Objective To investigate the additive effects of semaglutide on liver function indicators in patients with type 2 diabetes mellitus (T2DM) complicated by metabolic dysfunction-associated steatotic liver disease (MASLD), who received sodium-glucose cotransporter 2 inhibitors (SGLT2-Is).
Methods Liver function indicators at baseline were compared with those 6 months after the initiation of semaglutide.
Patients Forty-seven patients (mean age, 56.2±10.4 years; 74.5% men) with T2DM complicated by MASLD who were prescribed SGLT2-Is continuously for at least 12 months were retrospectively selected.
Results Six months of semaglutide treatment significantly decreased body weight (BW; 80.9±16.4 kg to 77.3±17.0 kg, p<0.001), body mass index (BMI; 28.9±4.5 kg/m2 to 27.7±4.6 kg/m2, p<0.001), and the levels of glycated hemoglobin (HbA1c; 7.4±0.7% to 6.9±0.7%, p<0.001), aspartate aminotransferase (AST; 30.4±15.9 U/L to 26.3±12.5 U/L, p=0.014), alanine aminotransferase (ALT; 42.4±28.7 U/L to 35.7±22.8 U/L, p=0.022), and gamma-glutamyl transpeptidase (γ-GTP; 46.5±36.8 U/L to 38.0±30.0 U/L, p=0.001). The fibrosis-4 index, an indicator of liver fibrosis, did not improve. Changes in ALT levels were significantly correlated with changes in metabolic parameters, including BW (p=0.004), BMI (p=0.004), HbA1c (p=0.005), and triglycerides (p=0.011).
Conclusion The addition of semaglutide to SGLT2-Is reduced liver enzyme levels in patients with T2DM complicated with MASLD. Future randomized controlled trials are needed to investigate the additive effects of semaglutide on MASLD in patients with T2DM receiving SGLT2-Is.
Objective The extent to which support benefits family caregivers and whether healthcare professionals effectively fulfill this role remains unclear. This study investigated whether the presence or absence of a supportive person affects the health-related quality of life (HRQOL) of family caregivers of patients with interstitial lung disease (ILD) and whether support from healthcare professionals influences HRQOL.
Methods This cross-sectional study was conducted at Kyoto University Hospital between April 2020 and March 2021. A supportive person was defined as someone available to discuss personal concerns. Caregivers' HRQOL was measured using the Medical Outcomes Study 36-Item Short-Form Health Survey version 2 (SF-36v2), and scores were compared between those with and without support. Further comparisons were made between caregivers who did and did not identify healthcare professionals as supportive persons.
Patients This study included outpatients with ILD and their caregivers.
Results Among the family caregivers, 78.3% reported having a supportive person. However, their presence was not significantly associated with the SF-36v2 scores. Compared to caregivers without a healthcare professional as a supportive person, those who identified healthcare professionals in this role had significantly higher vitality and mental component summary SF-36v2 scores (p=0.031 and p=0.012, respectively).
Conclusion Approximately 80% of the family caregivers of patients with ILD reported having a supportive person. While the general presence of a supportive person did not impact HRQOL scores, those who had healthcare professionals as supportive persons demonstrated a higher mental HRQOL.
Tumor-associated blood eosinophilia (TABE) and tumor-associated tissue eosinophilia (TATE) are rare in solid cancers, and reports of eosinophilic peritonitis in these cases are extremely limited. We present the case of a 68-year-old man with pancreatic and lung cancer who developed severe eosinophilia and eosinophilic peritonitis. Despite corticosteroid therapy, refractory ascites led to a decline in performance status. An autopsy confirmed undifferentiated pancreatic adenocarcinoma with TABE, TATE, and lung cancer. This case underscores the diagnostic challenges of multiple primary malignancies with eosinophilia and highlights the need for individualized management strategies for tumor-associated eosinophilic disorders.
We report an extremely rare case of recurrent Takotsubo syndrome (TTS) in an 83-year-old woman who initially experienced the mid-ventricular type and later developed the focal type. TTS is a transient cardiac condition that mimics acute coronary syndrome but lacks obstructive coronary lesions. While recurrence is uncommon, a change in morphological subtype is particularly rare. In this case, echocardiography was inconclusive, and the diagnosis was confirmed via left ventriculography. This case highlights the diagnostic challenges of focal-type TTS and emphasizes the importance of comprehensive imaging, especially left ventriculography, in patients with suspected recurrence and inconclusive non-invasive findings.
