A case of IgG4-related autoimmune disease with multiple organ involvement.

A 52-year-old male was admitted with autoimmune pancreatitis (AIP), showing mononuclear cell infiltration in both the pancreas and salivary glands with both normal sialography and anti-SS-A/SS-B antibodies. Although the AIP improved with glucocorticoid treatment, subsequent abdominal computed tomography (CT) revealed a nodular shadow in the bilateral kidneys, which was confirmed as interstitial nephritis by renal biopsy. The patient's serum immunoglobulin G4 (IgG4) level was 10 times higher than the upper limit of the normal range. IgG4-positive mononuclear cell infiltration was detected in the salivary gland, pancreas, and kidney. A new entity proposed as 'IgG4-related autoimmune disease' was considered.

RS3PE syndrome presenting as vascular endothelial growth factor associated disorder.

OBJECTIVES: To characterise serum concentrations of various cytokines and detection by magnetic resonance imaging (MRI) of synovial hypervascularity in patients with remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) syndrome before and after corticosteroid treatment. METHODS: Vascular endothelial growth factor(165) (VEGF(165)), tumour necrosis factor alpha (TNFalpha), and interleukin 1beta (IL1beta) were measured by enzyme linked immunosorbent assay (ELISA) in serum samples from three patients with RS3PE syndrome. As controls, serum samples from 26 healthy volunteers, 12 patients with rheumatoid arthritis, 10 patients with systemic lupus erythematosus, 13 patients with polymyositis/dermatomyositis, 13 patients with vasculitis syndrome, and 6 patients with mixed connective tissue disease were also analysed. Synovial hypervascularity of patients with RS3PE syndrome was estimated by rate of enhancement (E-rate) in a dynamic MRI study. RESULTS: Serum concentrations of VEGF(165) (mean (SD) 2223.3 (156.3) pg/ml) were significantly higher in patients with active RS3PE syndrome than in controls before corticosteroid treatment. TNFalpha and IL1beta levels were similar in patients and controls. Synovial hypervascularity in affected joints and subcutaneous oedema decreased during corticosteroid treatment, in parallel with the fall in serum VEGF(165). CONCLUSIONS: VEGF promotes synovial inflammation and vascular permeability in patients with RS3PE syndrome, suggesting that RS3PE can be classified as a VEGF associated disorder.

Detection of apoptosis-specific autoantibodies directed against granzyme B-induced cleavage fragments of the SS-B (La) autoantigen in sera from patients with primary Sjögren's syndrome.

The objective of this study was to detect autoantibodies against granzyme B cleavage products in sera from patients with primary Sjögren's syndrome (SS). Cell lysates derived from human salivary gland (HSG) cell lines were incubated with granzyme B. The susceptibility to the generation of cleavage fragments of SS autoantigens was assayed by immunoblotting using sera from 57 primary SS patients, 17 primary SS patients with malignant lymphoma (ML), 28 systemic lupus erythematosus (SLE) patients, and 20 healthy controls. A 27 kD protein was recognized by serum autoantibodies in 8 (14.0%) of 57 primary SS patients, 5 (29.4%) of 17 SS patients with ML, 2 (7.1%) of 28 SLE patients, but not in 20 normal subjects. This protein was recognized by anti-SSB (La) monoclonal antibodies. Granzyme B-treated recombinant La protein was also shown to migrate as a discrete 27 kD protein by SDS PAGE. Blocking studies demonstrated the existence of an apoptosis-specific B cell epitope present in sera from 2 of 8 primary SS patients and in 2 of 5 primary SS patients with ML which recognized the 27 kD protein. Granzyme B-induced La fragments are generated during cytotoxicity in vitro. This is the first report describing autoantibodies in sera from primary SS patients that specifically recognize fragments of the La protein that are produced by the granzyme B protease.

[Perioperative management of endovascular stent graft placement for abdominal aortic aneurysm].

We retrospectively examined the changes in hemodynamics, oxygen index and renal function along with the complications in 25 patients who had undergone endovascular stent graft placement (ESG) surgery for abdominal aortic aneurysm. During stent graft placement, mean arterial pressure decreased to 58 +/- 8 mmHg by increasing the dose of anesthetics and/or using vasodilators. Except for this intended hypotensive period, mean arterial pressure and heart rate were relatively stable and adequately maintained during surgical manipulation. Oxygenation index was well maintained. A patient with a high preoperative creatinine level underwent prophylactic hemodialysis postoperatively. In other patients except one who died in early postoperative period, both BUN and creatinine levels were kept within normal ranges. Four patients died postoperatively and the causes of the death in two patients are related to the surgical procedure; one with multiple emboli possibly due to released atheloma from the aortic wall during procedure, the other with sepsis due to infected stent graft. Although ESG is a well tolerated procedure, embolism is the most serious complication. Careful preoperative evaluation of the ascending arch and descending aortic wall and monitoring with transcranial doppler are necessary.

[An autopsy case of rheumatoid arthritis accompanied with acute exacerbation of interstitial pneumonia].

