Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
Volume 57 , Issue 18
Showing 1-29 articles out of 29 articles from the selected issue
EDITORIAL
REVIEW ARTICLE
  • Masayuki Amano, Taro Shimizu
    2018 Volume 57 Issue 18 Pages 2607-2612
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: May 18, 2018
    JOURNALS OPEN ACCESS

    Mondor's disease (MD) is a rare disease that manifests with a palpable cord-like induration on the body surface. In general, MD is a self-limited, benign thrombophlebitis that resolves in four to eight weeks without any specific treatment. Cases of MD can be roughly categorized into three different groups based on the site of the lesion as follows: original MD of the anterolateral thoracoabdominal wall, penile MD with dorsum and dorsolateral aspects of the penis, and axillary web syndrome with mid-upper arm after axillary surgery. The diagnosis of MD is rather straightforward and based on a physical examinations. However, some case occur "secondary" with another underlying disease, including malignancy, a hypercoagulative state, and vasculitis. Therefore, it is critical to identify MD precisely, evaluate any possible underlying disease, and avoid any unnecessary invasive tests or treatment. In this paper, we comprehensively review the clinical characteristics of MD.

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ORIGINAL ARTICLES
  • Kenichiro Majima, Yosuke Muraki, Takeshi Shimamoto
    2018 Volume 57 Issue 18 Pages 2613-2619
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    Objective Multiple white and flat elevated lesions (MWFLs) observed in the stomach have only been presented in abstracts at academic conferences over the last decade; therefore, relatively little is known about these lesions. Our aim was to prospectively clarify the clinical characteristics of MWFLs, to identify their risk factors and to retrospectively evaluate the clinical progression of these lesions.

    Methods A prospective analysis of clinical characteristics and risk factors was conducted in participants who underwent esophagogastroduodenoscopic screening at our hospital. A retrospective analysis of the medical chart of patients identified as having MWFLs was conducted to describe the clinical progression of these lesions.

    Results The prevalence rate of MWFLs was 10.4% (80/767), with the following risk factors identified on a logistic regression analysis: use of proton pump inhibitors [odds ratio (OR), 3.51; 95% confidence interval (CI), 1.92-6.43], female sex (OR, 1.92; 95% CI, 1.19-3.12) and a 1-year increase in age (OR, 1.05; 95% CI, 1.02-1.08). Among the 70 cases with MWFLs observed over a mean duration of 2.3 years, no progression of MWFLs was detected in 67 cases (96%). Among the 3 remaining cases, progression was mild, with none of the lesions progressing to malignancy.

    Conclusion The use of proton pump inhibitors (PPIs), female sex, and age are risk factors for MWFLs. We believe that endoscopists should recognize these lesions.

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  • Satoru Suzuki, Seigo Sugiyama
    2018 Volume 57 Issue 18 Pages 2621-2630
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    Objective B-type natriuretic peptide (BNP) and N-terminal pro-B-type natriuretic peptide (NT-proBNP) should be secreted from cardiomyocytes in response to increased myocardial wall stress in a molar ratio of 1.00; however, the calculated molar blood levels of NT-proBNP are often greater than those of BNP in routine clinical practice. The purpose of this study was to investigate the hypothesis that the molar ratio of NT-proBNP/BNP provides useful clinical information in stable outpatients with cardiovascular risk factors.

    Methods We measured both the BNP and NT-proBNP levels simultaneously in 551 consecutive, stable outpatients with at least one cardiovascular risk factor and then calculated the molar ratio of NT-proBNP/BNP. All patients were prospectively followed-up for the occurrence of heart failure (HF)-related events.

    Results Of those patients, 38 patients had an HF-related event. A multivariate Cox hazards analysis showed that the log (molar ratio of NT-proBNP/BNP) was an independent predictor of future HF-related events (p=0.039). A Kaplan-Meier analysis showed a significantly higher probability of HF-related events in patients with a higher molar ratio of NT-proBNP/BNP (≥1.70) (p<0.001). The area under the curve (AUC) of the receiver operating characteristic curve (ROC) for the molar ratio of NT-proBNP/BNP to predict HF-related events was 0.75 (p<0.001). The AUC of the ROC curve analysis with the molar ratio of NT-proBNP/BNP for the prediction of HF-related events was not significantly greater than that of BNP or NT-proBNP.

    Conclusion The molar ratio of NT-proBNP/BNP may be a significant prognostic factor for HF-related events.

