Objective Cardiac involvement defines the prognosis for patients with systemic sarcoidosis. Despite advancements in techniques for diagnosing cardiac lesions, there remains significant room for improvement in cardiac screening and prognostic prediction. The present study therefore assessed the prognostic factors associated with cardiovascular events in patients with sarcoidosis.

Methods We retrospectively studied 132 patients with systemic sarcoidosis and evaluated the clinical data obtained between 2009 and 2022. A Kaplan-Meier survival analysis and Cox proportional hazards models were used to evaluate the associations between cardiovascular events and prognostic factors.

Results The median age of the patients at the diagnosis was 64.0 (55.0-71.0) years old. During a mean follow-up period of 6.3±3.2 years, 28 patients suffered from cardiovascular events. Patients in the event group had more severe heart failure symptoms, more frequent ventricular tachycardia, higher serum high-sensitivity cardiac troponin T (hs-cTnT) values [0.025 (0.017-0.044) vs. 0.011 (0.007-0.019) ng/mL, p<0.001], and lower left ventricular ejection fraction values than those in the non-event group. These trends were observed even if the patients were not diagnosed with cardiac involvement at the time of enrollment. A multivariate analysis revealed that hs-cTnT was an independent biomarker for the prediction of cardiac events (hs-cTnT >0.014 ng/mL: HR: 7.31, 95% confidence interval: 2.20 to 24.28, p<0.001).

Conclusion Hs-cTnT is a useful biomarker for predicting cardiovascular events in patients with sarcoidosis, even if cardiac involvement is not detected at the initial evaluation.

Objective Sodium-glucose co-transporter-2 inhibitors (SGLT2is), such as dapagliflozin, have a diuretic effect, and their early initiation to treat acute heart failure (AHF) may improve outcomes; however, the significance of the timing of starting dapagliflozin after hospital admission remains unclear.

Methods We performed a post hoc analysis of a prospective, observational registry. Participants were divided into the early (E) group and late (L) group using the median time to the initiation of dapagliflozin (6 days) as the cut-off. We evaluated the relationship between the time to the initiation of dapagliflozin after hospital admission and patient characteristics and the length of the hospital stay.

Patients Study subjects were 118 patients with AHF admitted between January 2021 and April 2022 who were started on dapagliflozin treatment (10 mg/day).

Results Patients were divided into the E group (n=63) and L group (n=55). The HF severity as evaluated by the New York Heart Association class and the N-terminal pro-brain natriuretic peptide level was not significantly different between the groups. The time to the initiation of dapagliflozin and length of hospital stay showed a significant positive correlation (p<0.001, r=0.46). The hospital stay was significantly shorter in group E [median, 16.5 days; interquartile range (IQR): 13-22 days] than in group L (median, 22 days; IQR: 17-27 days; p=0.002). A multivariate logistic regression analysis showed that the early initiation of dapagliflozin was independently associated with a shorter hospital stay, even after multiple adjustments.

Conclusion Early initiation of dapagliflozin after hospital admission is associated with a shorter hospital stay, suggesting it is a key factor for shortening hospital stays.

Objective The presence of endotoxin (ET) in ascites at the time of cell-free and concentrated ascites reinfusion therapy (CART) is generally assessed in patients with infectious disease status, but the exact rate of ET positivity in ascites for patients treated with CART is unknown.

Methods We evaluated ET levels in ascites at the time of CART, regardless of the presence of infectious symptoms. The analysis was performed for 529 cases in 183 patients in whom ET levels in ascites were measured at 2 time points (pre- and post-processing).

Results ET in ascites was positive in 8 of 529 cases. In the positive cases, the ET level after CART was significantly decreased. ET-positive patients had a significantly higher white blood cell count, neutrophil count, and serum CRP level before CART than ET-negative patients.

Conclusion Collectively, our data suggest that ET may be present in ascites, regardless of the infectious symptoms, especially in patients with a high white blood cell count, neutrophil count, and serum CRP level. Although the ET level in the re-infusion ascites seems to be decreased by CART, the possibility of endotoxemia after CART should be considered for such patients.

Objective Dexamethasone, remdesivir (REM), and baricitinib (BAR) are commonly used to treat coronavirus disease 2019 (COVID-19). High-dose steroids have also been reported to be well tolerated, even when used in combination with multiple drugs. In this retrospective study, we assessed the safety and therapeutic efficacy of a three-drug combination of high-dose steroids, REM, and BAR in hospitalized COVID-19 patients.

Methods We retrospectively evaluated the safety and efficacy of three-drug combination therapy.

