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A 38-year-old woman was admitted to our university hospital with loss of muscle strength. She was diagnosed with dermatomyositis and underwent contrast-enhanced computed tomography of the entire body to check for malignant tumors. Computed tomography revealed multiple enhanced hepatic nodules and an extrahepatic portosystemic shunt. Although a needle biopsy of the nodule could not diagnose definitive hepatocellular carcinoma, some nodules increased in size after three months. Because of the inconclusive results of the second biopsy, we performed shunt embolization using a vascular plug. After another three months, the hepatic nodules shrank markedly, as expected.

A 36-year-old Japanese man presented with cavities and nodular shadows in the lower lobes of his lungs and osteolytic lesions in the thoracic spine. He was diagnosed with multisystem Langerhans cell histiocytosis (LCH). Three years earlier, he had been noted to have small cavities and granular lesions noted in the upper lobes of his lungs, which later improved with smoking cessation. It was likely that his single-system pulmonary LCH (PLCH) progressed to multisystem LCH despite smoking cessation. Relapse or progression may occur in cases where PLCH lesions improve after smoking cessation. Thus, close follow-up is vital.

Pulmonary nodular lymphoid hyperplasia (PNLH) is a very rare disease, and it is difficult to diagnose PNLH and distinguish it from mucosa-associated lymphoid tissue (MALT) lymphoma. In addition, information on bronchoalveolar lavage fluid (BALF) analyses is lacking. We herein report a 36-year-old Japanese woman diagnosed with PLNH by a surgical biopsy and analysis of BALF. The BALF showed an increase in B-cell marker-positive lymphocytes, normal patterns of B-cell clonality, mucosa-associated lymphoid tissue 1 gene, and immunoglobulin heavy chain at 14q32 translocations. We also reviewed Japanese cases of PNLH described in Japanese or English to explore the characteristics of such cases.

A 26-year-old Japanese woman was admitted with a 1-month history of diarrhea, a high fever for a few days, and exacerbation of dyspnea. She was treated with an antifibrotic drug and long-term oxygen therapy for Hermansky-Pudlak syndrome-related pulmonary fibrosis. New ground-glass attenuation appeared on chest computed tomography (CT), and a colon biopsy showed an inflammatory cell accumulation with a high titer of myeloperoxidase (MPO)-specific anti-neutrophil cytoplasmic antibodies (ANCA). Systemic inflammation related to MPO-ANCA titer elevation was suspected. Steroid pulse therapy and intravenous cyclophosphamide improved chest CT findings and diarrhea. Therefore, immunosuppressant treatment should be considered for systemic inflammation related to MPO-ANCA.

A 65-year-old Japanese man with interstitial pneumonia demonstrated honeycomb lung with thickened walls on chest high-resolution computed tomography (HRCT) and predominance of neutrophils in the cell fraction of the bronchoalveolar lavage fluid. Although there were no centrilobular nodular or branching shadows on chest HRCT suggestive of diffuse panbronchiolitis, he exhibited sinusitis and had the human leukocyte antigen (HLA)-B54 antigen. With long-term macrolide therapy, the cough and sputum production markedly improved, wall thickening of the honeycomb lung on chest HRCT decreased, and the forced vital capacity increased. Confirming the presence of HLA-B54 antigen may help determine the indication for long-term macrolide therapy in interstitial pneumonia patients.

We herein report a case of recurrent multifocal, distal-dominant-sensorimotor neuropathy with ophthalmoplegia, IgM anti-GM1 antibody, and pyrexia-associated relapse. The patient developed sensory disturbance in her limbs after febrile disease at 50 years old. She had experienced several similar episodes and was admitted to the hospital at 56 years old. Based on a pathological study and electrophysiological findings consistent with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), maintenance IVIg therapy was administered and produced partial improvement with no relapse at one-year follow-up. Immunohistochemical studies suggested the presence of IgG (not IgM) anti-myelin antibodies. Chronic neuropathy with ophthalmoplegia and pyrexia-associated relapse may be a unique variant of CIDP.