An autopsy case of rheumatoid arthritis (RA) with acute exacerbation of interstitial pneumonia is reported. A 57-year-old woman with longstanding RA was admitted to our hospital because of progressive dyspnea. On chest roentogenogram, diffuse interstitial shadow was confirmed in both lungs. Chest computed tomography (CT) showed diffuse lesion of elevated density of CT level in both lung. She was diagnosed as an acute exacerbation of interstitial pneumonia, and treated by methylpredonisolone pulse therapy (1,000 mg/day). Although cyclosporin A (2 mg/kg/day) was combined to steroid therapy, she was died of progressive respiratory failure. The histological findings of the lung showed extensive fibrosis with alveolar damage associated with hyaline membranes, edema and hemorrhage in alveolar space.

[The levels of FDP, FDP-E and D-dimer in patients with rheumatoid arthritis].

We studied the relationship of FDP and D-dimer levels of rheumatoid arthritis (RA) patients with their activities of RA. We evaluated FDP/D-dimer levels of thirty-six RA patients. And we also evaluated FDP/D-dimer levels of 14 patients with systemic lupus erythematosus (SLE) and 12 patients with other rheumatic diseases as control. RA patients were divided into two groups according to their activities. Nineteen patients, who fulfilled at least three of four activities criteria, were classified as active group [RA (A) group], and other 17 patients were classified as not-active group [RA (B) group]. FDP and D-dimer levels of RA patients were higher than those of SLE or other patients group significantly (P < 0.01). Furthermore, in RA patients, high levels of FDP and D-dimer were observed in RA (A) group compared to RA (B) group. In some RA patients, decrease of FDP and D-dimer levels were observed according to their RA activities. FDP and D-dimer levels were not correlated with the level of rheumatoid factor (RF). These results show that the FDP and D-dimer levels were elevated according to RA activity in RA patients.

[Three-dimensional CT angiography of the portal vein using multiple threshold display].

To evaluate the usefulness of three-dimensional (3D) CT angiography of the portal vein obtained by using a multiple threshold display, 3D reconstructions were performed in 15 patients. The CT scanner employed was the Toshiba Xvigor. A volume of 150 ml of lopamidol 300 mg1/ml was administered at 3.3 ml/sec intravenously. Portal venous phase helical scanning was initiated 80 seconds after the start of the injection. Helical CT data were acquired using up to 25 continuous 1.0-sec rotations during a single breath-hold with an X-ray beam width of 7 mm and a couchtop movement speed of 7 mm/sec. Axial images were reconstructed at a section interval of 3 mm. Both the shaded surface display (SSD) and multiple threshold display (MTD) were generated by using Xtension with Sparc20. MTD was performed as follows. First, voxels, with higher CT values than that of liver parenchyma, were selected. Then, selected voxels were divided into eight parts, which were each assigned gradations of white to grey. The highest part of selected voxels, with higher CT values than that of the second branch of the portal vein, were set to white and a transparency of 0%. The other seven parts were each assigned transparencies of more than 0%. MTD images were compared with SSD in 15 cases by two radiologists. MTD images were superior to SSD images in quality, because MTD diminished surrounding artifacts due to liver parenchyma and enabled small vessels to be depicted clearly. Based on the above results, it was considered that MTD was a useful method for 3D CT angiography using enhanced helical CT.

[Three-phase dynamic helical CT for hepatocellular carcinoma: optimal scanning protocol].

To assess the optimal scanning protocol for three-phase dynamic helical CT, 20 patients were examined according to the following 4 methods (5 cases each) : (1) 100 ml Iopamidol (300 mgI/ml), at a rate of 2 ml/sec ; (2) 120 ml,3 ml/sec, (3) 150 ml, 3 ml/sec ; (4) biphasic method, initial 100 ml, 3.3 ml/sec ; remaining 50 ml, 1.6 ml/sec. Assessment of the time-density curve of the aorta, and the liver parenchyma, indicated that protocol (4) was superior to the others. Using protocol (4), 32 patients (62 nodules) with hepatocellular carcinoma underwent three-phase scanning, consisting of early, late, and delayed phases. Out of 61 nodules, 43 nodules were detected as high density areas in the early phase, 7 nodules as low density areas only in the late phase, and 3 nodules as low density areas only in the delayed phase. Compared with MRI, three-phase dynamic helical CT demonstrated numerous nodules, especially those less than 10 mm in diameter, and, therefore, was useful for the detection of hepatocellular carcinoma.

[Three-dimensional helical CT angiography of the abdomen].

To evaluate the quality of three-dimensional (3D) images of the abdominal vasculature acquired using enhanced helical CT, 3D reconstructions were performed for 43 examinations (38 patients). Twenty-one of 43 examinations were also reconstructed by Maximum Intensity Projection (MIP). The CT scanner employed was the Toshiba Xforce. Helical CT data were acquired using up to 20 continuous 1.5-sec rotations with an X-ray beam width of 5 mm and a couchtop movement speed of 5 to 10 mm/1.5 sec. Axial images were reconstructed at a section interval of 2 mm. Optimal protocol on enhanced helical CT was as follows: Iopamidol 300 mg I/ml was administered intravenously using a biphasic technique (3-4 ml/sec for the initial 100 ml, followed by 0.7-1.5 ml for the remaining 50 ml), and delay times of the early and late phases were 25-35 and 90 sec, respectively. Aortic branches were clearly demonstrated on early phase, while portal branches were well defined on late phase. In the visualization of abdominal vessels, 3D images were nearly equal to MIP images. However, for anteroposterior images, MIP images were superior to 3D images in quality, because 3D images had some longitudinal direction artifacts. Three-dimensional images were considered to be useful for correctly evaluating overlapping abdominal vasculatures. From the above results, 3D and MIP images of the abdominal vasculature obtained using enhanced helical CT were considered to compensate for each other.

