Objective: To compare salivary gland function in autoimmune pancreatitis (AIP) patients with and without an elevated serum IgG4 concentration.
Patients and Methods: We allocated 14 AIP patients into 2 groups: one group had high (>135 mg/dL) serum IgG4 and the other low serum IgG4. Sialochemistry and submandibular and parotid gland scintigraphy were done in these patients.
Results: Serum IgG4 levels were elevated in 10 patients. Bilateral submandibular gland swelling was present in 5 patients with a high serum IgG4; there was no swelling in patients with a low serum IgG4. The salivary Na+ concentration was increased significantly in both patient groups (p<0.01) compared to controls. The β2-microglobulin concentration was significantly higher in patients with a high serum IgG4 than in those with a low serum IgG4 (p<0.05) and controls (p<0.01). On submandibular and parotid gland scintigraphy, both the ratio of the cumulative peak count to the injected radionuclide (PCR) and the washout ratio (WR) were significantly lower in the high serum IgG4 group than in controls (p<0.01). In the low serum IgG4 group, the PCR on submandibular gland scintigraphy, and the PCR and WR on parotid gland scintigraphy were significantly lower than in controls (p<0.05, p<0.01 and p<0.05, respectively). On submandibular gland scintigraphy, the PCR was significantly lower in the high serum IgG4 group than in the low serum IgG4 group (p<0.05).
Conclusions: Salivary gland function was impaired in all AIP patients, but it was more impaired in patients with a high serum IgG4 than in those with a low serum IgG4.

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Background: The potential to predict pancreatic necrosis within the first 48 h by using plasma soluble thrombomodulin (sTM) in 104 patients with acute pancreatitis (AP) was analyzed in a prospective 5-year investigation performed at a single institution.
Methods: According to Balthazar CT grade, pancreatitis was classified as no necrosis in 72 patients, one-third necrotic in 18 patients, one-half necrotic in 10 patients and more than one-half necrotic in 4 patients. Blood was collected at the first 48 hours after the onset of pain and analyzed for sTM.
Results: In the healthy volunteers, plasma levels of TM were 16.49±5.24 μg/L. By comparison, the mean plasma levels of TM in each group of pancreatitis patients were as follows: CT grade A group, 34.21±10.73 μg/L; CT grade B group, 36.18±12.50 μg/L; CT grade C group, 49.39±18.38 μg/L; CT grade D group, 114.46±39.44 μg/L; CT grade E group, 100.22±15.97 μg/L (p<0.01). And for the patients, the Pearson correlation coefficient between the CT grade and TM values was 0.784 (p<0.01). No necrosis group, 39.22±13.75 μg/L; one-third necrotic group, 71.44±18.02 μg/L; one-half necrotic group, 123.50±28.57 μg/L; more than one-half necrotic group, 129.00±33.28 μg/L (p<0.01); And for the patients, the Pearson correlation coefficient between the degree of necrosis and TM values was 0.888 (p<0.01). ROC analysis indicated the area under the ROC curve (AUC ± SE) for sTM was 0.949±0.020, clearly supportive of the high accuracy of this index in predicting the necrosis of AP.
Conclusion: Plasma soluble thrombomodulin (sTM) is a potential marker to predict pancreatic necrosis within the first 48 h, and further investigation in a multicentre study is necessary.

