Dermatomyositis Presenting with Generalized Subcutaneous Edema.

Generalized subcutaneous edema is rare in dermatomyositis. We herein report an 82-year-old woman with dermatomyositis who presented with generalized subcutaneous edema. Three weeks before admission, she first noticed facial edema, and the symptoms exacerbated, with limb edema occurring. On admission, muscle weakness in the limbs and skin rash were noted. Her muscle enzyme levels were elevated, and serum anti-transcriptional intermediary factor 1-γ antibody was detected. She was diagnosed with dermatomyositis and treated with corticosteroids and tacrolimus, which improved her muscle weakness, muscle enzymes, and edema. Thus, generalized subcutaneous edema can occur during dermatomyositis, with facial edema as the initial symptom.

Listeria rhombencephalitis mimicking stroke in a patient with giant cell arteritis.

Listeria monocytogenes sometimes causes central nervous system infections. However, rhombencephalitis is a rare form of L. monocytogenes infection. Its clinical symptoms and magnetic resonance imaging (MRI) findings are often similar to those of vertebrobasilar stroke. We present the case of a 79-year-old woman with Listeria rhombencephalitis presenting with rhinorrhea and productive cough. She had giant cell arteritis (GCA) treated with prednisolone and methotrexate. She was admitted for loss of appetite, rhinorrhea, and productive cough. These symptoms were alleviated without specific treatment; however, she suddenly developed multiple cranial nerve palsies, and MRI showed hyperintense signals on diffusion-weighted imaging and hypointense signals on apparent diffusion coefficient in the brainstem. Ischemic stroke due to exacerbation of GCA was suspected, and treatment with intravenous methylprednisolone was initiated; however, seizures occurred, and a lumbar puncture was performed. Cerebrospinal fluid and blood cultures revealed L. monocytogenes, and she was diagnosed with Listeria rhombencephalitis. Although antibiotic treatment was continued, the patient died. Thus, when patients with rhinorrhea or productive cough develop sudden cranial nerve palsy, Listeria rhombencephalitis should be considered as a differential diagnosis, and lumbar puncture should be performed.

Anti-synthetase Syndrome That Relapsed with Pulmonary Arterial Hypertension and Malignancy.

A 69-year-old man with a history of anti-synthetase antibody-positive polymyositis and interstitial lung disease (ILD) stable for more than 20 years suddenly developed pulmonary artery hypertension (PAH) with a mean PA pressure of 46 mmHg. At this stage, ILD was mild, but it became acutely exacerbated later, and high-dose corticosteroid and intravenous cyclophosphamide ameliorated both PAH and ILD. The tricuspid regurgitation pressure gradient decreased from 80 to 49 mmHg and ILD recovered almost completely. During a systemic examination, bone metastatic cancer of unknown origin was found. We herein report the relationship between anti-synthetase syndrome (ASS) and PAH as well as ASS and malignancy.

Four cases of dermatomyositis with abnormally high anti-MDA-5 antibody titres and not high levels of serum ferritin.

Anti-melanoma differentiation-associated gene 5 (MDA-5) antibody-positive dermatomyositis is a fatal disease presenting with rapidly progressive interstitial lung disease. High ferritin levels are a well-known poor prognostic factor. A high anti-MDA-5 antibody titre was also recently identified as a poor prognostic factor. We encountered four cases that had extremely high anti-MDA-5 antibody titres without high levels of ferritin in the initial examination. All cases were female with ages ranging between 29 and 54 years (mean age, 44 years). In the initial examination, anti-MDA-5 antibody titres were 2060-3040 (normal range, <32 index), ferritin levels were 87-480 ng/ml (normal range, 2.6-129.4 ng/ml), KL-6 level was 186-1806 U/ml (normal range, <500 U/ml), and creatine kinase level was normal in all patients. One patient had respiratory distress on exertion. Computed Tomography (CT) images showed mild ground-glass attenuation/reticular shadows near the pleura in all patients. Three patients were treated with a combination of high-dose glucocorticoids, intermittent intravenous cyclophosphamide, and calcineurin inhibitors, and two required plasma exchange due to the worsening of lung lesion. In these patients, ferritin and KL-6 levels tended to elevate after the beginning of treatment. Very mild pulmonary lesions disappeared in one patient treated with moderate doses of a glucocorticoid and calcineurin inhibitor. All patients survived, and one required oxygen on exertion at discharge. The condition of patients with abnormally high anti-MDA-5 antibody titres may deteriorate even though ferritin levels were not high and lung shadows are minimal at presentation. Therefore, intensive treatment needs to be considered early in the course of the disease regardless of the serum ferritin level.

