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We herein report a 78-year-old woman with Gaucher disease (GD) who was initially diagnosed with GD type 1, had been receiving long-term enzyme replacement therapy since 58 years old, and developed neurological manifestations in her 70s. The neurological manifestations included myoclonic seizures and progressive cognitive decline. Although it is rare for GD patients to first develop neurologic manifestations at such an advanced age, physicians engaged in long-term care for GD patients should be alert for this possibility.

A 24-year-old woman was admitted to our hospital due to abdominal pain and a high fever. She was diagnosed with ileocolonic Crohn's disease (CD), complicated with a gastro-colic fistula and splenomegaly. After initial treatment with an infliximab-biosimilar, all blood cell line counts markedly decreased. Three-dimensional reconstructed computed tomography revealed splenic vein narrowing. Thus, her pancytopenia was deemed to have likely been caused by hypersplenism. Surgery was performed, and clinical remission was maintained without pancytopenia. This is the first report of a CD patient with pancytopenia caused by hypersplenism that was triggered by gastro-colic fistula-associated splenic vein obstruction.

A 49-year-old woman presented with nephrotic-range proteinuria, microhematuria, and moderate renal dysfunction. Diuretic-resistant refractory ascites associated with nephrotic syndrome were observed. Based on the histopathological findings, the patient was diagnosed with proliferative glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID). Rituximab was administered due to steroid and immunosuppressive drug resistance, and partial remission was achieved after six months. Cell-free and concentrated ascites reinfusion therapy (CART) performed to treat the refractory ascites improved the ascites and anasarca. Rituximab successfully treated the PGNMID, while CART effectively treated the refractory ascites associated with nephrotic syndrome.

Objective Although aerobic exercise tests on cycle ergometry have long been used for initial assessments of cases of suspected mitochondrial disease, the test parameters in patients with final diagnoses of other diseases via the widely used 15 W for 15 minutes exercise protocol have not been fully characterized. Methods We retrospectively reviewed all patients who underwent the test at our institution. We classified the patients with genetic diagnoses or those who met previously reported clinical criteria as having mitochondrial diseases and those with a final diagnosis of another disease as having other diseases. Results were available from 6 patients with mitochondrial disease and 15 with other diseases. Results During the test, elevated venous peak lactate above the upper normal limit of healthy controls at rest [19.2 mg/dL (2.13 mM)] was observed in 3 patients with mitochondrial diseases (50.0%) and 5 with other diseases (33.3%). In the group of patients with elevated venous peak lactate, a lactate-to-pyruvate ratio of >20 was observed in all 3 patients with mitochondrial disease but in only 1 of the 5 with other diseases. More than a 2-fold increase in venous lactate from baseline was observed in 4 patients with mitochondrial disease (66.7%) and 1 with another disease (6.7%). Conclusion Elevated venous peak lactate levels were observed in patients with final diagnoses of other diseases, even under a low 15-minute workload at 15 W. The lactate-to-pyruvate ratio and increase in lactate level from baseline may add diagnostic value to venous peak lactate levels alone.

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 60-year-old man had received octreotide for a metastatic neuroendocrine tumor (NET) in the rectum. Computed tomography and ultrasonography revealed a cardiac tumor, diffuse thickness of the ventricular wall and pericardial effusion, which was diagnosed as cardiac metastasis. The metastatic lesions continued to grow despite the alteration of chemotherapy, and the patient complained of repeated syncope and was admitted to our hospital at 11 months after the diagnosis of cardiac metastasis. An electrocardiogram during syncope showed sustained ventricular tachycardia, which was considered to be caused by the cardiac metastasis. We herein report a case of NET with cardiac metastasis which caused lethal arrhythmia along with a review of the pertinent literature.

Objective We aimed to identify obstructive sleep apnea syndrome (OSAS) severity indices reflecting the anthropometric and metabolic characteristics of patients with OSAS. Methods A total of 76 patients with OSAS underwent nasal continuous positive airway pressure (nCPAP). We also investigated the effects of nCPAP on OSAS-associated muscle sympathetic nerve activity (MSNA), risk for cardiovascular diseases, and insulin secretion and sensitivity. Results Among the OSAS severity indices, HbA1c was significantly correlated with the apnea-hypopnea index, whereas HOMA-beta, HOMA-IR, and hepatic insulin resistance were significantly correlated with % SpO 2 <90%, independent of age, gender, and body mass index (BMI). Burst incidence of MSNA was independently associated with only a 3% oxygen desaturation index. nCPAP therapy significantly lowered the OSAS severity indices and reduced the burst rate, burst incidence, and heart rate. Conclusion The OSAS severity indices reflecting apnea/hypopnea are associated with glycemic control, whereas those reflecting hypoxia, particularly % SpO 2 <90%, are associated with hepatic insulin resistance independent of obesity. Both types of OSAS severity indices, especially the 3% oxygen desaturation index (reflecting intermittent hypoxia), are independently associated with MSNA, which is dramatically lowered with the use of nCPAP therapy. These findings may aid in interpreting each OSAS severity index and understanding the pathophysiology of OSAS in clinical settings.