Patients We evaluated 107 patients hospitalized with moderate or severe COVID-19 who underwent 3-drug combination therapy with high-dose steroids (80 mg of methylprednisolone or more, REM, and BAR) in our institution from December 2020 to June 2021. The mean age was 62.1±13.7 years old, and 71.2% were men. The severity of the study patients was as follows: 18 (16.8%) with an 8-category ordinal score of 4, 84 (78.5%) with a score of 5, and 5 (4.7%) with a score of 6.

Results The frequency of high-grade adverse events was low, except for hyperglycemia (n=59, 45.8%). The median duration from symptom onset to the start of three-drug combination therapy was eight days. All but one of the patients treated with the combination therapy improved. The median time to improvement by 1 category of the eight-category ordinal score was 6 days, and the 28-day mortality was 0.9%.

Conclusion This study showed the safety profile of three-drug combination therapy of high-dose steroids, REM, and BAR in moderate to severe COVID-19 patients. The three-drug combination therapy is well tolerated and has the potential to prevent exacerbation of severity.

Esophageal stricture caused by complete circumferential endoscopic submucosal dissection (ESD) of extensive esophageal squamous cell carcinoma (ESCC) is a major concern and can result in a low quality of life. Normal mucosa may remain within a complete circumferential lesion of ESCC in some cases. We herein report a case of ESCC in which a complete circumferential lesion was treated with ESD while leaving an island of normal mucosa within it. This case demonstrates that preserving areas of normal mucosa within lesions during complete circumferential ESD is not technically difficult and may be an effective measure for preventing esophageal stricture.

A 74-year-old man was admitted to our hospital with severe hematochezia. Abdominal enhanced computed tomography (CT) demonstrated extravasation of contrast material from the descending colon. Colonoscopy revealed recent bleeding in the descending colon diverticulum. Bleeding was stopped using detachable snare ligation. Eight days later, the patient developed abdominalgia, and CT revealed free air caused by delayed perforation. The patient underwent emergency surgery. Perforation at the ligation site was detected using intraoperative colonoscopy. This report is the first to describe a case of delayed perforation after endoscopic detachable snare ligation for colonic diverticular hemorrhage.

We reported a notable case of inflammatory hepatocellular adenoma that grew during pregnancy, consequently changing its appearance on magnetic resonance imaging remarkably. A 5-months-pregnant 35-year-old woman presented with a 37-mm liver nodule that had been diagnosed as focal nodular hyperplasia 3 years earlier. She had never used oral contraceptives. After 2 months, the nodule grew to 57 mm. The patient delivered a full-term infant without complications. Gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging performed after delivery revealed markedly different findings compared with the first images. A liver biopsy was performed, and the tumor was diagnosed as inflammatory hepatocellular adenoma.

A woman in her 70s presented with gallbladder carcinoma with liver metastases and peritoneal dissemination. After standard chemotherapy failed, a liver biopsy was performed. A FoundationOne CDx analysis showed that the tumor mutational burden (TMB) was high (34 mutations/megabase). Treatment with pembrolizumab, which is an immune checkpoint inhibitor (ICI), resulted in a partial response, and there were no significant immune-related adverse events. According to recently published reports, the frequency of TMB-high biliary tract cancer (BTC) is 3.4-4%, which makes it extremely rare. In conclusion, ICIs may be effective in patients with TMB-high BTC.

We encountered two consecutive cases of adult-onset Kawasaki disease (AKD) that were difficult to diagnose. In both cases, Kawasaki disease was not considered as a differential diagnosis in the early stages. However, it was possible to make a diagnosis by citing the disease as a differential diagnosis and introducing the patients to the pediatrics department. AKD has a minimal incidence rate and may have a clinical course different from that of childhood-onset Kawasaki disease. Therefore, it is necessary to incorporate Kawasaki disease into the differentiation of an adult fever and to consult with a pediatrician for its diagnosis.

Aortitis is a rare adverse event associated with granulocyte colony-stimulating factor (G-CSF). Contrast-enhanced computed tomography (CECT) is widely used to diagnose G-CSF-associated aortitis. However, the usefulness of gallium scintigraphy for the diagnosis of G-CSF-associated aortitis is unknown. We herein report a set of pre- and post-treatment gallium scintigrams of a patient with G-CSF-associated aortitis. During the diagnosis, gallium scintigraphy revealed hot spots on the arterial walls that appeared inflamed on CECT. Both the CECT and gallium scintigraphy findings disappeared. Gallium scintigraphy can be a supportive diagnostic tool for G-CSF-associated aortitis, especially in patients with an impaired renal function or allergy to iodine contrast.

