Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement
Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.
Antiphospholipid Antibodies in Children with Systemic Lupus Erythematosus 18 Years of Clinical Experience from North India.
Singh2, Surjit, Ahluwalia1, Jasmina, Naseem3, Shano, Suri2, Deepti, Rawat2, Amit
Department of Hematology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
Division of Pediatric Allergy and Immunology, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
Division of Pediatric Hematology, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
Pediatric systemic lupus erythematosus (p-SLE) is usually more severe than its adult counterpart and frequently involves vital organs (e.g. kidneys). Anti-phospholipid antibodies (APLA) have been reported in 3887% patients with p-SLE in and reports suggest that presence of APLA can modify disease expression. Higher incidence of neuropsychiatric, renal and hematological manifestations has been reported in APLA positive p-SLE, but influence of APLA on disease course remains unclear. While APLA have been extensively studied in adults with SLE, there is paucity of data on APLA in p-SLE.
Materials and Methods:
We report a single centre study on 64 patients with p-SLE seen during the period June 1992 - May 2010 in whom APLA testing was performed. Diagnosis of SLE was based on ACR criteria. Mean age at diagnosis was 10.3 years (range 317) with a female:male ratio of 3:1. Of these 24 (37.5%) had renal, 17 (26.6%) hematological (7-hemolytic anemia, 6-thrombocytopenia and 4-leucopenia), 14 (21.9%) neurological, 8 (12.5%) pulmonary, 29 (45.3%) musculo-skeletal and 42 (65.6%) had mucocutaneous involvement. APLA tested included: i) lupus anticoagulant [by kaolin clotting time, diluted Russell viper venom time (LA Screen and LA Confirm, Dade Behring) and STACLOT-LA kit] ii) anticardiolipin antibodies (ACLA) IgG, IgM and iii) anti-b2 glycoprotein I (b2 GPI) antibodies IgG, IgM (Orgentec GmBH by ELISA). Our laboratory is registered in World Health Organization's United Kingdom National External Quality Assurance Scheme for these tests.
Summary of Results:
Patients was tested for APLA on 155 occasions - minimum being once and maximum being 10 during a mean follow-up period of 59.3 months (range 1211 months). Thirty eight (59.4%) patients were positive for one or other APLA at some point of time of their disease. Overall LA was positive in 39.1%, IgG ACA in 23.4% and IgM ACA in 18.6%. At diagnosis, 37.8% children were positive for LA, 16.3% for IgG ACA and 20.9% for IgM ACA. During follow-up LA was positive in 26.2% patients, IgG ACA in 31%, IgM ACA in 19% patients. Anti-b2GPI (IgG and IgM) could be tested in 10 patients only and was found to be positive for anti-b2GPI IgG in 1 patient.
Thrombosis was seen in 5 patients - of these, 4 (80%) were positive for APLA. Of the 24 patients with nephritis, class II nephritis was seen in 4of these none was positive for any of the APLA; class III nephritis was seen in 1patient tested positive for APLA; class IV nephritis was seen in 15of these 9 (60%) were positive for 1 of the APLA; class V nephritis was seen in 3of these 2 (66.6%) were positive for APLA. Of the 17 patients with hematological involvement, 11 (64.7%) had positive APLA; of 14 with neurological involvement, 12 (85.7%) had positive APLA. Six (9.4%) children had a fatal course5 (83.3%) of these had positive APLA.
59.4% p-SLE patients had APLA positivity during the disease course. The commonest APLA was LA (39.1%), followed by IgG ACA (23.4%) and IgM ACA (18.6%). APLA positivity is more common in p-SLE patients with advanced nephritis, neurological involvement, thrombosis and a fatal course. Presence or persistence of these antibodies, however, may not always predict thrombosis in children with p-SLE.
To cite this abstract, please use the following information:
Singh, Surjit, Ahluwalia, Jasmina, Naseem, Shano, Suri, Deepti, Rawat, Amit; Antiphospholipid Antibodies in Children with Systemic Lupus Erythematosus 18 Years of Clinical Experience from North India. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :4