Objective Esophageal eosinophilia (EE), a histological hallmark of eosinophilic esophagitis, is classified into two endoscopic phenotypes: localized and diffuse EE. Our aim was to determine the prevalence of EE localized in the lower esophagus and to describe its clinical features in comparison with diffuse EE.
Methods Data from 81 consecutive patients with EE were retrospectively investigated. EE was histologically defined as ≥15 eosinophils per high-power field. Based on the endoscopic appearance with a histological assessment, EE was classified as either diffuse or localized type. We compared the clinical features, including the medical treatment and natural course, between the two types.
Results Of the 81 patients, 52 (64.2%) had diffuse EE, and 29 (35.8%) had localized EE. Among men patients, localized EE was significantly more common than diffuse EE. In localized EE, dysphagia and food impaction were less prevalent, and the presence of rings was significantly less common than in diffuse EE. Acid-suppressive therapy was administered to only 3 of the 29 patients with localized EE. In asymptomatic patients, especially those with localized EE, endoscopic abnormalities did not worsen but rather improved in some findings, such as with regard to furrows or exudate, during the natural course of three years without medical treatment.
Conclusion Localized EE has a strong predilection for men patients and accounted for more than one third of all cases of EE. This condition appears to be less symptomatic and necessitates milder medical treatment than diffuse EE and might not worsen progressively.
Objective Associations between aortic stiffness and cardiovascular disease events are mediated in part by pathways that include coronary microvascular dysfunction (CMD) and remodeling. However, the relationship between aortic stiffness and CMD remains unclear.
The present study aimed to determine whether aortic stiffness causes CMD as evaluated by the hyperemic microvascular resistance index (hMVRI) in patients with non-obstructive coronary artery disease (CAD).
Methods The intracoronary physiological variables in 209 coronary arteries were evaluated in 121 patients with non-obstructive CAD (fractional flow reserve >0.80) or reference vessels. The cardio-ankle vascular index (CAVI) as a measure of aortic stiffness and atherosclerotic risk factors were also measured.
Results Univariate analyses showed that hMVRI correlated with age (β=0.24, p=0.007), eicosapentaenoic acid (EPA; β=-0.18, p=0.048), EPA/arachidonic acid (AA) (EPA/AA) ratio (β=-0.22, p=0.014) and CAVI (β=0.30, p=0.001). A multivariate regression analysis identified CAVI (β=0.25, p=0.007) and EPA/AA ratio (β=-0.26, SE=0.211, p=0.003) as independent determinants of hMVRI.
Conclusion Aortic stiffness may cause CMD in patients with non-obstructive CAD via increased coronary microvascular resistance. Aortic stiffness is associated with CMD which is evaluated as hyperemic microvascular resistance in patients with non-obstructive CAD.
Objective The aim of the present study was to analyze the relationship between the patient characteristics and the timing of provision of an explanation about "Do Not Attempt Resuscitation (DNAR)" by attending physicians to advanced lung cancer patients.
Methods We conducted a retrospective analysis of patients with advanced or postoperative recurrent lung cancer in whom systemic therapy was initiated between 2015 and 2016.
Results The data of a total of 74 patients with lung cancer, including 59 patients with non-small cell lung cancer and 15 with small cell lung cancer were analyzed. The median overall survival of the patients was 10.0 months. Records of the explanation about DNAR by the physicians were available for 57 of the 74 (77.0%) patients. For 48 (64.9%) patients, the explanation was provided after the discontinuation of anticancer treatment, and for 9 (12.2%) patients, it was provided during the course of anticancer treatment. The provision of an explanation about DNAR during the course of treatment was associated with a poor performance status at the start of treatment (p=0.028), the tumor histology (p=0.037), the presence of driver gene mutation in the tumor (p=0.029), and shorter survival after the discontinuation of anticancer treatment (p<0.001).
Conclusion The results suggested that the timing of provision of an explanation about DNAR was associated with patient characteristics and the predicted prognosis.