Objective Environmental and climatic changes have been occurring throughout the past 20 years in Japan. Correspondingly, the antigens that cause hypersensitivity pneumonitis might be changing. In an epidemiological survey of Japan in the 1980s, summer-type hypersensitivity pneumonitis (SHP) accounted for 74.4% of the cases of hypersensitivity pneumonitis. The epidemiological characteristics of this disease have not been reported since then. We investigated the annual changes in the number of cases of SHP and the factors affecting the results. Methods Cases that were diagnosed as SHP were retrieved from the medical records of our institute between 1990 and 2015. The diagnostic criteria proposed by the Japanese Ministry of Health, Labour and Welfare in 1990 were applied to obtain the definite diagnosis. Patients The study population included 25 diagnosed patients, including one intrafamilial case. The subjects were predominantly non-smoking women in their 50s and all lived in wooden houses that had been constructed more than 10 years previously. Results The number of cases that were diagnosed as SHP tended to decrease during the study period. However, temporal increases tended to occur in years with increased rainfall and decreased daylight hours. No relationship appeared to exist between the number of cases and high temperatures or humidity levels. Conclusion The incidence of SHP currently appears to be decreasing; however, the weather conditions in any given year might cause a temporal increase in the incidence rate.

Objective For lung cancer complicated with sarcoidosis, there are no exact features that indicate whether lymphadenopathy is metastatic. This makes the validity of surgery uncertain for clinicians. The aim of this study was to clarify the clinical features of pulmonary sarcoidosis complicated by lung cancer, especially from the viewpoint of evaluating lymphadenopathy. Methods We retrospectively reviewed medical records from 2004 to 2013 at our institution, and 18 patients who were diagnosed with sarcoidosis and lung cancer were thus found to be eligible. We investigated the relationship between the clinical and pathological findings of their swollen lymph nodes. Results Of 18 patients, 11 conducted surgery, and the postoperative pathological evaluation of swollen lymph nodes was done in 8 of the patients. Postoperative N factor in all these patients was 0, even though lymphatic metastasis had been suspected preoperatively because of the unbalanced distribution of lymphadenopathy or the accumulation of fluorodeoxyglucose. Conclusion In patients with lung cancer complicated by sarcoidosis, the clinical assessment of the state of lymphadenopathy is difficult to make. However, as many of them tend to be benign, we suggest that surgical resection should be considered for a complete cure in the absence of any remote metastasis.

Objective: Long-term effects of pirfenidone have been poorly understood to date. This study investigated the clinical efficacy and safety of long-term pirfenidone use for idiopathic pulmonary fibrosis (IPF) in clinical practice., Methods: This survey study was a retrospective observational study. A survey was used to collect clinical information on IPF cases that were treated with pirfenidone. This survey sheet came from physicians belonging to the Diffuse Lung Diseases Research Group., Results: 502 patients at 22 institutes received pirfeidone treatment. Of the 502 cases, pirfenidone treatment was terminated in under one year in 186 cases (37.1%); adverse effect was the most frequent reason for termination. The pirfenidone treatment lasted for two years or longer in 111 cases (22.1%). The mean change in the forced vital capacity (FVC) was -30±224 (SD) mL in the first year of treatment, -158±258 mL in the second year, and -201±367 mL in the third year. FVC improved by 10% or more in the first year in 10 (14.7%) of 68 cases, and showed a change of ±10% in 47 (69.1%) cases. It showed a change of ±10% in the second and third years in 61.7% and 62.5% of the patients, respectively., Conclusion: The FVC improved in only a small percentage of patients who received pirfenidone treatment for a long period of time. However, a decrease in the FVC was prevented for three years in over half of the cases.

Squamous cell carcinoma of the extrahepatic bile duct is quite rare. A 77-year-old woman with jaundice and general fatigue was referred to our hospital. Multiphase contrast-enhanced computed tomography visualized a 17-mm solid mass in the junction of the cystic and common bile ducts. The patient underwent pylorus-preserving pancreaticoduodenectomy. The pathological findings demonstrated keratin-positive poorly differentiated squamous cell carcinoma of the extrahepatic bile duct (T3N0M0, stage IIIA). Although adjuvant chemotherapy with gemcitabine was administered, the patient exhibited local recurrence at the site of anastomosis of biliojejunostomy 20 months after resection and died 32 months after resection.

