Objective A direct renin inhibitor, aliskiren, has a longer stable antihypertensive effect compared with other renin-angiotensin-aldosterone system (RAAS) inhibitors. Methods This study was a 6-month, single-center, open trial conducted between December 2010 and November 2011 to assess the antihypertensive effect of adding aliskiren (300 mg) to the treatment of essential hypertension patients whose target blood pressure (BP) had not been achieved and to assess whether it was possible to reduce the amount of antihypertensive drugs used. Results The results showed an overall improvement in the target BP achievement rate of 60% for clinic BP and 52% for home BP measurements (75 cases total). The mean number of drugs before treatment with aliskiren was 3.28±1.52, whereas at the end of the six months the mean number of drugs prescribed other than aliskiren was 2.85±1.72 (p<0.0001). Moreover, no worsening of the renal function was observed in patients with diabetes or chronic kidney disease (CKD) who were being treated with other RAAS inhibitors in combination to aliskiren. Conclusion These results showed that when aliskiren was added to the treatment of poorly controlled hypertension, the BP achievement rate increased, and it was possible to reduce the amount of antihypertensive drugs used in combination with aliskiren. Moreover, as a result of careful monitoring of the renal function or decreasing the amounts of drugs used in combination, no worsening of the renal function was observed even in the cases complicated by diabetes or CKD being treated with other RAAS inhibitors.
Objective We herein assessed the utility of computed tomography (CT) for the diagnosis and ascertainment of the severity of community-acquired pneumonia (CAP) in the elderly. Methods The utility of CT compared with chest radiography (CR) for the diagnosis of CAP was prospectively studied among elderly inpatients with clinical symptoms and signs indicative of CAP at the Department of Respiratory Medicine in Nissan Tamagawa Hospital during the one-year period from January 2013 to December 2013. Additionally, we evaluated whether the findings of CT were useful as predictive factors related to the mortality rate associated with CAP. Results One hundred and forty-two patients, 65 years of age or older, were surveyed upon hospital admission for suspected CAP. Of the 142 patients included, 127 (89.4%) had pneumonic infiltration diagnosed by CT, however, CR could not recognize pneumonic infiltration in 9.4% (12/127) of these patients. In 127 CAP-positive patients, bilateral pneumonic infiltration was more frequently detected by CT in non-survivors than survivors (79.0% vs. 53.7%; p <0.05). By a multivariable analysis to determine the prognostic factors related to mortality from CAP, oxygen desaturation showed the greatest odds ratio among the other predictive factors, followed by comorbid neoplastic disease, blood urea nitrogen ≥21 mg/dL, male gender, and bilateral pneumonic infiltration diagnosed by CT. Conclusion We herein demonstrated that CT was superior to CR for diagnosing and evaluating the severity of CAP in elderly patients.
Objective Long-term effects of pirfenidone have been poorly understood to date. This study investigated the clinical efficacy and safety of long-term pirfenidone use for idiopathic pulmonary fibrosis (IPF) in clinical practice. Methods This survey study was a retrospective observational study. A survey was used to collect clinical information on IPF cases that were treated with pirfenidone. This survey sheet came from physicians belonging to the Diffuse Lung Diseases Research Group. Results 502 patients at 22 institutes received pirfeidone treatment. Of the 502 cases, pirfenidone treatment was terminated in under one year in 186 cases (37.1%); adverse effect was the most frequent reason for termination. The pirfenidone treatment lasted for two years or longer in 111 cases (22.1%). The mean change in the forced vital capacity (FVC) was -30±224 (SD) mL in the first year of treatment, -158±258 mL in the second year, and -201±367 mL in the third year. FVC improved by 10% or more in the first year in 10 (14.7%) of 68 cases, and showed a change of ±10% in 47 (69.1%) cases. It showed a change of ±10% in the second and third years in 61.7% and 62.5% of the patients, respectively. Conclusion The FVC improved in only a small percentage of patients who received pirfenidone treatment for a long period of time. However, a decrease in the FVC was prevented for three years in over half of the cases.