We herein report two cases of autoimmune gastritis without complete atrophy of the corpus. Both were positive for anti-parietal cell antibodies. Endoscopic examinations indicated that atrophic changes were predominant in the lesser curvature of the corpus in both cases. In one, the greater curvature was covered with pseudopolyp-like nodules, whereas the greater curvature of the other showed multiple similar nodules and mildly atrophic mucosa. Histopathological examinations of these nodules showed focal and patchy atrophy and preserved fundic glands with parietal cell pseudohypertrophy. Follow-up endoscopy and a repeated biopsy demonstrated the development of gastric atrophy on the greater curvature in both cases.
A 38-year-old Japanese man who had been diagnosed with appendiceal carcinoid and undergone ileocecal resection 8 years before presented with duodenal obstruction caused by a submucosal tumor-like appearance. He was diagnosed with long-term recurrence of appendiceal goblet cell carcinoid (GCC) with a multi-morphological pattern based on the histological assessment of a duodenal biopsy and his previously resected appendix. He underwent subtotal stomach-preserving pancreaticoduodenectomy combined with resection of an ileo-colic anastomotic lesion. The GCC recurred at the nearby ileo-colic anastomosis and invaded the duodenum. This late recurrence might have resulted from the unique features of his GCC, which contained cells with different degrees of malignancy.
Granulocyte and monocyte adsorptive apheresis (GMA) is occasionally introduced as an alternative combination therapy after loss of response to biologics in ulcerative colitis (UC) patients. However, there have been no reports of the concomitant use of vedolizumab (VDZ) and GMA for the initial induction of UC. A 20-year-old man with refractory UC was admitted for recrudescence. VDZ monotherapy had previously been introduced but was ineffective. Therefore, he received scheduled combination of VDZ and GMA and achieved clinical remission. The combination of two different approaches to inhibit the migration of leukocytes into the inflamed tissue led to satisfactory clinical outcomes.
We herein report four patients with desquamative esophagitis that developed one to nine days after peripheral blood stem cell transplantation (PBSCT). Three patients underwent allogeneic PBSCT for leukemia, and the other underwent autologous PBSCT for pineoblastoma. Esophagogastroduodenoscopy revealed mucosal sloughing and fresh blood in the esophagus. Fasting and intravenous proton pump inhibitor therapy in addition to blood transfusion improved the esophageal lesions within five to seven days in three patients. These cases indicate that desquamative esophagitis can occur in patients who receive hematopoietic stem cell transplantation. Although blood transfusions may be required, it can be resolved within seven days.
A man in his 60s visited a clinic with chief complaints of a fever and general malaise. Suspecting a liver abscess in the left lobe with infiltration into the subcutaneous fat tissue under the rectus abdominis muscle based on computed tomography findings, we performed fine-needle aspiration. An amoebic liver abscess was diagnosed. Remission was achieved by the oral administration of metronidazole alone without placement of a drainage tube. The results obtained in this case suggest that the first line of treatment should be a non-invasive approach with oral administration alone. Invasive intervention should then be considered depending on subsequent progress.
Pancreatic neuroendocrine neoplasms occasionally have a cystic component. We herein report a case of multiple hepatic tumors, including a huge one and a 24-mm sized pancreatic cystic lesion. The hepatic tumor showed an enhancement pattern consistent with hepatic adenoma. The pancreatic cystic lesion revealed a thickened outside border and a solid inside component, which was enhanced following a contrast study, suggesting cystic pancreatic neuroendocrine neoplasm. Surgical resection was performed. After the surgery, somatostatin receptor scintigraphy detected an occult lumbar spine metastasis. Huge multiple liver and bone metastases of the neuroendocrine neoplasm G2 component were seen, with a G1 small primary lesion.
We herein report a rare case presenting with severe hypercholesterolemia, massive Achilles tendon xanthomas, and multi-vessel coronary artery disease. Initially, the patient was misdiagnosed with familial hypercholesterolemia. However, a genetic analysis using our custom sequencing panel covering genes associated with Mendelian lipid disorders revealed him to have a genetic basis of sitosterolemia with compound heterozygous mutations in the adenosine triphosphate binding cassette subfamily G5 (ABCG5) gene. A comprehensive genetic analysis can be particularly useful for diagnosing cases with severe phenotypes, leading to appropriate and medical therapies. Our patient was refractory to statins, whereas ezetimibe and PCSK9 inhibitor with a low-plant-sterol diet successfully reduced his serum levels of low-density lipoprotein cholesterol.