A case of aortoduodenal fistula caused by IgG4-related periaortitis.

An 86-year-old man who underwent endovascular aortic repair for impending rupture of an abdominal aortic aneurysm a year ago presented to our hospital because of fatigue and black stools. Multiple bacterial specimens were detected in blood cultures, and computed tomography following oral administration of gastrografin demonstrated gastrografin in the abdominal aorta. The diagnosis of aortic duodenal fistula was confirmed and emergency abdominal aortic replacement was performed. The pathological findings of the aorta included a large number of immunoglobulin G4 (IgG4)-positive plasma cells infiltrating all layers of the aortic wall, with particularly marked thickening of the adventitia. The serum IgG4 level was 241 mg/dl and IgG4-related periaortitis was diagnosed. Aortoduodenal fistula is a rare but fatal complication of IgG4-related periaortitis. Patients should be followed carefully after endovascular aortic repair for inflammatory abdominal aortic aneurysms.

Encephalopathy induced by high levels of ceftriaxone in the blood and cerebrospinal fluid.

Ceftriaxone is commonly used to treat bacterial infections. Encephalopathy is a rare adverse effect of ceftriaxone therapy, and most cases have been diagnosed based on medical history. We report a case of a 73-year-old woman with ceftriaxone-associated encephalopathy, which was confirmed by measuring the ceftriaxone levels in the blood and cerebrospinal fluid. She regularly underwent hemodialysis. She received intravenous ceftriaxone at a dose of 1 g/day for 4 days for enteritis, and mental status began to be disturbed during the therapy. Six days after ceftriaxone discontinuation, her consciousness level rapidly improved. Thus, ceftriaxone-associated encephalopathy was suspected. High ceftriaxone levels in the blood and cerebrospinal fluid were observed while the patient had disturbed consciousness. This case indicated that high ceftriaxone levels in the blood and cerebrospinal fluid were related to development of encephalopathy. The estimation of ceftriaxone levels may be useful for an accurate diagnosis.

Case Report: Pulmonary Tuberculosis Accompanied by Pure Red Cell Aplasia and Autoimmune Hemolytic Anemia.

Tuberculosis is one of the most common infections worldwide. It has been associated with some hematologic disorders; however, pure red cell aplasia or autoimmune hemolytic anemia is rarely reported. We describe the case of a 68-year-old woman with pulmonary tuberculosis accompanied by pure red cell aplasia and autoimmune hemolytic anemia. These hematologic disorders were improved by treatment of pulmonary tuberculosis. This case suggests that physicians should consider the possibility of tuberculosis as a cause of pure red cell aplasia.

Rapidly destructive coxarthrosis accompanied by fluctuating C-reactive protein level.

Rapidly destructive coxarthrosis is a rare entity of unknown etiology that is characterized by rapid hip joint destruction. Blood tests are thought to be non-specific. However, we herein show a patient with rapidly destructive coxarthrosis, which was accompanied by fluctuating C-reactive protein level.

Case Report: Splenic Infarction in Infectious Mononucleosis due to Epstein-Barr Virus Infection.

Epstein-Barr virus (EBV) is the most common cause of infectious mononucleosis (IM) and IM is a clinical syndrome typically characterized by fever, pharyngitis, and cervical lymph node enlargement. We describe the case of a 19-year-old man with IM complicated by splenic infarction. The patient visited our hospital because of upper abdominal pain without a fever and sore throat. Abdominal computed tomography revealed a low-density area in the spleen, which indicated splenic infarction. The next day, he developed a fever. After diminishing abdominal pain and fever, he developed pharyngitis accompanied by fever. Acute EBV infection was confirmed by serological tests. The patient was successfully managed with no specific therapy. Splenic infarction is a rare complication of IM and this case showed that splenic infarction can precede a fever and pharyngitis.