A 66-year-old man presented with upper limb blood pressure. Imaging revealed a mural thrombus extending from the aortic arch to the descending aorta and multiple metastatic lesions. Fluorine-18 fluorodeoxyglucose-positron emission tomography/computed tomography showed a low FDG uptake, and gadolinium-enhanced magnetic resonance imaging ruled out malignancy. Despite progressive aortic luminal stenosis, the patient remained asymptomatic due to embolism. The patient died 22 months later, and an autopsy confirmed aortic intimal sarcoma. This case highlights the diagnostic limitations of imaging for malignancy-related aortic luminal stenosis and provides insights into the management of progressive aortic luminal stenosis when curative revascularization is not feasible.
A 73-year-old woman with bronchial asthma presented with a rash and peripheral neuropathy. Since 21 years old, her asthma had been treated with glucocorticoids. Benralizumab had been introduced six years ago, and glucocorticoids were discontinued one year later. Bronchial asthma had been in remission for 5 years. On admission, urinalysis findings were mildly abnormal, myeloperoxidase-specific antineutrophil cytoplasmic antibody was positive, and eosinophilia was observed. A renal biopsy showed signs of mild necrotizing vasculitis with eosinophilia. Eosinophilic granulomatosis with polyangiitis (EGPA) was diagnosed. Benralizumab was discontinued, glucocorticoids were restarted, and mepolizumab was administered. Since then, the EGPA and asthma have not recurred.
A 56-year-old man with nephrotic syndrome was diagnosed with recurrent complement 3 glomerulonephritis (C3GN) without preceding infection. Initial treatment with steroid pulse therapy and oral glucocorticoids failed, and he required hemodialysis due to progressive kidney dysfunction. After rituximab was added, his renal function gradually improved, and dialysis was discontinued. Relapse occurred months later, but combination therapy with steroids and rituximab was effective again. The patient remained dialysis-independent with biannual rituximab infusions. This case illustrates that combining corticosteroids and rituximab may induce remission and preserve the kidney function in relapsing C3GN, even in patients requiring temporary dialysis support.
Hereditary hemorrhagic telangiectasia (HHT) complicates pregnancy due to pulmonary arteriovenous malformations (PAVMs), which risk rupture from hemodynamic changes. A 26-year-old woman at 32 weeks' gestation with refractory cough, dyspnea, and hypoxemia was diagnosed with HHT based on recurrent epistaxis, telangiectasias, and an ENG deletion. Chest computed tomography revealed multiple PAVMs with a shunt fraction of 26%. Caesarean section at 33 weeks ensured maternal and fetal safety. Persistent postpartum hypoxemia necessitates endovascular embolization and home oxygen therapy. This case underscores the need for early detection and multidisciplinary management of HHT during pregnancy to mitigate life-threatening complications.
The "Cheerios sign" is a ring-shaped pulmonary nodule with central lucency, as observed on chest computed tomography. A 48-year-old woman with Turner syndrome presented with diffuse pulmonary nodules exhibiting the Cheerios sign. Although transbronchial lung biopsy is non-diagnostic, transbronchial lung cryobiopsy (TBLC) revealed nodules composed of meningothelial-like cells that were positive for epithelial membrane antigen, progesterone receptor, and CD56, thus leading to a diagnosis of diffuse pulmonary meningotheliomatosis. Estrogen and progesterone hormone therapy were discontinued, and the disease remained stable without treatment. This case highlights the diagnostic utility of TBLC for the evaluation of small pulmonary nodules that exhibit the Cheerios sign.
Dupilumab is widely used to treat type 2 inflammatory diseases; however, rare adverse events, including eosinophilic granulomatosis with polyangiitis (EGPA), may occur. We report the first case of central retinal artery occlusion (CRAO) as a manifestation of EGPA following dupilumab initiation after switching from mepolizumab in a 64-year-old man with severe asthma. The potential for rare but tragic complications such as CRAO should not be overlooked when using dupilumab, particularly when withdrawing or switching medications that suppress eosinophilic activity. Despite a generally poor visual prognosis, early recognition and multidisciplinary management are essential.
