Masamitsu Endo

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.

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.

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.

Efficacy and safety of on-pump beating heart surgery for valvular disease

BACKGROUND This study was conducted to assess the efficacy and applicability of on-pump beating heart valvular operations using retrograde coronary sinus perfusion. METHODS A prospective, randomized study was conducted. A total of 50 patients participated in this study after having been allocated to one of two groups. On-pump beating heart valvular operations using retrograde coronary sinus perfusion as myocardial protection were performed in 25 patients (beating heart procedure group: aortic = 8 patients, mitral = 15 patients, double = 2 patients). Twenty-five patients underwent conventional valvular operation using retrograde continuous warm blood cardioplegia (conventional procedure group: aortic = 9 patients; mitral = 13 patients; double = 3 patients). The remaining operative variables and early outcomes of these procedures were compared. In the beating heart procedure group, myocardial tissue oxygen was measured by near infrared spectroscopy, and partial oxygen pressure of coronary sinus perfusion was also measured. RESULTS The visual field of the on-pump beating heart was equal to that of conventional valvular operation, and technical accuracy was not compromised. In the beating heart procedure group, tissue oxygen saturation was maintained at 79% +/- 2%, and partial oxygen pressure of coronary sinus perfusion blood and returned blood were maintained at 383 +/- 29 mm Hg and 38 +/- 2 mm Hg, respectively. Postoperative peak creatine kinase-MB (measured every 3 hours postoperatively) and peak troponin T concentrations were significantly lower than those of conventional procedures (17.5 +/- 7.8 vs 32.1 +/- 9.3 IU/L and 0.12 +/- 0.04 vs 0.21 +/- 0.06 ng/mL, respectively; p < 0.05). There was no operative mortality and no major complications. CONCLUSIONS On-pump beating heart valvular operation is a good surgical option, and has advantages because conditions for the heart are more physiologic with beating tonus than with cardioplegia.

Endovascular Stent-Grafting for Infected Iliac Artery Pseudoaneurysms

We report two cases of acutely infected pseudoaneurysms of the iliac arteries, successfully treated with endovascular stent-grafting. Two patients underwent stent-graft treatment for erosive rupture of the iliac artery caused by surrounding infection. The first case is that of a 61-year-old man who had undergone Miles’ operation for an advanced rectal cancer. Postoperatively, he developed intrapelvic abscess formation, from which methicillin-resistant Staphylococcus aureus was cultured, followed by rupture of the right external iliac artery. The second case is that of a 60-year-old man who had a pseudoaneurysm of the left common iliac artery, which was contiguous with a left psoas muscle abscess, from which Streptococcus agalactiae was cultured. Both patients were successfully treated with only a stent-graft and antibiotic therapy, and remained symptom-free 12 months and 10 months later. Although endovascular stent-grafting should not be considered standard therapy for infected aneurysms, our cases suggest that it can result in repair of infected aneurysms even in the uncontrolled active stage.

Mediastinal Bronchial Artery Aneurysm Treated with a Stent-Graft

Purpose: To report a rare case of mediastinal bronchial artery aneurysm successfully treated with an endovascular stent-graft. Case Report: A 79-year-old man with a history of tuberculosis was admitted to our hospital complaining of worsening hoarseness. Examination revealed a large mediastinal bronchial artery aneurysm located near the origin of the artery. Since neither surgical intervention nor transcatheter embolization was feasible, an endovascular stent-graft repair was performed using a newly developed stent-graft constructed from a nitinol stent and a thin membrane of polyester. Postprocedural angiography showed satisfactory exclusion of the aneurysm. The patient made an uneventful recovery. The 18-month computed tomographic scan documented continued exclusion of the aneurysm, with no evidence of endoleak, graft thrombosis, or migration. Conclusions: When conventional procedures are too complicated for bronchial artery aneurysms, endovascular stent-graft repair can be a useful approach.

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.

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.

The use of a Greenfield filter to treat a pregnant woman for internal jugular venous thrombosis: report of a case.

Abstract.Internal jugular venous thrombosis is an unusual entity with the potential to develop into pulmonary embolism (PE). A 28-year-old woman at 15 weeks gestational age of pregnancy was referred to our hospital for pain and swelling on the left side of her neck. Magnetic resonance imaging and computed tomography of her neck revealed an occlusion of the left internal jugular vein. Left internal jugular venous thrombosis was thus diagnosed. She was successfully treated by placement of a Greenfield filter in the superior vena cava and delivered a full-term healthy infant. This procedure could be an effective and safe method to prevent PE in patients of internal jugular venous thrombosis in whom anticoagulation therapy has either failed or is contraindicated.