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.

The timing and choice of surgical method for type B aortic dissection, is still a topic of much debate. We performed Total arch replacement using Frozen elephant trunk (TAR-FET) as a means of preventing distant aortic events, such as retrograde type A aortic dissection (RTAD). We conducted analysis of 142 patients with acute type B dissection who were admitted between January of 2010 and July of 2017. Fifty-five cases required surgical intervention to treat enlargement of the false lumen diameter and ULP formation 2 weeks after the onset of symptoms. 17 TAR-FET were performed with a mean of 42±26 days period from onset to surgery. There were no complications of RTAD or paraplegic, and 90% of patient demonstrated aortic event free survival (5 years) and false lumen reduction ratio of 35%. Based on our analysis, using TAR-FET properly avoids serious complications like RTAD, and is a viable treatment option for type B dissection.

Spontaneous isolated dissection of the superior mesenteric artery (SMAD) is not still well known. We retrospectively analyzed our 30 patients with SMAD to elucidate the treatment strategy and long-term follow-up outcomes. Due to severe abdominal symptom we performed a stents deployment and surgical reconstructive surgery for each one case. Anerysmectomy and bypass surgery was performed for a patient with aneurysmal change. Other 27 patients were managed conservatively. SMAD patients had only two vascular events (renal infarction and graft occlusion), and showed good prognosis for 6–146 (mean 69) months follow-up. We found that there is a few SMAD patients necessary of invasive management at acute phase and that most patients are safely conservatively treated with good prognosis.