A multicenter report of biologic agents for the treatment of secondary amyloidosis in Turkish rheumatoid arthritis and ankylosing spondylitis patients

In this multicenter, retrospective study, we evaluated the efficacy and safety of biologic therapies, including anti-TNFs, in secondary (AA) amyloidosis patients with ankylosing spondylitis (AS) and rheumatoid arthritis (RA). In addition, the frequency of secondary amyloidosis in RA and AS patients in a single center was estimated. Fifty-one AS (39M, 12F, mean age: 46.7) and 30 RA patients (11M, 19F, mean age: 51.7) with AA amyloidosis from 16 different centers in Turkey were included. Clinical and demographical features of patients were obtained from medical charts. A composite response index (CRI) to biologic therapy—based on creatinine level, proteinuria and disease activity—was used to evaluate the efficacy of treatment. The mean annual incidence of AA amyloidosis in RA and AS patients was 0.23 and 0.42/1000 patients/year, respectively. The point prevalence in RA and AS groups was 4.59 and 7.58/1000, respectively. In RA group with AA amyloidosis, effective response was obtained in 52.2 % of patients according to CRI. RA patients with RF positivity and more initial disease activity tended to have higher response rates to therapy (p values, 0.069 and 0.056). After biologic therapy (median 17 months), two RA patients died and two developed tuberculosis. In AS group, 45.7 % of patients fulfilled the criteria of good response according to CRI. AS patients with higher CRP levels at the time of AA diagnosis and at the beginning of anti-TNF therapy had higher response rates (p values, 0.011 and 0.017). During follow-up after anti-TNF therapy (median 38 months), one patient died and tuberculosis developed in two patients. Biologic therapy seems to be effective in at least half of RA and AS patients with AA amyloidosis. Tuberculosis was the most important safety concern.     

Angiographic prevalence of myocardial bridging.

OBJECTIVE: Muscle fibers overlying the intramyocardial segment of an epicardial coronary artery are termed myocardial bridging. Variable prevalence has been described at autopsy and angiographic series with small and large sample sizes. The aim of the study was to investigate the angiographic prevalence of myocardial bridging in 25982 patients from Turkey. METHODS: We performed a retrospective study, evaluated the cases with myocardial bridging among patients undergone selective coronary angiography, and searched the angiographic prevalence of myocardial bridging in a very large sample size. We studied also the correlation between the severity of the bridging and risk factors for coronary artery disease. RESULTS: Among 25982 patients we found 316 cases of myocardial bridging in a retrospective manner. The total prevalence was 1.22%. Although, 96.52% of patients with myocardial bridging had the lesion in the left anterior descending coronary artery (LAD) as expected, distribution of bridges between mid- and distal segments were almost equal (52.79% and 47.21%, respectively). We subclassified patients in two groups, Group A (<50% of systolic compression) and Group B (>or=50% of systolic compression), according to the amount of systolic compression of LAD and studied relationship of risk factors for coronary artery disease between groups. Another subclassification was also made for patients having myocardial bridging without coronary or valvular heart disease and hypertrophic obstructive cardiomyopathy; Group 1 (<50% of systolic compression) and Group 2 (>or=50% of systolic compression). In these patients we studied correlation between the severity of the myocardial bridging and risk factors for coronary artery disease. The prevalence of bridges in circumflex and right coronary arteries individually and in all arteries as combination was also studied. CONCLUSION: In a very large group of patients from Turkey undergone selective coronary artery angiography, the angiographic prevalence of myocardial bridging was slightly higher than expected. Only diabetes mellitus as a risk factor for coronary artery disease was higher in groups representing <50% of systolic compression (Group A and 1) than in groups representing >or=50% of systolic compression (Group B and 2) but the importance of this result is not known.     

Scintigraphic evaluation of gallbladder motor functions in H pylori positive and negative patients in the stomach with dyspepsia

AIM：To evaluate the relationship between gallbladder （GB） motor function and H pylori infection in the stomach.
METHODS： All cases （86） underwent the 14C urea breath test （UBT）. ^14C-UBT was found as positive in 58 and negative in 28 dyspeptic patients. ^14C- UBT was accepted as a gold standard test. Clo test and histopathologic examination were compared with the results of ^14C-UBT in cases who tolerated upper gastrointestinal endoscopy procedure. Cholescintigraphy with ^99mTc-mebrofenin was used to determine the parameters of GB motor function （GB filling and emptying time, half of the emptying time, ejection fraction at 30th and 60th min） in all patients.
RESULTS： We found the sensitivity and specificity as 88% and 86% for Clo test and as 89% and 80% for histologic evaluation, respectively. The parameters of GB function were not significantly different in H pylori positive and negative patients. The GB emptying was normal in both groups. Minimum GB filling time was 30 min in 34 of 86 cases （39.5%）, filling was not observed in 2 cases. The GB ultrasonography （USG） results were normal for all cases and bile composition abnormality was not determined.
CONCLUSION： Our study showed that ^14C-UBT is highly reliable method to detect the presence of H pylori. The presence of H pylori infection does not directly affect the GB function.     

