Baseline and Updated Information on Nutritional Status in Patients With Chronic Limb Threatening Ischaemia Undergoing Revascularisation.

OBJECTIVE: The aim of this study was to assess the impact of baseline and updated nutritional status on prognosis in patients with chronic limb threatening ischaemia (CLTI) undergoing revascularisation. METHODS: The clinical database of the Surgical reconstruction versus Peripheral INtervention in pAtients with critical limb isCHemia (SPINACH) study, a prospective, multicentre, observational study, was used. The current analysis included 499 patients who underwent endovascular therapy or surgical reconstruction for CLTI. Nutritional status at baseline was evaluated using the Geriatric Nutritional Risk Index (GNRI; baseline GNRI). A GNRI <82 points indicates major nutrition related risk. GNRI was also calculated at 1, 3, 6, 12, 24, and 36 months after revascularisation (updated GNRI). The association between baseline and updated GNRIs and the mortality risk was analysed with the Cox regression model. RESULTS: Mean ± standard deviation (SD) GNRI at baseline was 89.9 ± 9.8 points. The proportion of patients alive with a GNRI ≥82 points was 78% (95% confidence interval [CI] 74-81) at baseline but gradually decreased during follow up, finally reaching 19% (95% CI 0-42) at 36 months. In patients with a GNRI <82 points at baseline, a GNRI of ≥82 points was increased to 37% (95% CI 6-68) 12 months after revascularisation. In the multivariable analysis, baseline and updated GNRIs were associated with a reduced mortality risk independently of each other; the adjusted hazard ratios per 1 SD were 0.80 (95% CI 0.65-0.98; p = .031) and 0.66 (95% CI 0.49-0.91; p = .015), respectively. Similar findings were observed when nutritional status was evaluated using the Controlling Nutritional Stats (CONUT) score, except for the association between its updated value and mortality risk, which marginally lost significance. CONCLUSION: There was still room for improvement in nutritional status after revascularisation for patients with CLTI. Updated GNRI was associated with death independently of baseline GNRI.

Increased Incidence of Cancer in Japanese Patients with Critical Limb Ischemia.

Objective: This multicenter observational study was conducted in order to investigate the incidence of cancer in patients with critical limb ischemia. Materials and Methods: We prospectively investigated the incidence of cancer in 68 patients with critical limb ischemia over a two-year period. Patients underwent an intensive examination at enrollment, which included tumor marker levels and chest and abdominal computed tomography, as well as one- and two-year follow-up examinations. We compared the observed incidence of cancer with the expected incidence calculated from national cancer rates by the standardized incidence ratio (SIR). Results: The majority (83.6%) of the patients were men, and 92.5% of the patients had a peripheral arterial disease that was classified as Fontaine stage III or IV. During enrollment, newly diagnosed cancers were detected in seven patients. Four additional cancers were detected during the follow-up period. All of the detected cancers were asymptomatic. We observed an increased risk of cancer (SIR, 4.04; 95% confidence interval, 1.31-9.42) in patients with critical limb ischemia. Conclusion: This study suggests that critical limb ischemia is associated with an increased risk of cancer. Our findings should be taken into serious consideration by future investigators considering the use of therapeutic angiogenesis.

Inflammatory features, including symptoms, increased serum interleukin-6, and C-reactive protein, in IgG4-related vascular diseases.

