Objective Fungal sensitization is known to be prevalent in chronic obstructive pulmonary disease (COPD) and it is recognized as a risk factor for severe asthma. However, its relationship to the development of asthmatic features in COPD remains unclear. Because the diagnostic criteria for asthma-COPD overlap (ACO) are not standardized, we focused on the specific asthmatic features that form the diagnostic basis of ACO, namely, blood eosinophilia and recurrent wheezing, to clarify how fungal sensitization contributes to their manifestation in COPD.
Methods We retrospectively analyzed data from 72 patients with COPD who had specific immunoglobulin E (IgE) levels measured at the Tokyo Medical University Hospital between October 2019 and January 2024. The associations between fungal sensitization and clinical features, including the blood eosinophil count and recurrent wheezing, were analyzed.
Results Fungal sensitization was observed in 38% of patients. Patients with fungal sensitization had significantly higher blood eosinophil counts than those without fungal sensitization, whereas no significant differences were found for other aeroallergens. Moreover, fungal sensitization was independently associated with both an elevated blood eosinophil count [≥300 cells/mm3; odds ratio (OR) 3.51, 95% confidence interval (CI) 1.21-10.20] and recurrent wheezing (OR 6.93, 95% CI 1.86-25.80).
Conclusion Fungal sensitization was independently associated with an elevated blood eosinophil count and recurrent wheezing in patients with COPD. These findings suggest that fungal sensitization may play a biological role in the emergence of the asthmatic features that characterize the ACO phenotype in COPD.
A 67-year-old man with type 1 autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis experienced a relapse, while presenting with pancreatic head swelling and distal bile duct stenosis. Despite an increase in the prednisolone dosage (from 7.5 to 20 mg/day), the disease worsened. A significant dilatation of the main pancreatic duct (8 mm) and new stenosis of the pancreatic tail duct were observed. Malignancy was ruled out by repeated endoscopic ultrasound-guided fine-needle aspiration biopsies. A further escalation of the prednisolone dose to 40 mg/day led to clinical and radiological improvements. This case underscores the difficulty in distinguishing a steroid-resistant AIP relapse from pancreatic cancer, and the importance of ruling out malignancy before intensifying immunosuppressive therapy.
Pneumatosis cystoides intestinalis (PCI) is characterized by the presence of gas in the gastrointestinal wall. It is rarely observed in patients with pulmonary Mycobacterium avium complex (MAC) infection, although the exact mechanisms are unclear. We, herein, present a case of PCI potentially associated with long-term antibiotic therapy for pulmonary MAC infection. An 83-year-old woman developed PCI after 3 years of antibiotic therapy for a pulmonary MAC infection, with subsequent improvement following the discontinuation of antibiotics. Based on the clinical course, we suggest that clinicians consider the development of PCI, especially in patients receiving long-term antibiotic therapy for pulmonary MAC infection.
Sodium zirconium cyclosilicate hydrate (SZC) is a novel potassium binder that can be administered either orally or via nasogastric tubes. We report the case of a 77-year-old woman who developed a 37-mm gastric bezoar after nasogastric SZC administration during intensive care for acute adrenal insufficiency. Endoscopic removal was performed, and the analysis confirmed that the SZC was the primary constituent. Impaired gastric motility, acidic gastric environment, and limited hydration are likely contributors. This case highlights the potential for SZC-induced gastric bezoar formation in critically ill patients, and underscores the importance of imaging monitoring, careful gastrointestinal management, and the awareness of this potential complication during SZC therapy.
A 49-year-old man presented to our hospital with anorexia and vomiting. Computed tomography revealed duodenal obstruction, whereas non-contrast magnetic resonance imaging (MRI) revealed a subacute intramural hematoma. Combined with the patient's history of abdominal trauma, this led to the diagnosis of traumatic intramural duodenal hematoma. A double elementary diet (W-ED) tube was endoscopically placed for gastric decompression and enteral nutrition. The patient was successfully managed conservatively, without surgery or central venous nutrition. Oral intake resumed after 1 month, and complete resolution was confirmed at 3 months. This rare adult case of traumatic intramural duodenal hematoma highlights the diagnostic utility of MRI and clinical potential of the W-ED tube.
