Objective To evaluate the effect of pemafibrate (PEM) on metabolic dysfunction-associated steatotic liver disease (MASLD).

Methods We retrospectively evaluated 43 patients with hyperlipidemia and MASLD to determine changes in clinical factors between the start of PEM treatment and 0.5 years later. Using FibroScan, 39 of 43 patients were evaluated for liver stiffness (LS; kPa) and controlled attenuation parameter (CAP; dB/m). None of the patients had decompensated cirrhosis.

Results Thirty patients were women, the median age was 66 years old, the median fibrosis-4 (FIB-4) score was 2.52, the median LS was 8.05 kPa, and the median CAP was 280.5 dB/m at the start of PEM treatment. AST, ALT, ALP, γGTP, and triglyceride levels decreased 0.5 years after starting PEM treatment, but FIB-4, LS, and CAP values did not decrease. However, LS decreased in patients with a FIB-4 index ≥1.3 at the start of PEM treatment, whereas it did not change in patients with a FIB-4 index <1.3. Similarly, LS decreased in patients with a value ≥8 kPa at the start of treatment and did not change in those with <8 kPa. The decreased LS group had higher baseline ALT and LS levels and lower ALT levels during 0.5 years of follow-up than the increased LS group.

Conclusion At the initiation of PEM treatment, the LS decreased in patients with MASLD complicated by hyperlipidemia and moderate LS (FIB-4>1.3 or LS >8 kPa). Although there is currently no approved treatment for MASLD, PEM may be a viable treatment option for MASLD with mild LS.

Objective Roxadustat, a hypoxia-inducible factor-prolyl hydroxylase inhibitor, increases the hemoglobin (Hb) levels in patients with chronic kidney disease (CKD). To date, limited clinical studies have focused on the excessive increase in the Hb levels in the early weeks after switching from erythropoiesis-stimulating agents (ESA) to roxadustat in adult non-dialysis patients. We conducted a retrospective study to examine whether early overshoot frequently occurs after switching to roxadustat.

Methods This 8-week retrospective pilot study examined patients with anemic, non-dialyzed CKD who switched from ESA (darbepoetin or epoetin beta pegol) to roxadustat or continued ESA. The Hb levels >12.5 g/dL after starting our observation was defined as Hb overshoot.

Patients: Twenty-three patients who switched to roxadustat (roxadustat group) and 63 who continued ESA (ESA group) were included.

Results The baseline median estimated glomerular filtration rate and mean Hb levels were 15.7 mL/min/1.73 m² and 10.77 g/dL in roxadustat group and 15.2 mL/min/1.73 m² and 10.64 g/dL in ESA group, respectively. Eight patients (34.8%) in the roxadustat group and two patients (3.2%) in the ESA group had Hb overshoot within the 8-week visit [odds ratio: 20.2 (95% confidence interval 3.13-130.0, p<0.01) in the background adjusted model]. Among the patients with Hb overshoot in the roxadustat group, the Hb levels were maintained close to baseline 4 weeks after roxadustat discontinuation. A younger age and higher baseline Hb and Hct levels were risk factors for Hb overshoot.

Conclusion Hb overshoot was frequently observed in patients switched to roxadustat. Clinicians should be aware of Hb overshoot and emphasize the importance of early Hb level checks.

Objective Short-term levodopa-carbidopa intestinal gel (LCIG) treatment using nasojejunal (NJ) tubes (NJ-LCIG test) is recommended for patients with advanced Parkinson's disease to ensure compatibility with this treatment system prior to permanent percutaneous endoscopic gastrojejunostomy. However, there have been no studies on NJ tube insertion by neurologists or on possible differences in treatment efficacy based on the NJ tube insertion method or tube tip position. We therefore investigated the effects of LCIG with NJ tube placement performed by a neurologist.

Methods This retrospective observational study included 13 patients with advanced Parkinson's disease and NJ tube placement between March 1, 2020, and October 31, 2023. A neurologist performed all NJ tube placements, and the daily off-time and dyskinesia time before and after NJ tube placement were compared. We also investigated the effects of differences in the NJ tube tip site.

Results NJ tubes were placed using either a combination of X-ray fluoroscopy-guided insertion and gastric motility methods (23.1%) or X-ray fluoroscopy-guided insertion alone (76.9%). All tubes were successfully placed in the descending duodenum (15.4%), ascending duodenum (23.1%), or jejunum (61.5%). The off-time decreased significantly after the NJ-LCIG test [pre-NJ-LCIG test, 6.6 h (5.1-8.1) vs. post-NJ-LCIG test, 2.0 h (0.8-3.5), p<0.01]. There was no difference in effectiveness based on the site of NJ tube tip placement.