A 73-year-old woman with a history of diarrhea for one year and other various symptoms was admitted to our hospital. Gastrointestinal endoscopy that included enteroscopy with multiple biopsies was performed. However, no significant findings were observed. Electrocardiography showed low voltage in all limb leads, and an echocardiogram showed thickened cardiac walls with granular sparkling pattern. A myocardial biopsy revealed amyloidosis, and a bone marrow biopsy showed multiple myeloma. This case suggests that we should suspect the possibility of amyloidosis in a patient with diarrhea and various symptoms involving multiple organ systems. Additionally, electrocardiograms and echocardiograms should be performed even when gastrointestinal biopsies reveal negative results.

A durable response after the discontinuation of immune checkpoint-inhibitor therapy has previously been reported in several cancers. We herein describe a patient with gastric cancer who maintained a durable response after the discontinuation of nivolumab. A 65-year-old man was treated with nivolumab as a sixth-line therapy for recurrent gastric cancer. After four cycles of nivolumab therapy, he showed a partial response. But the treatment was discontinued when two immune-related adverse events occurred after six cycles. Disease regression was sustained for approximately 2 years, without the re-administration of nivolumab. The characteristics leading to such responses are unclear, and further studies are warranted in this regard.

A 45-year-old woman was referred to our hospital for the evaluation of proximal muscle weakness and serum creatine kinase elevation [corrected]. She had atrial fibrillation and left ventricular asynergy. She was diagnosed with myopathy, accompanied by cardiomyopathy of unknown etiology. She was treated with prednisolone. After long-term follow-up and a detailed examination, the patient was diagnosed with antimitochondrial antibody (AMA)-associated myopathy with cardiac involvement. Although the patient received medical treatment, including beta-blockers and prednisolone, her cardiac function deteriorated progressively. Physicians should consider AMA-associated myopathy when diagnosing myopathies of unknown etiology. The presence of cardiac involvement should be proactively investigated in AMA-associated myopathy.

We report an unusual case of acute myocardial infarction in a high school girl. The patient was 17 years of age and had multiple coronary risk factors, including marked obesity with a body mass index (BMI) of 42.7 kg/m 2 , dyslipidemia and glucose intolerance. She had been an on and off smoker since she was 13 years of age. Due to the recent Westernization of the lifestyle, the prevalence of metabolic syndrome in the young generation has been increasing in Japan. Cardiovascular disease based on lifestyle-related diseases may become more common in young people.

We herein report three patients with cardiac angiosarcoma who were directly admitted to the intensive-care unit for hemodynamic instability with circulatory collapse. Using a multidisciplinary cardio-oncologic approach, we diagnosed their condition as angiosarcoma by an invasive biopsy and urgently started weekly paclitaxel administration despite their poor performance status. Their vital signs were soon stabilized, leading to the patients' discharge from the hospital. Although no treatment guidelines for cardiac angiosarcoma have been established, chemotherapy with paclitaxel can be an option for cases presenting with hemodynamic instability.

Liposomal-amphotericin B (L-AmB) is used for cutaneous leishmaniasis (CL); however, its treatment failure has not yet been described in detail. A 58-year-old man returned from the Republic of Venezuela with a cutaneous ulcer on his left lower leg. The causative pathogen was Leishmania braziliensis. We started L-AmB 3 mg/kg/day for 6 days; however, the ulcer did not resolve. The patient was successfully retreated with a higher dose L-AmB 4 mg/kg/day 9 times (total, 36 mg/kg). If L-AmB fails to treat CL and other therapeutics cannot be used, increasing the L-AmB dose is a viable option.

A 69-year-old Japanese woman was admitted to our hospital with progressive muscle weakness and dysphagia. She was taking pitavastatin for dyslipidemia. Her serum creatine kinase was 6,300 U/L. Pitavastatin was stopped, but her symptoms deteriorated, and cardiac congestion appeared. A muscle biopsy showed necrotizing myopathy (NM), and anti-signal recognition particle (SRP) antibody was positive. 18 F-fluorodeoxyglucose-positron emission tomography showed an abnormal uptake, and magnetic resonance imaging showed abnormal gadolinium enhancement in the left ventricular wall. An endomyocardial biopsy revealed inflammatory cardiomyopathy. Steroid, tacrolimus, and intravenous immunoglobulins were effective against the symptoms. This is the first case of biopsy-proven secondary cardiomyopathy due to anti-SRP-positive NM.