Immunoglobulin (IgG) 4-related diseases (IgG4-RDs) are fibro-inflammatory conditions characterized by tumorous swelling and serum IgG4 levels. Intrapelvic IgG4-RD has been subclassified according to the localization site and aortic shape as IgG4-related aortic aneurysms (IgG4-AAs), periaortitis (IgG4-PA), and retroperitoneal fibrosis (IgG4-RF). The IgG4-AA pathogenesis would involve interleukin (IL)-6 upregulation, and Th2-predominant and Treg-activated immune conditions. We characterized the features of intrapelvic IgG4-RD lesions, including presence of vascular lesions. The clinical, serological, and pathological features, including cytokines concerning Th1/2 and Treg (IL-4, IL-6, IL-10, IL-13, and interferon-gamma) of patients with IgG4-AAs (n = 24), IgG4-PA (n = 8), and IgG4-RF (n = 10) were retrospectively compared. Clinical symptoms, such as low-grade fever, abdominal/lumber pain, and anemia, were frequently detected in IgG4-AAs but rarely in IgG4-RF. Serum IL-6 and C-reactive protein (CRP) were significantly higher in IgG4-AAs and IgG4-PA than in IgG4-RF. Pathologically, IL-6+ cells were more frequently detected in IgG4-PA and IgG4-AAs than in IgG4-RF. There were no noteworthy differences in the clinical complications, white blood cell counts, serum IgE, and serum and immunopositive cells of other cytokines between the subgroups. Among IgG4-AAs and IgG4-PA, serum IL-6 and IL-6+ cells correlated with CRP, aortic diameter, and periaortic fibrosis. IgG4-AA and IgG4-PA, but not IgG4-RF, were characterized by "inflammatory" features, such as increased CRP and serum/pathological IL-6, and clinical inflammatory symptoms; thus, IgG4-AA and IgG4-PA belong to the same group as IgG4-related vascular disease. High levels of CRP and IL-6 would be hallmarks of IgG4-related vascular disease.

IgG4-Related Arterial Disease.

Immunoglobulin G4-related diseases (IgG4-RD) are systemic inflammatory conditions, characterized by high serum IgG4 concentrations, and pathologically IgG4-positive plasmacytes infiltrations and storiform fibrosis. We described IgG4-related inflammatory abdominal aortic aneurysm in 2008, and revealed the existence of vascular lesions. IgG4-related vascular lesions frequently occur in the aorta and branching medium-sized arteries with or without aneurysmal change. The inflammatory lesion mainly involves in the adventitia, indicating remarkable adventitial fibrous thickening with infiltration of inflammatory cells. Clinical symptoms associated with IgG4-related vascular lesions might be fever, abdominal pain, hydronephrosis, or few subjective symptoms. Comprehensive diagnostic criteria is applied according to image findings of thickening lesions, high serum IgG4 levels, and histopathological findings. As a treatment, open surgical repair or endovascular aneurysm repair is performed for the aneurysmal cases, and steroid administration is used for the cases with strong inflammation. This disease can lead to a lethal situation due to the rupture following aneurysmal formation, thus special attention is needed unlike IgG4-RD occupying in the other organs. (This is a translation of Jpn J Vasc Surg 2017; 26: 129-134.).

Upregulated interleukins (IL-6, IL-10, and IL-13) in immunoglobulin G4-related aortic aneurysm patients.

