Evaluation of myocardial perfusion in patients with Behçet’s disease

Aim

To estimate the prevalence of silent myocardial ischemia (SMI) in patients with Behçet’s disease (BD) and to identify a subgroup of patients at higher risk for the presence of SMI. Materials and Methods We evaluated 41 patients (mean age 42.8 ± 12.3 years) with BD and 35 healthy control subjects. Treadmill exercise test and thallium-201 myocardial perfusion single photon emission computed tomography (SPECT) were performed in all subjects. Coronary angiography was performed in all patients with a diagnosis of SMI in Behçet’s group.

Results

All subjects had normal resting electrocardiograms. Eight patients with BD (19.5%) had evidence of ischemia on exercise testing and myocardial perfusion SPECT. Only one SMI positivity (2.9%) was recorded in the control group. Significant coronary stenosis was not found with coronary angiography in the patients with a diagnosis of SMI in Behçet’s group. SMI positivity was recorded in 2 of 18 female patients (11%) and in 6 of 23 male patients (26.1%) with BD (p = 0.429). The mean duration of BD was 13.8 ± 2.6 years in patients with SMI and 7 ± 4.1 in patients without it (p < 0.001). Seven of the 8 patients (87.5%) with SMI had a duration of BD of greater than 10 years.

Conclusions

The results of this study show that the prevalence of SMI is high in patients with BD. Based on our findings, screening with myocardial perfusion scintigraphy may be recommended for patients with duration of BD greater than 10 years.     

MRI findings of myositis in Behçet disease

We report the magnetic resonance imaging findings in a case of localized myositis in a 23-year-old man with long-standing Behçet disease.     

Imaging pearls of pediatric Behçet’s disease

ObjectiveTo emphasize the diverse diagnostic imaging findings of pediatric Behçet's disease and to define the fundamental imaging clues for pulmonary, vascular, gastrointestinal and central nervous system involvements of Behçet’s disease in pediatric age group. We also aim to list the major imaging differences of Behçet's disease in childhood and adulthood.ConclusionThe diagnosis of pediatric Behçet’s disease is challenging. Imaging can narrow the differential diagnosis by using some substantial clues and prevent diagnostic delays.     

Evaluation of myocardial perfusion and function by gated SPECT in patients with Behçet’s disease

Background

Behçet’s disease (BD) is a systemic multi-system vasculitis that can have a wide range of effects on the cardiovascular system.

Objective

To determine the existence of myocardial perfusion defects caused by coronary microvascular dysfunction in BD and to evaluate coronary arterial distribution and left ventricular systolic function by gated single-photon emission computed tomography (SPECT).

Methods

The study population consisted of 23 (15 men and 8 women) patients with BD and 20 healthy controls (12 men and 8 women). Technetium-99m methoxyisobutylisonitrile (Tc-99m MIBI) gated SPECT studies were performed at stress and rest in a 2-day protocol. Stress and rest left ventricular ejection fraction (LVEF) were calculated. Using non-gated SPECT images myocardial perfusion scores [summed stress score (SSS), summed rest score (SRS), summed difference score (SDS), and fix defect score (FDS)] and perfusion defect extent as percentage (stress, rest ischemic, and fix %LV) were determined. Using gated SPECT images, wall motion score indices (stress wall motion score indices and rest wall motion score indices) were calculated. Coronary angiography (CAG) was applied to patients with abnormal myocardial perfusion scintigraphy (MPS).

Results

The mean ages of the BD and control groups were 39.3 ± 10.6 years and 36.2 ± 8.3 years, respectively. No statistically significant differences were observed between the two groups regarding clinical features and cardiologic findings. Abnormal MPS was found in 13 (56.5%) of the BD patients; 3 patients had non-transmural infarcts and 10 patients reversible perfusion defects. Reversible perfusion defects were also found in two controls (10.0%). When the two groups were compared regarding the gated SPECT findings, differences were determined in the following parameters; SSS, SRS, SDS, FDS, stress and rest LVEF, stress and rest %LV, and stress and rest WMSI. In the BD group, when gated SPECT results were compared between those with and without abnormal MPS, differences were determined in SSS, SRS, SDS, FDS, stress and rest %LV, and stress and rest WMSI. Epicardial coronary arteries were normal in CAG.

Conclusions

Myocardial perfusion and function are disturbed owing to influenced coronary microvascularity in BD, and CAG is frequently observed to be normal. Gated SPECT is a non-invasive reliable method that simultaneously evaluates the existence, extent and severity of myocardial ischemia or infarction and the wall movements in cardio-Behçet.