Cardiac amyloidosis is characterized by the deposition of amyloid fibrils, which cause complications such as mitral regurgitation (MR). The efficacy of transcatheter mitral edge-to-edge mitral repair (TEER) in patients with amyloidosis remains uncertain. We herein present four cases of TEER for transthyretin cardiac amyloidosis. TEER reduced the MR in all cases. However, two of the four individuals died within one year, and in one case, a recurrent MR deterioration occurred. Although TEER can reduce MR in individuals with cardiac amyloidosis, the long-term outcomes appear to be poor owing to disease progression or comorbidities, thereby underscoring the need for careful patient selection.
A 53-year-old man with Eisenmenger syndrome (ES), caused by a large secundum atrial septal defect (ASD) and severe pulmonary arterial hypertension (PAH), showed a marked hemodynamic improvement following an escalation of PAH-specific therapy. Surgical ASD closure using a fenestrated patch was successfully performed after a significant reduction in both the patient's pulmonary pressure and vascular resistance. This case highlights the potential utility of the "treat-and-repair" strategy in patients with ES and severe PAH, who exhibit a favorable response to medical therapy, resulting in both a symptomatic and hemodynamic improvement.
Drug-induced hypercholesterolemia is well known to cause side effects, such as secondary hypercholesterolemia. Many of these drugs are essential for long-term use and are difficult to replace; therefore, they are continued even when associated with hypercholesterolemia. Mesalazine is the first-line drug used for treating ulcerative colitis. We herein report a case of severe hypercholesterolemia following mesalazine administration in a patient with ulcerative colitis. In this case, a 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase inhibitor was effective in dramatically improving severe hypercholesterolemia. Clinicians should therefore be aware of the possibility of severe hypercholesterolemia caused by mesalazine for educational purposes.
We herein report a case of successfully treated synchronous advanced cancers: programmed death-ligand1 (PD-L1)-negative squamous cell carcinoma of the left upper lung lobe and adenocarcinoma of the ascending colon. As lung cancer was prioritized as the primary therapeutic target, the patient received a combination of anti-PD-L1 and anti-cytotoxic T-lymphocyte-associated protein-4 (CTLA-4) antibodies, along with chemotherapy (the POSEIDON regimen). This approach proved effective against both lung and colon cancer. Subsequent testing revealed high-frequency microsatellite instability (MSI-H) in the colon cancer. These findings highlight the therapeutic potential of combined anti-PD-L1 and anti-CTLA-4 antibodies in the treatment of PD-L1-negative lung and MSI-H colorectal cancers.
Occasionally, sarcoidosis can progress to fibrotic lung disease. Pleuroparenchymal fibroelastosis (PPFE) is a rare fibrotic pattern characterized by upper lobe-predominant pleural and subpleural fibroelastoses. However, pathologically confirmed cases of PPFE in sarcoidosis patients have not been reported. We herein describe the case of a 59-year-old woman with long-standing pulmonary sarcoidosis who developed progressive upper lobe-predominant fibrosis and cystic changes, culminating in living-donor lung transplantation. Histopathological examination of the explanted lungs revealed pleural thickening and subpleural intra-alveolar fibroelastosis, consistent with PPFE in the absence of typical granulomatous lesions of sarcoidosis. This case provides the first pathological evidence that PPFE represents a fibrotic manifestation of end-stage pulmonary sarcoidosis.
Epidermal growth factor receptor (EGFR) mutations are uncommon in lung squamous cell carcinoma (SQC). However, the role of third-generation EGFR-tyrosine kinase inhibitors (TKIs) remains unclear. The present study provides a case description and a systematic literature review of 34 eligible cases. Third-generation EGFR-TKIs demonstrate superior initial responsiveness compared to earlier-generation TKIs. The efficacy of TKIs was consistent across SQC forms (including de novo or adenocarcinoma-transformed cases) and EGFR mutation types. The findings indicate that, compared with early-generation TKIs, third-generation TKIs are effective against de novo SQCs and should be considered a plausible treatment option for transformed SQCs.
A 19-year-old woman with acute myeloid leukemia who had received a subcutaneous implantable cardioverter defibrillator (SICD) was scheduled to undergo allogeneic hematopoietic stem cell transplantation (HSCT). The conditioning regimen included total body irradiation (TBI) at a dose of 12 Gy, under which the SICD was considered at high risk for malfunction. We planned to deactivate and reactivate the SICD before and after each TBI session to mitigate the risk of device malfunction. Additionally, SICD shielding measures were implemented during TBI to minimize the risk of malfunction. Through these procedures, HSCT could be performed without significant adverse events linked to the SICD.