OBJECTIVE: Immunoglobulin (Ig) G4-related aortic aneurysms (IgG4-AAs) are a special aortic aneurysm among IgG4-related diseases (IgG4-RDs), which are inflammatory and fibrous conditions characterized by tumorous swelling of affected organs and high serum IgG4 concentrations. Recently, IgG4-RD pathogenesis was shown to be associated with T-helper-2 (Th2) and regulatory T (Treg) dominant cytokine production, such as interleukin (IL)-4, IL-10, and IL-13. IL-6 is a key proinflammatory cytokine contributing to lymphocyte and plasmacyte maturation and to atherosclerosis and aneurysm development. We serologically and histopathologically evaluated the cytokine profile in IgG4-AA patients. METHODS: Patients with IgG4-AAs (n = 10), non-IgG4-related inflammatory abdominal aortic aneurysms (non-IgG4-AAAs; n = 5), atherosclerotic AAAs (aAAAs; n = 10), and normal aortas without dilatation (n = 10) were examined for serum IL-10, IL-13, and IL-6 levels. Resected aortic tissues were evaluated for cluster of differentiation (CD) 34 (in the endothelial cells and mesenchymal cells) and CD163 (by macrophages) expression using immunohistochemistry and in situ hybridization. RESULTS: Serum IL-10 levels were rather higher in IgG4-AA patients (median, 1.3 pg/mL) than in non-IgG4-AAA and aAAA patients and in patients with normal aortas. Elevated serum IL-13 levels relative to standard values were detected in two IgG4-AA patients but not in the other groups. Cells immunopositive for IL-10 and IL-13 were more frequent in IgG4-AAs and significantly correlated with serum IgG4 levels. Serum IL-6 levels (median, 78.5 pg/mL) were also significantly higher in IgG4-AA patients than in non-IgG4-AAA and aAAA patients and control patients with normal aortas (P = .01, P = .001, and P = .004, respectively). They positively correlated with serum IgG4 levels and adventitial thickness, but other cytokines did not. The number of IL-6-immunopositive cells in the adventitia was significantly higher in IgG4-AA patients (median, 17.8/high-power field) than in aAAA patients or patients with normal aortas (P =.001 and P = .002, respectively). In situ hybridization confirmed frequent IL-6 messenger (m)RNA expression in the endothelium, mesenchymal cells, and histiocytes in IgG4-AA adventitia. In the same cells of IgG4-AAs, coexpression of IL-6 and CD34 mRNA or CD163 mRNA was detected. CONCLUSIONS: The cytokine profiles of IgG4-AA patients had two characteristics: local IL-10 and IL-13 upregulation in IgG4-AAs was related to Th2 and Treg-predominant cytokine balance, similar to other IgG4-RDs, and IL-6 upregulation in the adventitia was characterized by activated immune reactions in IgG4-AA patients. IL-6 synthesis, through contributions of mesenchymal cells and macrophages in the adventitia, is strongly involved in IgG4-AA pathogenesis or progression, or both.

Bailout Endovascular Stent Grafting for an Ascending Aortic Pseudoaneurysm Using an Infrarenal Aortic Extension Cuff.

We report successful thoracic endovascular repair of a pseudoaneurysm rupture in the ascending aorta using infrarenal endovascular devices after an aortic valve replacement. Complete exclusion of the pseudoaneurysm was achieved with no endoleak or postoperative complications. Despite limitations of the current technology, this endovascular technique was a relatively less invasive, feasible lifesaving surgical option for the repair of a pseudoaneurysm of the ascending aorta with a diameter ≤32 mm.

Clinical Outcomes After Endovascular Repair and Open Surgery to Treat Immunoglobulin G4-Related and Nonrelated Inflammatory Abdominal Aortic Aneurysms.

PURPOSE: To compare the follow-up results of endovascular aneurysm repair (EVAR) vs open surgery (OS) for inflammatory abdominal aortic aneurysms (IAAAs) with regard to immunoglobulin G4-related diseases (IgG4-RD), which are fibrous inflammatory conditions characterized by elevated serum IgG4 concentrations and numerous infiltrations of IgG4+ plasmacytes. METHODS: Between January 2005 and December 2015, 91 patients were treated with EVAR (begun in 2008) and 166 patients underwent OS for AAA. Forty of these 257 patients had IAAAs identified by a >2-mm thickness of periaortic fibrosis (PAF). Of these 40, 21 had pathologically confirmed IgG4-RD and/or serum IgG4 concentrations ≥135 mg/dL (classified IgG4+); 8 (mean age 76 years; 8 men) were treated with EVAR and 13 (mean age 71 years; 11 men) underwent OS. Of the 19 IgG4- patients with IAAA, 9 (mean age 71 years; 8 men) had EVAR and 10 (mean age 75 years; 9 men) had OS. The 4 subgroups were compared in terms of symptoms, complications, inflammation markers, PAF, and aneurysm diameter using the latest midterm follow-up data (12-24 months). RESULTS: Preoperative aneurysm diameter, PAF, gender, median age, symptoms, and median follow-up period were similar in all groups. Preoperative serum IgG4 was equal in EVAR and OS IgG4+ groups. Compared with the OS IgG4+ group, EVAR IgG4+ patients more frequently had postoperative IgG4 increase (5/8; p=0.006) and PAF progression (5/8; p=0.027), higher postoperative serum IgG4 levels (median 141 mg/dL; p=0.034), a thicker postoperative PAF (median 5.1 mm; p=0.016), and persistent clinical symptoms (p=0.006). Compared with EVAR IgG4- patients, the EVAR IgG4+ patients showed significantly thicker postoperative PAF (p=0.024) and larger increases in postoperative sac diameter (median +13.1 mm; p=0.030). Postoperative PAF and sac diameter frequently and synchronously became worse in the EVAR IgG4+ subgroup with increased IgG4 during follow-up. The rate of change in IgG4 significantly positively correlated with the rates of change in PAF (R=0.555, p=0.03) and sac diameter (R=0.902, p=0.003). CONCLUSION: Though sample sizes were rather small, this pilot study suggested that EVAR-treated IgG4+ IAAA patients have a higher risk of persistent symptoms and increases in PAF, sac diameter, and IgG4 levels. Therefore, OS should be preferred for complete recovery. Frequent monitoring of the postoperative serum IgG4 is necessary following EVAR in IgG4+ patients to detect these complications.