The MYH7 R453 variant has been identified in inherited hypertrophic cardiomyopathy (HCM) and is associated with sudden death and a poor prognosis. The detailed clinical course of HCM with the MYH7 R453 variant, from a preserved to a reduced left ventricular ejection fraction, has not been reported. We identified the MYH7 R453C and R453H variants in three patients who progressively developed advanced heart failure requiring circulatory support and summarized the clinical course and echocardiographic parameters of these patients over the years. Because of the rapid disease progression, we consider genetic screening for patients with HCM imperative for future prognosis stratification.

IgG4-related membranous nephropathy (MN) is often refractory to glucocorticoid (GC) therapy, and treatment remains unclear. We herein report a 67-year-old Japanese man with IgG4-related MN and tubulointerstitial nephritis. A post-gastroscopy antibody test revealed Helicobacter pylori infection. After eradication, his proteinuria decreased indefinitely. We started prednisolone (30 mg/day), long-term GCs, and immunosuppressant therapy. However, remission proved challenging to achieve, with persistent proteinuria present at 1.0-2.0 g/gCr. We performed multitarget therapy for refractory IgG4-related MN, achieving proteinuria remission (<0.3 g/gCr). Multitarget therapy with low-dose GCs can resolve refractory IgG4-related MN through remission induction of proteinuria and minimize the risks associated with GC therapy.

A 58-year-old man with human immunodeficiency virus (HIV) infection presented with a week-long history of gross hematuria, nephrotic proteinuria, and acute kidney injury. The patient was non-adherent with combination antiretroviral therapy. A kidney biopsy showed cellular crescents with disruption of Bowman's capsule, C3-dominant immune complex deposition, consistent with HIV-associated immune complex kidney disease (HIVICK). During the course, his worsening kidney function warranted initiation of hemodialysis. This case highlights the fact that HIV patients are at an increased risk of developing HIVICK, especially in the setting of non-adherence. A greater understanding of HIVICK among HIV patients should promote additional investigation into its etiology and viable treatments.

Exostosin 1 (EXT1) and exostosin 2 (EXT2)-associated membranous nephropathy (MN) may be associated with active autoimmune disease. We encountered an elderly man who presented with EXT1/EXT2-associated lupus-like MN with full house immune deposits, monoclonal gammopathy of uncertain significance and Sjögren's syndrome. The patient exhibited various other immune abnormalities. Although he did not fulfill the criteria of clinical systemic lupus erythematosus (SLE), he met a stand-alone renal criterion of the Systemic Lupus International Collaborating Clinics (SLICC) 2012. Whether or not a stand-alone renal criterion with EXT1/EXT2 positivity, as in the present patient, can efficiently guide decisions regarding the diagnosis and treatment of SLE remains a clinical dilemma.

The number of patients with syphilis has been rapidly increasing. Without treatment, syphilis can damage various organs and become life-threatening. We herein report a 29-year-old woman diagnosed with neurosyphilis, acute hydrocephalus, syphilitic uveitis combined with hypertensive retinopathy, and malignant hypertensive nephropathy. To our knowledge, this is the first report of syphilis complicated with malignant hypertensive nephropathy proven by a renal biopsy. Neurosyphilis was successfully treated with intravenous penicillin G, and severe hypertension subsequently resolved. However, delayed medical examinations and complications of syphilitic uveitis and hypertensive retinopathy resulted in irreversible visual loss. To prevent irreversible organ damage, early treatment is essential.

Encapsulating peritoneal sclerosis (EPS) is a fatal complication of peritoneal dialysis. A 68-year-old man undergoing peritoneal dialysis for 10 years started receiving daily 50 mg of glucocorticoids for idiopathic pulmonary sclerosis. At the transition to hemodialysis, a peritoneal biopsy was performed, which demonstrated mild histological changes, including no fibrin formation and mild T lymphocyte infiltration at the time of 6.5 mg glucocorticoids. However, five months later, he developed EPS when receiving 2.5 mg glucocorticoids. Afterward, over 5 mg daily glucocorticoids were required to avoid the recurrence of EPS. These findings suggest that glucocorticoids may conceal peritoneal inflammation, a main contributor to EPS.

A 44-year-old man with coronavirus disease 2019 (COVID-19) and nephrotic syndrome relapse was admitted to our intensive-care unit for respiratory failure. Despite receiving mechanical ventilation and immunomodulators, the patient experienced refractory hypoxemia, necessitating venovenous extracorporeal membrane oxygenation (VV-ECMO) therapy. Due to a worsening renal function, continuous hemodiafiltration was initiated. After 11 days, his respiratory status gradually improved, and VV-ECMO was withdrawn. The kidney function and proteinuria improved, and hemodialysis was subsequently discontinued. The patient was discharged 64 days after admission. This case highlights the potential benefit of early ECMO application in dramatically promoting recovery in severe COVID-19 cases.

