We report a case of performing a 4-stage operations, including TEVAR through the descending aorta as an access route, for multiple aortic aneurysms complicated by severe COPD. The patient was a 71-year-old woman. A chest X-ray suggested a thoracic aortic aneurysm (TAA). CT scans revealed significant aortic tortuosity and six aortic aneurysms, including a TAA with a maximum diameter of 65 mm. However, due to severe mixed ventilatory impairment with an FEV1 of 39% and a %VC of 64%, a multi-stage surgery including TEVAR was chosen from the perspective of surgical tolerance. Additionally, due to severe calcification and stenosis extending from both iliac arteries to the femoral arteries and significant aortic tortuosity, careful planning for endovascular access was necessary. In the first stage, TEVAR was performed through the descending aorta as the access route for the TAA. In the second stage, a prosthetic graft replacement (abdominal four-branched reconstruction) was performed for the thoracoabdominal aortic aneurysm. In the third stage, TEVAR was performed using a prosthetic graft branch as the access route for the remaining TAA. In the fourth stage, additional TEVAR was performed for the endoleak, and EVAR was performed for the abdominal aortic aneurysm and common iliac artery aneurysm, completing the treatment in four stages. By carefully designing treatment strategies, such as access routes for endovascular stent-graft insertion with a focus on minimal invasiveness, severe postoperative complications, including respiratory issues, were successfully avoided.

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of autoimmune diseases characterized by muscle involvement and various extramuscular manifestations. Interstitial lung disease (ILD) is one of the most common extramuscular manifestations of IIM and is associated with significant mortality and morbidity. The clinical phenotypes, treatment responses, and prognosis of IIM-ILD are significantly related to myositis-specific antibody (MSA) profiles, with some racial differences. The features associated with MSA in IIM-ILD could also be relevant to cases of ILD where MSA is present but does not meet the criteria for IIM. The anti-melanoma differentiation-associated gene 5 antibody is highly associated with rapidly progressive ILD (RP-ILD), especially in Asian populations, and with characteristic cutaneous manifestations, such as skin ulcers. Radiologically, ground-glass opacities, consolidations, and nonsegmental linear opacities were more predominant than reticular opacities and honeycombing. While the mortality rate is still around 30%, the prognosis can be improved with early intensive therapy with corticosteroids and multiple immunosuppressants. In contrast, anti-aminoacyl-tRNA synthetase (ARS) antibodies are associated with chronic ILD, although RP-ILD is also common. Patients with anti-ARS antibodies often show lung-predominant presentations, with subtle muscle and skin involvement. Radiologically, reticular opacities, with or without consolidation, are predominant and may progress to honeycombing over time. Combination therapy with corticosteroids and a single immunosuppressant is recommended to prevent relapses, which often lead to a decline in lung function and fatal long-term outcomes. Significant advances in immunology and genetics holds promise for fostering more personalized approaches to managing IIMILD.

Background/Aims: The factors affecting the detection rate of lymphoplasmacytic sclerosing pancreatitis (LPSP) using endoscopic ultrasound-guided tissue acquisition (EUS-TA) in patients with type 1 autoimmune pancreatitis (AIP) have not been thoroughly studied. Therefore, we conducted a retrospective study to identify the predictive factors for histologically detecting level 1 or 2 LPSP using EUS-TA. Methods: Fifty patients with AIP were included in this study, and the primary outcome measures were the predictive factors for histologically detecting level 1 or 2 LPSP using EUS-TA. Results: Multivariate analysis identified the use of fine needle biopsy (FNB) needles as a significant predictive factor for LPSP detection (odds ratio, 15.1; 95% confidence interval, 1.62–141; ¬¬p=0.017). The rate of good-quality specimens (specimen adequacy score ≥4) was significantly higher for the FNB needle group than for the fine needle aspiration (FNA) needle group (97% vs. 56%; p<0.01), and the FNB needle group required significantly fewer needle passes than the FNA needle group (median, 2 vs. 3; p<0.01). Conclusions The use of FNB needles was the most important factor for the histological confirmation of LPSP using EUS-TA in patients with type 1 AIP.

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of autoimmune diseases characterized by muscle involvement and various extramuscular manifestations. Interstitial lung disease (ILD) is one of the most common extramuscular manifestations of IIM and is associated with significant mortality and morbidity. The clinical phenotypes, treatment responses, and prognosis of IIM-ILD are significantly related to myositis-specific antibody (MSA) profiles, with some racial differences. The features associated with MSA in IIM-ILD could also be relevant to cases of ILD where MSA is present but does not meet the criteria for IIM. The anti-melanoma differentiation-associated gene 5 antibody is highly associated with rapidly progressive ILD (RP-ILD), especially in Asian populations, and with characteristic cutaneous manifestations, such as skin ulcers. Radiologically, ground-glass opacities, consolidations, and nonsegmental linear opacities were more predominant than reticular opacities and honeycombing. While the mortality rate is still around 30%, the prognosis can be improved with early intensive therapy with corticosteroids and multiple immunosuppressants. In contrast, anti-aminoacyl-tRNA synthetase (ARS) antibodies are associated with chronic ILD, although RP-ILD is also common. Patients with anti-ARS antibodies often show lung-predominant presentations, with subtle muscle and skin involvement. Radiologically, reticular opacities, with or without consolidation, are predominant and may progress to honeycombing over time. Combination therapy with corticosteroids and a single immunosuppressant is recommended to prevent relapses, which often lead to a decline in lung function and fatal long-term outcomes. Significant advances in immunology and genetics holds promise for fostering more personalized approaches to managing IIMILD.