A 58-year-old man was diagnosed with acute myeloid leukemia with myelodysplasia-related changes. Induction therapy with CPX-351 achieved complete remission, and a second course of consolidation therapy was administered. Shortly after the second course, he developed aphthous ulcers on the tongue that progressively worsened. Although a tongue biopsy showed no specific findings, serum anti-bullous pemphigoid 180 antibodies were positive. Mucous membrane pemphigoid (MMP) was diagnosed, and corticosteroid therapy led to a rapid improvement. Given the temporal relationship between CPX-351 administration and the MMP onset, CPX-351 was considered to be the likely causative agent.
Deep cerebral venous thrombosis (DCVT) is an uncommon cerebrovascular disease with diverse neurological deficits and a poor prognosis. Therefore, the early diagnosis and treatment of DCVT are needed. We herein present a case of DCVT with edematous lesions in the bilateral thalami with Korsakoff syndrome (KS) as the initial symptom. Although KS mainly occurs in patients with thiamine deficiency, it can also result from cerebrovascular disease. Because thalamic dysfunction may be associated with KS, DCVT with thalamic lesions should be considered a cause of KS to facilitate its early diagnosis and treatment.
The acute immune-mediated disorder, Bickerstaff brainstem encephalitis (BBE), is characterized by consciousness disturbance, external ophthalmoplegia, and ataxia. BBE is usually readily diagnosed and patients recover well, but rare features may sometimes obscure the diagnosis, and some patients develop prolonged weakness. We herein describe a Japanese patient with BBE who presented with uncharacteristic features of a decorticate-like posture, involuntary movements, and impaired visual acuity, entered a coma requiring intubation, and later required orthosis-assisted ambulation for 10 months. Despite these uncharacteristic features, the typical triad and other BBE characteristic features can be valuable for making an early diagnosis. BBE with a coma or intubation may therefore occasionally lead to prolonged weakness.
A 30-year-old woman with history of bipolar disorder presented to the emergency department with impaired consciousness and myoclonus following regular lithium treatment. Brain magnetic resonance imaging revealed a hyperintense lesion in the splenium of the corpus callosum, consistent with cytotoxic lesions of the corpus callosum (CLOCCs). Serum lithium levels were markedly elevated (3.54 mmol/L), and acute-on-chronic lithium toxicity was diagnosed. Hemodialysis and supportive care resulted in clinical and radiological recovery. This case highlights that CLOCCs may serve as important neuroimaging evidence of lithium toxicity and may support an early diagnosis and treatment, especially in patients with long-term lithium use.
A 42-year-old woman with positive anti-citrullinated protein antibody (ACPA) and rheumatoid factor (RF) levels exhibited arthralgia, but she did not fulfill the criteria for rheumatoid arthritis (RA). Subsequently, the patient exhibited episodic monoarthritis with a remarkable response to colchicine. An analysis of the MEFV gene identified heterozygous variants, L110P and E148Q, thus indicating a colchicine-responsive autoinflammatory condition. The patient was later diagnosed with RA and achieved remission with abatacept and colchicine. This case illustrates that ACPA-positive arthralgia evolves into RA through an autoinflammatory mechanism, thereby underscoring the diagnostic difficulties arising from the convergence of both autoimmune and autoinflammatory characteristics.
We herein describe the case of a 20-year-old Japanese woman with adult-onset Still's disease (AOSD) complicated by macrophage activation syndrome (MAS). Despite the administration of prednisolone and tocilizumab, MAS developed with hyperferritinemia, thrombocytopenia, hypertriglyceridemia, and liver dysfunction. Steroid pulse therapy, cyclosporine A, and plasma exchange (PE) improved the patient's condition. However, diabetes hinders glycemic control under high-dose steroids. Subcutaneous canakinumab (300 mg/month) was initiated, thus enabling rapid prednisolone tapering to 10 mg within one month without relapse. This case highlights that PE followed by canakinumab is a potentially effective strategy for treating severe AOSD with MAS, particularly when minimizing steroid exposure is critical.