Objective Autoimmune cerebellar ataxias were recently reported to be treatable. However, the proportion of patients with cortical cerebellar atrophy of unknown etiology with autoimmune-associated cerebellar ataxia and the actual effectiveness of immunotherapy in these diseases remain unknown. Methods We measured the level of autoantibodies (including anti-gliadin antibody, anti-glutamic acid decarboxylase (GAD) antibody, and anti-thyroid antibody) in 58 Japanese patients with cerebellar ataxia, excluding those with multiple system atrophy, hereditary spinocerebellar ataxia, cancer, or those who were receiving phenytoin, and the efficacy of immunotherapy was assessed. Results Thirty-one of 58 (53%) patients were positive for anti-GAD antibody, anti-gliadin antibody, or anti-thyroid antibody. Seven of the 12 anti-gliadin antibody-positive patients, three of the four anti-GAD antibody-positive patients, and three of the six anti-thyroid antibody-positive patients responded well to immunotherapy, indicating that 59% of patients with ataxia-associated antibody-positive cerebellar ataxia undergoing immunotherapy responded well. Conclusion Some patients with cerebellar ataxia have autoimmune conditions and diagnosing autoimmune cerebellar ataxia is therefore an important component in the care of patients with this disease entity.
Objective Primary Sjögren's syndrome (pSS) is a common chronic autoimmune disease that primarily affects the salivary and lacrimal glands. Arterial stiffness is one of the earliest detectable manifestations of adverse structural and functional changes within the vessel wall. The aim of this study was to evaluate the relationship between arterial stiffness and pSS. Methods In this study, 75 female patients with pSS who fulfilled the American European Consensus Criteria for Sjögren's syndrome, were included. A total of 68 age-, sex- and body mass index-matched subjects were recruited as the control population. Arterial stiffness was assessed by measurement of the carotid-femoral pulse wave velocity (PWV). Results The mean age of the patients was 54.0±9.3 years and the median duration of the disease was 10 years. Compared with the control subjects, patients with pSS had a higher mean PWV (8.2±1.5 m/s vs. 7.5±1.4 m/s; p=0.01). Correlation analysis showed that the PWV was positively correlated with age, body mass index, serum cholesterol, low-density lipoprotein (LDL) and C-reactive protein levels, blood pressure, mean arterial pressure (MAP), pulse pressure and left ventricular mass index. A multiple linear regression analysis revealed that arterial stiffness was associated with age, MAP and LDL levels in pSS patients. Conclusion Although patients with pSS appear to have increased arterial stiffness, risk factors associated with arterial stiffness in these patients are similar to the general population. However, we cannot exclude the possibility that a higher PWV in pSS patients is caused, not by pSS itself, but by the use of steroids, hypertension and dyslipidemia.
Objective To devise an effective method to assess the peripheral circulation using an infrared thermographic analysis. Methods Sequential measurements of the skin temperature before and after cold-water immersion of the hands were analyzed by a thermographic examination in healthy controls and patients diagnosed to have Raynaud phenomenon (RP). The skin temperatures of the dorsum of all fingernail folds and the metacarpophalangeal (MCP) joints were measured at baseline. Then the hands were immersed in 10°C water for 10 s, and the skin temperatures were measured at 0, 3, 5, 10, 15, 20 and 30 min after immersion. The mean temperature, recovery rate and disparity (coefficient of variation) of the nail fold temperatures were calculated. The distal-dorsal difference (DDD) was calculated by subtracting the mean MCP temperature from the mean nail fold temperature. Receiver operating characteristic (ROC) curves were generated to compare these parameters in terms of their capability to differentiate patients with RP. Results Thirty-one RP patients and 25 controls were included in the study. The baseline nail fold temperature was significantly lower in RP patients than in the controls. The RP patients had a lower recovery rate, lower DDD and higher disparity than the controls. The disparity and DDD were negatively correlated (r=-0.63, p<0.01), whereas the recovery rate and DDD were positively correlated (r=0.91, p<0.01). The ROC curve analysis revealed that the disparity in nail fold temperature effectively differentiated RP patients from controls (area under the curve: recovery rate 0.72; disparity 0.88; DDD 0.79). Conclusion The temperature disparity between fingers is a useful thermographic parameter for evaluating disturbed peripheral circulation in patients with Raynaud phenomenon.