We treated a man with co-incident Marfan-like connective tissue disease with morphologic left ventricular non-compaction (LVNC). He underwent valve-sparing aortic root replacement because of aortic root dilation at 43 years old. Pathological findings of the aorta revealed cystic medio-necrosis, consistent with Marfan syndrome. He developed congestive heart failure caused by LVNC at 47 years old. His daughter had scoliosis, and he had several physical characteristics suggestive of Marfan syndrome. We herein report a rare case of a patient who had Marfan-like connective disease with an LVNC appearance.
A 76-year-old man developed repeated fulminant myocarditis in a short period, and immunosuppressive therapy was remarkably effective. A pathologic evaluation showed that inflammatory cells had infiltrated the myocardium. Not only invasion of inflammatory cells but also the formation of lymphoid follicle was noted. Chronic myocardial inflammation was proven, but cardiac sarcoidosis was negative according to the results of various examinations. This is the first report of recurrent autoimmune myocarditis with a lymphoid follicle in the myocardium. These findings may suggest a novel pathogenesis of myocarditis.
An 83-year-old woman with a 1-year history of scheduled intravitreal injection of vascular endothelial growth factor (VEGF) inhibitor (aflibercept) was diagnosed with nephrotic syndrome due to focal segmental glomerulosclerosis with histopathological findings of segmental infiltration of foam cells in the glomerular capillaries. Her nephrotic syndrome improved immediately following the termination of aflibercept intravitreal injection without steroid therapy. Although widely used to treat ophthalmic diseases, we should keep in mind that even intravitreal injection of VEGF inhibitor, as opposed to systemic administration, can cause kidney injury.
A 69-year-old man with stage III lung squamous cell carcinoma developed immune-related hepatitis following treatment with durvalumab, and was given high-dose corticosteroids and immunosuppressive drugs (mycophenolate mofetil, azathioprine, tacrolimus) but without demonstrating any improvement. Two cycles of infliximab (5 mg/kg) were then administered and thereafter the hepatitis improved. At the time of writing (9 months after the initiation of first course of durvalumab), the patient is alive without either any hepatitis symptoms nor any lung cancer progression.
Infliximab may be effective for treating non-small cell lung cancer (NSCLC) patients who develop immunosuppressive drug-resistant immune-related hepatitis caused by durvalumab.
An inguinal lymph node biopsy of a woman with a one-month history of a progressive fever, fatigue, dyspnea, skin rash, and lymphadenopathy revealed a well-preserved basic structure, hyperplastic germinal centers, and an interfollicular region containing polyclonal plasma cell sheets, suggesting plasma cell-type multicentric Castleman disease (MCD). We initiated prednisolone and anti-interleukin (IL)-6 antibody (tocilizumab), without success. A biopsy specimen re-evaluation detected CD20-positive atypical large B cells infiltrating the small vessels within and around the lymph node and its capsule. We diagnosed her with intravascular large B-cell lymphoma (IVLBCL). Lymphoma cells were weakly positive for IL-6 by immunohistochemical staining. IL-6 from lymphoma cells may have caused the MCD-like presentation as a paraneoplastic etiology. Malignant lymphoma should be excluded before diagnosing MCD.
Rotatory vertigo is known to have not only peripheral causes, e.g., Meniere's disease, vestibular neuritis, and benign paroxysmal positional vertigo, but also central causes, e.g., stroke, hemorrhage, and tumor. In most cases, central rotatory vertigo is caused by a lesion in the brainstem or cerebellum, but rare cases with a cerebral lesion have also been reported. We herin describe a unique case with acute rotatory vertigo following a small hemorrhage in the left superior temporal gyrus, which probably led to a dysfunction of the visual-vestibular system.