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  • Katsuhiko Ogawa, Yutaka Suzuki, Takayoshi Akimoto, Keiji Shiobara, Mak ...
    2018 Volume 57 Issue 18 Pages 2631-2639
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    Objective An abnormal high intensity area (HIA) on diffusion-weighted imaging (DWI) indicates the presence of cytotoxic edema and has been reported to be observed in the hippocampus of patients with transient global amnesia (TGA). The appearance of an HIA on DWI is usually delayed after the onset of patients with amnesia in TGA; thus, the significance of the HIA was evaluated in patients with TGA.

    Methods Three adult TGA patients who had a unilateral HIA on DWI (right, n=2; left, n=1) were enrolled. These patients were hospitalized due to acute-onset amnesia. Amnesia subsided within 24 hours of hospitalization in all three patients.

    Results The HIA was confined to the upper lateral zone of the body in the unilateral hippocampus where the CA1 region exists. The lesions were confirmed after the improvement of amnesia in the three patients. The location of the lesions corresponded to the watershed area where the upper and lower hippocampal arteries were anastomosed.

    Conclusion Cytotoxicity caused by glutamate-mediated calcium influx in the neurons of the CA1 region was recently reported in the pathogenesis of TGA. Based on the pathogenesis, the cytotoxicity was considered to have been caused by calcium overload throughout the entire CA1 region, and amnesia occurred due to this cytotoxicity. The cytotoxicity was more marked in the lesions because of the lower blood flow in the watershed area and was prolonged after the function of the CA1 region (excluding the watershed area) improved, which led to cytotoxic edema in the lesions.

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  • Tetsuro Konishi
    2018 Volume 57 Issue 18 Pages 2641-2645
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: May 18, 2018
    JOURNALS OPEN ACCESS

    Objective The aim of this study was to clarify the clinical conditions related to the depressive mental states in Japanese patients with subacute myelo-optico-neuropathy (SMON), caused by clioquinol intoxication more than 40 years previously.

    Methods The changes in the mental states with aging were investigated in 25 Japanese SMON patients (mean age: 77.2 years old, range: 53-90) using a Japanese version of the Zung Self-rating Depression Scale (J-SDS) questionnaires with supportive interviews by the clinical psychotherapist and medical checkup records. These mental and medical examinations were repeated more than twice within 2 to 11 years' interval. The J-SDS questionnaires were also examined in 25 age-matched non-SMON elderly people.

    Results The total J-SDS scores of most of the SMON patients decreased with age without significant changes in the mean Barthel index scores during this study period. The mean J-SDS scores at the first and latest studies were significantly higher than in the age-matched healthy elderly people. The total J-SDS scores of the latest study were significantly correlated with the degree of physical disability, such as the inverse total Barthel index scores, severity of SMON or gait disturbance, but not with the age.

    Conclusion The total J-SDS scores of most of the SMON patients tended to decrease with age. Repeating mental supportive interviews and medical examinations by experts helped to improve the depressive mental state and revealed close relationship between the mental state and the physical disabilities of the SMON patients.

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  • Kazunori Sato, Masaaki Niino, Atsushi Kawashima, Moemi Yamada, Yusei M ...
    2018 Volume 57 Issue 18 Pages 2647-2655
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    Objective In Japan, following the launch of dimethyl fumarate (DMF) after fingolimod as a disease-modifying drug in multiple sclerosis (MS), some patients switched from fingolimod to DMF. The aim of this study was to determine the follow-up status of MS patients who switched to DMF after fingolimod cessation.

    Methods Clinical and magnetic resonance imaging (MRI) data in 19 patients with MS who switched to DMF were collected for at least for 6 months after fingolimod cessation.

    Results Ten patients (52.6%) experienced clinical or MRI exacerbation after fingolimod cessation. The peripheral blood lymphocyte counts at the time of fingolimod cessation in those with disease exacerbation were significantly lower than in those without exacerbation. The patients with disease exacerbation were further classified into three groups based on MRI findings: those with some new T2-weighted lesions with or without gadolinium (Gd) enhancement (group I), those with more new and/or enlarged T2-weighted lesions with Gd enhancement compared to pre-fingolimod induction (group II), and those with multifocal tumefactive demyelinating lesions. In group II, the clinical disease activity, which was similar to that at fingolimod initiation in group I, was higher than the clinical disease activity observed before fingolimod initiation. Conversely, group III exhibited unexpected new MRI findings that were not evident before fingolimod initiation.