Conclusion Our results suggest that neurologists can place NJ tubes and that the NJ-LCIG test can also improve off-time, regardless of the placement site.

A 78-year-old man was diagnosed with advanced poorly differentiated gastric adenocarcinoma, presenting with jaundice and diffuse thickening of the extrahepatic bile duct. No obstructive biliary sites or liver masses were observed. The serum concentrations of proteins induced by the absence of vitamin K or antagonist-II were markedly high. Samples of the extrahepatic bile duct and liver were obtained by endoscopic examination. The patient was diagnosed with lymphangiosis carcinomatosa of the liver and extrahepatic bile duct but died 28 days after hospitalization. As the disease progresses rapidly with uncharacteristic imaging findings, biopsy samples should be obtained early using several diagnostic tools.

Choroidal malignant melanoma is a rare malignant tumor that develops in adult eyeballs. It causes early lymph node and distant metastasis. Fluorodeoxyglucose (FDG)-positron emission tomography/computed tomography (CT) is widely used for screening malignant melanoma metastases. We encountered a 58-year-old man with choroidal malignant melanoma in whom liver metastasis recurred 12 years after surgery, without any observable FDG accumulation. Immunostaining revealed the absence of glucose transporter type 1 (GLUT-1) expression, crucial for intracellular FDG uptake. The lack of FDG accumulation in the lesion could be attributed to the diminished cellular FDG uptake due to the absence of GLUT-1 expression.

A 75-year-old woman visited to our hospital with liver dysfunction. The patient's liver function was normal. She had been treated with tocilizumab for rheumatoid arthritis for two years. One year after initiation of tocilizumab treatment, liver dysfunction was observed. Serum ceruloplasmin concentration was low. We diagnosed hepatic iron overload because of a high ferritin concentration and a liver biopsy. The cessation of tocilizumab and phlebotomy improved the liver function. We believe that tocilizumab induced iron accumulation. We should be aware of the possibility that tocilizumab induces iron overload in susceptible patients and monitor iron status in patients treated with tocilizumab.

A boy in his late teens had undergone proctocolectomy for refractory ulcerative colitis eight months ago. Two months earlier, he had developed pouchitis and received intensive treatment with infliximab (IFX). Two days after the IFX injection, a fever and generalized rash appeared. Varicella zoster virus (VZV) antigen positivity in the blisters led to a diagnosis of varicella. Based on the history of VZV vaccination, environmental conditions, and VZV antibody titers in the early stages of the disease, VZV reactivation was suggested. The possibility of varicella should be considered when blisters occur in immunosuppressed patients, although this is rare.

We herein present the case of a 30-year-old Japanese male patient with ulcerative colitis (UC) who was admitted to our hospital because of significant ascites. Upon evaluation, the patient was diagnosed with unresectable UC-associated colon cancer (UCAC), localized in the transverse colon. Using gene profiling of the tumor tissue, anti-epidermal growth factor receptor (EGFR) antibody combination chemotherapy was selected. Subsequently, the patient exhibited a temporary response to this regimen, with an enhancement in his quality of life and he was able to survive for 12 months. This case underscores the potential benefits of aggressive chemotherapy tailored to the gene profile in UCAC treatment, offering insights into potential avenues for improving the patient prognosis.

Primary neuroendocrine carcinoma (NEC) of the anal canal is a rare, highly malignant tumor with a poor prognosis. Despite the standard first-line treatment with etoposide or irinotecan combined with cisplatin, effective second-line therapies are lacking. In 2019, Japan approved cancer genome profiling (CGP) tests for solid tumors to enhance genomic understanding. We present the case of a 79-year-old woman with NEC of the anal canal, treated with etoposide, carboplatin, and amrubicin. As Post-standard therapy, CGP suggested pemigatinib, a tyrosine kinase inhibitor; however, the patient died before receiving it. This case highlights the potential of personalized medicine to improve outcomes in such cases.

A delayed diagnosis of polyarteritis nodosa may lead to critical limb-threatening ischemia (CLTI). A 74-year-old woman presented with left-foot pain and was treated with oral vasodilators and antiplatelet agents. However, the distal ischemia progressed to CLTI, including gangrene of the fingers and toes, and bilateral foot dropping appeared because of peroneal nerve paralysis. Angiography of the extremities revealed obstruction and stenosis of medium-sized arteries. Based on the progressive distal gangrene, mononeuropathy multiplex, and pathological findings of necrotic vasculitis, polyarteritis nodosa was diagnosed, and the patient's condition improved. A biopsy and neurological examination are essential for the appropriate diagnosis of PAN and immediate treatment.

