Objective Autoimmune pancreatitis (AIP) and Mikulicz's disease have recently been recognized as pancreatic or salivary gland lesions of IgG4-related systemic disease. These are frequently associated with elevated serum IgG4 levels. This study aimed to clarify clinical implications of serial changes of elevated serum IgG4 levels in IgG4-related systemic diseases. Methods Serial changes of elevated serum IgG4 levels were examined in patients with IgG4-related systemic diseases. Patients Serial changes of elevated serum IgG4 levels were examined in 44 patients: AIP (n=24), Mikulicz's disease (n=8), pancreatic cancer (n=5), bile duct cancer (n=1), sclerosing cholangitis (n=1), hypereosinophilic syndrome (n=1), chronic thyroiditis (n=1), hypophysitis (n=1), idiopathic pancreatitis (n=1), and Behcet's disease (n=1). Results The serum IgG4 levels decreased in all patients with AIP and Mikulicz's disease after steroid therapy. The serum IgG4 levels were normalized in 46% of AIP patients and 38% of Mikulicz's disease patients. The serum IgG4 levels were not normalized at remission in 3 of 4 relapsed AIP patients, and re-elevation of serum IgG4 levels was detected in all relapsed patients. Elevated serum IgG4 levels decreased in 3 patients with pancreatic cancer after resection or chemotherapy, and decreased in patients with hypereosinophilic syndrome, sclerosing cholangitis, and hypophysitis after steroid therapy. Conclusion Measurement of serial serum IgG4 levels is useful to determine the disease activity of IgG4-related systemic diseases.
Background Acute exacerbation (AE) of idiopathic interstitial pneumonia (IIP) is occasionally observed after lung surgery. However, the risk of lung surgery in patients with IIPs is not yet clearly known. Subjects and Methods We conducted a retrospective study of consecutive patients who underwent lung surgery for cancer or for the diagnosis of interstitial pneumonia (IP) between 2000 and 2006. Patients who developed AE following the lung operation were assessed. Results The data of 68 consecutive patients (males: 56, females: 12) with IP who underwent lung surgery were analyzed. The lung surgery included lobectomy for lung cancer in 48 patients [idiopathic pulmonary fibrosis (IPF) 31, non-IPF 17], and lung biopsy in 20 patients [IPF 8, non-specific interstitial pneumonia (NSIP) 8, unclassified 4]. Three patients with IPF (4.4% in total) developed AE after the operation (2 lobectomy, 1 biopsy). The triggers of AE were considered to be prolonged ventilation at a large tidal volume with oxygen supplementation at a high concentration. At the time of the AE, the extent of parenchymal involvement on the HRCT images was greater on the non-operated side. All three patients died of respiratory failure 12 to 82 days after the onset of AE despite corticosteroid therapy. Conclusion It is essential to be aware of the risk of AE of IPF following lung operation. Intraoperative respiratory management, such as oxygen supplementation at a high concentration and/or prolonged mechanical ventilation are likely possible etiologic factors.
Background Forced expiratory volume in 6 seconds (FEV6) is becoming a substitute of forced vital capacity (FVC). However, the Japanese predictive equation for FEV6 has not been established, and the validity for the use of FEV1/FEV6 for diagnosing airflow limitation in Japanese has not been confirmed. Methods Subjects aged 40 or older, who had participated in a community-based health check in Takahata, Japan, from 2004 through 2005, were enrolled. The smoking histories of these subjects were investigated using a self-reporting questionnaire. FVC, FEV1, and FEV6 were measured using spirometric machines. Predictive equations of FEV6 were obtained from never-smoking subjects without history of pulmonary diseases by multiple linear regression assay. Results FEV6 and FEV1/FEV6 were significantly correlated with FVC (r=0.998, p<0.001) and FEV1/FVC (r=0.989, p<0.001), respectively. The cutoff values of percent predicted (%) FEV6 and FEV1/FEV6 for discrimination of having the restrictive lung disorder determined by %FVC <0.8 and having the airflow limitation determined by FEV1/FVC <0.7 were 0.80 and 0.72, respectively (%FEV6: sensitivity=0.995, specificity=0.983, positive predictive value <PPV>=0.832, negative predictive value <NPV>=1.000; FEV1/FEV6: sensitivity=0.942; specificity=0.971; PPV=0.787; NPV=0.993). When the 5th percentile the lower limit of normal values was used as criterion for discrimination of having airflow limitation, sensitivity, specificity, PPV, and NPV of FEV1/FEV6 were 0.932, 0.985, 0.808, and 0.995, respectively. Conclusion The results of the present study suggest that %FEV6 and FEV1/FEV6 are excellent substitutes for %FVC and FEV1/FVC, respectively. We confirmed the validity of the use of FEV6 and FEV1/FEV6 for identifying pulmonary diseases in Japanese individuals.