    Conclusion Cessation of fingolimod might precipitate rebound or reactivation of clinical disease in patients with MS, and careful follow-up is necessary for patients who discontinue fingolimod.

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CASE REPORTS
  • Shinya Kawaguchi, Masataka Kikuyama, Tatsunori Satoh, Shuzo Terada
    2018 Volume 57 Issue 18 Pages 2657-2662
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    Five patients complaining of severe pain due to severe post-endoscopic retrograde cholangiopancreatography pancreatitis (PEP) underwent nasopancreatic drainage (NPD) placement. Pain relief was achieved on the second, fourth, and fifth day in three, one, and one patients, respectively. Four patients underwent pancreatic juice culture; all were positive. Our results suggest that NPD can relieve severe PEP with severe pain. Bacteria-induced protease-activated receptor-2 activation may be associated with PEP.

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  • Masanari Sekine, Hiroyuki Miyatani, Keita Matsumoto, Hitomi Kashima, Y ...
    2018 Volume 57 Issue 18 Pages 2663-2668
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: May 18, 2018
    JOURNALS OPEN ACCESS

    The patient was a "73" -year-old woman who visited our hospital with the chief complaint of weight loss. Upper gastrointestinal endoscopy revealed an enlarged ampulla of Vater, and a biopsy led to a diagnosis of Group "4" gastric carcinoma; suspicious of adenocarcinoma. There were no findings suggesting invasion into the muscle layer of duodenum, despite tumor mass formation being observed in the sphincter of Oddi. We performed endoscopic papillectomy for both diagnostic and therapeutic purposes. Pathologically, a well-differentiated adenocarcinoma existed in the superficial layer of the mucous membrane of the papilla of Vater, and gangliocytic paraganglioma was present in the deep portion. The resected margins of both lesions were negative.

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  • Kotaro Matsumoto, Kentaro Kikuchi, Yusuke Kajiyama, Yuichi Takano, Mas ...
    2018 Volume 57 Issue 18 Pages 2669-2673
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    An 81-year-old woman developed liver dysfunction after two months' treatment with direct-acting antivirals (DAAs) for chronic hepatitis C virus (HCV) infection. She was positive for serum anti-nuclear antibody, with an elevated immunoglobulin G level. A liver biopsy revealed high-grade interface hepatitis and infiltrate of lymphocytes and plasma cells. DAA-associated drug-induced autoimmune hepatitis (DI-AIH) was considered. Her liver dysfunction improved after discontinuing DAA therapy and starting prednisolone treatment. The differential diagnosis for AIH should include liver injury during DAA therapy for chronic HCV infection.

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  • Takafumi Nakayama, Shunsuke Murai, Nobuyuki Ohte
    2018 Volume 57 Issue 18 Pages 2675-2679
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare type of systemic vasculitis. Cardiac involvement is the main cause of death in patients with this disease. We herein report a case of congestive heart failure in a patient with EGPA. Neither 67Ga scintigraphy nor cardiac magnetic resonance imaging detected inflammation of the myocardium; however, myocardial biopsy revealed numerous infiltrating inflammatory cells, thereby fulfilling the criteria of inflammatory dilated cardiomyopathy. We improved the left ventricular systolic function by increasing the patient's prednisolone dosage. This case shows that in some cases the detection myocardial inflammation - which allows for appropriate therapy - may only be achieved by myocardial biopsy.

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  • Yoshihiro Tanaka, Hayato Tada, Yoshimichi Takeda, Kenji Iino, Kenshi H ...
    2018 Volume 57 Issue 18 Pages 2681-2684
    Published: September 15, 2018
    Released: September 15, 2018
    JOURNALS OPEN ACCESS

    A 52-year-old man with a history of hypertension was referred to our hospital due to persistent abdominal pain. Abdominal palpation revealed remarkable rigidity and rebound tenderness all over the abdomen. Enhanced computed tomography demonstrated the superior mesenteric artery (SMA) dissection with a complete obstruction at the middle part of the SMA. Intraoperative findings showed significant necrosis in the most small intestine and surgical resection was performed. Emergent operation is warranted once abdominal pain becomes uncontrollable or intestinal necrosis is suspected. Physicians should pay careful attention to patients' symptoms and repeatedly perfume physical examinations.

