Pregnancy outcomes among Egyptian women with systemic lupus erythematosus: A prospective cohort study.

Pregnant patients with systemic lupus erythematosus (SLE) represent a high-risk group. The aim of this study is to describe the pregnancy outcomes among SLE patients who were followed prospectively at a conjoint high-risk pregnancy/rheumatology clinic from 2007 to 2021 and to identify predictors of adverse maternal and fetal outcomes. This study included 201 singleton pregnancies of 123 women with SLE. Their mean age was 27.16 ± 4.80 years, and their mean disease duration was 7.35 ± 5.46 years. Secondary antiphospholipid syndrome (APS) was diagnosed in 77 (38.3%) pregnancies. The pregnancy was planned in 104 (51.7%) pregnancies. Flares occurred in 83 (41.3%) and pre-eclampsia in 15 (7.5%) pregnancies. Full-term pregnancy occurred in 93 (46.3%), fetal loss (miscarriage and intra-uterine fetal death) in 41 (20.4%), and prematurity in 67 (33.3%) of the pregnancies, respectively. Seven neonates died from complications of prematurity, and another one died from cardiac congenital anomalies. In the multivariate analyses, unplanned pregnancy was associated with eight times higher risk of disease flare OR = 7.92 (p < 0.001), lupus nephritis flare during pregnancy increased the odds of pre-eclampsia occurrence four times OR = 3.98 (p = 0.02), while disease flares during pregnancy predicted prematurity OR = 2.49, p = 0.049. Patients with secondary APS had three times increased risk of fetal loss OR = 2.97, p = 0.049. To conclude, unplanned pregnancy, disease flares, and APS have been identified as predictors for adverse maternal and/or fetal outcomes. Pregnancy planning is necessary to reduce maternal and fetal complications.

Evaluation of treatment adherence in patients with Behçet's disease: its relation to disease manifestations, patients' beliefs about medications, and quality of life.

INTRODUCTION/OBJECTIVES: Despite its importance, adherence to treatment has not been sufficiently studied in Behçet's disease (BD). The aim of this study was to evaluate medication adherence in BD using the Compliance Questionnaire of Rheumatology (CQR) and factors potentially affecting it. METHOD: This cross-sectional study included 67 consecutive BD patients including 57 (85%) males with a mean age of 35.1 ± 9.27 years and mean disease duration of 129 ± 91 months. The cumulative clinical manifestations, the Behçet's Disease Current Activity Form (BDCAF) score, and the Vasculitis Damage Index (VDI) were recorded. The CQR, Socioeconomic Status Questionnaire for Health Research in Egypt (SES), the Beliefs about Medication Questionnaire (BMQ), and the Short Form 36 (SF-36) quality of life assessment questionnaire were administered to the patients. Linear regression analysis was done to determine independent predictors of CQR. RESULTS: The mean BDCAF score was 3.27 ± 3.54 and the VDI was 3.36 ± 2.21. The mean CQR score was 69.2 ± 11.79. The CQR score varied significantly among different health sources (p = 0.02), with no relationship detected with other sociodemographic characteristics, nor with clinical characteristics or the SF-36. Among the investigated medications' complexities, severity of side effects showed significantly different CQR scores (p = 0.004), and a weak positive correlation between medications' numbers and the CQR was detected. Predictors for higher CQR scores included the necessity beliefs score of the BMQ (ß = 1.1, p < 0.001); whereas, predictors for lower CQR scores were the harm and concern BMQ subscales ((ß = - 1.5, p = 0.004) and (ß = - 0.72, p = 0.032), respectively). CONCLUSIONS: Beliefs about medications were the only predictor for adherence in our cohort.

Psychiatric and functional neuroimaging abnormalities in chronic hepatitis C virus patients: Is vasculitis a contributing factor?