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Background: In hemodialysis patients, adynamic bone disease has been reported to be closely associated with low levels of parathyroid hormone (PTH) due to exposure to high levels of serum calcium following the administration of calcium carbonate (CaCO₃) or vitamin D agents. This study was conducted to clarify the therapeutic effect of a non-calcemic phosphate binder, sevelamer hydrochloride (sevelamer), for hypoparathyroidism in hemodialysis patients with or without diabetes mellitus.
Methods: Based on entry criteria, 40 Japanese chronic hemodialysis patients (22 males and 18 females with a mean age of 60.6, 14 diabetic patients and 26 non-diabetic patients) were switched from CaCO₃ to sevelamer for 48 weeks. Serum calcium, phosphate, intact (i) PTH and PTH-(1-84) were analyzed. Bone remodeling activity was evaluated by determining intact osteocalcine (iOC), bone-specific alkaline phosphatase (BAP).
Results: The switch from CaCO₃ to sevelamer significantly decreased the serum levels of calcium, resulting in the elevation of iPTH levels from 31±18 pg/mL to 95±96 pg/mL by 48 weeks. In contrast, serum phosphate levels remained similar to those in patients with CaCO₃ treatment. Concomitantly, the levels of BAP and iOC were elevated. Further, these beneficial effects on bone turnover were observed in both diabetic and non-diabetic patients.
Conclusion: Sevelamer reduced the calcium concentration and thereby increased PTH levels, resulting in the improvement of markers of bone turnover. The administration of sevelamer is of therapeutic benefit for the improvement of bone remodeling activity even in hemodialysis patients with diabetes.

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Objective: Adiponectin has attracted great attention because of its anti-atherogenic properties; however, to date the relationship between serum adiponectin and arteriolosclerosis has not been reported. In our study, we aimed to examine whether or not serum adiponectin levels are associated with arteriolosclerosis in patients with IgA nephropathy which is the most common form of chronic glomerulonephritis.
Materials and Methods: We enrolled 35 patients aged 35.0±14.6, who underwent renal biopsy from August 2004 to February 2006 in our hospital, and were confirmed to have IgA nephropathy. We examined serum adiponectin, high-sensitive C-reactive protein, total cholesterol and triglyceride level, urinary protein excretion, body mass index (BMI), and the presence of arteriolosclerosis in the renal specimens. Since the serum adiponectin level is strongly affected by renal function, we classified the patients by creatinine clearance.
Results: Multiple regression analysis showed the associations of adiponectin with creatinine clearance (p<0.001), BMI (p<0.001), serum triglyceride (p=0.001) and urinary protein excretion (p=0.001). We observed a positive relation of adiponectin with urinary protein excretion and an inverse relation of adiponectin with creatinine clearance, serum triglyceride, and BMI. We could not detect any relation between the presence of arteriolosclerosis and adeponectin in the IgA nephropathy patients as a whole; however, in patients whose creatinine clearance was 90-120 ml/min/1.73 m², the serum adiponectin level of patients with arteriolosclerosis was lower than in those without arteriolosclerosis (p=0.025).
Conclusion: The serum level of adiponectin was related to arteriolosclerosis in IgA nephropathy patients whose renal function was almost normal. Adiponectin may prevent renal arteriolosclerosis.

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Legionella spp are well recognized as one of the etiologic factor in pneumonia, but it is difficult to distinguish the clinical features of Legionella infection from pneumonia due to other causes. The objective of the present study was to examine the clinical characteristics of community-acquired Legionella pneumonia in elderly patients. We reviewed the clinical, laboratory and radiographic findings in 8 patients diagnosed as having pneumonia caused by Legionella. The diagnosis was confirmed by the presence of urinary antigen, bacterial culture, polymerase chain reaction (PCR) and serum antibody. There were 6 men and 2 women, whose ages ranged from 76 to 85 years. All patients had fever and hypoxia, four patients had respiratory symptoms. The initial chest X-ray findings were varied-consolidation, ground glass opacity, pleural effusion and linear shadow. Urinary antigen was positive in 4 patients, bacterial culture in 2, PCR on the sputum in 3 and serum antibody in 2 patients. As pneumonia caused by Legionella often becomes life-threatening, especially in elderly people, it is imperative to diagnose it at the initial stage. In this study, urinary antigen proved to be the most useful diagnostic means. However, it is important to confirm the diagnosis through plural examinations.