Ewing's sarcoma (ES)/primitive neuroectodermal tumors (PNETs) are highly malignant neoplasms that usually affect the bones and soft tissues in children and young adults. ES/PNET of the lung is very rare and is associated with a poor prognosis. We herein report a case of ES/PNET of the left lung in a 45-year-old man. He was treated with neoadjuvant chemotherapy and pneumonectomy, but unfortunately his disease recurred 1.5 months after surgery. He was started on pazopanib, which resulted in a five-month progression-free survival. To our knowledge, this is the first demonstration of pazopanib efficacy in ES/PNET of the lung.

Clinical trials and real-world data have proven that hepatitis C virus (HCV) in most infected patients can be eradicated by direct-acting antivirals (DAAs). However, the proper retreatment regimen for hemodialysis patients with HCV infection who have previously failed to respond to DAAs has not been clarified. We herein report, for the first time, the successful retreatment with glecaprevir and pibrentasvir, of three hemodialysis patients with genotype 1 or 2 HCV infection, who had previously failed to respond to combination therapy with an HCV-NA5A inhibitor (daclatasvir) and an HCV protease inhibitor (asunaprevir).

We herein report a case of a 75-year-old woman who presented with a low-grade fever, repeated cold-induced urticaria, and painful leg edemas with neutrocytosis. Because her mother also had cold-induced urticaria and her skin lesions histologically showed neutrophilic dermatitis, we suspected that she had familial cold autoinflammatory syndrome, a subtype of cryopyrin-associated periodic syndromes. Sequencing of the NLRP3 and MEFV genes revealed that she carried both the p.A439V missense mutation and p.E148Q homozygous mutation, which is commonly detected in familial Mediterranean fever patients. The administration of colchicine reduced the frequency and severity of her skin rash and leg edema.

The efficacy and safety of glecaprevir and pibrentasvir in Japanese patients with human immunodeficiency virus (HIV) and/or genotype 3 hepatitis C virus (HCV) infection is yet to be clarified. This is because no or only a few patients have been included in Japanese phase 3 trials. We herein report for the first time the successful treatment of glecaprevir and pibrentasvir in three Japanese patients with HIV and genotype 3 HCV coinfection as well as hemophilia. Glecaprevir and pibrentasvir treatment is safe and effective for Japanese patients with genotype 3 HCV and HIV coinfection.

Anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody is associated with rapidly progressive interstitial lung disease (RP-ILD) in patients with clinically amyopathic dermatomyositis (CADM) or dermatomyositis (DM). We herein report three Japanese cases of anti-MDA5 antibody-positive RP-ILD without signs of CADM or DM. High-resolution computed tomography revealed patchy or subpleural distribution of consolidations and/or ground-glass opacities accompanied by traction bronchiectasis. All patients succumbed to respiratory failure within two months. Anti-MDA5 antibody-positive RP-ILD without signs of CADM or DM should be included in the differential diagnosis of acute/subacute ILD. Measurement of anti-MDA5 antibody and an intensive immunosuppressive regimen might rescue these patients from RP-ILD.

We report the case of a 36-year-old previously healthy woman who presented with fever and headache. Blood and cerebrospinal cultures and a bacterial analysis revealed the presence of β-lactamase non-producing ampicillin-resistant Haemophilus influenzae type f (Hif) with sequence type 124. Accordingly, the patient was diagnosed with bacterial meningitis with bacteremia caused by Hif. She had normal humoral immunity, and antibiotic therapy rapidly improved her condition. Our case indicates that serotype replacement can occur in Japan and suggests that a certain sequence type causes invasive Haemophilus influenzae disease, regardless of host immunity.

A 65-year-old Japanese man was referred to our hospital for the further assessment of cough and dyspnea. He had a history of ulcerative colitis for which he was receiving treatment. Chest computed tomography showed a crazy-paving pattern. His bronchoalveolar lavage fluid had a milky appearance, and a transbronchial lung biopsy specimen revealed acellular periodic acid-Schiff stain-positive bodies. The serum anti-granulocyte macrophage-colony stimulating factor (GM-CSF) antibody titer was elevated. The diagnosis was autoimmune pulmonary alveolar proteinosis (PAP). There are few reports of autoimmune PAP in patients with ulcerative colitis. Some reports suggest that PAP and inflammatory bowel disease might have a common pathogenesis involving the anti-GM-CSF antibody.