We herein report the rare case of a 76-year-old woman who underwent cholecystectomy with bile duct resection for advanced gallbladder cancer associated with pancreaticobiliary maljunction (PBM) and subsequently developed multiple cancers of the pancreaticobiliary system (the distal bile duct, intrahepatic duct and pancreatic duct) after the operation. We performed conventional endoscopic retrograde cholangiopancreatography (ERCP) using a side-viewing scope to evaluate the masses in the distal bile duct and the pancreatic duct. We also performed ERCP using double-balloon enteroscopy (DBE) to observe the mass in the intrahepatic duct. It was possible to directly observe the lesion using DBE and to perform a biopsy under visual control. All lesions were correctly diagnosed by the combination of ERCP using different endoscopes. The present case suggests that it is necessary to pay close attention (with regard to carcinogenesis) to the whole pancreaticobiliary system in patients with PBM. In addition, the combination of ERCP using DBE and a side-viewing scope may be useful for making a precise diagnosis in patients with altered biliary anatomy who have multiple cancers of the pancreaticobiliary system.

Epidermoid cysts arising from both the pancreas and spleen are rare. We herein report a case of a surgically resected epidermoid cyst in the pancreatic tail invaginated from the spleen. A multi-locular cyst, 2 cm in diameter, without a solid component was discovered incidentally in the pancreatic tail. During the 11-year follow-up, the emergence of satellite cystic lesions with distinct appearances was seen, and surgical resection was selected despite the lack of any associated symptoms or evidence of cytological abnormalities. Histologically, these cysts were lined with benign multi-layered flattened epithelium surrounded by a thin layer consisting of cells positive for CD8 and CD68 and connecting to the spleen.

Objective The influence of smoking on the pathogenesis and clinical course of interstitial pneumonia has recently attracted attention. To clarify the influence of smoking on the clinical patient characteristics and therapeutic effects in patients with interstitial pneumonia presenting with a non-specific interstitial pneumonia (NSIP) pattern, we compared the clinical patient characteristics and therapeutic effects in smokers and nonsmokers in this study. Methods We divided 31 NSIP (16 idiopathic nonspecific interstitial pneumonia and 15 collagen vascular disease-associated nonspecific interstitial pneumonia) patients into smoker and non-smoker groups for each case. The patient characteristics, pulmonary function tests, Krebs von den Lungen 6 (KL-6), surfactant protein D (SP-D), bronchoalveolar lavage fluid findings, and clinical courses for two years were compared between the smoker and non-smoker groups. Results The smoking subgroup (n=15) of NSIP patients had a significantly lower % diffusing capacity for carbon monoxide/ alveolar ventilation (DL CO /VA) and tended to have higher SP-D values than the nonsmoking subgroup (n=16). Although no difference was observed regarding the prognosis, 5 of 6 cases with NSIP, which had worsening of lung disease were heavy smokers with a pack-year history of 40 or greater. Conclusion Smoking is thus suggested to negatively influence the diffusing capacity caused by damage to alveolar epithelial cells. In addition, smoking may also be potentially related to resistance to therapy in NSIP cases.

Multifocal fibrosclerosis (MFS), which causes systemic and chronic connective tissue inflammation, has been associated with IgG4 and regarded as an identical entity with "IgG4-related disease (IgG4-RD)". Although a few cases of MFS mimicking IgG4-RD histopathologically, despite the absence of a serum IgG4 elevation and IgG4-positive plasma cell infiltration, have been reported, there is, so far, little information regarding such exceptional cases. We herein demonstrate a case of non-IgG4-related MFS presenting with periaortitis and parotiditis, whose histological findings were consistent with IgG4-RD despite the absence of elevated serum and tissue IgG4 levels.

A 51-year-old woman diagnosed with Crohn's disease developed drug-induced hypersensitivity syndrome (DIHS) 12 and six weeks after starting the oral intake of mesalazine and trimethoprim/sulfamethoxazole, respectively. Chest CT showed centrilobular nodular shadows and a transbronchial lung biopsy (TBLB) revealed infiltration of inflammatory cells predominantly in the small pulmonary artery walls and bronchiolar walls. Regarding pulmonary lesions of DIHS, infiltrative shadows have sometimes been reported, whereas nodular shadows have rarely been documented. This is a valuable case report for considering the mechanism underlying the development of pulmonary lesions in case of DIHS.