Background The natural course of HIV-1 infection includes 10 years of an asymptomatic period before the development of AIDS. However, in Japan, the disease progression process seems faster in recent years. Methods The study subjects were 108 new patients with primary HIV-1 infection during the period from 1997 through 2007. We evaluated their clinical symptoms and laboratory data, and then analyzed disease progression in 82 eligible patients. Disease progression was defined as a fall in CD4 count below 350/μL and/or initiation of antiretroviral therapy. Results Ninety percent of the patients were infected via homosexual intercourse. All patients had at least one clinical symptom (mean; 4.75±1.99) related to primary HIV-1 infection, with a mean duration of 23.2 days (±14.8) and 53.3% of them had to be hospitalized due to severe symptoms. The mean CD4 count and viral load at first visit were 390/μL (±220.1) and 4.81 log10/mL (±0.78), respectively. None developed AIDS during the study period. Estimates of risk of disease progression were 61.0% at 48 weeks and 82.2% at 144 weeks. In patients who required antiretroviral therapy, the median CD4 count was 215/μL (range, 52-858) at initiation of such therapy. Among the patients with a CD4 count of <350/μL at first visit, 53% never showed recovery of CD4 count (>350/μL) without antiretroviral therapy. Conclusion Despite possible bias in patient population, disease progression seemed faster in symptomatic Japanese patients with recently acquired primary HIV-1 infection than the previously defined natural course of the disease.
Objective Previous research suggests that sleep duration is related to metabolic syndrome, based on Western criteria. However, the criteria for metabolic syndrome vary among countries and populations. We therefore evaluated the relationship between sleep duration and the presence of metabolic syndrome in Japan. Methods A cross-sectional study was conducted involving 44,452 apparently healthy individuals aged 20 years or older who underwent annual health checkup the Center for Preventive Medicine at St. Luke's International Hospital in 2008. Participants were divided into 4 groups according to their average reported nightly sleep duration (<6 hours, 6-6.9 hours, 7-7.9 hours, ≥8 hours). Based on criteria by the Japan Society for the Study of Obesity, we identified individuals with metabolic syndrome. Multivariable logistic regression was used to explore the relationship adjusting for age, gender, alcohol consumption, current smoking, past medical history, and level of physical activity. Results The prevalence of metabolic syndrome was 8.7% (3,876/44,452; 95% CI, 8.5-9.0%). The mean age of participants was 44.8 years (SD, 12.8) and 49.5% were male. Those with sleep duration less than 6 hours were more likely to meet metabolic syndrome criteria (OR=1.42; 95% CI=1.20-1.68) than those with longer sleep duration (≥8 hours). Conclusion Short sleep duration is associated with the presence of metabolic syndrome despite the difference between Japanese and Western criteria.
A 19-year-old Japanese male with a BMI of 55.4 kg/m2 who also had liver dysfunction, dyslipidemia and hyperuricemia underwent repeated intragastric balloon therapy. The percent excess weight loss was 22.5% at the first balloon removal and 28.6% at the second balloon removal. The hepatic dysfunction resolved after the second balloon therapy, however, the dyslipidemia and hyperuricemia did not improve. The Japanese population is regarded as a high-risk race for obesity-related diseases at lower BMI values, and morbidly obese Japanese patients may need more serious weight reduction protocols to improve the comorbidities than similarly obese Americans or Europeans.
We report an 18-year-old woman, who had undergone surgical repair of tetralogy of Fallot, and then underwent successful intracardiac recordings within the zone of slow conduction (ZSC) of the reentry circuit on the initiation of premature ventricular contractions (PVC) and reentrant ventricular tachycardia (VT), obtained with multielectrode basket catheter. PVC a) were caused by wavefronts of sinus cycles entering and exiting the ZSC, and b) were not repetitive because the wavefront of the PVC could not reenter the ZSC. Similarly, VT developed after delayed potentials of sinus cycles, and was sustained because the wavefront following the 1st cycle of VT was able to repetitively reenter and exit the ZSC. Thus, spontaneous changes in the conductivity of the ZSC, like an on-off switch, appeared to determine the onset and sustenance of reentrant VT.
A 45-year-old man with chronic active Epstein-Barr virus (EBV) infection (CAEBV) with natural killer cell type developed pulmonary arterial hypertension (PAH). After chemotherapy, he showed marked depression of the EBV DNA genome in the peripheral blood, but PAH sustained. He died of heart failure due to PAH, and the histo-pathological examination revealed pulmonary vascular abnormalities without lung disease on autopsy. Although the EBV DNA genome and the infiltrating lymphocytes were not detected in the lung, his clinical course suggested that his PAH might be caused by CAEBV. This is the first reported case of PAH associated CAEBV in an adult.