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Objective: Benzodiazepines are commonly used for the treatment of tension-type headache (TTH), however, there are few randomized controlled trials recommending the use of these drugs in Japan. This study was undertaken to evaluate the efficacy of etizolam, a thienodiazepine derivative, in combination with a non-steroidal anti-inflammatory drug (NSAID) as an acute treatment for TTH.
Methods: The study design was a multi-center randomized control trial and included 144 patients. The diagnosis of TTH was based on the criteria of the International Classification of Headache Disorders-1 and all patients were diagnosed with episodic tension-type headache (ETTH). Changes in the severity of headache and shoulder pain were graded using a Visual Analogue Scale (VAS) before and after administration of drugs. Patients were randomized into NSAID alone (NSAID, mefenamic acid, 250 mg)group and NSAID (mefenamic acid, 250 mg) plus etizolam (0.5 mg) (NSAID-ET) group prior to treatment.
Results: Although both groups showed a significant drop in VAS for headache and shoulder pain (p<0.01), there was no overall significant difference between the NSAID-ET and NSAID groups. However, headache was improved significantly in female patients (p<0.05), and shoulder pain was improved in young and female patients (p<0.05, p<0.04) in the NSAID-ET group.
Conclusion: This study indicates that the combination treatment of etizolam and NSAID is useful in young or female patients.

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A 66-year-old woman developed systemic sclerosis (SSc) after receiving interferon alphacon-1 (IFNαcon-1;consensus interferon) therapy for chronic hepatitis C. She suffered from Raynauds phenomenon about 1 year after a course of IFNαcon-1 therapy. The combination of proximal scleroderma, Raynauds phenomenon, and ANA positivity led to a diagnosis of systemic sclerosis (SSc). IFN has multiple effects on the immune system and is known to trigger the development of autoantibodies, as well as the onset or exacerbation of autoimmune disease. We suspect that the immunomodulatory effects of IFNαcon-1 triggered the clinical manifestations of SSc in this patient. To our knowledge this is only the third case of SSc after IFNα therapy, and it is the first case associated with IFNαcon-1 therapy.

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Acute aortic dissection of Stanford type A with intramural hematoma was diagnosed based on computed tomography (CT) findings in a 60-year-old man. During medical treatment, pulmonary embolism and deep vein thrombosis developed. CT revealed thrombosis in the right pulmonary artery, and ^99mTc pulmonary perfusion scintigraphy showed defects in the right lung field. CT showed thrombus in the common iliac vein. An inferior vena caval filter was placed because anticoagulation therapy was contraindicated. A CT scan before discharge showed no thrombus in the pulmonary artery or common iliac vein, but a newly captured thrombus was found inside the filter.

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A 69-year-old woman presented with a harsh systolic murmur and severe anemia. Echocardiography demonstrated hypertrophic obstructive cardiomyopathy with a peak pressure gradient of 154 mmHg. Endoscopic examinations disclosed an angiodysplasia and multiple diverticula in the colon, but no active bleeding was noted in these lesions. A selective defect of large multimers of von Willebrand factor was detected by electrophoresis. After collection of anemia and Ca antagonist therapy, left ventricular obstruction was relieved and cessation of the occult gastrointestinal bleeding was obtained. This is the first report whereby acquired type 2A von Willebrand syndrome was caused by hypertrophic obstructive cardiomyopathy.

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Hypersensitivity vasculitis (HSV) has been used to describe several forms of vasculitis of small blood vessels, including Henoch-Schönlein purpura (HSP), mixed cryoglobulinemia, and allergic vasculitis, etc. HSP is a disease occasionally seen in childhood, and is characterized by dermatological and abdominal symptoms. Here, we report a rare case of HSV which showed a clinical course similar to HSP after pneumococcal pneumonia in an elderly adult. Generally, Streptococcus pneumoniae is the most common pathogen in adult community-acquired pneumonia. Therefore, it is critical to recognize HSV as one of the important complications after bacterial infection, especially Streptococcus pneumoniae.

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A 37-year-old man was referred to our hospital with complaints of dyspnea and general fatigue. Chest radiograph and CT scan revealed thickness of bronchovascular bundles in both lungs. In spite of various examinations, the primary lesion was not identified. He received chemotherapy containing TS-1 and cisplatin. Pulmonary lymphangitic carcinomatosis disappeared and the patient achieved a good partial response. He survived for 14 months after the chemotherapy. We believe the combination of TS-1 and cisplatin is one of the attractive options for patients with cancer of unknown primary site.