A 56-year-old man with gastroesophageal reflux disease (GERD) was referred to our hospital. Esophagogastroduodenoscopy (EGD) revealed no evidence of any polypoid lesions in the stomach, and the patient had no history of Helicobacter pylori infection. He received omeprazole (20 mg) once daily for the GERD. EGD was performed at 1 year after the start of omeprazole administration, and this time, gastric hyperplastic polyps (GHPs) were detected. The GHPs increased in size as the omeprazole treatment continued, but they markedly decreased in size following omeprazole discontinuation. Thus, the administration of proton pump inhibitors may be a risk factor for the development of GHP independent of H. pylori infection.

Although pneumothorax has been reported to be a major pulmonary adverse event in patients treated with pazopanib, a multikinase inhibitor, drug-induced interstitial lung disease (DILD) has not been reported. A 74-year-old Japanese man who received pazopanib for the treatment of femoral leiomyosarcoma and lung metastasis presented with dyspnea and fatigue. He had mild interstitial pneumonia when pazopanib treatment was initiated. Chest computed tomography revealed progressive bilateral ground-glass opacity (GGO) and traction bronchiectasis. We diagnosed DILD due to pazopanib. The patient's pazopanib treatment was interrupted and a steroid was administered. The symptoms and GGO were improved with treatment. Physicians should be aware of DILD due to pazopanib in patients with pre-existing interstitial lung disease.

Central nervous system graft-versus-host disease can present quite a diagnostic challenge. We herein present a case of histologically-confirmed chronic graft versus host disease (GVHD) involving the central nervous system that occurred at 19 months after peripheral blood stem cell transplantation. Cranial magnetic resonance imaging showed areas of confluent hyperintensity in the deep/subcortical white matter with multiple punctate and curvilinear gadolinium enhancements, suggesting the disruption of the blood-brain barrier. A brain biopsy revealed perivascular CD3-positive T cell infiltration around the small vessels. We propose that the detection of punctate-enhanced lesions by magnetic resonance imaging may be a useful finding that facilitates the early diagnosis of chronic GVHD involving the central nervous system.

Two men, 48 and 54 years of age, were referred for medical checkups without any particular symptoms. Upper gastrointestinal endoscopy showed a normal gastric body, but white marbled appearance in the lesser curvature of the gastric angle and antrum. Biopsy specimens revealed relatively long and tightly coiled organisms. The two patients were diagnosed as having non-Helicobacter pylori helicobacter (NHPH) infection according to the findings of pathological and quantitative reverse transcription-polymerase chain reaction (qRT-PCR) analyses. After triple therapy (amoxicillin, clarithromycin, and rabeprazole), endoscopy showed an improvement of the white marbled lesions and biopsy specimens showed no NHPH. The white marbled appearance limited to the gastric angle and antrum may be a potential characteristic finding of NHPH-infected gastritis.

Seminomas rarely metastasize to the gastrointestinal tract. In general, these lesions metastasize to the lungs or retroperitoneal lymph nodes. A 34-year-old Japanese man who had undergone orchiectomy for seminoma two years earlier experienced shortness of breath and tarry stools. The patient presented at our hospital and was diagnosed with metastatic seminoma to the third portion of the duodenum on double balloon endoscopy. He was effectively treated with chemotherapy and continues to progress well, with no episodes of recurrence.

A 47-year-old man was referred to our hospital with an abnormal shadow on a chest X-ray. He had a history of untreated chronic sinusitis and suspected ulcerative colitis (UC). Chest CT revealed a diffuse centrilobular granular shadow, while laboratory tests demonstrated an increased proportion of neutrophils; however, no microorganisms were detected in bronchoalveolar lavage fluid. Therefore, sinobronchial syndrome or small airway disease associated with UC was diagnosed, and the patient was treated with long-term erythromycin therapy. Small airway disease associated with UC is usually treated with steroids. Our experience shows that airway involvement in patients with inflammatory bowel disease can be treated with macrolides.

