Antifosfolipidsyndrom
Senast reviderad:
Sakkunnig:Eva Zetterberg
Definition:
Antifosfolipidsyndrom kännetecknas av arteriella och venösa tromboser, graviditetskomplikationer och förhöjda titrar av antifosfolipidantikroppar.
Förekomst:
Incidensen i den generella befolkningen är uppskattad till cirka 0,05 % per år. Tillståndet är vanligare bland fertila kvinnor samt personer med systemiska autoimmuna sjukdomar, framförallt SLE. Kan även förekomma hos barn.
Symtom:
De vanligaste manifestationerna är djup ventrombos, lungemboli och graviditetskomplikationer. Andra manifestationer är stroke, hjärtinfarkt och småkärlstromboser i njure, hud och hjärna.
Kliniska fynd:
Kliniska fynd förenliga med trombossjukdom eller graviditetskomplikationer.
Diagnostik:
Utredning på specialistenhet med bland annat antifosfolipidantikroppar.
Behandling:
Antitrombotiska läkemedel i första hand.
- Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4(2):295-306. doi:10.1111/j.1538-7836.2006.01753.x. PMID: 16420554 PubMed
- El Hasbani G, Taher AT, Sciascia S, Uthman I. Antiphospholipid syndrome: the need for new international classification criteria. Expert Rev Clin Immunol. 2021;17(4):385-394. doi:10.1080/1744666X.2021.1900733. PMID: 33682558 PubMed
- Ünlü O, Zuily S, Erkan D. The clinical significance of antiphospholipid antibodies in systemic lupus erythematosus. Eur J Rheumatol. 2016;3(2):75-84. doi:10.5152/eurjrheum.2015.0085. PMID: 27708976 PubMed
- Cohen D, Berger SP, Steup-Beekman GM, Bloemenkamp KWM, Bajerna IM. Diagnosis and management of the antiphospholipid syndrome. Clinical review. BMJ 2010; 340: c2541. PMID: 20472677 PubMed
- Cervera R. Antiphospholipid syndrome. Thromb Res. 2017;151 Suppl 1:S43-S47. doi:10.1016/S0049-3848(17)30066-X. PMID: 28262233 PubMed
- Cervera R, Piette JC, Font J, et al. Antiphospholipid syndrome: clinical and immunological manifestations and patterns of disease expression in a cohort of 1000 patients. Arthritis Rheum 2002; 46: 1019-27. PubMed
- Avcin C, Cimaz R, Silverman ED, et al. Pediatric antiphospholipid syndrome: clinical and immunologic features of 121 patients in an international registry. Pediatrics 2008; 122: 1100-7. PMID: 18955411 PubMed
- Garcia D, Erkan D. Diagnosis and Management of the Antiphospholipid Syndrome. N Engl J Med. 2018;378(21):2010-2021. doi:10.1056/NEJMra1705454. PMID: 29791828 PubMed
- Galli M, Luciani D, Bertolini G, Barbui T . Lupus anticoagulants are stronger risk factors for thrombosis than anticardiolipn antibodies in the antiphspholipid syndrome: a systematic review of the literature. Blood 2003; 101: 1827-32. PMID: 12393574 PubMed
- Opatrny L, David M, Kahn SR, Shrier I, Rey E. Association between antiphospholipid antibodies and recurrent fetal loss in women without autoimmune disease: a metaanalysis. J Rheumatol 2006; 33: 2214-21. PubMed
- Clark EA, Silver RM, Branch DW. Do antiphospholipis antibodies cause preeclampsia and HELLP syndrome?. Curr Rheumatol Rep 2007; 9: 219-25. PubMed
- Danowski A, de Azevedo MN, de Souza Papi JA, Petri M. Determinants of risk for venous and arterial thrombosis in primary antiphospholipid syndrome and in antiphospholipid syndrome with systemic lupus erythematosus. J Rheumatol. 2009;36(6):1195-1199. doi:10.3899/jrheum.08119. PMID: 19447935 PubMed
- Matyja-Bednarczyk A, Swadźba J, Iwaniec T, et al. Risk factors for arterial thrombosis in antiphospholipid syndrome. Thromb Res. 2014;133(2):173-176. doi:10.1016/j.thromres.2013.11.012. PMID: 24321419 PubMed
- Erkan D, Harrison MJ, Levy R, et al. Aspirin for primary thrombosis prevention in the antiphospholipid syndrome: a randomized, double-blind, placebo-controlled trial in asymptomatic antiphospholipid antibody-positive individuals. Arthritis Rheum. 2007;56(7):2382-2391. doi:10.1002/art.22663. PMID: 17599766 PubMed
- Devreese KM, Pierangeli SS, de Laat B, et al. Testing for antiphospholipid antibodies with solid phase assays: guidance from the SSC of the ISTH. J Thromb Haemost. 2014;12(5):792-795. doi:10.1111/jth.12537. PMID: 24589091 PubMed
- Lim W, Crowther MA, Eikelboom JW. Management og antiphospholipid antibody syndrome: a systematic review. JAMA 2006; 295: 1050-7. PMID: 16507806 PubMed
- Davis WD, Brey RL. Antiphospholipid antibodies and complement activation in patients with cerebral ischemia. Clin Exp Rheumatol. 1992;10(5):455-460. PMID: 1458697 PubMed
- Hamulyák EN, Scheres LJ, Marijnen MC, Goddijn M, Middeldorp S. Aspirin or heparin or both for improving pregnancy outcomes in women with persistent antiphospholipid antibodies and recurrent pregnancy loss. Cochrane Database Syst Rev. 2020;5(5):CD012852. Published 2020 May 2. doi:10.1002/14651858.CD012852.pub2. PMID: 32358837 PubMed
- Girón-Gonzáles JA, García del Rio E, Rodriguez C, et al. Antiphospholipid syndrome and asymptomatic carriers of antiphospholipid antibody: prospective analysis of 404 individuals. J Rheumatol 2004; 31: 1560-7. PubMed
- Ruiz-Irastorza G, Crowther M, Branch W, Khamashta MA. Antiphospholipid syndrome. Seminar. Lancet 2010; 376: 1498-1509. PubMed
- Empson M, Lassere M, Craig J, Scott J. Prevention of recurrent miscarriage for women with antiphospholipid antibody or lupus anticoagulant. Cochrane Database of Systematic reviews 2005; 2: CD002859. PMID: 15846641 PubMed
- Bramham K, Hunt BJ, Germain S, et al. Prfegnancy outcome in different clinical phenotypes of antiphospholipid syndrome. Lupus 2010; 19: 58-64. PubMed
- Eva Zetterberg, docent och överläkare, Koagulationsmottagningen, VO Hematologi, Onkologi och strålningsfysik, Skånes universitetssjukhus.
- Anna Nager, docent och specialist i allmänmedicin, Karolinska institutet