A 48-year-old woman with a history of bronchial asthma and allergic rhinitis presented with cardiac arrest due to ventricular fibrillation and was resuscitated by defibrillation. Coronary angiography revealed coronary vasospasm. Various antispastic agents were unable to control recurrent coronary spasms. We searched for other points of intervention and found a significant increase in the eosinophil count. The patient received mepolizumab for bronchial asthma with eosinophilia, which normalized the eosinophil count. Since then, the patient has remained free from coronary vasospasm, suggesting that mepolizumab may be a viable option for treating refractory coronary angina associated with bronchial asthma and eosinophilia.

A 70-year-old man with diabetes was treated with a sodium glucose cotransporter 2 (SGLT2) inhibitor. He developed vomiting and epigastric pain and was diagnosed with diabetic ketoacidosis (DKA). Computed tomography (CT) revealed mediastinal emphysema. As Boerhaave syndrome could not be ruled out, treatment was initiated in parallel with DKA treatment. After the DKA healed, the mediastinal emphysema disappeared. DKA combined with mediastinal emphysema is known as Hamman syndrome. There have been no reports of Hamman syndrome in elderly patients with diabetes caused by SGLT2 inhibitors. His symptoms mimicked the course of Boerhaave syndrome, and such cases have a high risk of misdiagnosis.

A 47-year-old man who presented with scalp hair loss was transferred to our dialysis facility 3 months after hemodialysis initiation. He noticed systemic hair loss one month after the initiation of dialysis. Because the antipruritic drug nalfurafine was the only drug that had been newly added to his regular medication after he started hemodialysis, we stopped its prescription. His hair loss was completely ameliorated for the next five months. We speculated that κ-opioid receptor activation by nalfurafine caused blood capillary regression around the hair follicles, leading to cessation of hair growth and subsequent hair fallout.

Nephrotic syndrome (NS) predisposes patients to immunocompromised hosts owing to the loss of immunoglobulins, immunosuppressant use, and edema complications. In addition, aging impairs the immune system; thus, elderly individuals with NS are vulnerable to infection. Nocardiosis is not a common disease; however, once infected, it can disseminate hematogenously, causing serious health problems. An 88-year-old woman with amyloid light chain amyloidosis-induced NS was treated with prednisolone and tacrolimus and developed nocardiosis and invasive aspergillosis. Protecting the skin and wounds from direct exposure to nocardia is important. Physicians should consider the safe dose and treatment period of immunosuppressants in elderly patients with NS.

A 71-year-old woman developed nephrotic syndrome during 10-year follow-up for chronic lymphocytic leukemia. A renal biopsy sample analysis revealed IgG1-lambda-positive monoclonal immunotactoid glomerulopathy (mITG). The patient was treated with ibrutinib, a Bruton tyrosine kinase inhibitor, and complete renal remission was achieved after 24 months. ITG is a rare disease that is characterized by glomerular deposition. In particular, mITG, which presents immune deposits that exhibit light-chain restriction, is often associated with hematologic disorders. Most patients with mITG receive immunosuppressive therapy and/or chemotherapy; however, to our knowledge, there have been no reports of treatment with ibrutinib.

Acquired pure red cell aplasia (PRCA), caused by thymic hyperplasia, is extremely rare. We herein report a previously healthy 41-year-old man who presented with severe anemia, lymphadenopathy, an upper mediastinal mass, and hypogammaglobulinemia. The patient was eventually diagnosed with PRCA and adult T-cell leukemia/lymphoma (ATLL). The mediastinal mass was pathologically diagnosed as thymic hyperplasia without clear ATLL invasion. Although his anemia improved rapidly after thymectomy, PRCA recurred approximately 500 days later and was accompanied by ATLL exacerbation. The findings in this patient suggest that the Good's syndrome-like symptoms (thymic hyperplasia and hypogammaglobulinemia) in this patient and PRCA may have been paraneoplastic syndromes caused by ATLL.

A 65-year-old man with generalized lymphadenopathy was diagnosed with classical Hodgkin lymphoma-mixed cellularity via left cervical lymph node biopsy. Initial treatment with doxorubicin, bleomycin, vinblastine, and dacarbazine led to complete metabolic remission (CMR); however, recurrence developed after 6 months. Brentuximab vedotin induced partial remission followed by systemic relapse after 10 months. Nivolumab led to a second CMR, but disease progression persisted over nearly 4 years, despite treatment adjustments and local radiotherapy. Eventually, the patient was diagnosed with diffuse large B-cell lymphoma during routine esophagogastroduodenoscopy. Four courses of rituximab-CHOP therapy led to a CMR. This case highlights the importance of performing re-biopsies to detect the recurrence or progression of lymphoma.