Amplification of the mesenchymal-epithelial transition (MET) gene plays an important role in anticancer drug resistance to anaplastic lymphoma kinase-tyrosine kinase inhibitors (ALK-TKIs) in echinoderm microtubule-associated protein-like 4-anaplastic lymphoma kinase (EML4-ALK)-rearranged lung cancer cells. We encountered an ALK-rearranged lung cancer patient who developed MET amplification after alectinib treatment and showed an effective response to fifth-line crizotinib. First-line alectinib treatment was effective for 2.5 years; however, liver metastases exacerbated. Liver biopsy specimens revealed MET and human epidermal growth factor receptor 2 (HER2) amplifications. Switching to the MET inhibitor crizotinib improved liver metastases. Crizotinib may be effective in ALK-positive patients with MET amplification.

Pulmonary and extrapulmonary complications after coronavirus disease 2019 (COVID-19) have been major public health concerns during the COVID-19 pandemic. Although post-COVID-19 pulmonary manifestations cover a wide spectrum, eosinophilic pneumonia (EP) has rarely been reported. To date, only four cases of EP potentially triggered by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection have been reported, all of which required systemic corticosteroid therapy. We herein report the first case of post-COVID-19 EP resolution without systemic corticosteroid therapy. We also review the literature regarding EP associated with SARS-CoV-2 infection and vaccination.

We herein report a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) resembling adult-onset Still's disease (AOSD). A 40-year-old woman presented with a fever, erythema, and painful subcutaneous nodules on the trunk. Laboratory data and a bone marrow analysis showed hemophagocytic syndrome. Although AOSD was suspected, based on a histopathological evaluation of the erythema, she was diagnosed with SPTCL. She was refractory to combination chemotherapy but achieved durable remission with cyclosporine monotherapy. Genetic testing revealed a homozygous HAVCR2 c.245A>G variant (rs184868814) that had caused NLRP3 inflammasome activation. SPTCL and AOSD share a pathogenesis in terms of NLRP3 inflammasome activation, so the clinical phenotype of SPTCL reasonably mimics AOSD.

We herein report a 79-year-old man diagnosed with primary gastric diffuse large B-cell lymphoma (DLBCL) with gastropleural fistula (GPF), successfully treated by chemotherapy without surgery. If primary gastric DLBCL perforates during chemotherapy, surgery is often warranted. Our patient's computed tomography findings showed loculated pleural effusion with air foci in the left lower lobe, suggesting GPF. After six cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) chemotherapy, the fistula fully closed, and complete remission was achieved. In conclusion, while gastric DLBCL can exhibit spontaneous GPF, it can be treated with chemotherapy alone, which was well-tolerated in our patient.

We report a case of granulomatosis with polyangiitis (GPA) presenting with hypertrophic pachymeningitis with a huge brain tumor-like lesion. A 57-year-old man acutely developed consciousness disturbance. Magnetic resonance imaging revealed a right frontal lobe mass with thickened, contrast-enhanced dura. Computed tomography revealed sinusitis and multiple lung nodules. The presence of proteinase 3-anti-neutrophil cytoplasmic antibody indicated GPA. Histopathology of the excised brain tissues revealed thrombovasculitis with heavy neutrophilic infiltration in the pachy- and leptomeninges covering an ischemic cerebral cortex. The patient improved with corticosteroids and rituximab. Our case warrants considering GPA as a cause of hypertrophic pachymeningitis with brain-tumor like lesions.

Rivastigmine is a highly effective drug for treating Alzheimer's disease. However, its addiction can be fatal, so proper use of this transdermal drug is needed. We herein report an 85-year-old woman with Alzheimer's disease who inappropriately placed rivastigmine patches on the back of her neck. She suffered from acute cholinergic syndrome, hypersalivation, anorexia, dyspnea, and vomiting. These symptoms disappeared when the improper use of rivastigmine patches was ceased. This case serves as a warning to physicians and pharmacists of the risk associated with the improper placement of rivastigmine patches.

An 80-year-old woman with rheumatoid arthritis during treatment with etanercept, a tumor necrosis factor (TNF) inhibitor, showed swelling of the salivary glands and retroperitoneal fibrosis, which was diagnosed as IgG4-related disease. Although some reports have shown the efficacy of TNF inhibitors for IgG4-related disease or retroperitoneal fibrosis, TNF inhibitors sometimes cause paradoxical reactions like psoriasis, and the mechanisms are considered to involve the upregulation of plasmacytoid dendritic cells and IFN-α, which is also common in patients with IgG4-related disease. This is a case report of IgG4-related retroperitoneal fibrosis with the possibility of a rare paradoxical reaction by a TNF inhibitor.