Objective: Acute respiratory worsening is defined as the unexpected rapid deterioration of idiopathic pulmonary fibrosis (IPF), and idiopathic acute respiratory worsening is known as an acute exacerbation of IPF. Torque teno virus (TTV) is a circular single-stranded DNA virus whose pathological significance remains unclear. The aim of the present study was to investigate the prevalence and titer of TTV DNA in IPF patients with acute respiratory worsening., Methods: The serum TTV DNA titer was measured using real-time PCR in nine IPF patients (two treated with steroids and immunosuppressants; seven treated without steroids or immunosuppressants) who developed acute worsening, including five patients with acute exacerbation. The serum TTV DNA titer was also measured in eight stable IPF cases and four IPF cases of lung cancer. In addition, in order to examine time course changes in the TTV DNA titer, the titer was measured more than once, with an interval of four weeks or longer, in eight patients., Results: Among the nine IPF patients with acute worsening, the TTV DNA titer was above 1×10(6) copies/mL in two subjects without acute exacerbation who had been continuously treated with steroids and immunosuppressants. Meanwhile, the mean TTV DNA titer was 2.4±2.6 (×10(4) copies/mL) in the five patients with acute exacerbation and 3.1±3.4 (×10(4) copies/mL) in the eight patients with stable IPF. Moreover, the TTV DNA titers were increased in all three IPF patients who started treatment with steroids and immunosuppressants., Conclusion: Our results suggest that it is unlikely that TTV is directly involved in the onset of acute exacerbation of IPF and that the serum TTV DNA titer potentially reflects the immunosuppressive state of the host due to treatment.

Objective: We retrospectively investigated the clinical features of pulmonary aspergillosis associated with interstitial pneumonia., Methods: We reviewed the medical records of all patients treated for interstitial pneumonia with or without pulmonary aspergillosis at our institution between April 2006 and August 2012 and evaluated the clinical features as well as risk and prognostic factors for pulmonary aspergillosis associated with interstitial pneumonia., Results: Among 539 patients with interstitial pneumonia, 15 who suffered from pulmonary aspergillosis were identified. The median age was 69.2±7.0 years, and fourteen patients were men. The subtypes of pulmonary aspergillosis were chronic pulmonary aspergillosis (n=14) and invasive pulmonary aspergillosis (n=1). The forms of interstitial pneumonia included idiopathic pulmonary fibrosis (n=9), rheumatoid arthritis-related interstitial pneumonia (n=4) and pleuroparenchymal fibroelastosis (n=2). The underlying conditions were emphysema (n=9) and a history of oral corticosteroid and/or immunosuppressive use (n=4). Home oxygen therapy (HOT) was administered in 11 patients. Following the diagnosis of pulmonary aspergillosis, all patients were treated with antifungal drugs. Ten patients (66.6%) died. A comparison of the interstitial pneumonia patients with and without pulmonary aspergillosis showed that the presence of emphysema, use of HOT and death were significantly associated with pulmonary aspergillosis., Conclusion: Pulmonary aspergillosis is one of the major complications of interstitial pneumonia and its prognosis is poor. Therefore, providing careful monitoring and proper treatment is extremely important.

Background: Pulmonary arteriovenous malformations (PAVMs) are rarely encountered in clinical practice. The prevalence of PAVMs associated with hereditary hemorrhagic telangiectasia (HHT) has been estimated based on the rate in the family members of HHT patients, but the prevalence of PAVMs in the general population remains unknown., Methods: We retrospectively examined the prevalence and clinical characteristics of PAVMs as detected by a low-dose thoracic CT screening program for lung cancer at the Hitachi Medical Center and the Hitachi General Health Care Center in the northern part of Ibaraki Prefecture, Japan., Results: From 2001 to 2007, we identified eight patients (seven females and one male) with PAVMs among 21,235 initial screening participants (the mean age of the patients with PAVMs and that of the screening participants was 60.6 years). The prevalence of PAVMs was estimated at 38 per 100,000 individuals [95% confidence interval (CI)=18-76]. The diameter of the PAVMs was a mean of 6.6 mm, and none of the lesions could be detected by chest X-ray. Females older than 60 years tended to have larger PAVMs than younger women did (p=0.06). Two patients (25%) were diagnosed with HHT. One patient had previously undergone surgery for a brain abscess., Conclusion: PAVMs are more prevalent than previously reported, especially among females.