We describe a 77-year-old man with Wolff-Parkinson-White syndrome exhibiting double coronary sinus (CS) potentials during retrograde conduction over accessory pathway (AP). The first, low-frequency potential (DP1) was first recorded in the left posterolateral region, while the second, higher frequency signal (DP2) was recorded in a lateral-to-septal direction. The two signals were fused near the left lateral wall. Successful ablation of the AP was obtained at its ventricular insertion site in the postero-septal region. The unexpectedly delayed activation of the paraseptal RA following activation of the paraseptal left atrium (DP1) can be explained by the absence of a LA-CS musculature (CSM) electrical connection at the proximal CS, which forces a detour of the activation wavefront from LA to RA via the distal CS (DP2). This is a rare case exhibiting unique double CS potentials which unmasked the absence of a LA-CSM electrical connection at CS ostium.
Invasive aspergillosis, a major problem during the post-transplant period, typically presents with pneumonia or tracheobronchitis in lung transplant recipients. In contrast, primary cutaneous aspergillosis is very rarely observed in lung-transplant recipients. In this report, we describe a case of tracheobronchial aspergillosis following primary cutaneous aspergillosis in a lung-transplant recipient. Early diagnosis of tracheobronchial aspergillosis is important because occult tracheobronchial aspergillosis can be potentially lethal. Our report suggests that surveillance bronchoscopy may facilitate identification of occult tracheobronchial invasion in lung-transplant recipients with primary cutaneous aspergillosis.
Extreme caution should be taken to avoid uncontrollable bleeding in treating hypervascular tumors via bronchoscope. We report two cases of endobronchial metastasis of renal cell carcinoma treated with bronchial artery embolization (BAE) before endoscopic treatments. The intraluminal lesions were removed swiftly and safely. Although arterial embolization is not always efficacious in cases of tracheal lesions, BAE is effective for tumors located in the carina, bilateral main bronchus or intermediate bronchus. The addition of BAE before endoscopic tumor removal should be considered a treatment option in patients suffering from airway obstructions due to hypervascular tumors such as renal cell carcinoma.
Cerebrovascular diseases in patients with Klippel-Trenaunay Syndrome (KTS) are uncommon, and the mechanism of stroke has remained elusive. We describe a patient with KTS who experienced a transient ischemic attack (TIA). Contrast-transcranial Doppler with the Valsalva maneuver revealed a right-to-left shunt and contrast-transesophageal echocardiography confirmed patent foramen ovale. Ultrasonography revealed dilated superficial and deep veins in the lower extremities; the D-dimer level was high and indicated hypercoagulability. Therefore, the mechanism of TIA was diagnosed as paradoxical embolism. To the best of our knowledge, this is the first case report of paradoxical embolism in a patient with KTS.
Marinesco-Sjögren syndrome (MSS) is a rare autosomal recessive disorder characterized by cerebellar ataxia, congenital cataracts, mental retardation, primary hypogonadism, skeletal abnormalities and myopathy, and patients with MSS are considered to be at risk of falls and bone fractures. We report a patient with MSS who received testosterone replacement therapy and risedronate administration. Muscle strength and the MRI features of the skeletal muscles were not changed, but low bone mass was improved by these treatments, and improvement has continued after risedronate treatment alone. This case suggests that treatment of MSS-related low bone mass using bisphosphonates is likely beneficial.
A 46-year-old female with a past history of ulcerative colitis (UC) was diagnosed with subacute thyroiditis (SAT), which improved with prednisolone (PSL) treatment (60 mg/day). The dose of PSL was gradually decreased, however upper back and neck pain and chest discomfort developed. The patient was diagnosed with Takayasu's arteritis (TA) based on wall thickening and luminal narrowing of the left common carotid artery and the left subclavian artery. The result of human leukocyte antigen typing analysis was A24 and B52 positive. These findings suggested that common genetic factors may be important for the etiology of TA, UC and SAT. This is the first report of TA that developed following UC and SAT.
IgG4-related disease is a recently recognized fibroinflammatory disorder characterized by extensive IgG4-positive plasma cell and lymphocyte infiltration of various organs. The pancreatic manifestation of IgG4-related disease is called autoimmune pancreatitis (AIP), in which autoimmune mechanisms are likely involved. On the other hand, some autoimmune and chronic inflammatory disorders, such as Sjögren's syndrome and rheumatoid arthritis, are associated with increased risks of non-Hodgkin lymphoma (NHL). There have been a few reports of cases with IgG4-related disease that had subsequently developed NHL, however, all of them suffered from B-cell lymphoma. We describe the first case of NHL, compatible with a subtype of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), which arose in IgG4-related sclerosing cholangitis. As patients with IgG4-related disease may be at an increased risk of developing NHL, such presentation during the follow-up of IgG4-related disease should be carefully scrutinized to exclude NHL.