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  • Tomoe Kinoshita, Hideaki Kaneto, Fumiko Kawasaki, Takatoshi Anno, Take ...
    2018 Volume 57 Issue 18 Pages 2685-2688
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    Fulminant type 1 diabetes mellitus (T1DM) is idiopathic T1DM with the rapid destruction of pancreatic β-cells. We herein report a 48-year-old man who developed fulminant T1DM complicated with a life-threatening electrolyte abnormality and abnormal electrocardiogram findings. He had no remarkable medical history, but one day, he developed general fatigue. His blood glucose level and HbA1c were 806 mg/dL and 6.3%, and his insulin secretion was markedly suppressed. He had ketoacidosis, hyponatremia and hyperkalemia. Furthermore, a life-threatening abnormality was noted on electrocardiogram. After fluid infusion and insulin therapy, the abnormality disappeared. In conclusion, we should bear in mind the possibility of fulminant T1DM in patients complaining of general malaise.

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  • Ikki Sakuma, Jun Saito, Yoko Matsuzawa, Masao Omura, Seiji Matsui, Tet ...
    2018 Volume 57 Issue 18 Pages 2689-2694
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
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    A 33-year-old man was admitted to our hospital to undergo an evaluation to determine the cause of secondary hypertension. Computerized tomography angiography (CTA) showed bilateral multiple renal arteries with significant stenosis of the right extra-renal artery due to fibromuscular dysplasia and segmental impairment of renal perfusion. Although the plasma aldosterone concentration and plasma renin activity were within the normal ranges, percutaneous balloon dilatation of the stenotic lesion resolved his hypertension, leading to a diagnosis of renovascular hypertension caused by segmental renal ischemia due to extra-renal artery stenosis. CTA should be considered during the examination of patients with early-age hypertension, even if the plasma renin activity is not sufficiently elevated.

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  • Naoya Nagaya, Mayuko Kanayama, Masayoshi Nagata, Shigeo Horie
    2018 Volume 57 Issue 18 Pages 2695-2700
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    Circulating tumor cells (CTCs) are a promising biomarker for several cancers. We streamlined the experimental procedure of CTC immunofluorescent staining. We encountered a 72-year-old woman with metastatic right renal cell carcinoma (RCC) (clinical stage: T4N0M1), whose CTC number rapidly increased after the administration of sunitinib and then gradually decreased. The change in the CTC number appeared to coincide with laboratory data and hypertension, suggesting that a CTC analysis may be useful for promptly monitoring the treatment response. Our data provided the first evidence of an association between the CTC numbers and the treatment response in a metastatic RCC patient.

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  • Eun Jin Kim
    2018 Volume 57 Issue 18 Pages 2701-2704
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
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    The author reports the case of a patient with a tuberculosis-associated endobronchial inflammatory polyp. Acid-fast bacillus (AFB) staining and culturing of sputum and bronchial washing fluid specimens were negative on three occasions. Biopsy results twice showed chronic inflammation. The patient was finally diagnosed with Mycobacterium tuberculosis based on a polymerase chain reaction (PCR) of a biopsy tissue specimen, along with the finding of chronic granulomatous inflammation. The author herein reports a rare case of a tuberculosis-associated endobronchial inflammatory polyp that was AFB smear- and culture-negative and the patient's clinical course after treatment.

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  • Noriho Sakamoto, Shota Nakashima, Hiroshi Ishimoto, Tomoyuki Kakugawa, ...
    2018 Volume 57 Issue 18 Pages 2705-2708
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    A 65-year-old Japanese man was referred to our hospital for the further assessment of cough and dyspnea. He had a history of ulcerative colitis for which he was receiving treatment. Chest computed tomography showed a crazy-paving pattern. His bronchoalveolar lavage fluid had a milky appearance, and a transbronchial lung biopsy specimen revealed acellular periodic acid-Schiff stain-positive bodies. The serum anti-granulocyte macrophage-colony stimulating factor (GM-CSF) antibody titer was elevated. The diagnosis was autoimmune pulmonary alveolar proteinosis (PAP). There are few reports of autoimmune PAP in patients with ulcerative colitis. Some reports suggest that PAP and inflammatory bowel disease might have a common pathogenesis involving the anti-GM-CSF antibody.