This report concerns a 62-year-old man with primary cutaneous diffuse large B-cell lymphoma (PCLBCL), leg type that developed during methotrexate (MTX) treatment for rheumatoid arthritis (RA). Several tumors were observed on the left lower leg. A histological analysis showed diffuse proliferation of large neoplastic B-cells that were immunophenotypically CD10-/MUM1+/BCL6-/BCL2+ and cytogenetically had IgH/c-MYC translocation without translocation involving BCL6 or IgH/BCL2. No evidence of Epstein-Barr virus (EBV) infection was found. The discontinuation of MTX resulted in a 20-month disease-free period. No previous cases of PCLBCL, leg type associated with RA or MTX therapy have been reported. The phenotypes of our patient were partly different from those of typical PCLBCL, leg type or RA/MTX-associated lymphoma.

Hepatosplenic gamma-delta T-cell lymphoma (HSTCL) is a rare, aggressive subset of peripheral T-cell lymphoma. It has been reported that Epstein-Barr virus (EBV) infection can cause HSTCL; however, such cases are extremely rare, with only a few cases having been reported to date. We herein report an autopsy case of HSTCL associated with EBV infection. The presence of EBV infection was confirmed in serum EBV DNA and on in-situ hybridization, and cytotoxic molecules, such as granzyme B, perforin and T-cell intracytoplasmic antigen (TIA)-1, were all positive in lymphoma cells. These findings indicate that stimulation of persistent EBV infection may have caused HSTCL in this patient.

We herein report the case of 41-year-old homosexual man who presented to our hospital with typical acute retroviral syndrome. Complications of bilateral facial nerve palsy and appendicitis appeared eight days after admission. The bilateral facial nerve palsy spontaneously recovered one month later; however, the appendicitis required surgical intervention. To our knowledge, this is the first reported case of appendicitis related to acute retroviral syndrome.

Hermansky-Pudlak syndrome (HPS) is a rare genetic disorder, the most common complication of which influencing the prognosis is pulmonary fibrosis. In the present report, we describe an autopsy case of a Japanese woman with HPS. The patient was diagnosed at 50 years of age based on the presence of oculocutaneous albinism, hemorrhagic diathesis, ceroid-lipofuscin accumulation and pulmonary fibrosis. Although systemic steroids, immunosuppressants and pirfenidone were administered for pulmonary involvement, she died from respiratory failure two years later. Obtaining an early diagnosis and taking into consideration the need for lung transplantation is necessary in order to improve the prognosis of HPS. We herein report this very rare Japanese case of HPS with a review of the treatment approaches for HPS complicated with pulmonary fibrosis.

A 13-year-old boy was brought to our hospital after recovering from ventricular fibrillation that occurred after an episode of chest pain during training with his soccer team. Subsequent 64-slice multidetector computed tomography revealed the left coronary artery arising from the right sinus of Valsalva, which coursed between the ascending aorta and root of the main pulmonary artery. Surgical correction including unroofing of the left coronary ostium and pulmonary artery translocation was performed successfully. One year later, he remained asymptomatic and was back on his soccer team.

Solid-pseudopapillary neoplasms (SPN) are rare pancreatic tumors. The etiology of SPN involves mutations in the gene that encodes β-catenin (CTNNB1). We herein report the case of a 23-year-old woman with a large SPN with proliferating tumor cells that displayed both solid and pseudo-papillary patterns. The simultaneous nuclear accumulation and loss of membrane localization of β-catenin and E-cadherin was specifically observed in the tumor cells. Further, the tumor cells were shown to harbor a missense mutation in exon 3 of CTNNB1. We also present a review of the literature describing the clustering of CTNNB1 mutations in patients with SPN.

Ankylosing spondylitis (AS) primarily affects the spine and axial skeleton. Various pulmonary manifestations have been reported; however, diffuse alveolar hemorrhage (DAH) has not been previously described in a patient with AS. A 49-year-old man with longstanding AS visited our hospital complaining of progressive dyspnea and hemoptysis. DAH was diagnosed based on the findings of chest computed tomography and bronchoscopy. No positive findings suggested any cause of DAH other than weakly positive results for perinuclear antineutrophil cytoplasmic antibodies on indirect immunofluorescence. Following the administration of steroid and plasmapheresis therapy, the patient's symptoms and chest radiographic findings improved. Further clinical case reports and investigations are needed to clarify whether DAH represents a possible pulmonary manifestation of AS.