[Evaluation of AMI-25 enhanced MR imaging and enhanced helical CT for liver tumors].

To evaluate the diagnostic capability of superparamagnetic iron oxide enhanced MRI and enhanced helical CT, 17 patients with histologically or clinically diagnosed hepatocellular carcinoma or metastatic liver tumor and a total of 208 nodules were studied. The pulse sequences used were SE 500/20 (T1WI), SE 2000/20 (PDWI) and SE 2000/70 (T2WI). Contrast medium was administrated intravenously for more than 30 minutes at a dose of 10 mumoles Fe/kg. Enhanced MR images were obtained 30-90 minutes after the injection of contrast medium. With helical CT, 133 nodules were detected. With unenhanced MRI, 62 were detected on T1WI, 60 on PDWI and 77 on T2WI. On the other hand, with enhanced MRI, 165 were detected on T1WI, 190 on PDWI and 186 on T2WI. Enhanced MRI revealed the most nodules among these images (P < 0.05), and was especially effective in detecting small nodules less than 10 mm in diameter (P < 0.05). In conclusion, it was considered that superparamagnetic iron oxide enhanced MRI was useful for the detection of hepatic nodules and that the most adequate pulse sequence was PDWI.

[Clinical evaluation of asymmetric film-screen system for thoracic lesions].

We investigated the clinical usefulness of the asymmetric film-screen system on postero-anterior chest roentgenograms for thoracic lesions. The asymmetric film-screen system, the so-called Kodak Insight thoracic imaging system, records a different image on each side of double-emulsion film. An image displaying the lung field is captured on the anterior side of the film, while one displaying the mediastinal, retrocardiac and retrodiaphragmatic structures is captured on the posterior side. The clinical usefulness of the asymmetric film was evaluated in 20 patients for visibility of the anatomical structures and abnormal lesions in comparison with conventional film. In the asymmetric film-screen system, the front and rear screens were HC (high contrast) and Regular, respectively. Chest radiography was performed at 200mA and 100kVp. As conventional film, we used SRC film (Konica, orthotype film), and chest radiography was performed at 100mA and 130kVp. The chest roentgenograms obtained with the asymmetric film-screen system provided better information on normal structures and abnormal lesions in the lung field, but slightly inferior information on mediastinal structures. In conclusion, it was considered that the asymmetric film-screen system was useful for thoracic lesions.

Helical scanning CT for hepatocellular carcinoma.

To evaluate the clinical usefulness of helical scanning CT for hepatocellular carcinoma, 39 patients were examined using a Toshiba CT system, the Xforce. Helical scanning CT data were acquired using up to 20 continuous 1.5-sec rotations, with contrast medium bolus injection of 100-150 ml at a rate of 1.5-3 ml/sec, and during a single breath-hold. Scanning was begun 35 to 50 sec after the start of injection. Helical scanning CT permits axial images to be reconstructed at any desired position within the scanned area, and provides images without interslice gaps caused by respiratory movement. Contrast enhanced helical scanning CT permits blood vessels to be clearly displayed following the bolus injection of contrast medium using an automatic injector. Furthermore, the shorter scanning time makes it possible to scan the target area in the desired vascular phase. In all 39 patients who were examined using contrast enhanced helical scanning CT, high-quality three-dimensional images were obtained. Such images were particularly useful for assessing the three-dimensional relationships among high density tumors and vascular structures. In conclusion, helical scanning CT was considered to be useful for hepatocellular carcinoma.

[Clinical evaluation of two- and three-dimensional imaging of helical scanning CT in the upper abdomen].

To evaluate the usefulness of helical scanning CT of the upper abdomen, 30 patients with hepatic, adrenal, pancreatic and renal disease were examined using a Toshiba CT system, the Xforce. Helical scanning CT data were acquired using up to 20 continuous 1.5-second rotations, with a 1.5-3 ml bolus injection of contrast medium, and during a single breath-hold. Helical scanning provided both better data continuity and resolution than conventional scanning. The axial images with a slice thickness of 5 mm were not inferior in quality to equivalent images acquired by conventional CT, and multiplanar reconstruction images were superior. Targeted structures could be easily obtained because helical scanning CT permits image reconstruction in any direction. Using a CEMAX-VIPstation, we generated three-dimensional display images of high-density structures from the postcontrast data acquired with helical scanning. On these images, it was easy to observe the high-density tumors three-dimensionally and to determine the position of the tumors relative to the high-density surrounding organs. In conclusion, helical scanning CT was considered to be useful in clinical diagnosis involving the upper abdomen.