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  • Minami Okayama, Yoshihiro Kanemitsu, Tetsuya Oguri, Takamitsu Asano, S ...
    2018 Volume 57 Issue 18 Pages 2709-2712
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: May 18, 2018
    JOURNALS OPEN ACCESS

    A 36-year old man was referred to our hospital due to isolated chronic cough that was refractory to anti-asthma medications, including inhaled corticosteroids/long-acting β2 agonists. Chest X-ray showed diffuse nodular and enhanced vascular shadows with Kerley lines in both lungs. A blood analysis showed elevated serum carcinoembryonic antigen (CEA) and CA19-9 levels. A transbronchial biopsy revealed well to moderately differentiated adenocarcinoma, the origin of which was immunohistochemically suspected to be the gastrointestinal tract. Colonoscopy confirmed the diagnosis of primary rectum carcinoma. Pulmonary lymphangitic carcinomatosis was therefore regarded as the origin of the cough. Lymphangitic carcinomatosis is an uncommon diagnosis but important to consider in patients with persistent cough.

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  • Maki Watanabe, Ken Ohyama, Masashi Suzuki, Yasunobu Nosaki, Takashi Ha ...
    2018 Volume 57 Issue 18 Pages 2713-2718
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    Aceruloplasminemia is an autosomal recessive inherited disorder caused by ceruloplasmin gene mutations. The loss of ferroxidase activity of ceruloplasmin due to gene mutations causes a disturbance in cellular iron transport. We herein describe a patient with aceruloplasminemia, who presented with diabetes mellitus that was treated by insulin injections, liver hemosiderosis treated by phlebotomy therapy, and neurological impairment. A genetic analysis of the ceruloplasmin gene revealed novel compound heterozygous mutations of c.1286_1290insTATAC in exon 7 and c.2185delC in exon 12. This abnormal compound heterozygote had typical clinical features similar to those observed in aceruloplasminemia patients with other gene mutations.

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  • Takuya Fukuoka, Yoshihiko Nakazato, Masaomi Yamamoto, Akifumi Miyake, ...
    2018 Volume 57 Issue 18 Pages 2719-2722
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    We report a rare case of fatal familial insomnia in a 58-year-old man who initially developed parkinsonism, secondary dementia, and visual hallucinations that were suspected to be due to dementia with Lewy bodies. We evaluated the function of the striatum via dopamine transporter single-photon emission computed tomography (DAT SPECT) using 123I-ioflupane and found marked presynaptic dopamine dysfunction in the bilateral striatum. This is the first reported case in which the initial symptom of fatal familial insomnia was parkinsonism and in which the dopamine transporter function was evaluated by DAT SPECT.

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  • Jun Ueda, Hajime Yoshimura, Nobuo Kohara
    2018 Volume 57 Issue 18 Pages 2723-2726
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    Chronic inflammatory demyelinating polyradiculoneuropathy is a relapsing-remitting or chronic progressive demyelinating polyradiculoneuropathy. We report the case of a patient with chronic inflammatory demyelinating polyradiculoneuropathy who experienced relapses on four occasions after experiencing pyrexia and flu-like symptoms. Our patient showed characteristic features, such as relapse after pyrexia and flu-like symptoms, remission after pyretolysis without treatment, and the absence of remarkable improvement in a nerve conduction study in the remission phase. The serum level of tumor necrosis factor-α was elevated in the relapse phase and reduced in the remission phase; thus, the induction of cytokine release by viral infection might have caused the relapses.

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  • Junko Ishii, Yukiko Shishido-Hara, Michi Kawamoto, Satoru Fujiwara, Yu ...
    2018 Volume 57 Issue 18 Pages 2727-2734
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    A 37-year-old woman with systemic lupus erythematosus presented with gait disturbance and cognitive dysfunction. Brain magnetic resonance imaging (MRI) revealed small, punctate, T2-/fluid-attenuated inversion recovery-hyperintense and T1-hypointense lesions without gadolinium enhancement, which is atypical for progressive multifocal leukoencephalopathy (PML). On a pathological examination of biopsied brain tissues, JC virus-infected cells were hardly detected via immunohistochemistry but were certainly detected via in situ hybridization, conclusively verifying the PML diagnosis. After tapering off the immunosuppressant and mefloquine administration, the MRI findings revealed gradual improvement, and she has been stable for over 18 months. A punctate MRI pattern is not specific to natalizumab-associated PML but may be a ubiquitous early sign useful for the early diagnosis of PML.