Drug-induced immune thrombocytopenia (DITP) caused by ampicillin (ABPC) is rarely reported. An 87-year-old woman developed severe thrombocytopenia on postoperative day 3 during treatment with multiple drugs, including ABPC, for chronic periprosthetic infection following total knee arthroplasty. Although the platelet counts were unresponsive to oral prednisolone and intravenous immunoglobulin, they recovered promptly after the discontinuation of ABPC. Flow cytometry demonstrated ABPC-dependent IgG binding to healthy donor platelets. This is the first report to confirm ABPC-dependent platelet-reactive IgG using flow cytometry, thereby expanding the diagnostic tools for suspected DITP and highlighting the need for early recognition, prompt drug withdrawal, and appropriate supportive care.
Adult T-cell leukemia/lymphoma (ATL) has a poor prognosis, with mogamulizumab effectively controlling the disease but increasing graft-versus-host disease (GVHD) risk during transplantation if residual levels persist. We herein report a 61-year-old patient with acute-type ATL who underwent mogamulizumab concentration-guided transplantation to avoid severe GVHD despite human leukocyte antigen-mismatched donor transplantation. Post-transplant central nervous system (CNS) relapse was successfully managed with intrathecal chemotherapy alone, achieving long-term remission. Two years post-transplantation, the patient remains in complete remission without relapse or chronic GVHD. This case highlights the importance of assessing mogamulizumab levels for optimal transplantation timing and intrathecal chemotherapy as a strategy for CNS-limited ATL relapse.
We herein report a case of recurrent Guillain-Barré syndrome presenting with tetraplegia, ophthalmoplegia, respiratory failure, and autonomic dysfunction following Campylobacter jejuni infection. A glycoarray analysis revealed IgG antibodies against ganglioside complex antigens, including GQ1b and GD1b. Despite multiple immunotherapies, the patient required prolonged ventilatory support and remained bedridden for over one year. These antibodies may be associated with disease severity and serve as biomarkers.
Heterozygous HTRA1 gene variants are associated with hereditary cerebral small vessel disease (CSVD). HTRA1 also plays an important role in bone metabolism; however, its association with abnormal bone formation remains unclear. A 51-year-old man with minimal vascular risk factors was hospitalized for acute ischemic stroke, and magnetic resonance imaging demonstrated scattered deep cerebral white matter lesions. Anthropometric measurements revealed short stature and limb length. A genetic analysis revealed a heterozygous missense variant (c.889G>A, p. V297M) in HTRA1. The patient was diagnosed with heterozygous HTRA1-related CSVD. Our case suggests that this HTRA1 variant may contribute to shorter limbs and height.
Mepolizumab, which selectively suppresses Interleukin-5, has been proven to be effective in treating eosinophilic granulomatosis with polyangiitis. However, the medium- to long-term outcomes of mepolizumab initiation in the early remission induction phase remain unclear. We investigated the outcomes up to 104 weeks in 10 patients in whom mepolizumab was initiated early in remission (mean, 14 days after glucocorticoid initiation). We found that the disease activity was promptly suppressed at 4 weeks and remained controlled for up to 104 weeks without relapse or adverse events. Glucocorticoids were discontinued at 104 weeks in 50% of patients, and prednisolone ≤4 mg/day was achieved in 70% of patients.
Signal transducer and activator of transcription-3 (STAT3) gain-of-function (GOF) syndrome predisposes patients to autoimmunity diseases. Yet, nontuberculous mycobacterial (NTM) infections have rarely been described. We herein report a teenage Japanese patient with genetically confirmed STAT3-GOF syndrome who developed Mycobacterium intracellulare disease. Combination antimycobacterial chemotherapy reduced the bacterial burden; however, the refractory NTM infection relapsed after initiation of adjunctive tofacitinib therapy. The present case demonstrates that host-directed therapy must be combined with prolonged antimycobacterial regimens to control drug-resistant NTM in patients with inborn errors of immunity.
A 75-year-old woman was hospitalized because of sudden impairment of mobility, fever, and abdominal tenderness. Abdominal ultrasonography and computed tomography revealed a tumorous mass in the left adnexa. Escherichia coli, and Bacteroides fragilis were detected in urine and blood cultures, respectively. Despite ceftriaxone therapy, the fever persisted. B. fragilis was detected in the uterine pus after separation of vulvar adhesions and uterine drainage. The symptoms persisted, despite changing to ampicillin-sulbactam. Citrobacter amalonaticus was detected in the abscess after resection of the left fallopian tube and intra-abdominal drainage. After changing to cefepime, there was no recurrence of the abscess. C. amalonaticus should be considered for patients with urinary tract abnormalities.