Impact of Guidewire Route on Severe Dissection After Balloon Angioplasty for Femoropopliteal Chronic Total Occlusion Lesions: An Intravascular Ultrasound Analysis.

OBJECTIVE: To determine the impact of the guidewire route on severe dissection after balloon angioplasty for femoropopliteal chronic total occlusion (CTO) lesions using a new intravascular ultrasound (IVUS) assessed classification scheme corresponding to a conventional angiographic classification scheme. METHODS: Images for 21 femoropopliteal CTO lesions treated endovascularly between May 2018 and December 2019 were used for analysis. IVUS images after guidewire passage and those after balloon angioplasty were evaluated at 1 cm intervals. Cross sectional images were obtained (n = 219) and divided into two groups by the guidewire route: those in which the guidewire passed through the inner half of the luminal radius (central wiring group, 139 cross sectional images) and those in which the guidewire passed through the outer half of the luminal radius (eccentric wiring group, 80 cross sectional images). Angiographically severe dissection was defined as Type C or greater according to the National Heart, Lung, and Blood Institute classification, to which six dissection morphology patterns were applied based on IVUS images (Types A - E2). RESULTS: Central wiring was achieved in an average of 69.6 ± 28.0% of the CTO length from per limb analysis. Among the IVUS assessed dissection morphology patterns, Types D - E2 were more frequently correlated with angiographically severe dissection than were Types A - C (57.5% vs. 13.7%, p < .001). Multivariable analysis showed that soft plaque was a predictive factor for (odds ratio [OR] 2.14; 95% confidence interval [CI] 1.007 - 4.72; p = .048) and central wiring was a protective factor (OR 0.27; 95% CI 0.14 - 0.49; p < .001) against Type D - E2 dissection patterns assessed by IVUS after balloon angioplasty. CONCLUSION: Lesions with Type D - E2 dissection patterns assessed by IVUS were correlated with angiographically severe dissection. Central wiring may be useful for preventing severe dissection after balloon angioplasty for femoropopliteal CTO lesions.

Noteworthy Cardiovascular Involvement with Sporadic Late-onset Nemaline Myopathy.

Cardiac involvement has recently been the focus of sporadic late-onset nemaline myopathy (SLONM). However, right ventricular failure and pulmonary hypertension, in addition to repetitive cardiac arrest, are noteworthy characteristics of SLONM. We herein report a 66-year-old woman with SLONM whose main symptoms were cardiac arrest, right ventricular failure, and pulmonary hypertension. Despite permanent pacemaker replacement, cardiac arrest occurred repetitively, and even with continuous positive airway pressure, right ventricular failure and pulmonary hypertension persisted. The patient was finally diagnosed with SLONM by a muscle biopsy. Our case suggests the possibility of cardiovascular involvement in SLONM, especially right ventricular failure and pulmonary hypertension.

Rapid Response System for In-Hospital Large Vessel Occlusion: A Case-Control Study.

Objective: Acute ischemic stroke due to large vessel occlusion (LVO) in hospitalized patients is relatively rare but important condition. However, unlike community-onset cases, there are only few time-saving protocols for in-hospital LVO. This study aimed to evaluate the time-saving effects of rapid response system (RRS) for the management of in-hospital LVO. Methods: We retrospectively evaluated consecutive in-hospital LVO patients who underwent mechanical thrombectomy (MT) between April 2015 and January 2020. In November 2017, we added "acute hemiparesis, eye deviation, and convulsive seizures" to the activation criteria for RRS. In this protocol, the patient is immediately transported from the ward to the emergency room (ER) by Medical Emergency Team (MET). The stroke team can then start assessment in the same manner as for community-onset cases. The time metrics between those with and without RRS intervention were compared. The primary outcome was time from detection to the first assessment by stroke team and to initial CT. To investigate the validity of the revised criteria, we also analyzed all RRS-activated cases. Results: In total, 26 patients (RRS group, 11 patients; non-RRS group, 15 patients) were included. The median time from detection to stroke team assessment (10.0 [interquartile range: IQR, 8-15] minutes vs 65.5 [18-89] minutes) and to CT (22.0 [16-31] minutes vs. 46.5 [35-93] minutes) were significantly shorter in the RRS group. RRS was activated in 34 patients (mean, 1.3/month) according to the added criteria, of whom 20 (58.8%) had cerebral infarction and 9 underwent MT. About two-thirds of the other patients developed neurological emergencies (e.g., epileptic seizure, syncope, or hypoglycemia) that required acute care. Conclusion: RRS has the potential to shorten response time efficiently in the management of in-hospital LVO. Prompt transportation of the patient to the ER by MET enables faster intervention by the stroke team.

