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Despite limited evidence, IgA-aCL and IgA anti-\u03b22-glycoprotein-I (anti-\u03b22GPI) were included in the 2012 Systemic Lupus International Collaborating Clinics criteria. The present study aimed to evaluate IgG-\/IgA-\/IgM-aCL and anti-\u03b22GPI occurrence in relation to disease phenotype, smoking habits, pharmacotherapy, anti-phospholipid syndrome (APS) and organ damage among 526 Swedish SLE patients meeting ACR-97. Patients with rheumatoid arthritis (n\u00a0=\u00a0100), primary Sj\u00f6gren's syndrome (n\u00a0=\u00a050) and blood donors (n\u00a0=\u00a0507) served as controls. Anti-phospholipid antibodies (aPL) were analysed by fluoroenzyme-immunoassays detecting aCL\/anti-\u03b22GPI. Seventy-six (14%) SLE cases fulfilled the Sydney APS-criteria, and \u2265\u00a01 aCL\/anti-\u03b22GPI isotype (IgG\/IgA\/IgM) occurred in 138 SLE patients (26%). Forty-five (9%) of the SLE cases had IgA-aCL, 20 of whom (4%) lacked IgG-\/IgM-aCL. Seventy-four (14%) tested positive for IgA anti-\u03b22GPI, 34 (6%) being seronegative regarding IgG\/IgM anti-\u03b22GPI. Six (1%) had APS manifestations but were seropositive regarding IgA-aCL and\/or IgA anti-\u03b22GPI in the absence of IgG\/IgM-aPL and LA. Positive LA and IgG-aPL tests were associated with most APS-related events and organ damage. Exclusive IgA anti-\u03b22GPI occurrence associated inversely with Caucasian ethnicity [odds ratio (OR)\u00a0=\u00a00\u00b721, 95% confidence interval (CI)\u00a0=\u00a00\u00b706\u20130\u00b772) and photosensitivity (OR\u00a0=\u00a00\u00b719, 95% CI\u00a0=\u00a00\u00b705\u20130\u00b772). Nephritis, smoking, LA-positivity and statin\/corticosteroid-medication associated strongly with organ damage, whereas hydroxychloroquine-medication was protective. In conclusion, IgA-aPL is not rare in SLE (16%) and IgA-aPL analysis may have additional value among SLE cases with suspected APS testing negative for other isotypes of aPL and LA.<\/jats:p>","DOI":"10.1111\/cei.13180","type":"journal-article","created":{"date-parts":[[2018,9,12]],"date-time":"2018-09-12T22:28:52Z","timestamp":1536791332000},"page":"27-38","source":"Crossref","is-referenced-by-count":18,"title":["Immunoglobulin A anti-phospholipid antibodies in Swedish cases of systemic lupus erythematosus: associations with disease phenotypes, vascular events and damage accrual"],"prefix":"10.1093","volume":"194","author":[{"given":"M","family":"Frodlund","sequence":"first","affiliation":[{"name":"Division of Neuro and Inflammation Sciences, Department of Clinical and Experimental Medicine, Link\u00f6ping University, Link\u00f6ping, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A","family":"Vikerfors","sequence":"additional","affiliation":[{"name":"Unit of Rheumatology, Department of Medicine Solna, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G","family":"Grosso","sequence":"additional","affiliation":[{"name":"Unit of Rheumatology, Department of Medicine Solna, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T","family":"Skogh","sequence":"additional","affiliation":[{"name":"Division of Neuro and Inflammation Sciences, Department of Clinical and Experimental Medicine, Link\u00f6ping University, Link\u00f6ping, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J","family":"Wetter\u00f6","sequence":"additional","affiliation":[{"name":"Division of Neuro and Inflammation Sciences, Department of Clinical and Experimental Medicine, Link\u00f6ping University, Link\u00f6ping, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K","family":"Elvin","sequence":"additional","affiliation":[{"name":"Unit of Clinical Immunology, Department of Clinical Immunology and Transfusion Medicine, Karolinska Institutet, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I","family":"Gunnarsson","sequence":"additional","affiliation":[{"name":"Unit of Rheumatology, Department of Medicine Solna, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A","family":"Kastbom","sequence":"additional","affiliation":[{"name":"Division of Neuro and Inflammation Sciences, Department of Clinical and Experimental Medicine, Link\u00f6ping University, Link\u00f6ping, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00d6","family":"Dahlstr\u00f6m","sequence":"additional","affiliation":[{"name":"Swedish Institute for Disability Research, Department of Behavioural Sciences and Learning, Link\u00f6ping University, Link\u00f6ping, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J","family":"R\u00f6nnelid","sequence":"additional","affiliation":[{"name":"Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E","family":"Svenungsson","sequence":"additional","affiliation":[{"name":"Unit of Rheumatology, Department of Medicine Solna, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C","family":"Sj\u00f6wall","sequence":"additional","affiliation":[{"name":"Division of Neuro and Inflammation Sciences, Department of Clinical and Experimental Medicine, Link\u00f6ping University, Link\u00f6ping, 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