Antiphospholipid Syndrome in Patient with Portal Venous Thrombosis: Case Report
Antiphospholipid syndrome (APS) is defined by the presence of arterial and venous thrombosis, recurrent fetal death, cerebrovascular accidents, hemolytic anemia, thrombocytopenia and various manifestations on different organs in the presence of anticardiolipin antibodies (aCL) and or lupus anticoagulant (LA).
It was reported in early 1980's. This syndrome is the most common cause of acquired thrombophilia. There is no consensus for treatment among physicians. Overall there is a general agreement that patients with recurrent thrombotic episodes require life-long anticoagulation therapy and those with recurrent spontaneous abortions require anticoagulation therapy (low molecular weight heparin) and low dose aspirin during most of gestation. Immunosuppresion seems to be ineffective exept in patients with fulminate multiple organ failure i.e. catastrophic antiphospholipid syndrome where plasmapheresis can also be used.
We present a case of 31 year old woman with primary APS and portal venous thrombosis (PVT), without any recognizable autoimmune disease. She has 4 spontaneous abortions, calf thrombosis, gangrene of one toe, refractory cutaneous ulcer on the heel and livedo reticularis. She is positive for aCL and LA, with hypergammaglobulinemia.
The Diagnostic Value of Anti-Cyclic Citrullinated Peptide Antibodies (anti-CCP) in Patients with Rheumatoid Arthritis
A cross-sectional study was carried out to analyze the prevalence of anti-CCP antibodies and IgM RF in the sera of 160 randomly selected patients from the Rheumatology Department: 60 with RA, 50 with other rheumatic diseases (non-RA), 50 healthy controls (HC). The mean age of the group was 50.06+/-11.9 years. There were 141 females (88.1%) and 19 males (11.9%). RA patients fulfilled the revised ACR criteria. The mean duration of the disease was 82.4 months. Anti-CCP ELISA kit and IgM RF Latex test were used. The mean anti-CCP values were as follows: RA 60.4+/-57.6, non-RA 2.1+/-3.6, HC 1.3 +/-0.4 U/mL. Respectively, the mean values of IgM RF were: RA 515.8+/-525, non-RA 102+/-294, HC 15+/-57.5. Forty out of 60 (66.6%) RA patients were anti-CCP positive. Forty one out of 60 (68.3%) RA patients were positive for IgM RF. As expected, anti-CCP showed comparable sensitivity (66.8% vs. 68.3%) and higher specificity (98% vs. 87%) than IgM RF, at optimal cut-off values. The presence of either anti-CCP or IgM RF increased the testing sensitivity for the diagnosis of RA to 76.6%. AUC was greater for anti-CCP than for IgM RF (0.92 vs. 0.82).
Rheumatoid arthritis is an inflammatory arthritis characterized by synovial tissue inflammation that leads to structural damage and disability. There are several treatment options available, which include glucocorticoids, DMARDs and biologics given alone as monotherapy or in a variety of combinations. Recent evidence has shown that early treatment is important in reducing the rate of progression of erosions and decreasing disability. The lack of adequate statistical data on number of patients that are eligible for first-line therapy/monotherapy of rheumatoid arthritis in Macedonia, triggered this epidemiological analyse describing eligible patients for first-line treatment/monotherapy distributed by gender, age and geographical allocation. The study was conducted by fulfilling a tailored questionnaire every two months in a period of six months (September 2017-February 2018) by including summarized data not related to personal data of patients nor specific drug information. The results have shown that a total of 115 patients in Macedonia are eligible for first-line therapy, whereby 54 (46%) patients were eligible for monotherapy of rheumatoid arthritis. Precise determination of these data provides patients’ determination by geographical allocation and proper selection of the best treatment option and optimized therapy for each patient, furthermore when subcutaneous formulation of tocilizumab is available as an effective clinically proven treatment option for RA
Introduction: Atherosclerosis in young and premenopausal women with systemic lupus erythematosus (SLE) is frequent, premature and progressive. Although asymptomatic or with atypical clinical presentation, the patients are at high risk of cardiac events. Aim of this study is to estimate the risk profile for atherogenesis and the prevalence of myocardial perfusion abnormalities with 99mTc myocardial perfusion scintigraphy (MPS) in young and premenopausal women.
Material and methods: Sixty female patients, aged 30-72 years (divided into two subgroups - patients under 45 years of age and patients over 45 years), diagnosed with SLE for over of 5 years, in active phase of the disease were analyzed for disease activity scores (SLEDAI), the immunologic status of the disease (ANA and a-DNA antibodies in the serum), procoagulant tendency (antiphospholipid antibodies-APhL and lupus-anticoagulant-LAC), the activity of the inflammatory process (hsCRP), the anti-SLE therapeutic approach and the presence of traditional risk factors for atherosclerosis (BMI, smoking, hypertension, hyperlipidemia, diabetes, and familial history for the CAD). Using one-day Dipyridamol – Rest 99mTc SPECT Gated MPS SPECT the extent, severity and reversibility of myocardial perfusion abnormalities were estimated, along with summed scores at stress, rest and summed difference scores and left ventricle volumes and ejection fraction.
Results: Abnormal MPS SPECT were detected in 27/60 or in 45% of patients, with one vessel affection of 66.7% (18/27pts) of LAD and 14.8% (4/27pts) o RCA and with two vessel disease of LAD/RCA in 2/27 pts (7.4%) and LAD/Cx in 3/27pts (11.1%). Myocardial perfusion abnormalities were equally prevalent in subgroups of patients younger than 45 years (44,4%) and in patients older than 45 years (45.5%) (ns). The subgroups did not differ significantly concerning the extent of perfusion abnormalities (9,8±3.2% of LV myocardial mass vs. 9,8±7.1%,ns), their severity (with predominance of mild perfusion defects, 48,6% vs. 51,3%,ns) and reversibility (reversible in 41.3% and 58.6%, ns). The differences between the summed scores of severity and the extent of ischemia in the two subgroups were statistically nonsignificant. Younger patients had significantly higher end-diastolic, end-systolic and stroke volumes during stress and rest conditions, compared to older patients (p<0,01) although there were no differences in systolic function, which was not affected in either of the groups as expressed threw ejection fraction.
Although nonsignificant, younger patients had higher values of hsCRP and higher procoagulant activity (positive aPhL, LAC) while they were with more active disease activity, with higher SLEDAI score compared to older patients (p=0.028). Higher SLEDAI score and LV volumes, especially EDV at stress were identified as predictor of abnormal MPS in younger groups and more aggressive multidrug anti SLE treatment as predictor of normal MPS.
Conclusion: The prevalence and characteristics of myocardial perfusion abnormalities in young SLE are equal as the same in older SLE patients, which indicates the presence of premature, accelerated atherosclerosis in young cohort of patients with SLE. Younger SLE patients with pure disease control (higher SLEDAI score, less aggressive treatment, high hsCRP values and pronounced procoagulant tendency) should undergo screening for myocardial perfusion abnormalities s using 99mTc MIBI MPS)