Late Sac Behavior after Endovascular Aneurysm Repair for Abdominal Aortic Aneurysm.

BACKGROUND: Sac behavior after endovascular aneurysm repair (EVAR) for abdominal aortic aneurysms (AAAs) is considered as a surrogate for the risk of late rupture. The purpose of the study is to assess the sac behavior of AAAs after EVAR. METHODS AND RESULTS: Late sac enlargement (LSE) (≥5 mm) and late sac shrinkage (LSS) (≥5 mm) were analyzed in 589 consecutive patients who were registered at 14 national centers in Japan. The proportions of patients who had LSE at 1, 3 and 5 years were 2.6% ± 0.7%, 10.0% ± 1.6% and 19.0% ± 2.9%. The proportions of patients who had LSS at 1, 3 and 5 years were 50.1% ± 0.7%, 59.2% ± 2.3% and 61.7% ± 2.7%. Multiple logistic regression analysis identified two variables as a risk factor for LSE; persistent endoleak (Odds ratio 9.56 (4.84-19.49), P <0.001) and low platelet count (Odds ratio 0.92 (0.86-0.99), P = 0.0224). The leading cause of endoleak in patients with LSE was type II. CONCLUSIONS: The incidence of LSE is not negligible over 5 year period. Patients with persistent endoleak and/or low platelet count should carefully be observed for LSE. CLINICAL TRIAL REGISTRATION: UMIN-CTR (UMIN000008345).

Is Endovascular Aneurysm Repair a Relative Contraindication for Patients with Preoperative Renal Dysfunction?

UNLABELLED: Whether endovascular aneurysm repair (EVAR) for abdominal aortic aneurysm (AAA) is a relative contraindication in patients with preoperative renal dysfunction (Pre-RD), remains controversial because the contrast medium may induce nephrotoxicity. In this study 1658 patients were treated at ten Japanese medical centers between January 2005 and March 2011 (Open surgery (OS) vs. EVAR: n = 1270 vs. n = 388). They were retrospectively analyzed. Multiple logistic regression analysis (MLRA) with pre- and intra-operative variables was applied to all patients. The endpoints induced onset of new dialysis and postoperative renal dysfunction (Post-RD), were defined as a 50% decrease or more from the preoperative estimated glomerular filtration rate (eGFR) level. RESULTS: Early mortality, Post-RD, incidence of new dialysis in all patients were 1.6% (OS: EVAR = 1.9%:0.8%), 6% (OS: EVAR = 8%:2.3%) and 1.4% (OS: EVAR = 1.5%:1.0%) respectively. MLRA identified operation time, clamp of renal artery as risk factors for Post-RD, and operation time and Pre-eGFR level as risk factors for new dialysis. CONCLUSION: Although Post-RD was more frequently observed in the OS group, MLRA showed that the choice of OS or EVAR was not a risk factor for Post-RD and new dialysis. It was strongly suggested that using contrast medium during EVAR is not a contraindication to AAA repair in patients with Pre-RD. (This article is a translation of J Jpn Coll Angiol 2014; 54: 13-18.).