Objective Low-dose trimethoprim-sulfamethoxazole (TMP-SMX) is commonly used to prevent pneumocystis pneumonia in daily practice. Previous reports have shown a relationship between high- or standard-dose of TMP-SMX and hyperkalemia, however it remains unclear whether this is true for low-dose TMP-SMX. In this study we sought to determine the risk factors for hyperkalemia associated with low-dose TMP-SMX. Methods In this retrospective cohort study, 186 consecutive adult patients who received TMP-SMX as prophylaxis for pneumocystis pneumonia from January 2014 to January 2015 were evaluated. Data on the patients' age, gender, baseline estimated glomerular filtration rate (eGFR), baseline serum potassium, maximum serum potassium, duration reaching the maximal serum potassium level, dosage, and concomitant use of angiotensin-converting enzyme inhibitors (ACEi)/angiotensin receptor blockers (ARB), β-blockers, non-steroidal anti-inflammatory drugs and potassium-sparing diuretics were retrospectively collected. Hyperkalemia was defined as a serum potassium level ≥5 mEq/L. Univariate and multivariate analyses were performed. Results The median age of the patients was 66 years and 51.1% were men. Hyperkalemia associated with low-dose TMP-SMX was observed in 32 patients (17.2%). The median duration to reach the maximal serum potassium level was 12 days. The multivariate logistic regression analysis identified renal insufficiency to be a major risk factor for hyperkalemia associated with low-dose TMP-SMX (eGFR <60 mL/min/1.73 m2, adjusted OR 4.62). Moreover, in the subpopulation of patients with renal insufficiency, ACEi/ARB use was considered to be a major risk factor for hyperkalemia (adjusted OR 3.96). Conclusion Renal insufficiency in concert with ACEi/ARB use is a major risk factor for hyperkalemia induced by low-dose TMP-SMX.
Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical syndrome of varying etiologies with similar neuroimaging findings. This is a case report of a 25-year-old woman who developed typical, neurological symptoms and magnetic resonance imaging abnormalities after treatment for the acute exacerbation of ulcerative colitis (UC), which included blood transfusion, the systemic administration of prednisolone, and the administration of metronidazole. It has been reported that these treatments may contribute to the development of RPLS. RPLS should therefore be considered in the differential diagnosis of UC patients who exhibit impaired consciousness, seizures or visual deficits during treatment. We report a rare case of RPLS in a patient with UC.
A 49-year-old man was admitted to our hospital with a chief complaint of dyspnea. He had a history of mitral valve replacement (MVR) with a Starr-Edwards (SE) caged-disc valve at the age of 14. Echocardiography revealed elevated trans-valvular pressure gradient of the mitral prosthetic valve with neither disk motion abnormality nor abnormal structure. Catheterization confirmed an elevation of the mean diastolic gradient of the mitral valve to 12.3 mmHg. Re-MVR was performed, and abnormal tissue attached to the cage of the valve and proliferating beneath the valve was observed. Histologic examination revealed them as fibrinous tissue and mild pannus proliferation, respectively. This rare case report focuses on long-term follow-up and the complication of a SE caged-disc valve. A SE caged-disc valve may become stenotic, only detected with a trans-valvular pressure gradient without any disk motion abnormality or abnormal structure during a prolonged follow-up period.
A 59-year-old Japanese woman developed diabetes mellitus without ketoacidosis in the presence of glutamic acid decarboxylase autoantibody (GADA) (24.7 U/mL). After the amelioration of her hyperglycemia, the patient had a relatively preserved serum C-peptide level. Her endogenous insulin secretion capacity remained almost unchanged during 5 years of insulin therapy. The patient's GADA titers normalized within 15 months. The islet-related autoantibodies, including GADA, are believed to be produced following the autoimmune destruction of pancreatic beta cells and are predictive markers of type 1 diabetes mellitus. Therefore, the transient appearance of GADA in our patient may have reflected pancreatic autoimmune processes that terminated without progression to insulin deficiency.
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare, smoking-related, interstitial lung disease, and pulmonary hypertension (PH) is associated with mortality. We herein report a case of PLCH complicated by severe PH and respiratory impairment. After developing PH, the patient displayed a cystic pattern on chest high-resolution computed tomography (HRCT). This, in turn, corresponded with the scarring stage of PLCH. However, the patient's PH and respiratory impairment improve dramatically following smoking cessation. PLCH patients with a cystic pattern on chest HRCT may still be able to improve their PH and respiratory impairment when they are able to quit smoking.
The patient was a 68-year-old man presenting with body weight loss and exertional dyspnea. High-resolution computed tomography of the chest showed dense subpleural consolidation with traction bronchiectasis and volume loss predominantly in bilateral apical lesions and upper lobes. A histopathological analysis of a specimen of the right upper lobe showed histological patterns which were consistent with idiopathic pleuroparenchymal fibroelastotis (IPPFE). Treatment with pirfenidone was introduced with the expectation of its potential benefit. The effect of pirfenidone was satisfactory, and a decline in forced vital capacity was inhibited during treatment. This is the first case report suggesting the efficacy of pirfenidone for patients with IPPFE.
A 38-year-old woman with sustained right chest pain was referred to our hospital. She showed pleural effusion and peripheral blood eosinophilia. Thoracentesis revealed eosinophilic pleural effusion in which the smear, culture and cytological examinations were all negative. Although she had no notable dietary history, chest CT revealed linear opacities, which suggested the migration tracks of paragonimiasis. The diagnosis was confirmed using enzyme-linked immunosorbent assays, which showed elevated Paragonimus westermani and Paragonimus miyazakii antibody levels. After the initiation of praziquantel therapy, all clinical findings were promptly improved. The detection of a migration track may therefore be useful in the diagnosis of paragonimiasis.