A 26-year-old man presented at our hospital in 2008 to undergo detailed investigations as part of a routine health examination. Chest computed tomography (CT) showed linear and reticular opacities with, in part, diffuse calcification in the lung fields bilaterally. A surgical lung biopsy was performed and the histological findings were compatible with a diagnosis of diffuse pulmonary ossification (DPO) of the dendriform type. DPO usually occurs as a secondary disease. As the histological changes in interstitial fibrosis were minimal rather than diffuse and not significant enough to be regarded as interstitial pneumonia, we considered this to be an idiopathic case. However, the findings appear to suggest that inflammation and fibrosis were associated with ossification.

Objective: The prognosis of idiopathic pulmonary fibrosis (IPF) patients with acute exacerbation (AE) is reported to be extremely poor. Several clinical studies suggest that direct hemoperfusion with polymyxin B-immobilized fiber (PMX) may have beneficial effects on AE in patients with interstitial pneumonia (IP). The aim of this multicenter retrospective analysis was to investigate whether PMX treatment could provide improvement of oxygenation and survival benefits in IPF patients with AE., Methods: We conducted a retrospective study of 160 IP patients (including 73 IPF) with AE treated by PMX at 18 institutions in Japan. PMX treatment was carried out twice. The total hemoperfusion time of PMX treatment was, on average, 12 hours. Data concerning oxygenation on PMX treatment and survival after AE were collected and analyzed., Results: In IPF patients with AE, arterial oxygen tension (PaO(2))/inspiratory oxygen fraction (FiO(2)), (P/F) ratio was significantly improved at the end of the 2nd treatment with PMX (173.9 ± 105.4 to 195.2 ± 106.8 Torr, p=0.003). White blood cell count was significantly reduced at the end of the 2nd treatment (13,330 ± 7,002 to 9,426 ± 5,188 /mm(3), p<0.001). These clinical changes were also observed on analysis of all 160 IP patients with AE. The one- and three-month survival rates of IPF patients after AE were 70.1% and 34.4%, respectively., Conclusion: PMX treatment may improve oxygenation and survival in IPF patients with AE. Prospective, controlled trials of PMX treatment for IPF with AE are warranted to verify this potential benefit.

A 40-year-old man was admitted with a continuous high grade fever accompanying a relatively large solitary liver abscess with septations. A puncture of the abscess revealed gram-negative rods that could be identified histologically as Fusobacterium necrophorum, which was later confirmed by tissue culture. The patient was switched to meropenem and penicillin, and cured of the infection. Fusobacterium necrophorum is a rare bacterium causing potentially fatal liver abscesses in humans. Clinicians should bear Fusobacterium necrophorum in mind when treating patients with an enlarged solitary liver abscess.

A 70-year-old woman was admitted to our hospital with pleuritis and pericarditis. Cytological examination of pleural and pericardial effusion, and pleural biopsy specimens under thoracoscopy revealed no specific pathological findings. The pleural effusion was drained continuously; however, she died of circulatory insufficiency at day 45 from admission. At autopsy, a fragile hemorrhagic mass arising from the right auricle had invaded bilateral pleura and the pericardium directly without distant metastasis. Immunohistochemical staining showed that the tumor cells expressed endothelial markers such as CD31 and CD34 antibodies, and factor VIII-related protein. These findings supported the diagnosis of a poorly differentiated angiosarcoma.

Objective: Renal angiomyolipomas (R-AMLs) are major complications of lymphangioleiomyomatosis (LAM). The objective of this study was to better understand the influence of R-AMLs in patients with LAM on the prognosis and other clinical factors related to respiration, and to investigate the management of R-AMLs in patients with LAM., Patients and Methods: We retrospectively investigated the clinical features of 7 patients with LAM [4 were TSC (Tuberous sclerosis complex)-LAM and 3 were S (sporadic)-LAM] complicated by R-AMLs admitted to our hospital from 1997 to 2008., Results: All patients were females and the mean age at diagnosis of LAM was 40.7 years (31.7 years for TSC-LAM and 52.7 years for S-LAM). Although 5 patients had symptoms related to R-AMLs, only 1 patient experienced symptoms related to R-AMLs at the time of diagnosis. Five patients had bilateral and 2 patients had unilateral R-AMLs. R-AMLs ruptured in 4 cases (3 patients were TSC-LAM) including 2 patients in whom they ruptured bilaterally, and who underwent bilateral nephrectomy. In 1 case, unilateral R-AMLs grew larger and appeared on the other side during the follow-up period., Conclusion: Although only rare cases of LAM show symptoms related to R-AMLs initially, R-AMLs are a notable complication. To avoid nephrectomy, R-AMLs should be diagnosed when they are small and should be followed up carefully by periodic echograms or CT scans., (© 2011 The Japanese Society of Internal Medicine)