Immunoglobulin G4-related periaortitis complicated by aortic rupture and aortoduodenal fistula after endovascular AAA repair.

PURPOSE: To report a rare and complicated case of immunoglobulin (Ig) G4-related periaortitis involving both the aortic wall and the retroperitoneum without aneurysmal formation. CASE REPORT: A 79-year-old man with IgG4-related periaortitis suffered aortic rupture despite a normal caliber aorta after 6 months of steroid therapy (20 mg/d). Endovascular repair with an aortic cuff sealed the rupture. Steroid therapy was halted 2 weeks later due to infection. Four months later, a biopsy during esophagogastroduodenoscopy to investigate gastrointestinal bleeding suggested a relapse of IgG4-RD in the duodenum. Subsequent aortoduodenal fistula formation proved fatal. Generally, IgG4-related periaortitis does not result in such complications due to the absence of aneurysm formation and a thick aortic wall. CONCLUSIONS: Our report highlights a rare case of IgG4-related periaortitis where complications resulted following steroid therapy and surgical intervention, emphasizing the difficulties in dealing with IgG4-related cardiovascular lesions.

Impact of introducing endovascular aneurysm repair on treatment strategy for repair of abdominal aortic aneurysm--National Hospital Organization network study in Japan.

BACKGROUND: The objective of the present study was to assess the hypothesis that the introduction of endovascular aneurysm repair (EVAR) into Japan has expanded the indication of abdominal aortic aneurysm (AAA) repair without increasing surgical mortality. METHODS AND RESULTS: From 10 national hospitals, we registered a total of 2,154 consecutive patients (Open surgery [OS]: n=1,577, EVAR: n=577) over 8 years, divided into 4 time periods: Group I (2005-2006: n=522), Group II (2007-2008: n=475), Group III (2009-2010: n=551), Group IV, (2011-2012: n=606). Mean age increased over the 4 time periods (P<0.0001). The incidences of COPD, smoking history, history of abdominal surgery and concomitant malignancy significantly increased as well, while the numbers of patients with preoperative shock or high ASA status reduced over time. The proportion of EVAR in AAA repair increased from: 0% in Group I, 11.6% in Group II, 41.0% in Group III, to 48.8% in Group IV (P<0.0001). Early mortality was 0.8% in the EVAR and 3.4% in the OS (P<0.001) groups. Survival rates among the 4 groups free of all-cause death and aneurysm-related death at 1 year were 92.1-96.3% (P=0.1555) and 95.5-96.8% (P=0.9891), respectively. Multiple logistic regression analysis for surgical death failed to demonstrate survival advantage of EVAR over OS. CONCLUSIONS: Introduction of EVAR expanded the indication of AAA repair without increasing mortality, while high risk for anesthesia and emergency cases reduced over time. UMIN-CTR (UMIN000008345).

A clinicopathologic study of immunoglobulin G4-related disease of the femoral and popliteal arteries in the spectrum of immunoglobulin G4-related periarteritis.