Optic disc and retinal nerve fiber layer parameters as indicators of neurodegenerative brain changes in patients with obstructive sleep apnea syndrome

Purpose

Retina is a unique part of the central nervous system (CNS) for visualizing the processes of axonal and neuronal degeneration. Optical coherence tomography (OCT) allows direct visualization and measurement of retinal nerve fiber layer (RNFL) thickness, macular volume, and optic disc (OD) parameters. One of the disorders associated with atrophy in different brain regions is obstructive sleep apnea syndrome (OSAS). In the present study, we aimed to determine OD and RNFL changes measured by OCT for investigating the progress of neurodegeneration development in OSAS, excluding all the other conditions that can directly affect RNFL thickness and optic nerve parameters.

Methods

Both eyes of 101 patients with OSAS and 20 controls were investigated by OCT. Full-night polysomnography (PSG) and ophthalmologic examination including automated visual field (VF) examination and OCT were performed in all of the patients.

Results

According to the OSAS grading, patients were grouped as mild (n?=?15), moderate (n?=?27), and severe (n?=?59). We found significant decrease in RNFL thickness only in the patients with severe OSAS compared with the other groups and decreased macular ganglion cell thickness in the severe OSAS group compared with the control group. VF parameters were significantly worsened in all the OSAS subgroups compared to the control group. We found different data such as normal or increased optic nerve parameters as result of subtle OD edema, which may mask possible peripapillar axonal loss.

Conclusions

We think that evaluation of neurodegeneration in OSAS is not always possible by examining OD and RNFL because there are difficulties due to the confounding issues of cerebral atrophy and OD edema.     

Rapid pleurodesis is an outpatient alternative in patients with malignant pleural effusions: a prospective randomized controlled trial

Background

Chemical pleurodesis can be palliative for recurrent, symptomatic pleural effusions in patients who are not candidate for a thoracic surgical procedure. We hypothesized that effective pleurodesis could be accomplished with a rapid method of pleurodesis as effective as the standard method.

Methods

A prospective randomized ‘non-inferiority’ trial was conducted in 96 patients with malignant pleural effusion (MPE) who are not potentially curable and/or not amenable to any other surgical intervention. They were randomly allocated to group 1 (rapid pleurodesis) and to group 2 (standard protocol). In group 1, following complete fluid evacuation, talc slurry was instilled into the pleural space. This was accomplished within 2 h of thoracic catheter insertion, unless the drained fluid was more than 1,500 mL. After clamping the tube for 30 min, the pleural space was drained for 1 h, after which the thoracic catheter was removed. In group 2, talc-slurry was administered when the daily drainage was lower than 300 mL/day.

Results

No-complication developed due to talc-slurry in two groups. Complete or partial response was achieved in 35 (87.5%) and 33 (84.6%) patients in group 1 and group 2 respectively (P=0.670). The mean drainage time was 40.7 and 165.2 h in group 1 and group 2 respectively (P<0.001).

Conclusions

Rapid pleurodesis with talc slurry is safe and effective and it can be performed in an outpatient basis.     

The effect of local corticosteroid injection on F-wave conduction velocity and sympathetic skin response in carpal tunnel syndrome

The aim of this study was to evaluate the efficacy of steroid injection for the treatment of the carpal tunnel syndrome (CTS), with F-wave parameters and sympathetic skin response (SSR). Seventeen hands of 10 women patients were treated with local steroid injection with 2-month follow-up. All patients underwent single injection into the carpal tunnel. Response to injection was measured nerve conduction studies (NCSs), median nerve F waves, and SSR before and after treatment. To determine the normal values, 42 hands of 21 healthy women were also studied. There was a significant improvement of sensory and motor nerve conduction values when compared to baseline values (P < 0.01). At the end of follow-up period, the median sensory distal latency and the sensory latency differences between the median and the ulnar nerve were improved 35 and 65%, respectively. The maximum, mean F-wave amplitudes and chronodispersion showed a slight improvement with respect to baseline values and controls, but statistical significance was not achieved after treatment. Although no statistically significant improvements were observed in SSR parameters, slightly decreased amplitudes and increased habituation of SSR were noted at the end of the treatment. The present study shows that the local steroid injection results in improvement in NCSs values, but the F-wave parameters were not effectual in short-term outcome of CTS treatment. These findings suggest that the sensory latency differences between the median and the ulnar wrist-to-digit 4 are better parameters in the median nerve recovery after treatment than the median sensory distal latency. Furthermore, the SSR does not seem to be a sensitive method in follow-up of CTS treatment.     