A 67-year-old man, on oral therapy for type 2 diabetes mellitus since 1990, had sustained proteinuria since 2005. When hematuria was first discovered in 2008, renal dysfunction [creatinine (Cr), 1.2 mg/dL], inflammation [C-reactive protein (CRP), 12 mg/dL] and high myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA) levels [546 ELISA units (EU)] were observed. Renal biopsy showed the diagnosis of ANCA-associated nephritis combined with diabetic nephropathy. For this patient, there was pathological proof of the combination of diabetic nephropathy and ANCA-associated vasculitis.

Objective: Capsule endoscopy (CE) allows direct examination of the small bowel in a safe, noninvasive and well-tolerated manner. Nonetheless, experience indicates failure to reach the cecum in 20-30% of patients within the 8 hour battery life. Attempts to improve the completion rate (CR) as defined by reaching the cecum have been unsuccessful. This study was to investigate the relationship between patients' physical activity and CR., Methods: Between January 2009 and January 2010, 76 patients (44 men, 32 women; median age 64.5 yr) underwent CE for the diagnosis of small intestinal disorders. Indications for CE were obscure gastrointestinal bleeding/anemia (62 cases), others (14 cases). Patients were divided into an outpatient group (n=23), mild bed rest group (n=35) and strict bed rest group (n=18)., Results: For all patients, the average gastric transit time was 65.5 minutes, small bowel transit time was 301.4 minutes and the CR was 86.8%. However, the CR was 100% (23/23) in the outpatient group, an 85.7% (30/35) in the mild bed rest group, and 72.2% (13/18) in the strict bed rest group. The CR increased with physical activity of patients by Cochran-Armitage Trend Test (p=0.009). In multivariate logistic regression analyses, low physical activity was a significant risk factor for failure to reach the cecum during CE examination; adjusted OR: 3.39, 95% CI: 1.01-11.42 (p=0.048)., Conclusion: Our observations suggested that increasing physical activity would increase the likelihood of a complete bowel examination by CE. Further, for CE, inconvenient bowel preparations like the use of polyethylene glycol may be avoided.

We herein report the case of a 61-year-old woman with dilated phase of hypertrophic cardiomyopathy (D-HCM) who had been diagnosed with HCM 17 years previously. On admission, her left ventricle (LV) had marked dilation, dyssynchrony with diffuse severe hypokinesis, and ventricular tachycardia. She had two mutations in the cardiac myosin binding protein-C gene, which were suspected to be the causes of the D-HCM. We performed LV reconstruction surgery and cardiac resynchronization therapy with a defibrillator for her drug-resistant severe heart failure. After surgery, her New York Heart Association class dramatically improved, and she has not been re-hospitalized since these treatments.

The prognosis of patients with chronic respiratory tract infections, especially diffuse panbronchiolitis, is remarkably improved by long-term administration of low-dose macrolides. However, in some cases, patients are refractory to macrolide treatment and show a low or no response; therefore, new treatment strategies are required. Here we present a patient refractory to either single low-dose clarithromycin or azithromycin but responded remarkably to the combination usage of both macrolides.

Diaphragmatic paralysis is commonly caused by surgical and traumatic injuries, malignant neoplasm, and neurodegenerative disorders. However, in rare instances, diaphragmatic paralysis due to herpes-zoster virus infection has been reported. Here, we describe an 85-year-old woman who developed left hemidiaphragmatic paralysis within 19 days of the appearance of a typical herpes-zoster rash involving the C4-5 dermatome on the left side. Clinical and radiological findings revealed no local causes of phrenic nerve lesion. The hemidiaphragmatic paralysis was thought to be caused by herpes-zoster virus infection.

Background and Aim: The incidence of ischemic colitis (IC) in Japan has been increasing due to the westernization of diet and the aging population. The aim of this study was to evaluate the relationship between endoscopic findings and clinical severity in IC., Methods: This retrospective analysis included 106 cases diagnosed with IC that were divided into two groups based on endoscopic findings in the acute stage: redness and erosion (RE) versus longitudinal and circumferential ulcers (LCU). The clinical variables were compared between the two groups. In addition, we investigated the risk factors of IC associated with the severity of the endoscopic findings by multivariate logistic regression analysis., Results: The percentage of cases presenting abdominal pain was significantly higher in the LCU group than that in the RE group (p=0.002), as were the baseline serum CRP levels (p=0.0001). The periods of hospitalization in LCU group were longer than in the RE group (p=0.0001). Multivariate logistic regression analysis indicated that ischemic heart disease (IHD) and connective tissue disease were the independent explanatory factor associated with the endoscopic severity of IC (p<0.05)., Conclusion: We showed clearly that the two endoscopic classifications were accurate indicators of severity and could be used to anticipate severity of IC. Furthermore, we confirmed that IHD and connective tissue disease were the exacerbating factor associated with the severity of endoscopic findings in IC.