BACKGROUND AND STUDY AIMS: Central nervous system (CNS) involvement in hepatitis C virus (HCV) infection has different facets such as anxiety, depression, cognitive impairment and vasculitis. We were interested in detecting subclinical CNS involvement in chronic HCV infected subjects with and without systemic vasculitis. PATIENTS AND METHODS: Nineteen patients (15 females and 4 males) with chronic HCV infection (mean age 46.5 ±â€¯7 and mean duration since diagnosis of HCV infection 4.7 ±â€¯4 years, including 6 (32%) Child-Pugh class A cirrhotic patients) and 30 age, sex and education matched healthy control subjects were studied. Thirteen patients had associated vasculitis. Patients and control subjects were assessed using the block design and comprehension subtests of Wechsler Bellevue Adult Intelligence Scale, Wechsler Memory scale (WMS), Beck Anxiety Inventory (BAI) and Beck Depression Inventory (BDI). Brain HMPAO Single Photon Emission Computed Tomography (SPECT) was performed for HCV patients. RESULTS: Patients with HCV had lower scores on the block design test compared to control subjects (8.37 ±â€¯1.89 versus 10.37 ±â€¯1.47, p < 0.001), lower total WMS scores (43.15 ±â€¯10.49 versus 60.27 ±â€¯8.08, p < 0.001) and higher anxiety and depression scores (16.94 ±â€¯10.46 and 37.17 ±â€¯10.38 versus 10.3 ±â€¯4.67 and 28.9 ±â€¯5.99, p = 0.004 and 0.001, respectively). Total WMS were lower in HCV patients with vasculitis compared to those without vasculitis (39.14 ±â€¯9.3 versus 51.17 ±â€¯8.3, p = 0.019) while the block design and comprehension tests, BAI and BDI were not significantly different between both groups. The block design and comprehension tests, WMS, BAI and BDI were not significantly different between cirrhotic and non-cirrhotic patients. Seven patients had different patterns of cerebral hypoperfusion on SPECT, and all of them had associated vasculitis. Abnormal SPECT was associated with lower total WMS scores (35.87 ±â€¯10.8 versus 46.79 ±â€¯8.6 in those with normal SPECT, p = 0.049). CONCLUSIONS: Vasculitis may contribute to the development of neuropsychiatric involvement in HCV patients.

Cardiac findings in patients with Behçet's disease: Facts and controversies.

Behçet's disease (BD) is a systemic vasculitis of unknown etiology. It is characterized by recurrent oral and genital ulcers, uveitis, and a number of systemic manifestations. Although the disease is recognized worldwide, its prevalence is highly variable. A detailed review and analysis of the worldwide published reports on BD showed that not only the prevalence of the disease but also its cardiac complications differ according to the geographic distribution of the studied population. With the exception of France, Greece, Spain, and Italy, very few reports and studies regarding BD have been published from the western countries. Cardiovascular complications are an important cause of poor outcome in patients with BD. Over the past few years, many case reports and studies have been published, providing more facts about these complications. For example, peculiar echocardiographic findings in patients with aortic valve regurgitation and intracardiac thrombi secondary to BD were recently described. The role of these findings in the initial diagnosis of the disease, however, remains to be evaluated. On the other hand, some reports present contradictory results, especially concerning the left ventricular diastolic function, pathogenesis of coronary artery disease, and proper management of the cardiac complications in BD. Importantly, management of these complications is based mainly on the discretion of the treating physician due to the absence of large controlled studies and clear guidelines. This approach sometimes creates inconsistent data and allows controversies to persist. The review presented here will discuss some of the facts and controversies related to cardiac complications in BD.

Assessment of Left Ventricular Diastolic Function in Egyptian Patients With Behçet's Disease.

OBJECTIVES: This study aims to evaluate left ventricular diastolic function in Egyptian patients with Behçet's disease using variable echocardiographic parameters by combining both transmitral and tissue Doppler velocities to overcome the occasional false interpretation of preload dependent transmitral parameters and to detect the correlation between various left ventricular diastolic parameters and different disease manifestations. PATIENTS AND METHODS: Forty patients (35 males, 5 females; mean age 31±8 years; range 19 to 52 years) with Behçet's disease and 32 age- and sex- matched healthy controls (26 males, 6 females; mean age 28±6 years; range 19 to 60 years) were enrolled. Left ventricular diastolic function was assessed using an algorithm that combined transmitral and tissue Doppler derived parameters. RESULTS: Mean disease duration of the patients was 7.4±6 years. Left and right ventricular systolic function was normal in both groups. The transmitral E/A was<1 in six patients (15%). There was no difference between patients and controls regarding the other left ventricular diastolic parameters. Compared to patients with an E/A ratio >1, patients with E/A<1 were significantly older and had a longer disease duration (p=0.001 and p<0.001, respectively). All the other echocardiographic parameters in both groups were comparable. CONCLUSION: Egyptian patients with Behçet's disease have normal left ventricular diastolic function regardless of disease duration and activity.

Silent central nervous system involvement in Egyptian Behçet's disease patients: clinical, psychiatric, and neuroimaging evaluation.

