Bibliographic Details
| Title: |
Clinical Spectrum and Outcome in Primary Antiphospholipid Antibody Syndrome: A Retrospective Observational study from a South Indian tertiary care centre. |
| Authors: |
Tiwari, Vishwa Prakash1, Sankarlingam, Rajeswari2, Sandhu, Akanksha1, Chilukuri, Balaji3 vishwaprakashtiwari430@gmail.com, Prabhu, Nidhi Ramesh1, Muniraju, Tejas1, Memon, Sharmin1, Ibrahim, Tariq4 |
| Source: |
European Journal of Cardiovascular Medicine. 2024, Vol. 14 Issue 6, p111-115. 5p. |
| Subject Terms: |
*FACTOR V Leiden, *PROTEIN S deficiency, *VENOUS thrombosis, *PHOSPHOLIPID antibodies, *SYMPTOMS, *CLINICAL epidemiology |
| Abstract: |
Aim and Objective: To describe the epidemiology, clinical-serological profile, and outcome of primary APS in a South Indian tertiary care center. Material and methods: This retrospective study, conducted in the Clinical Immunology and Rheumatology Department at SRMC/SRIHER Chennai from 2018 to 2024, included 40 patients with the primary anti-phospholipid syndrome (PAPS) as defined by the modified Sapporo criteria. Patients with conditions like protein C or S deficiency, hyperhomocysteinemia, Factor V Leiden mutation, or other autoimmune diseases were excluded. Data on demographics, clinical presentation, lab results, and treatment outcomes were gathered. Anticardiolipin (aCL) and anti-β-2 glycoprotein I (β-2GPI) antibodies (IgG and IgM) were measured by ELISA, with positivity cut-offs of 12 GPL-U/ml for aCL and 20 GPL-U/ml for β-2GPI. Lupus anticoagulant (LAC) was assessed using the diluted Russell Viper Venom Test (dRVVT), with a normalized ratio >1.2 indicating positivity. This approach ensured standardized PAPS diagnosis and characterization in the study cohort. Result: In this study of 40 primary anti-phospholipid syndrome (PAPS) patients, 82.5% were female, with an average diagnostic age of 35.9 years (range 16--65). Venous thrombosis was the most frequent presentation, seen in 60% of cases, followed by arterial thrombosis (37.5%) and mixed thrombosis (22.5%), while 12.5% had obstetric complications. Deep vein thrombosis (DVT) and ischemic stroke were the most prevalent venous and arterial events, at 47.5% and 27.5%, respectively. Diagnostic testing revealed lupus anticoagulant (LAC) in 55% of patients, anti-β-2 glycoprotein I (β-2GPI) in 47.5%, and anticardiolipin (aCL) antibodies in 37.5%; 20% were positive for all three antibodies. Non-thrombotic manifestations included thrombocytopenia (27.5%) and hemolytic anemia (22.5%). Catastrophic anti-phospholipid syndrome (CAPS) affected 12.5% of patients, with one fatality, while 12.5% experienced thrombosis recurrence. Conclusion: Data on primary anti-phospholipid syndrome (PAPS) alone is limited, as most studies combine primary and secondary APS cases. Our study uniquely focuses on PAPS, including cases with catastrophic APS (CAPS), where triple-positive APS antibodies were more prevalent. Venous thrombosis was the most common presentation, with LAC as the leading antibody, followed by β2GPI and aCL, while thrombotic microangiopathy (TMA), frequently observed in our study, is rarely reported elsewhere. [ABSTRACT FROM AUTHOR] |
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