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Objectives: Lupus enteritis (LE) is a rare but well-known gastrointestinal manifestation of systemic lupus erythematosus (SLE). This study was conducted to identify prognostic factors associated with poor responses in patients with LE., Methods: We consecutively registered patients diagnosed with LE between January 2009 and October 2019, and retrospectively compared their clinical characteristics based on whether they had good or poor responses to treatment., Results: A total of 13 patients (17 episodes) were included. The median age was 41 years, and 12 patients were female. A comparison of clinical characteristics between groups revealed similar computed tomography (CT) findings. However, serum CH50 levels were significantly lower in the poor response group (median [interquartile ranges (IQR)]; 29.2 [25.3-46.9] U/mL vs 19.3 [7.8-24.0] U/mL, p  = .0095). More patients in the poor response group had higher titers of anti-cardiolipin β2-glycoprotein I antibody (anti-CL β2GPI Ab) and were started on glucocorticoids (GCs) at moderate doses. In multivariable analysis, serum CH50 level was independently associated with poor response to induction therapy., Conclusion: Lower levels of CH50 at the time of initial treatment predicted inadequate treatment response in patients with LE.

A 33-year-old male with a history of bronchial asthma and allergic rhinitis was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA) eight years ago. The diagnosis was based on the presence of fever, remarkable eosinophilia, and painful digital ulcer. His signs and symptoms improved with a moderate dose of glucocorticoids. Thereafter, he was lost to follow-up, failing to attend any of the scheduled appointments. Three years later, he presented with painful digital gangrene on the lateral fold of the right ring fingernail and abdominal pain triggered by meals. Angiography showed multiple occlusions and severe stenoses of the peripheral arteries and coronary aneurysms, which confirmed the diagnosis of medium vessel vasculitis of the coronary and peripheral arteries due to flare up of EGPA. EGPA predominantly affects the small-sized vessels, but rarely the medium-sized vessels. Coronary vasculitis might occur asymptomatically, until the coronary stenosis becomes severe or myocardial infarction develops; hence, its prevalence is underestimated. In this case, a digital gangrene prompted us to perform a systemic angiography, leading to the diagnosis of coronary vasculitis. Careful observation for coronary lesions is necessary in patients with EGPA who develop digital gangrene.

Objectives: Surgical strategies for patients with aortic arch aneurysm extending to the descending aorta remain controversial. The antero-lateral partial sternotomy (ALPS) approach has been developed as a less invasive alternative single-stage strategy for extensive thoracic arch aneurysm (ETAA)., Methods: From September 2007 to April 2011, 18 patients underwent elective total arch replacement for ETAA by the ALPS approach (ALPS group). In this approach, a skin incision was made from the bottom of the xiphoid to the anterior axillary line at the third intercostal space with a convex curved line. The thorax was entered through the third intercostal space and a partial lower sternotomy was done. Surgical outcomes were compared with those of 22 patients with ETAA who underwent elective total arch replacement by median sternotomy alone (MS) with regard to the level of distal anastomosis, postoperative complications and mortality., Results: In the ALPS group, no hospital mortality occurred and one patient experienced pneumonia. No significant difference between the ALPS and MS groups was seen in operative time (384.1 ± 41.6 min vs 402.3 ± 85.3 min P = 0.423) and cardiopulmonary bypass time (220.8 ± 47.1 min vs 236.9 ± 45.4 min P = 0.286). In contrast, distal anastomosis was at a significantly lower vertebral level in the ALPS than in the MS group (5.5 ± 0.4 vs 4.3 ± 0.9, respectively: P < 0.0001)., Conclusions: The ALPS approach provides good surgical exposure for distal aortic arch aneurysms extending to the descending aorta and ensures the accurate reconstruction of the distal anastomosis without major complications.

Surgical treatment is effective to exclude all infected tissue in patients with infective endocarditis. Although various techniques have been reported, it has remained a great challenge for patients with extensive infected regions. A patient with extensive bivalvular endocarditis including the aortic and mitral valve and aorto-mitral continuity is described. A handmade aorto-mitral bioprosthetic valve was created to reconstruct the defect after extensive debridement. The patient was discharged on the 30th postoperative day without inflammatory signs.

A successful apico-aortic bypass for a patient with a porcelain aorta suffering from aortic stenosis is reported. A sewing cuff with an outflow graft to the apex and a hand-made composite graft were used instead of a rigid apical connector.

The activation of interleukin 1 receptor-associated kinase (IRAK)-1 is a key event in the transmission of signals from Toll-like receptors (TLRs). The catalytic activity of the protein kinase is not essential for its ability to activate nuclear factor (NF) kappaB, because transfection of a kinase-dead mutant of IRAK-1 (IRAK-1KD) is able to activate NF-kappaB in HEK293T cells. In the present study, we observed that the effect of IRAK-1KD was impaired by simultaneous expression of IRAK-4. The effect of IRAK-4 was accompanied by the phosphorylation and degradation of IRAK-1KD. Expression of IRAK-4KD instead of IRAK-4 did not cause these events. In IRAK-4-deficient Raw264.7 macrophages that were prepared by introducing short-hairpin RNA probes, the basal level of IRAK-1 was increased markedly. Stimulation of these cells with TLR ligands did not cause the degradation of IRAK-1, which was clearly observed in the parent cells. These results suggested that the expression of IRAK-4 alone is sufficient to cause the degradation of IRAK-1; the autophosphorylation of IRAK-1 is not necessary to terminate the TLR-induced activation of NF-kappaB. IRAK-4 has an ability to induce the degradation of IRAK-1 in addition to its role as an activator of IRAK-1.