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  • Masahiro Oomura, Yuto Uchida, Keita Sakurai, Takanari Toyoda, Kenji Ok ...
    2018 Volume 57 Issue 18 Pages 2735-2738
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    We herein report a patient with Miller Fisher syndrome mimicking Tolosa-Hunt syndrome. A 47-year-old man presented with right orbital pain and diplopia. On a neurological examination, he had right oculomotor nerve palsy and diminished deep tendon reflexes. Brain magnetic resonance imaging failed to show any parenchymal lesions; however, the bilateral oculomotor nerves were gadolinium-enhanced. The presence of a triad of orbital pain, ipsilateral oculomotor nerve palsy, and a rapid response to steroid therapy met the diagnostic criteria for Tolosa-Hunt syndrome. After discharge, antibodies against GQ1b and GT1a were reported to be positive only with phosphatidic acid. The present case was ultimately diagnosed as an incomplete phenotype of Miller Fisher syndrome.

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  • Jumpei Hasegawa, Sachiko Wakai, Momoko Kono, Yusuke Imaizumi, Sakiko M ...
    2018 Volume 57 Issue 18 Pages 2739-2745
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and cryoglobulinemic vasculitis (CV) rarely coexist. An 83-year-old woman was admitted with rapidly progressive renal failure, gastrointestinal hemorrhage and purpura with myeloperoxidase (MPO)-ANCA positivity and cryoglobulinemia. Despite intensive immunosuppressive treatment, she died of aspergillus pneumonia. Autopsy revealed necrotizing crescentic glomerulitis in the majority of the glomeruli, accompanied by partially membranoproliferative-like glomerular changes. Immunofluorescence staining revealed the presence of neutrophil extracellular trap (NET) formation in the glomeruli and cutaneous arteries. These pathological findings suggested that MPO-AAV and/or CV caused NET formation, leading to lethal systemic vasculitis.

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  • Hirofumi Toko, Hiroto Tsuboi, Naoto Umeda, Fumika Honda, Ayako Ohyama, ...
    2018 Volume 57 Issue 18 Pages 2747-2752
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: May 18, 2018
    JOURNALS OPEN ACCESS

    Hemophagocytic syndrome (HPS) associated with systemic lupus erythematosus (SLE), dubbed acute lupus hemophagocytic syndrome (ALHS), is an intractable complication of SLE. A 24-year-old man who had been diagnosed with SLE three months previously, presented with fever, rash, hallucination, and pancytopenia accompanied with hyperferritinemia and bone marrow hemophagocytosis. He was diagnosed with ALHS and neuropsychiatric (NP)-SLE. Although 4 courses of methylprednisolone pulse therapy and 1 course of intravenous cyclophosphamide (IVCY) improved his NP-SLE, his ALHS did not respond. However, the addition of cyclosporine A (CsA) led to a rapid remission from ALHS. This suggests the usefulness of CsA in the treatment of intractable, corticosteroid- and IVCY-resistant ALHS.

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  • Yoshinori Mori, Shiori Kinoshita, Takashi Kanamori, Hiromi Kataoka, Ta ...
    2018 Volume 57 Issue 18 Pages 2753-2757
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: May 18, 2018
    JOURNALS OPEN ACCESS

    The clinical efficacy and outcomes of pazopanib treatment for metastatic extraosseous Ewing sarcoma remain unclear. We herein report a case of heavily pre-treated metastatic extraosseous Ewing sarcoma in which pazopanib treatment achieved a significant improvement. A 17-year-old girl was referred to our hospital due to metastatic extraosseous Ewing sarcoma. The initial cytotoxic chemotherapy was temporarily effective, however, her disease eventually progressed, and she was subsequently treated with pazopanib. The recurrent tumor showed a marked response to pazopanib therapy; the therapeutic effect has lasted for more than 26 months. The present case suggests that pazopanib may be a therapeutic option for extraosseous Ewing sarcoma.

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  • Takayasu Uchida, Mami Kanzaki, Toshiyuki Kakumoto, Yoshikazu Uesaka
    2018 Volume 57 Issue 18 Pages 2759-2761
    Published: September 15, 2018
    Released: September 15, 2018
    [Advance publication] Released: April 27, 2018
    JOURNALS OPEN ACCESS

    The diagnosis of longus colli tendinitis (LCT) is sometimes challenging, especially when laboratory data show marked inflammation and neuroimaging studies do not indicate calcification within the tendon of the longus colli muscles. We herein report a case of LCT that presented with elevated inflammation parameters without calcification on imaging. Findings characteristic of LCT, such as prevertebral hyperintensity areas on T2-weighted images and no signs of purulent diseases, informed our diagnosis of LCT. Enhanced computed tomography and magnetic resonance imaging are useful procedures when diagnosing LCT after ruling out other critical purulent diseases.

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