     

Proton MRS in Behçet’s disease with and without neurological findings

Our aim was to investigate whether neurological impairment in Behçets disease (BD) can be assessed by means of proton MRS and whether it can assist in prognosis. We used single-voxel MRS to measure metabolites in regions of normal-appearing pons, basal ganglia and periventricular white matter (PWM) in 32 patients with chronic BD patients with and without neurological deficits and 29 control subjects. Patients had significantly higher N-acetylaspartate (NAA)/creatine (Cr) and choline (Cho)/Cr ratios in the basal ganglia than the controls. The Cho/Cr ratio in the PWM was also significantly higher in the patients. MRS enabled clear discrimination of patients and controls and also revealed spectral differences between non-neuro-Behçets disease and neuro-Behçets disease in the basal ganglia. MRS can be used to assess brain involvement in BD even if structural changes are absent.     

Evaluation of gastrointestinal involvement of Behçet’s disease by nuclear medical techniques

To evaluate the value of nuclear medicine procedures in the diagnosis of gastrointestinal involvement of Behcet's disease in asymptomatic patients, Tc-99m human immunoglobulin (HIG) and Tc-99m leucocyte (LC) whole body scintigraphies were performed on 30 patients with major symptoms of the disease. Comparison of the results with other diagnostic techniques showed that Tc-99m HIG whole body scanning can be a useful diagnostic aid before the disease becomes clinically active in the gastrointestinal system.     

The usefulness of Tc-99m-MDP bone scintigraphy in detection of articular involvement of Behçet’s disease

Articular involvement was reported to be present in approximately 5-76% of Behçet patients. Therefore, we need a useful non-invasive method to detect articular involvement early in Behçet patients with nonspecific complaints. We aimed to evaluate the usefulness of^99mTc-methylene diphosphonate (Tc-99m-MDP) bone scintigraphy in the detection of the articular involvement of Behçet’s disease (BD). Bone scintigraphy with Tc-99m-MDP was performed in 32 (17 male, 15 female) consecutive patients with BD. The sacroiliac (SI) joints with SI index higher than 1.34 were diagnosed as having sacroiliitis. Although joint complaints were present in only 8 (25%) patients, we detected joint involvement by scintigraphy in 27/32 (84.4%) Behçet patients mostly affecting the knees (62.5%), ankles (59.4%), SI joints (25%), wrists (21.9%), shoulders (18.7%), elbows (12.5%) and hips (3.1%). The articular involvement was monoarticular in four cases (12.5%) and was oligoarticular in the remaining. There was no correlation between joint involvement and age, gender, disease duration, drug usage or other clinical manifestations. Despite the fact that our patients were clinically asymptomatic and had normal pelvis radiography, sacroiliitis was found in 8 patients (25%). Bone scintigraphy is sensitive in the diagnosis of joint involvement allowing earlier diagnosis and showing the presence of articular involvement, especially in SI joints.     

Arthropathy in Behçet disease: MR imaging findings in two cases

Arthropathy is part of the protean manifestations of Beh?et disease. Imaging findings reported thus far have been based on those of conventional radiography. Magnetic resonance imaging in two cases of Beh?et disease with arthropathy demonstrated synovial thickening and effusion, as well as myositis manifested by high signal intensity on T2-weighted images.     

Use of technetium-99m hexamethylpropylene amine oxime SPET for the study of cerebral blood flow reactivity after acetazolamide infusion in patients with Behçet’s disease

The purpose of this study was to characterise the nature of the baseline perfusion defects found in patients with Beh?et’s disease using hexamethylpropylene amine oxime single-photon emission tomography in conjunction with acetazolamide test (Acz SPET). Eleven patients underwent both baseline and Acz SPET. Regions of interest (ROIs) were drawn on the areas with decreased perfusion (D-ROI) and, in the same section, on areas with normal perfusion (N-ROI). The ROIs were then repositioned on the corresponding section on Acz SPET. The mean ROI counts were then transformed into a perfusion index value (PIV) with reference to the global brain counts. In total we found 24 D-ROIs (17 in the cortical and 7 in subcortical grey matter). The influence of Acz infusion was selectively registered in the D-ROIs, where PIVs changed from 1.23±0.17 (baseline SPET) to 1.63±0.23 (Acz SPET) (P<0.001). No significant difference was seen in the N-ROIs (1.46±0.21 and 1.40±0.17, respectively, on baseline SPET and Acz SPET). Our results demonstrate that Acz infusion increases the regional cerebral blood flow within baseline grey matter perfusion defects. This finding suggests that baseline perfusion abnormalities could reflect a disconnection rather than local vasculitic involvement. Received 10 November 1999 and in revised form 29 January 2000     