An 84-year-old man developed motor aphasia and right hemiparesis on postoperative day 1 after orchiectomy for suspected malignant lymphoma. He had a history of thoracic endovascular aortic repair for aortic aneurysm using a bypass graft from the right subclavian artery to the left common carotid artery (CCA); however, the graft had become occluded six months later. Brain magnetic resonance imaging revealed acute cerebral infarctions in the left frontal lobe. Carotid ultrasonography revealed a stump at the left CCA, just below the bifurcation, formed by the occluded graft with an oscillating thrombus. This case was rare in that a CCA stump was identified as the embolic source of ischemic stroke.
We herein report a 33-year-old woman who was an asymptomatic hepatitis B virus (HBV) carrier and presented with distal muscle weakness in the legs and asymmetrical paresthesia in the distal extremities. A nerve biopsy specimen revealed fibrinoid necrosis associated with inflammatory infiltration in the perineural space, and deposition of hepatitis B core antigen and C4d complement was detected in the vascular endothelial cells as well as around the vessels. She was diagnosed with HBV-related vasculitic neuropathy and treated with intravenous immunoglobulin (IVIG). Her symptoms completely subsided after eight weeks. Vasculitic neuropathy rarely develops in the chronic inactive stages of HBV infection. This is the first report of an HBV-inactive carrier with vasculitic neuropathy successfully treated with IVIG.
A 55-year-old woman with neuromyelitis optica (NMO) had recurrent myalgias with hyperCKemia. A muscle biopsy suggested nonspecific myopathic changes. Regarding immunohistochemistry, the expression of both major histocompatibility complex class I and myxovirus resistance protein A was observed in the endomysial capillaries, suggesting immunological involvement of these capillaries, whereas both C5b9 (membrane attack complex) and aquaporin 4 immunofluorescence stainings were normal. The present findings led us to conclude that one possible mechanism for hyperCKemia in NMO underlying the immunological involvement of the endomysial capillaries was an as-yet-unidentified factor that triggered damage to the integrity of the sarcolemma and thereby cause CK leakage into the serum.
The frequency and risk of embolism by Lambl's excrescences (LEs) remain unclear. We herein report an autopsy case of LEs that caused cardioembolic stroke. A 74-year-old man with colon cancer was hospitalized for ischemic stroke. His D-dimer levels were elevated. Thus, a diagnosis of ischemic stroke with Trousseau syndrome was made. At the autopsy, we found LEs in the aortic valves and thromboembolism of the brain blood vessels. This finding demonstrated that fibrin clots had adhered to the LEs because of coagulation abnormalities associated with Trousseau syndrome and became embolized. This case highlights the risk of LEs in patients with coagulation abnormalities.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare condition of systemic vasculitis of small to medium-sized blood vessels. We herein report the case of a 75-year-old man who presented with hemiplegia on his right side due to cerebral infarction following myalgia and a high fever. He had no history of asthma or allergic rhinitis. He was diagnosed with EGPA based on the presence of eosinophilia, sinusitis suggested by magnetic resonance imaging, and muscle pathology. His hemiplegia improved rapidly after corticosteroid therapy. This case suggests that EGPA should be a differential diagnosis of cerebral infarction with myalgia and eosinophilia.
This case concerned a 39-year-old woman diagnosed with uterine fibroids. Upon initiation of gonadotropin-releasing hormone (GnRH) agonist therapy, she experienced various neurological deficits but did not seek medical attention because of gradual spontaneous symptom improvement. Upon completing four courses of GnRH agonist therapy, she began experiencing severe neurological symptoms and was diagnosed with multiple sclerosis (MS). Although her symptoms improved with steroid pulse therapy, serious sequelae remained. GnRH agonist therapy can exacerbate the disease activity of MS; therefore, awareness of the potential emergence of neurological symptoms during GnRH agonist therapy is important.
A 40-year-old man with systemic lupus erythematosus taking consecutive oral corticosteroids developed a high-grade fever and disorder of consciousness following acute rhinitis. Haemophilus influenzae type f (Hif) was found and isolated from the blood and cerebrospinal fluid by culture, leading to a diagnosis of meningitis. The prevalence of H. influenzae type b (Hib) infections has decreased due to routine immunization. As a result, the prevalence of invasive non-Hib, including Hif infection, is increasing as a common H. influenzae infection in children and adults. Physicians should be aware of non-Hib H. influenzae infection, even though the Hib vaccine is widely used in Japan.