We herein report a case of reversible cerebral vasoconstriction syndrome (RCVS) with an unusual presentation of hyperdense blood vessels. A 53-year-old woman developed thunderclap headache. Brain computed tomography (CT) showed hyperdensity of the anterior cerebral artery. Brain magnetic resonance imaging revealed cerebral infarctions in the left anterior cerebral artery (ACA) territory and cerebellum. The left ACA presented with a hyperintense vessel sign, although magnetic resonance angiography (MRA) appeared normal. One week later, stenotic changes were confirmed using MRA. The vasoconstriction disappeared on day 20, and the patient was diagnosed with RCVS. CT-defined hyperdense vessel signs can be observed at an early stage of RCVS, leading to ischemic events.

Cases of neuronopathy associated with immune checkpoint inhibitors (ICIs) have rarely been reported. We herein report a case of ICI-associated neuronopathy. A 54-year-old man underwent chemotherapy for right maxillary sinus cancer. Two months after pembrolizumab treatment, diarrhea, worsening of abnormal sensations, and severe ataxia of the lower limbs were observed. Somatosensory evoked potentials (SEPs) with tibial nerve stimulation showed disappearance of the N21 waveform. A colonic biopsy suggested ICI-associated colitis. Based on these findings, the patient was diagnosed with ICI-associated neuronopathy. Clinical symptoms and SEP findings improved markedly after two courses of intravenous methylprednisolone.

We present a 76-year-old man with cryptogenic new-onset refractory status epilepticus (C-NORSE) with an initial abnormal signal in the nucleus accumbens and a remarkable hyperintense signal on T1-weighted magnetic resonance imaging in the bilateral basal ganglia (BG). His status epilepticus did not respond to most anti-epileptic therapies or immunotherapies, and he died of sepsis. An autopsy revealed severe neuronal loss and hypertrophic astrocytes in the BG and limbic system, with no signs of inflammation or malignancy. This case suggests that lesions in the BG may reflect secondary degeneration and predict poor outcomes in C-NORSE.

A 73-year-old man who presented with nonspecific general symptoms and cognitive impairment was initially diagnosed with mild cognitive impairment due to dementia with Lewy bodies (DLB) based on a reduced blood flow in the parietal and occipital lobes on single-photon emission computed tomography (SPECT) imaging. However, the patient later presented with hyponatremia and hypoglycemia, leading to impaired consciousness, and was diagnosed with isolated adrenocorticotropic hormone deficiency (IAD). Hydrocortisone treatment improved the blood test scores and general symptoms, including cognitive impairment. IAD may show a DLB-like presentation on cerebral blood flow SPECT; therefore, caution is required for the correct diagnosis of IAD.

The interleukin (IL)-5 inhibitor mepolizumab is beneficial in eosinophilic granulomatosis with polyangiitis (EGPA), and the inhibition of antineutrophil cytoplasmic antibody (ANCA) production has been suggested as a possible mechanism. We herein report a 78-year-old Japanese man with EGPA who received solo mepolizumab 300 mg twice for elevated ANCA levels, which led to subsequent glucocorticoid (GC) discontinuation after achieving remission. The patient was able to be freed from the adverse events associated with long-term GC treatment, and the sole addition of mepolizumab also proved that mildly elevated ANCA could be converted to a negative result, thus leading to GC discontinuation.

We herein report three cases of microscopic polyangiitis (MPA). Two patients were administered avacopan in combination with glucocorticoid (GC), whereas one patient was treated with avacopan monotherapy; none of the patients were co-administered either rituximab or cyclophosphamide. The doses of GC were successfully reduced after the introduction of avacopan in the two patients, and the serum C-reactive protein levels decreased in the patient treated with avacopan monotherapy. Avacopan may therefore be effective either in combination with GC or as monotherapy, even for patients at a high risk of developing adverse effects when administered rituximab or cyclophosphamide.

We herein report an 81-year-old woman with no significant medical history who developed a fever, headache, and right eyelid swelling. Magnetic resonance imaging (MRI) showed eye proptosis, sphenoid opacity, enlarged cavernous sinus, and dilated right superior ophthalmic vein (SOV). Subsequent enhanced MRI revealed intraventricular debris and thrombosis in the right SOV and the left transverse and sigmoid sinuses. Blood cultures were positive for Aggregatibacter aphrophilus, as identified by mass spectrometry. The patient responded well to antibiotics, anticoagulants, and surgical drainage of sphenoid sinusitis. To our knowledge, this is the first case of A. aphrophilus sphenoid sinusitis causing orbital cellulitis, meningitis, and venous sinus thrombosis.