Pulmonary involvement in Behçet's disease: evaluation of 123 I-MIBG retention

Pulmonary involvement in Beh?et's disease (BD) is reported to indicate poor prognosis and high mortality. Our aim was to evaluate the pulmonary vascular status in BD. As 123I-meta-iodobenzylguanidine (123I-MIBG) shares the same uptake, storage and release mechanisms as norepinephrine, associated with the functional state of pulmonary endothelium, it may reflect endothelial injury. Twenty-five patients (13 males, 12 females; mean age, 36.44 years) and 12 age-matched controls were included. After intravenous injection of 123I-MIBG, thorax images were taken at 15 min and 4 h. Appropriate regions of interest were drawn, and heart to mediastinum (H/M) ratios and lung retention indices (LRI) were calculated. The difference between the LRI of controls (75.6+/-4%) and patients (87.7+/-10%) was found to be extremely significant (P<0.005). The LRIs of active (92.85+/-12%) and inactive (83.65+/-5%) states of BD were significantly different (P<0.05) from each other. There was no significant difference between the H/M ratios of controls (2.4+/-0.27) and patients (2.39+/-0.31) or between patients in active and inactive disease states. Our study revealed prolonged lung retention of 123I-MIBG in BD, probably reflecting the severity of the disease. In conclusion, 123I-MIBG lung retention is a valuable finding in the evaluation of pulmonary vascular status and may be a potential marker of prognosis in BD.     

Hemoptysis revealing a pulmonary arterial aneurysm on Behçet disease in children: role of computed tomography

Beh?et's disease is a systemic condition whose discovery during hemoptysis on a pulmonary artery aneurysm is rare, especially in children. Through the case of a 11-year-old boy, the authors emphasize the importance of imaging in diagnosis of this disease, whose progression can be fatal if hemoptysis is violent.     

Imaging of the small bowel: Crohn’s disease in paediatric patients

In more than 20% of all patients, the Crohn’s disease presents before the age of 18years. The diagnosis and management of Crohn’s disease in children has changed dramatically over the last decade, mainly due to increased awareness, availability of newer diagnostic modalities such as magnetic resonance imaging (MRI) and newer, more powerful treatments such as biologics. Imaging of the small bowel is needed for diagnosis, management, follow-up and also evaluation of the disease in terms of location, extent, activity and complications. We review all the methods (barium examinations, ultrasonography, computed tomography, MR, and computed tomography- positron emission tomography) commonly used for imaging the small bowel in paediatric patients with Crohn’s disease analyzing the advantages and disadvantages of each modality, with particular emphasis on MR imaging.     

Multiple Intrahepatic Artery Aneurysms in a Patient with Behçet’s Disease: Use of Transcatheter Embolization for Rupture

Intrahepatic artery aneuryms are a rare and potentially life-threatening condition. We present the first case in the English literature of multiple intrahepatic artery aneuryms in a patient with Behçet’s disease who presented acutely with rupture. The ruptured aneurysm was treated successfully with transcatheter arterial coil embolization-CT and clinical follow-up confirming a good result. We discuss the management dilemma with regard to prophylactic embolization of the numerous other small asymptomatic intrahepatic aneurysms in this same patient.     

Diffusion-weighted imaging in chronic Behçet patients with and without neurological findings

Our aim was to investigate whether neurological impairment in chronic Behçets disease (BD) patients with normal appearing brain can be assessed by means of diffusion-weighted imaging (DWI). The averaged apparent diffusion coefficient (ADC) values were calculated in 22 different radiologically normal appearing brain regions in 32 patients with and without neurological findings and 20 control subjects. The ADC values in bilateral frontal, temporal and occipital normal appearing white matter were significantly higher in the patient groups compared with the control subjects (p<0.05). In these brain regions, DWI revealed differences in the ADC values between patients with neurological findings (including symptomatic and neuro-Behçet patients) and the asymptomatic patient group. The similarity of the ADC values of patients without symptoms to those of the control group allowed clear discrimination between patients with and without neurological findings. DWI may serve to assess subclinical neurological involvement in BD, even when structural changes are absent.     

Pseudoaneurysm of the renal interlobar artery in Behçet's disease

Beh?et's disease is a rare clinical event characterized by recurrent oral and genital ulcers, and iridocyclitis. Vascular involvement is a rare but serious form of the disease. We present a case of renal interlobar artery pseudoaneurysm occurring in a 20-year-old male patient with a 5-year history of Beh?et's disease, who had been admitted because of right flank pain. Findings obtained on abdominal ultrasonography, abdominal computed tomography, and renal angiography confirmed a right renal pseudoaneurysm and subcapsular hematoma.     