Crizotinib is highly effective for anaplastic lymphoma kinase (ALK)-positive non-small cell lung cancer (NSCLC). However, it remains unclear whether crizotinib has a beneficial effect on elderly patients with ALK-positive NSCLC with a poor performance status (PS). We herein present the case of an 85-year-old man with stage IV ALK-positive NSCLC, whose PS score was 4 due to pericardial and pleural effusions. After initiating crizotinib therapy, a drastic response was observed and the PS score improved to 0. Adverse effects were manageable. Our results indicate that crizotinib could be an important choice when treating elderly patients with ALK-positive NSCLC with poor PS.
A 76-year-old woman was referred to our hospital because of anemia. The laboratory findings revealed hemolysis. Although a direct Coombs test was negative, a high titer of RBC-bound IgG was detected, and a diagnosis of Coombs-negative autoimmune hemolytic anemia was made. She was successfully treated with prednisolone. One year and five months later, she again presented anemia and was diagnosed with pure red cell aplasia. Anti-erythropoietin receptor antibody was detected in the serum. She was treated with cyclosporine and obtained prompt recovery. We herein report this rare case and review the pertinent literature.
Behçet's disease (BD) is a polysymptomatic and recurrent systemic vasculitis with a chronic course and unknown cause. We herein report a 27-year-old woman who had suffered from a recurrent fever and tonsillitis for nearly ten years with BD for who tonsillectomy was effective.
We herein present the case of a 56-year-old woman who presented with symptoms of psoriatic arthritis (PsA) with erythema that progressed to annular pustular psoriasis. The patient had a 15-year history of polyarthritis. Annular pustular psoriasis is not typically observed in cases of arthritis. This is the first reported case of PsA with annular pustular psoriasis.
We herein describe a rare case of temporal arteritis associated with hypertrophic pachymeningitis. An 81-year-old man presented with a right temporal headache that had persisted for one month. A right superficial temporal artery biopsy revealed intimal hypertrophy with increased elastic fibers, consistent with temporal arteritis. Brain MRI using gadolinium enhancement showed thickened dura mater on the right frontal and temporal lobes, which led to the diagnosis of hypertrophic pachymeningitis. Intravenous methylprednisolone and oral prednisolone improved the patient's symptoms. According to our findings, hypertrophic pachymeningitis may be a potential cause of an ipsilateral temporal headache associated with temporal arteritis.
A 68-year-old man visited an emergency department complaining of dyspnea. He was diagnosed to have Legionella pneumonia with multiple organ failure. Although his multiple organ failure improved, he suffered from persistent abdominal pain and diarrhea with continuous minor bleeding. Colonoscopy revealed a longitudinal ulcer of the rectum, below the peritoneal reflection. He was diagnosed with cytomegalovirus (CMV) colitis. Antiviral therapy with ganciclovir was initiated. He finally underwent a colostomy after a bowel stricture caused an intestinal outlet obstruction, which made oral intake impossible. Based on the present case, we believe that CMV colitis must be considered as one of the differential diagnoses when critically ill patients develop continuous diarrhea and abdominal pain.
A 41-year-old nurse was referred to our hospital with a fever and disturbed consciousness. She tested positive for influenza antigen. CT and MRI findings revealed low density and intensity areas in the right occipital and lateral lobes with remarkable brain edema, which led to a diagnosis of influenza encephalopathy. Influenza A antibodies in the serum were below the detection limit despite the patient receiving previous vaccination three months earlier. A PCR analysis revealed that the influenza HA gene was classified into clade 3C.2a, subclass AH3N2. The present case indicates the potential development of encephalopathy in adults under certain conditions.
Good's syndrome is an immunodeficiency disease involving thymoma accompanied by hypogammaglobulinemia. We encountered a case of Good's syndrome accompanied by agranulocytosis that followed a rapid clinical course. A 72-year-old man visited our hospital with a two-week history of a sore throat. Candida albicans was detected in the pharynx, and hypogammaglobulinemia was detected in addition to granulocytopenia. The patient subsequently developed septic shock and followed a rapid clinical course which ended in death. Good's syndrome with agranulocytosis was diagnosed at autopsy. Good's syndrome accompanied by agranulocytosis can follow a rapid clinical course and some cases remain asymptomatic until old age. Its prompt treatment is crucial.