Background: Inhalation of N-acetylcysteine (NAC) has been carried out in our department since 1994 for treating interstitial pneumonia such as idiopathic pulmonary fibrosis (IPF). In this study, the clinical efficacy and safety of long-term NAC inhalation monotherapy for IPF was investigated., Methods: NAC inhalation was carried out in 23 of 34 cases diagnosed as IPF by surgical lung biopsy in our department between 1994 and 2008. The treatment was continued for one year or longer in 14 cases. In these 14 cases and in 11 cases without treatment, the clinical courses, prognosis, lung function (%FVC, %DLco, and %TLC), and changes in serum markers for interstitial pneumonia (KL-6 and SP-D) were examined., Results: There were no significant differences in survival curves between the two groups. Acute exacerbation was observed in 4 of 14 cases (28.6%) receiving NAC inhalation. Compared with the results just before the beginning of NAC inhalation, Δ%FVC and Δ%DLco in the treated cases were -4.7% and -2.9% one year later and -4.0% and -5.8% two years later, respectively. In cases without treatment, Δ%FVC and Δ%DLco were -3.5% and +5.3% one year later and +0.2% and +1.0% two years later, respectively., Conclusion: Since this study is an open case-control study in a single institute and the number of cases is not large, its use in evaluating the efficacy of NAC inhalation monotherapy is limited. In addition, the role of NAC inhalation in combination with a steroid, an immunosuppressive agent, and a new anti-fibrosis drug should also be investigated.

Emergency coronary angiography of a 53-year-old man with acute coronary syndrome revealed stenosis in right coronary artery. During angioplasty, the ECG change showed three kind of ST deviation, namely inferior ST elevation with precordial ST depression, inferior ST elevation with precordial ST elevation, and inferior ST depression with precordial ST elevation. The present case shows that the ST deviation of the inferior ischemia with right ventricular ischemia takes various patterns. This phenomenon is decided by degree of right main coronary and right side branch blood flow.

Herein, we encountered an 89-year-old woman with pancreatic cancer who presented with fever without infective focus, leukocytosis of 45,860 /microL, and elevation of serum granulocyte-colony stimulating factor (G-CSF). The patient could not receive any curative therapy due to an extremely aggressive clinical course. Specimens taken at necropsy revealed an adenosquamous carcinoma positive for G-CSF by immunohistochemistry; it was only the second reported case to date. She was finally diagnosed with G-CSF-producing pancreatic cancer. In light of the above, clinicians should consider the presence of G-CSF-producing tumors, including pancreatic cancer, when presented with patients showing leukocytosis of unknown origin and fever without infective focus.

Objective: The purpose of this study was to analyze the CT findings of interstitial lung diseases that are associated with collagen vascular disease (CVD), with particular attention to nonspecific interstitial pneumonia (NSIP), and to examine whether it is possible to predict the clinical diagnosis of CVDs based on the CT findings alone., Methods: CT scans of 49 patients with NSIP associated with CVD (15 males, 34 females; mean age, 55+/-10 years; age range, 25-76 years) were included in this retrospective study. All patients underwent a surgical biopsy. The clinical diagnosis comprised rheumatoid arthritis (RA) (n=15), systemic sclerosis (SSc) (n=8), polymyositis and dermatomyositis (PM/DM) (n=18), Sjögren's syndrome (SjS) (n=4), and mixed connective tissue disease (MCTD) (n=4). Each CT was reviewed by two independent observers who made a clinical diagnosis based on the CT findings alone., Results: The observers made a correct diagnosis for 22 (45%) of the 49 patients. A correct diagnosis was made for: RA in 7 (47%) of 15 patients; SSc in 3 (38%) of 8 patients; PM/DM in 11 (61%) of 18 patients; SjS in 1 (25%) of 4 patients. None of the 4 MCTD cases was diagnosed., Conclusion: It is difficult to make a correct clinical diagnosis of the various types of CVDs based solely on CT findings. However, it is probable to make a reasonably accurate clinical diagnosis in cases that show the typical CT findings, especially for PM/DM patients.