Assessment of alveolar epithelial permeability in Behçet’s disease with <Superscript>99m</Superscript>Tc-DTPA aerosol scintigraphy

Objective

Behçet’s disease (BD) is a multisystem disorder characterized by vasculitis, and consists of a triad of recurrent ulcers of the oral and genital mucosa with relapsing uveitis. The prevalance of pulmonary involvement varies in the range of 1–10% in various studies and its complications are severe and life threatening. In this study, we investigated the changes of pulmonary epithelial permeability of patients with BD using technetium-99m diethylene triamine penta-acetic acid (^99mTc-DTPA) aerosol scintigraphy, so as to begin the therapy regimen as soon as possible.

Methods

Twenty-one nonsmoking patients with BD (8 women, 13 men; mean age 38.67 ± 8.86 years) and 15 healthy volunteer nonsmoking controls (8 women, 7 men; mean age 50.87 ± 12.45 years) underwent ^99mTc-DTPA aerosol inhalation scintigraphy and pulmonary function tests (PFTs). Subjects inhaled 1480 MBq of ^99mTc-DTPA for 4 min in the supine position. Scintigraphic data were recorded dynamically (1 frame/min) in the posterior projection on a 64 × 64 matrix for a 30-min period using a double-headed gamma camera (Infinia, GE, Tirat Hacarmel, Israel) equipped with a low-energy all-purpose parallel hole collimator. Half time of ^99mTc-DTPA clearance (T _1/2) was calculated by placing a mono-exponential fit on the curves. Penetration index (PI) was also calculated by dividing the peripheral total counts by the sum of the peripheral and central total counts on the first minute image, in order to quantify the distribution of the inhaled aerosol.

Results

The clearance half time of ^99mTc-DTPA radioaerosols in the BD patients (24.81 ± 6.22 min) was faster than in the normal control group (46.53 ± 22.41 min) (P = 0.004). There was also a significant difference between PI of the patients with BD (0.15 ± 0.03) and that of the controls (0.21 ± 0.06) (P = 0.002). No correlation was found between the mean T _1/2 values of ^99mTc-DTPA clearance or the spirometric measurements in the BD patients. Penetration indices were not correlated with PFT in the BD patients.

Conclusions

Lung epithelial permeability of the patients with BD was significantly higher than that of the normal subjects. The results of this study demonstrated that the assessment of lung epithelial permeability using ^99mTc-DTPA aerosol scintigraphy could predict the presence of lung involvement in the early stages of BD.

     

Doppler ultrasonography findings in ocular Behçet's disease

The aim of this prospective study was to determine and compare the orbital Doppler ultrasonography parameters of patients with Behçet's disease (with or without ocular involvement) with those of healthy subjects. We evaluated ophthalmic artery (OA), central retinal artery, posterior ciliary artery (PCA), central retinal vein, and superior ophthalmic vein (SOV) flow velocities and resistance indices (RIs). Detection of the decreased flow velocities in the OA and SOV and the increased RI in the OA and PCA might allow the identification of active period of patients with Behçet's disease.     

Use of ACE-inhibitors and falls in patients with Parkinson’s disease

Falls represent a major concern in patients with Parkinson’s disease (PD); however, currently acknowledged treatments for PD are not effective in reducing the risk of falling. The aim was to assess the association of use of ACE-inhibitors (ACEIs) and angiotensin receptor blockers (ARBs) with falls among patients with PD.We analysed data of 194 elderly with PD attending a geriatric Day Hospital. Self-reported history of falls that occurred over the last year, as well as use of drugs, including ACEIs and angiotensin II receptor blockers (ARBs) were recorded. The association of the occurrence of any falls with use of ACEIs, and ARBs was assessed by logistic regression analysis. The association between the number of falls and use of ACEIs, and ARBs was assessed according to Poisson regression.In logistic regression, after adjusting for potential confounders, use of ACEIs was associated with a reduced probability of falling over the last year (OR = 0.15, 95% CI = 0.03–0.81; P = 0.028). This association did not vary with blood pressure levels (P for the interaction term = 0.528). Also, using Poisson regression, use of ACEIs predicted a reduced number of falls among participants who fell (PR = 0.31; 95% CI = 0.10–0.94; P = 0.039). No association was found between use of ARBs and falls.Our results indicate that use of ACEIs might be independently associated with reduced probability, and a reduced number of falls among patients with PD. Dedicated studies are needed to define the single agents and dosages that might most effectively reduce the risk of falling in clinical practice.