BACKGROUND: Immunoglobulin (Ig) G4-related disease has recently been recognized to occur in the cardiovascular system in the aorta and main branching arteries, often manifesting as aneurysms and arteritis/periarteritis. Peripheral arteries (the femoral and popliteal arteries) are frequent sites of arteriosclerosis obliterans (ASO) and occasionally show aneurysms or arteritis. This study re-examined peripheral arterial lesions from the standpoint of IgG4-related disease. METHODS: The study comprised 104 patients who underwent surgical treatment of peripheral arterial lesions, including 30 patients with peripheral arterial aneurysms (PAAs) and 74 with ASO. IgG4-related disease was identified on the basis of diffuse infiltration of numerous IgG4-positive plasmacytes as revealed by immunohistochemical examination. Clinicopathologic features were compared between IgG4-related and IgG4-unrelated lesions. RESULTS: IgG4-related disease was found in four of the 30 patients with PAAs (13.3%; two in the deep femoral artery, two in the popliteal artery) but not in any patients with ASO. IgG4-related PAA displayed clinicopathologic features resembling those of other IgG4-related diseases and a characteristic saccular appearance (P = .002). CONCLUSIONS: IgG4-related disease was detected in PAA patients but not in ASO patients. IgG4-related disease thus represents one potential etiology of aneurysm in the peripheral arteries.

Early outcomes of open abdominal repair versus endovascular repair for abdominal aortic aneurysm: report from national hospital organization network study in Japan.

OBJECTIVE: Early outcomes of open abdominal repair (OS) versus endovascular repair (EVAR) for abdominal aortic aneurysm were retrospectively analyzed, after commercialized devices for EVAR had become available in Japan. PATIENTS AND METHODS: A total of 781 consecutive patients (OS, n = 522; EVAR, n = 259) were treated at ten medical centers between January 2008 and September 2010. The OS group comprised patients with preoperative shock (SOS, n = 34) and without shock (NOS, n = 488). RESULTS: Patients in the EVAR group were 3 years older than those in the NOS group. There was greater prevalence of hostile abdomen, on dialysis, chronic obstructive pulmonary disease on inhaled drug, and cerebrovascular disease in the EVAR group than in the NOS group. Surgical mortality was 16 cases (2.0% in all patients, EVAR: 0.8%, NOS: 1.4%, SOS: 21%). Hospital stay >30 days was documented in 52 (11%) with NOS, 11 (33%) with SOS, and 8 (3%) with EVAR. Thirty late deaths included 6 aneurysm related death and 14 cardiovascular causes at a mean follow up of 1.0 year. The survival rates freedom from all cause death at one year, were 95 ± 1% in NOS and 94 ± 2% in EVAR respectively. CONCLUSION: Though significant differences in patient characteristics among three groups were noted, early results were satisfactory.

Early outcomes of endovascular aneurysm repair for abdominal aortic aneurysm: first preliminary report of national hospital organization network study in Japan.

PATIENTS AND METHODS: In order to assess the early outcomes of endovascular aneurysm repair (EVAR) for abdominal aortic aneurysm (AAA) in the Japanese population, a total of 183 patients who had EVAR at eight medical centers of the National Hospital Organization were retrospectively reviewed and registered. The mean number of registered cases in each center was 23 ± 17 (4-50 cases). Patient characteristics were male sex, 84%; mean age, 77 years; age ≥ 80 years, 40%. RESULTS: In-hospital mortality was one case (0.5%). Endoleaks were observed at the end of the procedure in 35 patients (19%: type I: n = 4, II: n = 22, III, n = 3, IV: n = 6). Early morbidity included delayed wound healing or infection (n = 7), deterioration of renal dysfunction (n = 3), stroke (n = 2), postoperative bleeding (n = 2), gastrointestinal complications (n = 2), and peripheral thromboembolism (n = 2). Eleven late deaths included one of unknown cause and six cardiovascular causes at a mean follow up of 1.0 year. Survival rates of freedom from all causes of death and from aneurysm-related death at one year were 95.4% ± 1.7% and 99.5% ± 0.5%, respectively. INTERPRETATION: Although registered patients carry a variety of risks, early outcomes were satisfactory. EVAR is an acceptable alternative treatment modality for treating AAA.

A clinicopathologic study of immunoglobulin G4-related sclerosing disease of the thoracic aorta.

