exon 19 deletion and L858R mutation, respectively. Median time interval between the first diagnosis of LC and T790M mutation evaluation was 30.8 months; 7 patients were diagnosed with positive T790M mutation by using body cavity effusion cell block methods. The T790M mutation was highly detected by examination of body cavity effusion cell blocks. Further evaluation is necessary with respect to variations in T790M detection rate based on the specimen collection site and/or progressive disease pattern in different patients.

Since hand deformities in rheumatoid arthritis (RA) are relatively not very painful, the worsening of the deformities often goes unnoticed and the functional impairment progresses irreversibly. Herein, we report a case of boutonnière deformity of the left middle and ring fingers treated with rehabilitation since an early stage. The patient was a 58-year-old woman who was referred to our hospital due to joint pain in the fingers and feet, following which a diagnosis of RA as made；however, she could not be administered methotrexate due to complications. PIP joint deformity of the left middle and ring fingers developed later. The middle finger was in -50-degree extension and difficult to correct passively, while the ring finger was in -35-degree extension and correctable. Surgery for the left middle finger was proposed based on the diagnosis of boutonnière deformity；however, consent was not obtained. After a steroid injection in the painful middle finger, she was managed using a Capener splint and ROM exercises with finger stretching. The symptoms improved five months following the rehabilitation intervention. Nalebuff et al. classified the severity of the boutonnière deformity based on the limited PIP joint extension and recommended treatment accordingly. In this case, surgical treatment was believed to be required；however, since the patient refused surgery, conservative treatment was chosen. Although the extension was severe, there was little joint destruction, due to which the symptoms improved with early and active intervention. Orthotic treatment and occupational therapy were effective in improving ADL.

Since hand deformities in rheumatoid arthritis (RA) are relatively not very painful, the worsening of the deformities often goes unnoticed and the functional impairment progresses irreversibly. Herein, we report a case of boutonnière deformity of the left middle and ring fingers treated with rehabilitation since an early stage. The patient was a 58-year-old woman who was referred to our hospital due to joint pain in the fingers and feet, following which a diagnosis of RA as made；however, she could not be administered methotrexate due to complications. PIP joint deformity of the left middle and ring fingers developed later. The middle finger was in -50-degree extension and difficult to correct passively, while the ring finger was in -35-degree extension and correctable. Surgery for the left middle finger was proposed based on the diagnosis of boutonnière deformity；however, consent was not obtained. After a steroid injection in the painful middle finger, she was managed using a Capener splint and ROM exercises with finger stretching. The symptoms improved five months following the rehabilitation intervention. Nalebuff et al. classified the severity of the boutonnière deformity based on the limited PIP joint extension and recommended treatment accordingly. In this case, surgical treatment was believed to be required；however, since the patient refused surgery, conservative treatment was chosen. Although the extension was severe, there was little joint destruction, due to which the symptoms improved with early and active intervention. Orthotic treatment and occupational therapy were effective in improving ADL.

A 68-year-old man was admitted to our hospital with complaints of fatigue, polyuria, and loss of appetite, and was diagnosed with diabetic ketosis. Chest and abdominal computed tomography (CT) showed a pulmonary tumor on the right S3 and multiple liver tumors. Blood chemistry revealed elevated levels of amylase and hepatobiliary enzymes. Pathological examination of a biopsy specimen from the liver tumor showed a small cell carcinoma. Based on the imaging and pathological findings, we made a diagnosis of extensive disease small-cell lung cancer (ED-SCLC), cT1aN3M1b (HEP, ADR). Treatment with carboplatin and etoposide evoked partial response and the serum level of amylase decreased. Immunohistochemical staining of liver biopsy specimen was positive for amylase, leading to a diagnosis of SCLC with amylase production. About 22 months after the diagnosis of SCLC, he was admitted to our hospital with fatigue, muscular weakness, edema, and hyperpigmentation. Laboratory findings showed elevated serum levels of hepatobiliary enzymes, adrenocorticotropic hormone (ACTH), and cortisol, and a decreased serum potassium level. Urinary potassium level was elevated. Pituitary magnetic resonance imaging showed a normal morphology. We made a diagnosis of SCLC complicated by Cushing’s syndrome. We report this rare case of SCLC with amylase and ACTH production, which was detected in the course of treatment of SCLC.

A 38-year-old man was admitted to our hospital with fever and skin rash, and he was diagnosed as having dermatomyositis. He was treated with anti-inflammatory steroid and immunosuppressive agents. On hospital day 48, chest computed tomography (CT) revealed a nodule measuring approximately 2 cm in size in the lower lobe of the right lung (S9). Bacterial and/or fungal infection was suspected, but there was no response to antibiotic or antifungal treatment. A week later, repeat chest CT revealed the tumor now measuring approximately 6 cm in size in the lower lobe of the right lung. We performed bronchoscopy, and bacteriological examination of the transbronchial biopsy specimen revealed pulmonary tuberculosis. Interferongamma release assay (IGRA) before the initiation of immunosuppressive treatment was negative, so we did not administer treatment for latent tuberculosis infection. He was, however, treated with isoniazid, rifampicin, ethambutol, and pyrazinamide for 9 months, following which radiological features improved gradually. Here we describe in detail this rare case of a negative IGRA result before immunosuppressive therapy in a relatively young Japanese man who went on to develop active tuberculosis with a rapidly-growing pulmonary lesion during hospitalization.