Wegener's granulomatosis (WG) is a multisystemic disease of unknown etiology characterized by necrotizing vasculitis and granulomatous inflammation (1-3). The disease typically involves the upper airways, lungs and kidneys, and gastrointestinal involvement is uncommon. Described here is a 33-year-old man who presented at the hospital with abdominal pain. Colonoscopy revealed multiple ulcers, including round ulcers, throughout the large intestine. Small bowel ulcers were detected by double balloon enteroscopy (DBE). Further study confirmed that these ulcers were caused by gastrointestinal complications of WG. The patient was administered prednisolone and cyclophosphamide and remains in remission. This case indicates the importance of considering a gastrointestinal complication of WG as the potential cause of abdominal symptoms among WG patients as well as the use of DBE in detecting such a complication.

Systemic capillary leak syndrome (SCLS) is a life-threatening disorder which presents with periodic episodes of hypovolemic shock, due to plasma leakage to the extra-vascular space reflected by accompanying hypoalbuminemia, hemoconcentration and edema often with associated monoclonal gammopathy. We describe a 28-year-old woman with SCLS who required aggressive fluid resuscitation and was successfully treated with corticosteroid, terbutaline, and theophylline. At exacerbation, the levels of serum granulocyte colony-stimulating factor (G-CSF) were increased. Thus, G-CSF might play an important role and can be a useful biomarker for the severity of attacks in SCLS.

We describe a case of advanced atrioventricular (AV) block, in which treatment with cilostazol was effective in recovering the AV conduction. The patient was referred to our hospital for close examination of the advanced AV block and permanent pacemaker implantation. Although the patient had experienced third-degree AV block with occasional AV synchrony for more than two days, the AV conduction completely recovered after treatment with oral cilostazol at 200 mg/day. Here we discuss the possible mechanism of the improvement in the AV conduction by cilostazol.

A 73-year-old woman who had been diagnosed with systemic sclerosis was admitted for further examination of bilateral hilar lymphadenopathy. Sarcoidosis was confirmed based on elevated serum levels of angiotensin-converting enzyme, a high proportion of lymphocytes and a high CD4/CD8 ratio in bronchoalveolar lavage fluid, abnormal (67)Gallium uptake in the mediastinum and noncaseating granulomas in skin biopsy specimens. In addition, high levels of antimitochondrial M2 antibodies and alkaline phosphatase indicated primary biliary cirrhosis (PBC). Here we describe a rare triplex of sarcoidosis, SSc and PBC. Although the etiology of this complex remains unknown, these three diseases might share some pathogenesis.

A 46-year-old man was referred to our hospital with hemoptysis. He had been diagnosed with chronic sinusitis since childhood, but had received no treatment. Chest CT showed a diffuse centrilobular granular shadow and thickened bronchial walls. Otitis media and decreased spermatic motor ability were identified. In addition, electron microscopy of a biopsy specimen of the nasal mucosa showed a deficiency of inner dynein. Based on these clinical findings, primary ciliary dyskinesia (PCD) was diagnosed and successfully treated with long-term, low-dose clarithromycin. Although the effects of macrolide therapy remain controversial, long-term treatment with low-dose clarithromycin might confer clinical benefits upon patients with PCD.

Cystic fibrosis (CF) is rare in Japan. We encountered a CF case with drug-resistant Pseudomonas aeruginosa infection and successfully performed lung transplant from living related donors. A combination of beta-lactams and aminoglycosides for drug-resistant P. aeruginosa infection was administered before lung transplantation. Intravenous colistin was also used immediately before and after transplant surgery. Gram staining of respiratory specimens was performed every day after surgery and it was useful in monitoring infection status. Strict monitoring of infections by the Gram staining and culture of respiratory specimens is considered to be effective in preventing lower respiratory infection in lung transplantation.