OBJECTIVE: Immunoglobulin G4-related sclerosing disease (IgG4-SD) has recently been reported to occur in the cardiovascular system and manifest as inflammatory abdominal aortic aneurysm. Thoracic aortic lesions are often associated with aortitis in several divergent etiologies. Thus, this study was performed to review thoracic aortic lesions from the aspect of IgG4-SD and to elucidate the clinicopathologic characteristics of this subgroup in the thoracic aorta. METHODS: The study comprised 125 patients, including 71 with thoracic aortic aneurysm (TAA), 44 with aortic dissection, 7 with Takayasu aortitis, and 3 with infectious aortitis. IgG4-SD was identified by diffuse infiltration of numerous IgG4-positive plasmacytes by immunohistochemical examinations. Clinicopathologic features were compared between IgG4-related and IgG4-unrelated lesions. RESULTS: Among the 125 patients, IgG4-SD was found in 5 patients with TAA but was not detected in the other subgroups of thoracic aortic lesion. IgG4-related TAA included one case of lymphoplasmacytic aortitis, 1 case of inflammatory aneurysm, and three cases of atherosclerotic aneurysms. Patients with IgG4-related TAA showed clinicopathologic features similar to patients with IgG4-SD: male gender, old age, history of bronchial asthma and allergies, elevation of white blood cell counts, C-reactive protein levels, and IgG4 and IgE concentrations (in one patient); eosinophilic infiltration, obliterative phlebitis, lymph follicle formation, and perineural inflammation. In addition, compared with IgG4-unrelated TAA, IgG4-related TAA was characterized by clinically more frequency of involvement of the aortic arch (P = .002), saccular formation (P = .003), and fibrous adhesion to surrounding tissue (P < .001), and histopathologically thicker entire aortic wall and adventitia (P < .001 each). CONCLUSIONS: IgG4-SD is involved in 4% of all thoracic aortic lesions and uniformly presents in the form of an aneurysm with distinct histologic and clinicopathologic features. IgG4-SD represents one, albeit rare, etiology of TAA, especially those originating in the aortic arch.

A new clinicopathological entity of IgG4-related inflammatory abdominal aortic aneurysm.

OBJECTIVE: Recently, the relationship between immunoglobulin (Ig)G4 and idiopathic sclerosing lesions has attracted much attention. IgG4-related disease was first described with regard to the pancreas (autoimmune pancreatitis), and has been expanded to various organ systems. We previously reported that inflammatory abdominal aortic aneurysm (IAAA) could be one of the manifestations of IgG4-related disease. In this study, we tried to elucidate the clinical characteristics of IgG4-related IAAA. METHODS: This study consisted of 23 cases of IAAA and 40 cases of atherosclerotic abdominal aortic aneurysm (AAA). Clinical presentation, laboratory findings, and pathological features were examined. Aneurysms of 13 cases histologically corresponded to IgG4-related IAAA. RESULTS: Those cases accounted for 5% of all surgical AAAs, and 57% of IAAAs. Compared to non-IgG4-related IAAA, IgG4-related cases were characterized by less frequent association with abdominal or back pain. Serum IgG4 concentrations were significantly elevated in IgG4-related cases. Interestingly, patients with IgG4-related IAAA frequently showed an allergic constitution, such as drug allergy, autoimmune diseases, high serum IgE concentrations, and a high titer of antinuclear antibody. Pathologically, IgG4-related cases were characterized by more significant thickening of the adventitia and more numerous IgG4-positive plasma cell infiltrations. Three non-IgG4-related cases showed aneurysmal rupture at the time of first presentation, whereas no IgG4-related cases showed rupture. CONCLUSION: Recognizing a new disease entity of IgG4-related IAAA seems important because this was clinically and pathologically different from conventional aAAA and non-IgG4-related IAAA.

A case of multiple immunoglobulin G4-related periarteritis: a tumorous lesion of the coronary artery and abdominal aortic aneurysm.