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A 47-year-old man presented with hemophagocytic syndrome (HPS) without any obvious underlying diseases. On computed tomography, his liver was occupied by multiple ill-defined low intensity lesions. Liver biopsy revealed diffuse infiltration of numerous histiocytes without cytologic atypism and prominent fibrotic changes. These histiocyes showed S100(+), CD68(+), CD1a(-), and lysozyme(+) and Langerhans cell granules were not observed by electron microscopic examination. He failed to respond to immunosuppressive and chemotherapeutic treatments and progressed to severe liver failure. At autopsy, his liver exhibited veno-occulusive disease (VOD). Since VOD is regarded as a rare complication of HPS, the presence of VOD associated with HPS may be easily overlooked.

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We describe a Japanese family with transthyretin Val107-related familial amyloid polyneuropathy (FAP). The clinical features were high-aged onset, sensorimotor polyneuropathy, carpal tunnel syndrome (CTS) and trigger finger. In addition, the proband showed cardiac conduction block and amyloid deposition in the sural nerve and dermis. Trigger finger may be a so far unknown clinical manifestation of Val107 FAP due to amyloid deposition in the connective tissue like CTS.

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A 34-year-old man visited the hospital with chief complaints of headache, fever, and disturbance of consciousness. In view of his clinical condition, the course of the disease, and results of examination, he was diagnosed with viral meningitis and treated accordingly. However, his clinical condition worsened, and MRI revealed abnormal signals in the splenium of the corpus callosum, in the basal ganglia and in the internal capsule, as well as the presence of severe inflammation in the base of the brain. Since he had a high ADA level in the cerebrospinal fluid and was consequently suspected to have tuberculous meningitis, he was placed on antitubercular agents. Then, his clinical condition began to improve. Additional steroid pulse therapy further improved his condition, and abnormal signals in the splenium of the corpus callosum and the basal ganglia resolved. This valuable case suggests that an immune mechanism contributed to the occurrence of central nervous system symptoms associated with tuberculous meningitis.

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A 23-year-old man complained of weakness of the right arm that he first noted six years prior to his visit. Neurological examination revealed atrophy and weakness of the triceps and serratus anterior muscle on the right side, which resulted in scapular winging on that side. MRI with neck flexion revealed compression of the cervical cord enabling a diagnosis of flexion myelopathy. Proximal muscle weakness and atrophy in flexion myelopathies including Hirayama disease are extremely rare. Here, we report a case of unilateral, proximal upper limb atrophy with scapular winging, attributed to middle cervical flexion myelopathy.

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We report a case of O'Sullivan-McLeod syndrome in a 59-year-old man, who had experienced slowly progressive weakening of both hands since he was 20 years of age. Mild hyperIgEemia and eosinophilia were present. Nerve conduction studies revealed reduced F wave-evoked frequencies for the median and ulnar nerves. Intravenous immunoglobulin (IVIG) at a dose of 400 mg/kg/day was given for 5 days. After IVIG, the muscle weakness of the distal upper extremities improved together with increased F wave-evoked frequencies. These effects lasted for a few months. These observations suggest that immune-mediated neural damage partially contributes to O'Sullivan-McLeod syndrome.

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We report a case of pulmonary cryptococcosis in an immunocompromised patient who had focal endobronchial lesions. When we first examined her, she complained of a productive cough that had lasted for two months. Seventeen months prior to this examination, she had been diagnosed with Sjogren syndrome and Sweet syndrome. Since that diagnosis, her condition had been maintained with low-dose prednisolone. We performed a chest CT scan, which revealed a mass, 3 cm in diameter, in the upper lobe of her left lung. A bronchoscopic examination revealed 3 white, elevated lesions in the upper lobe bronchus of her left lung. After 40 days of treatment with fluconazole, the shadow of her lung mass decreased in size to that of a scarred lesion and her white, elevated bronchial lesions disappeared.

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