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.

Seasonal variation of lupus nephritis in a cohort of Egyptian patients.

INTRODUCTION: Systemic lupus erythematosus is an autoimmune multisystem disease; renal affection is one of its most common manifestations. The effect of environmental factors on lupus nephritis flares is not fully understood. METHODS: This is a retrospective study that included 200 patients with lupus nephritis flares. All patients had confirmed diagnosis of lupus nephritis on histopathological examination. Lupus nephritis flares were defined by either (1) nephritic flare: defined as increased proteinuria or serum creatinine concentration; abnormal urinary sediment or a reduction in creatinine clearance, or (2) proteinuria flare defined as persistent increase in proteinuria > 0.5-1.0 g/day after achieving complete remission; doubling to > 1 g/day after achieving partial remission. The time of renal flare (month of the year) was recorded to determine the effect of seasonal variation on lupus nephritis flares. RESULTS: The median age for the patients was 33 years (IQR = 13); 92% of patients were females. The median duration of lupus was 7 years (IQR = 6). The median serum creatinine was 1.4 mg/dl, median serum urea level was 32, and median UPCR was 2.4 gm/dl. The highest incidence of flares occurred in June (14%) and July (12.5%) (p = 0.003). CONCLUSION: Seasonal pattern of LN flare was observed in our study in Egyptian cohort of patients, with most flares observed during meteorological summertime. Larger studies are needed to confirm this seasonal pattern. Key Points • Flares of lupus nephritis are common in patients with systemic lupus erythromatosus. • A seasonal pattern of flares of lupus nephritis was observed in our study. This seasonal pattern has been observed by previous studies in variable ethnicities and variable climatic circumstances.

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.

Value of certain echocardiographic findings in the initial suspicion of Behçet's disease.

PURPOSE: To describe the echocardiographic findings detected as first manifestations of Behçet's disease (BD) and compare these findings with those detected in established cases of BD receiving regular medical treatment. METHODS: Two groups of patients were studied. Group 1 comprised 41 patients with BD on regular medical treatment. Group 2 comprised 5 previously healthy patients who presented to the cardiology department because of cardiac symptoms and were diagnosed in retrospect during hospitalization as BD. Thirty-two age- and sex-matched individuals served as control subjects (control group). All patients and controls underwent complete M-mode, two-dimensional, and Doppler transthoracic echocardiographic examinations. RESULTS: Of the 41 treated patients with BD (mean age: 32 ± 8 years, 90% males, mean duration since diagnosis: 14.5 years), only 1 patient was found to have severe aortic regurgitation secondary to aortic root dilation. On the other hand, the 5 previously healthy patients who presented with cardiac symptoms (mean age: 24 ± 6 years, all males) had significant cardiac involvement and evident echocardiographic findings (P < 0.001). Four cases had intracardiac masses: 3 in the right atrium (RA), 1 in the right ventricle (RV), while the last patient had pericardial effusion (PE). All these patients were diagnosed in retrospect as BD. The RA masses disappeared on medical therapy, while the RV mass was surgically excised and proved to be multiple thrombi histopathologically. The patient with PE had recurrent attacks of massive effusion so a pericardial window was performed surgically. CONCLUSION: Diagnosis of BD might be initially suspected by the cardiologists based on certain echocardiographic findings, namely the presence of right-sided masses. Diagnosis of BD in such patients has important therapeutic implications and accordingly prognostic value.

Camptodactyly, arthropathy, coxa vara, and pericarditis syndrome among egyptians.

OBJECTIVE: To highlight the clinical, radiological, and pathological presentations of 10 Egyptian patients with camptodactyly, arthropathy, coxa vara, and pericarditis (CACP) syndrome. Methods. Ten cases underwent a full history, complete clinical examination, laboratory and radiological investigations (and magnetic resonance imaging, MRI, of knee joints); arthroscopic histopathological synovial studies were performed in 6 cases. Results. Camptodactyly and large joint arthropathies were present in all cases. The onset was at birth in 5 cases, and consanguinity was present in all cases. Laboratory investigations were normal in all cases (normal complete blood cell count, erythrocyte sedimentation rate, absent rheumatoid factor, and antinuclear antibody). Synovial fluid analyses were acellular in 3, hypocellular in 4, and moderately cellular in 2 cases. Histopathology revealed noninflammatory synovial hyperplasia in the 6 synovial biopsies obtained, with multinucleated giant cells in 4 of them. Plain hip radiology revealed short broad femoral neck and widening of joint space in all cases, with coxa vara in 9 cases. MRI of the knees showed rim-like enhancement of the lining of the fluid filled bursae in 7 cases, and homogenous enhancement pattern in 2 cases. No evident cartilage destruction existed in any case. CONCLUSION: Our cases represent a familial syndrome of noninflammatory arthropathies associated with camptodactyly and coxa vara. The complete picture of the syndrome may be related to disease duration, and MRI is a useful tool in diagnosis. Physicians should be aware of the syndrome, to avoid misdiagnosis with other pediatric connective tissue diseases.

Tumor necrosis factor-alpha and neuropsychiatric lupus erythematosus: relation to single photon emission computed tomography findings.

Abstract This study was designed to highlight the relation of tumor necrosis factor-α (TNF-α) to neuropsychiatric lupus (NPLE) manifestations. The relation of TNF-α to the type of single photon emission computed tomography (SPECT) findings in this context was also studied. Twenty-one systemic lupus erythematosus (SLE) females, mean age 27.57 ± 9.89 years, and twenty age-matched normal females (controls), were subjected to TNF-α assessment. Different clinical and neuropsychiatric manifestations were evaluated. SPECT was carried out for all patients. The results showed that the mean TNF-α level (pg/ml) was significantly raised in patients compared with controls (167.8 ± 102.5 versus 64 ± 50.2, respectively, P < 0.005). Thirteen patients (69.1%) had NPLE manifestations. NPLE patients had a significantly higher mean TNF-α than patients without NPLE (203 ± 102.8 versus 109 ± 47.3, respectively, P < 0.03). Positive SPECT findings were found in 18 lupus patients (85.7%), including all 13 patients with NPLE (100% sensitivity), with a multiple focal pattern of hypoperfusion being the most frequent type (9/13), followed by diffuse (3/13), and then single focal pattern (1/13). The mean TNF-α was significantly higher in patients with multiple focal pattern (P < 0.001). In conclusion, results of this work support the hypothesis that TNF-α could be involved in the pathogenesis of NPLE, and hence, it could be speculated that the evolving anti-TNF therapy can play a potential role in the management of this disease.