Immunoglobulin G4 (IgG4)-related disease can occur in various organs, most of which are glandular or ductal tissues. Here, we report a case of multiple IgG4-related vascular lesions. A 63-year-old patient was found to have an abdominal aortic aneurysm and a tumorous lesion around the right coronary artery. The surgically resected aneurysmal wall and a tumorous lesion of the right coronary artery showed similar histologic features including diffuse lymphoplasmacytic infiltration, occasional eosinophils, and obliterative phlebitis. Immunohistochemically, numerous IgG4-positive plasma cells were evident within the lesions. The serum concentrations of IgG4 in the preoperative period was 456 mg/dL (reference range, <135), which decreased to 242 mg/dL 2 weeks after surgery. We made a diagnosis of multiple IgG4-related periarteritis manifesting as an abdominal aortic aneurysm and a tumorous nodule of the coronary artery. This case report suggested that IgG4-related disease can occur in the vascular system and manifest as an aneurysm or a periarterial mass lesion.

Inflammatory abdominal aortic aneurysm: close relationship to IgG4-related periaortitis.

Inflammatory abdominal aortic aneurysm (AAA) is a member of a family of disorders referred to as "chronic periaortitis" together with retroperitoneal fibrosis. Retroperitoneal fibrosis is included in IgG4-related disease, which is characterized by numerous infiltrating IgG4-positive plasma cells and high serum IgG4 concentrations. However, the relationship between IgG4-related disease and inflammatory AAA has not been documented. In this study, we examined the clinicopathologic characteristics of inflammatory (10 cases) and atherosclerotic (22 cases) AAAs, based on the hypothesis that inflammatory AAA might be related to IgG4-related disease. Cases of inflammatory AAA could be classified into 2 groups based on immunostaining of IgG4. Four patients showed diffuse infiltration of abundant IgG4-positive plasma cells (IgG4-related cases), whereas the remaining 6 cases of inflammatory AAA and all cases of atherosclerotic AAA had only a few IgG4-positive plasma cells (non-IgG4-related cases). IgG4-related inflammatory AAA was pathologically characterized by the frequent infiltration of eosinophils, lymph follicle formation, perineural inflammatory extension, and inconspicuous infiltration of neutrophils compared with non-IgG4-related inflammatory AAA. Obliterative phlebitis, which is venous occlusion with inflammatory cell infiltration, is observed in all IgG4-related cases. In addition, serum IgG4 concentrations were significantly higher in IgG4-related inflammatory AAA (109 to 559 mg/dL, normal range: 4 to 110 mg/dL) than non-IgG4-related inflammatory AAA (32 to 59 mg/dL) and all atherosclerotic AAA (12 to 83 mg/dL). In conclusion, inflammatory AAAs might be classified into 2 groups: IgG4-related or nonrelated. The former might be one of the IgG4-related diseases, and could be included in IgG4-related periaortitis together with retroperitoneal fibrosis.

Surgical treatment of a left ventricular neurofibroma.

Primary cardiac neurofibroma is a rare occurrence. We describe a case of left ventricular neurofibroma in a 56-year-old woman with Von Recklinghausen disease. Resection of the tumor with concomitant mitral valve replacement yielded a satisfactory clinical result, and histological examination of the resected tissue confirmed benign neurofibroma. The anatomic distribution of the vagus nerve plexus, which penetrates the epicardium and myocardium and courses over the left ventricular subendocardial surface, provides a tissue source from which this neurogenic tumor may arise.

[Diagnosis and treatment for limb edema due to venous disease].

We described the diagnosis and treatment for limb edema due to venous disease in this paper. Venous limb edema is caused by vein pressure elevation, which is induced by venous reflux, flow disturbance and overflow. Valve incompetence causes varicose vein and deep venous insufficiency of lower extremities. Deep vein thrombosis is the most popular disease among the venous obstruction morbidity. Arterio-venous fistula for hemodialysis sometimes induces venous arm edema due to overflow. Arm edema due to venous hypertension appears more clearly when it is associated by subclavian vein stenosis or occlusion. There are several causes for venous edema. So, we have to make an appropriate treatment based on the clear diagnosis.