Objective: To examine whether exercise training using nontreadmill walking is effective for the improvement of exercise performance of patients with pulmonary tuberculosis sequelae (PTS) characterized by restrictive ventilatory defect., Patients and Methods: Fourteen patients with stable PTS hospitalized for assessment of exertional dyspnea in Fukujuji Hospital from April 1997 to March 1999 were enrolled in this observational study. All patients underwent baseline pulmonary function tests, arterial blood-gases analysis and exercise tests for initial assessment. Four patients were excluded because of hypoxemia during the initial treadmill test. The remaining 10 patients who stopped exercising because of symptom limitations became candidates for the exercise training. The patients were instructed to perform daily walking exercise training in a hallway in the hospital for 2 weeks. The training was started at their maximum walking speeds during the treadmill test, and walking speed was gradually increased as the patients gained confidence. After finishing the exercise training, the patients performed pulmonary function tests, arterial blood-gases analysis was done, and exercise tests were conducted in identical fashion to the baseline protocol., Results: There were no significant changes in pulmonary function tests and arterial blood-gases analysis after the exercise training. Exercise tolerance improved with a significant increase in peak oxygen uptake (from 13.6+/-2.8 to 14.8+/-2.8 ml/kg/min, p<0.01) and distance covered in a 6-minute walk (from 399+/-62 to 467+/-65 m, p<0.01) after the exercise training., Conclusion: The exercise training we conducted is shown to be a safe and effective modality for the improvement of exercise performance of patients with PTS.

We report two cases in whom inhaled corticosteroid rapidly improved pulmonary sarcoidosis. In the first case, fluticasone at 400 microg/day was initiated, because dry cough and small nodular shadows on chest X-ray persisted for six months. But her cough and the nodular shadows were persisted, therefore the treatment was replaced with budesonide at 800 microg/day. Two months later, her dry cough subsided and pulmonary shadows improved. Serum angiotensin-converting enzyme (ACE) level was decreased and pulmonary function improved. In the second case, bumethasone was already administered at a local clinic. Budesonide at 400 microg/day was combined with oral steroid, because pulmonary shadows continued for eight years. Also two months later, the serum ACE level was decreased and the pulmonary shadows slightly improved. Inhaled corticosteroid therapy for two to three months is tolerable, and may be a useful treatment option in some patients with sarcoidosis.

Object: The serum levels of KL-6, surfactant protein A (SP-A), and SP-D are useful biomarkers and prognostic factors for the activity of interstitial pneumonias. The aim of this study was to determine the clinical roles of the levels of KL-6, SP-A, and SP-D in the serum and bronchoalveolar lavage fluid (BALF) of patients with acute eosinophilic pneumonia (AEP)., Materials and Methods: We researched 5 cases of AEP. The levels of KL-6, SP-A, and SP-D in the sera and BALF of those patients were measured by enzyme-linked immunosorbent assay., Results: KL-6 levels in BALF did not differ between AEP patients and the healthy control group, while SP-A and SP-D levels in BALF were significantly higher in the AEP patients than in the healthy control group. In sera, AEP patients had significantly higher than normal levels of SP-A and SP-D, but not of KL-6. Only in sera there was a positive correlation between SP-A and SP-D, but no apparent correlations in BALF and also between KL-6 and the others. Furthermore, the BALF levels of SP-D, but not of SP-A or KL-6, statistically correlated with the concentration of albumin in BALF. After clinical improvement, the elevated levels of serum SP-A or SP-D in AEP patients decreased until normal levels were reached within 2 months., Conclusion: These results suggest that the serum or BALF levels of SP-D appear to be more sensitive than those of SP-A or KL-6 at reflecting the inflammatory response in AEP lungs.

Objective: To investigate whether or not the newly revised classification of the severity of idiopathic interstitial pneumonia (IIP) is appropriate with respect to quality of life (QOL)., Methods: The association between the subscale of Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36) and pulmonary function or serum marker was analyzed using Pearson's correlation coefficient. The association between the subscale of SF-36 and the previous or newly revised classification of the severity of IIP was analyzed using Spearman's rank correlation test., Patients: Forty patients with idiopathic pulmonary fibrosis (IPF) were enrolled., Results: The mean deviation value scores for 7 items, excluding bodily pain (BP) in SF-36 were below the national reference values. % vital capacity (VC) was correlated with the 7 items excluding BP. However, neither serum LDH nor KL-6 values were correlated with any item in SF-36. According to the new or previous classification of the severity, severity was correlated with physical function, limitation of role functioning related physical problems and general health (GH); the correlation coefficient with the new one was slightly higher than the previous one. Based on these results, we established a unique draft on the classification of the severity. %VC <70% was added as an item for the newly revised classification in our draft. In our draft, there was rank correlation between the 7 items, excluding BP, in SF-36 and severity., Conclusion: With respect to QOL, the newly revised classification of the severity of IIP was not satisfactory, but the hypoxemia during exercise in patients with resting PaO(2) >80 Torr and reduction of VC were found to be important factors.