Cardiovascular outcome and home blood pressure in relation to silent myocardial ischemia in a clinical population: The J-HOP study.

Several guidelines recommend measuring home blood pressure (BP) and lowering blood pressure than ever before. But several studies reported that lowering diastolic blood pressure (DBP) increased the incidence of coronary artery disease (CAD). We analyzed 3605 individuals who underwent both home and office BP monitoring over 14 days and baseline Hs-cTnT measurement and identified follow-up data of the Japan Morning Surge-Home Blood Pressure (J-HOP) study who had a history of or risk factors for cardiovascular disease. During a mean follow-up period of 6.4 years (23 173 person-years), 114 coronary artery disease and 81 stroke events occurred. Elevated Hs-cTnT (≥0.014 ng/mL) was observed in 298 patients (8.3%). In the group with non-elevated Hs-cTnT (<0.014 ng/mL, n = 3307), an adjusted Cox hazard model showed that home systolic BP (SBP) was associated with a risk of stroke incidence (hazard ratio [HR] per 1 SD, 1.62; 95% confidence interval [CI], 1.29-2.03). This association was also observed in office SBP (HR per 1 SD, 1.43; 95%CI, 1.07-1.91). There was no association between office or home BP and CAD events in the group with non-elevated Hs-cTnT. In the group with elevated Hs-cTnT, an adjusted Cox hazard model showed that home DBP was associated with a risk of CAD incidence (HR per 1 SD, 0.54; 95%CI, 0.30-0.99). However, this association was not observed in office DBP. In patients with elevated Hs-cTnT, which is a marker of subclinical myocardial ischemia, excessive lowering of home DBP may be associated with a risk of incident CAD.

Differential Impact of the Renal Resistive Index on Future Cardiovascular Events in Hospitalized Atherosclerotic Cardiovascular Patients According to Left Ventricular Ejection Fraction　- The Jichi Vascular Hemodynamics in Hospitalized Cardiovascular Patients (J-VAS) Study.

BACKGROUND: Determinants of poor outcome in atherosclerotic cardiovascular disease (ASCVD) according to left ventricular ejection fraction (LVEF) are unclear. The renal resistive index (RRI) correlates well with atherosclerotic vascular damage, which, in turn, is correlated with cardiovascular outcomes. This study investigated whether high RRI is associated with poor cardiovascular outcomes in ASCVD patients classified by LVEF.Methods and Results:Records of 1,598 acute coronary syndromes (ACS) and acute decompensated heart failure (ADHF) patients, categorized into preserved (p), mid-range (mr), and reduced (r) ejection fraction (EF) groups (EF ≥50% [n=1,130], 40-50% [n=223], and <40% [n=245], respectively), were analyzed retrospectively. The primary endpoint was any cardiovascular-related event: fatal and non-fatal ACS, ADHF, stroke, and sudden cardiac death. Over 1.9-years follow-up (3,030 person-years), 233 events occurred: 122, 37, and 74 in the pEF, mrEF, and rEF groups, respectively. Adjusted Cox regression analysis revealed RRI ≥0.8 was associated with the primary endpoint in the pEF group (hazard ratio [HR] 1.67; 95% confidence interval [CI] 1.09-2.56), but not in the mrEF or rEF groups. The primary endpoint risk of pEF patients with an RRI ≥0.8 was comparable to that of mrEF patients using the pEF+RRI <0.8 group as the reference (HR 1.89 [95% CI 1.26-2.83] and 1.77 [95% CI 1.19-2.63], respectively). CONCLUSIONS: RRI was associated with the risk of cardiovascular events in ASCVD patients with pEF.