Behçet's disease (BD) is an idiopathic multisystem disorder. Involvement of CNS occurs in 4-48% of cases. This study was designed to evaluate the prevalence of subclinical neuropsychiatric affection in asymptomatic Egyptian BD patients using psychometric tests and brain imaging with single photon emission computed tomography (SPECT) and magnetic resonance imaging (MRI), also to assess possibly associated clinical predictive variables. Twenty-five BD patients without overt CNS involvement and ten healthy controls matched for age, education, and sex completed a comprehensive neuropsychological battery including Hamilton scales for anxiety and depression and Wechsler memory scale-revised. Disease activity was assessed using Behçet's Disease Current Activity Form (BDCAF). SPECT was done for all subjects, and 12 patients underwent brain MRI. Compared to controls, 23 (92%) and 24 (96%) patients had anxiety and depression scores respectively above normal range; also, BD patients had significantly lower memory quotient (MQ). SPECT revealed abnormalities in 16/25 (64%), while in 3/12 patients (25%), MRI was abnormal. Subjects with abnormal SPECT had significantly higher ages than those with normal SPECT (P = 0.02) and were more frequently males (P = 0.03). No statistically significant differences between cases with normal or abnormal SPECT were found regarding disease duration, frequency of headache, BDCAF, frequency of active eye disease, major vascular involvement, mean Hamilton anxiety and depression scores, and mean MQ. Early diagnosis of neurological involvement in BD is important in reducing or preventing complications. Neuropsychiatric evaluation and HMPAO brain SPECT were found to be useful for detecting subclinical neurological abnormalities in BD patients.

A retrospective analysis of treatment outcomes in patients with hepatitis C related systemic vasculitis receiving intravenous methylprednisolone and cyclophosphamide.

The aim of this work is to describe the outcome of a series of patients with hepatitis C virus (HCV)-related vasculitis who were treated with corticosteroids and I.V. cyclophosphamide without receiving any antiviral therapy. The data of 16 patients with HCV infection and vasculitis were retrospectively analyzed for the treatment outcome in the present study. Eleven patients were females (68.8%) with a mean age of 49.6 ± 10.0 years. Nine patients (56.2%) had medium-sized vessel vasculitis (group A) and seven patients (43.8%) had small vessel vasculitis (group B). Disease activity was assessed using the Birmingham Vasculitis Activity Score (BVAS 2003) and organ damage was assessed by the Vasculitis Damage Index (VDI). HCV infection was confirmed in all patients by the detection of antibodies to HCV in serum by ELISA and HCV RNA using qualitative PCR. Quantitative PCR was done using the branched DNA technique. None of our study patients had received antiviral therapy, but they all received I.V.-pulsed cyclophosphamide monthly for 6 months, then every 3 months for six times if needed, preceded by I.V. methylprednisolone. Twelve patients (75%) had undetectable viral load by the quantitative technique. The drop in mean BVAS recorded at different intervals was highly significant. Although there was a drop in the VDI mean between the first and second reading, it was not statistically significant. All patients responded to treatment. Seven patients (43.8%) had relapse. Two patients died (12.5%). One patient died from renal failure (group B) and another died from sepsis (group A). The treatment outcomes were not statistically significant between the two vasculitis groups. A subset of patients with HCV-related vasculitis and with low levels of viremia can be safely treated with corticosteroids and cyclophosphamide alone. Despite successful treatment, a significant proportion of patients relapse and some develop severe complications and death.

Renal disease in systemic sclerosis with normal serum creatinine.

Prognosis of systemic sclerosis largely depends on involvement of internal organs. The aim was to evaluate renal impairment in patients with systemic sclerosis by measuring the Glomerular filteration rate (GFR) and then calculating the GFR using the Cockgroft and Gault formula and the Modification of Diet in Renal Disease Equation (MDRD) formula. Thirty one scleroderma patients were recruited from the Rheumatology and Rehabilitation Department, Cairo University Hospitals, mean age 43.25 +/- 11.28 years, 31 healthy controls were included. Disease severity was done using Medsger score. GFR was measured using classical Gates method TC99mDTPA. The modified Cockcroft and Gault formula and equation 7 from the MDRD were used for calculation of GFR. All patients had within normal serum creatinine levels. A normal GFR (>89ml/min) was found in 45.1%. Gates method showed reduced GFR was reported in 54.9%. Stage II chronic kidney disease (60-89 ml/min) found 32.3%, and stage III (30-59 ml/min) in 22.6%. The formulae used showed reduction of GFR in 35.29% of those affected by the Cockcroft-Gault and in 41.17% of those affected using the MDRD. No correlation to patients' age, disease duration, or severity. A positive correlation was also reported between the presence of renal involvement and pulmonary vascular involvement p = 0.04. Gates method showed reduction of the GFR in 54.9% of the systemic sclerosis patients. The formulae used were not as precise as the measured GFR in diagnosing all cases with subclinical renal involvement. Patients with systemic sclerosis should be screened for renal involvement irrespective of disease severity or duration.