DRESS syndrome with fatal results induced by sodium valproate in a patient with brucellosis and a positive cytoplasmic antineutrophilic cytoplasmic antibody test result

DRESS syndrome is a life-threatening adverse reaction characterized by skin rashes, fever, leukocytosis with eosinophilia or atypical lymphocytosis, lymph node enlargement, and liver or renal dysfunctions. DRESS syndrome related to valproic acid use is very rarely observed. We present a case of DRESS syndrome induced by sodium valproate, which developed and progressed fatally in a brucellosis patient with a positive c-ANCA test. A 19-year-old female patient presented with fever, cough, jaundice, and rash all over her body. Brucella Coombs test was positive at 1:1280 titers, and the Rose Bengal test was also positive. The involuntary movements were thought to be due to chorea, and the patient was started on sodium valproate 500 mg 2*1, as well as streptomycin 1?g flk 1*1 and tetradox capsules 2*1 for the brucellosis and was discharged. DRESS syndrome was suspected in the patient, and she was taken off sodium valproate and tetradox; N-acetylcysteine, ceftriaxon, prednizolone, and support treatment were started. When sodium valproate is used on its own, it carries no risk of inducing DRESS syndrome. However, in the case presented, another co-morbidity such as brucellosis and c-ANCA positivity was present. We believe that the presence of further co morbidity not yet reported in literature is important from the perspective of the risk of valproate-induced DRESS syndrome. Therefore, if sodium valproate treatment is to be started in patients, especially those with co morbidity, they must be closely monitored with clinical and laboratory observations. At the slightest suspicion of DRESS syndrome, all medication should be ceased immediately and the patient should be placed under continuous observation.     

Nasopharyngeal sarcoidosis: a rare involvement

Sarcoidosis is a chronic, multisystemic, non-caseating granulomatous disease of unknown etiology. Nasopharyngeal involvement is very rare in sarcoidosis. The objective of this report was to evaluate a rare involvement of sarcoidosis. This report includes a case of nasopharyngeal sarcoidosis. A 51-year-old female with nasopharyngeal sarcoidosis was treated as sarcoidosis, and she was better. Nasopharyngeal involvement is very rare in sarcoidosis but it must be kept in mind.     

Carbohydrate antigen-125 and N-terminal pro-brain natriuretic peptide levels: compared in heart-failure prognostication

Carbohydrate antigen-125 (CA-125) is emerging as a prognostic biomarker of risk in heart failure. In a prospective study, we compared the prognostic values of CA-125 and amino-terminal pro-brain natriuretic peptide (NT-proBNP) in patients with stable heart failure.We enrolled 102 consecutive chronic, stable, systolic-heart-failure patients (68 men and 34 women; median age, 71 yr) from November 2008 through February 2010. We measured baseline NT-proBNP and CA-125 levels and compared their prognostic values. The primary endpoint was all-cause death and other major adverse events, defined as hospitalization for decompensated heart failure or acute coronary syndrome.During a mean follow-up period of 14 ± 2 months, 12 patients died and 35 others sustained major adverse events. We found that CA-125 level significantly correlated with New York Heart Association functional class, pulmonary artery pressure, microalbuminuria, creatine kinase-MB fraction, and hemoglobin, albumin, and NT-proBNP levels. Upon receiver operating characteristic curve analysis, CA-125 and NT-proBNP had similar accuracy in predicting major adverse events and death: for major adverse events, area under the curve (AUC) was 0.699 for CA-125 (P=0.002) and 0.696 for NT-proBNP (P=0.002); for death, AUC was 0.784 for CA-125 (P=0.003) and 0.824 for NT-proBNP (P=0.001). Multivariate Cox regression analysis showed that CA-125 levels greater than 32 U/mL and NT-proBNP levels greater than 5,300 pg/mL had independent prognostic value for major adverse events and death.We conclude that baseline CA-125 and NT-proBNP levels are comparably reliable as heart-failure markers, and that CA-125 can be used for prognosis prediction in heart failure.