This is a report of a patient with occupational hypersensitivity pneumonitis (HP) caused by Konjak flour and powdered Hijikia fusiforme. A 56-year-old man had worked as a Konnyaku manufacturer for 38 years, and suffered from dyspnea on exertion. Konnyaku is a paste made from Konjak flour, powdered Hijikia fusiforme, quicklime, and powdered scallop shell. The diagnosis of HP was confirmed immunologically by the detection of serum precipitins to powdered Hijikia fusiforme, and by the positive result of in vitro lymphocytic proliferative response for Konjak flour using peripheral blood lymphocytes. To our knowledge, this is the first case report of Konnyaku manufacturer's lung.

A 40-year-old woman undergoing prednisolone and cyclosporine therapy for subcutaneous panniculitic T-cell lymphoma complained of a cough for a few weeks. A chest X-ray revealed bilateral diffuse granular shadows. Additionally, the patient was discovered to have multiple subcutaneous abscesses. Gram-stained smears of sputum and pus from the abscess showing branched gram-positive rods led to a diagnosis of pulmonary nocardiosis with dissemination to the lungs and subcutaneous tissues. Combination therapy consisting of sulfamethoxazole/trimethoprim and panipenem/betamipron produced rapid improvement of radiographic abnormalities. It is suggested that pulmonary nocardiosis should be considered in the differential diagnosis of diffuse granular shadows on chest X-rays, especially in immunocompromised patients.

Following a high fever, a healthy woman became comatose within a few days. Severe cerebellar symptoms appeared when she regained consciousness. The brain MRIs revealed abnormal signal intensity of the cerebellar cortex and brainstem gray matter, however, no abnormalities were revealed in the cerebral hemispheres. Acute inflammation due to direct viral or autoimmune involvement of the cerebellar and brainstem gray matter was a likely explanation and thus acute cerebellitis may in fact be a rhombencephalitis. Among the previous reports of acute cerebellar ataxia, this is perhaps one of the most profoundly affected cases and appears important for the understanding of the target of this particular form of encephalitis.

We measured the levels of interleukin-8 (IL-8) and monocyte chemoattractant protein-1 (MCP-1) in the bronchoalveolar lavage (BAL) fluids from 27 patients with active pulmonary sarcoidosis and examined the relationship between chemokine levels and some clinical manifestations. The levels of two chemokines were assessed by enzyme-linked immunosorbent assay. There were significant positive correlations between the absolute number of lymphocytes and MCP-1 levels. The level of MCP-1 was significantly higher in the group with age at onset of over 50 year than that in the group with age at onset under 30 year. There were no significant differences between the non-smokers and smokers, or among the groups of patients classed according to stages. We conclude that MCP-1 can play an important role in the pathogenesis and clinical course of pulmonary sarcoidosis, although further analysis is needed to delineate the exact role of IL-8.

A case of isolated angiitis of the central nervous system (IACNS) in puerperium with a clinical presentation of bilateral frontal lobe hemorrhage is described. Her symptoms and arteriographic abnormalities were resolved by institution of corticosteroid. After cessation of steroid treatment she showed no evidence of relapse. This case indicates that there appears to be a benign form of IACNS, although the etiology of postpartum IACNS is not known. The postpartum cerebral angiopathy described in the literature may be an identical disorder, since there are no clinical points of specific distinction between them.

We report siblings of late-onset Charcot-Marie-Tooth disease type 1A (CMT1A) of whom the elder brother presented with drop foot. DNA analysis confirmed the 17p11.2-p12 duplication. Estimation of the age of onset is difficult in CMT1A because of the subtle and slow progress of the symptoms. These cases are unique in that the initial clinical manifestation was a pressure palsy in the brother and both siblings were quite delayed in their clinical presentations. The peripheral myelin protein-22 gene duplication is uniformly found in CMT1A patients. However, other factors influencing the disease manifestations are yet to be determined.

We analyzed the HLA antigens in two patients with rheumatoid arthritis accompanied by diffuse panbronchiolitis in whom HLA typing could be done and found the presence of B54 and DR4 alleles. We previously reported that the frequency of HLA-B54 is significantly increased in patients with diffuse panbronchiolitis. This allele demonstrates a linkage disequilibrium with DR4 (or DR4.1) in Japanese individuals. The association of rheumatoid arthritis with HLA-DR4 is well established in various ethnic groups including Japanese people. Since both diseases show the same pattern of HLA correlation, more Japanese patients with these two diseases are likely to be encountered.