Clinical and Pathological Characteristics of IgG4-Related Periaortitis/Periarteritis and Retroperitoneal Fibrosis Diagnosed Based on Experts' Diagnosis.

IgG4-related disease is a systemic disease, characterized by elevation of serum IgG4 and, histopathologically, massive infiltration of IgG4+ lymphocyte and plasma cell infiltration, storiform fibrosis, causing enlargement, nodules or thickening. It may affect various organs simultaneously or metachronously. Here we analyzed the clinical and pathological characteristics of 99 patients diagnosed with IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis. Of 99 patients (women/men, 15/84; mean age 67.3±9.5 years), 33 were diagnosed based on the histopathological findings of perivascular/retroperitoneal lesions, 50 were diagnosed based on the characteristic imaging findings of perivascular/retroperitoneal lesions and the presence of definitive IgG4-related disease in other organ(s), and the remaining 16 patients were diagnosed by experts based on the characteristic imaging findings of perivascular/retroperitoneal legions, serological findings, response to glucocorticoid treatment, and/or the presence of suspected IgG4-related disease in other organ(s). According to the new organ-specific criteria proposed by experts, 73 (73.7%) diagnoses were categorized to be definitive, and 6 (6.1%) and 17 (17.2%) diagnoses were categorized to be probable and possible, respectively. Further analyses are needed to clarify the optimal diagnostic and therapeutic strategy of IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis. (This is a translation of J Jpn Coll Angiol 2018; 58: 117-129.).

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.).

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.