Abstract
Secondary antiphospholipid syndrome (SAPS) is APS that occurs in the context of another medical condition. Although antiphospholipid antibodies (aPL) can occur in patients with viral, bacterial, or protozoal infections, they are rarely associated with thrombosis. An exception is catastrophic antiphospholipid syndrome which, although extremely rare in itself, is precipitated by infection in a quarter of the cases where a specific trigger can be identified. Antiphospholipid antibodies are also found in patients with malignancy and who are taking certain drugs, but very few of these patients develop SAPS.
The majority of SAPS cases occur in association with systemic lupus erythematosus (SLE). In fact many authors now prefer the term SLE-associated APS. Estimates for how commonly this combination occurs vary widely, but aPL occur in up to 30–40 % of patients with SLE, and 30–70 % of these aPL-positive patients may develop APS. The clinical features and management of SLE-associated APS are similar to those of primary APS though some symptoms such as arthritis are more common in the SLE-associated form. It can sometimes be difficult to work out whether the clinical manifestations in an individual patient with SLE-associated APS are due to inflammation or thrombosis.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Giannakopoulos B, Krilis SA (2013) The pathogenesis of the antiphospholipid syndrome. N Engl J Med 368(11):1033–1044
Miyakis S et al (2006) International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 4(2):295–306
Wilson WA et al (1999) International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum 42(7):1309–1311
Asherson RA et al (2009) History, classification, and subsets of the antiphospholipid syndrome. In: Antiphospholipid syndrome in systemic autoimmune diseases, vol 10. Elsevier Science, Amsterdam/London, pp 1–11
Asherson RA et al (2003) Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus 12(7):530–534
Gomez-Puerta JA, Cervera R (2014) Diagnosis and classification of the antiphospholipid syndrome. J Autoimmun 48–49:20–25
Swaak AJ et al (1979) Anti-dsDNA and complement profiles as prognostic guides in systemic lupus erythematosus. Arthritis Rheum 22(3):226–235
Mehrani T, Petri M (2009) Epidemiology of the antiphospholipid syndrome. In: Antiphospholipid syndrome in systemic autoimmune diseases, vol 10. Elsevier Science, Amsterdam/London, pp 13–34
Meroni PL et al (2004) Anti-beta 2 glycoprotein I antibodies in centenarians. Exp Gerontol 39(10):1459–1465
Andreoli L et al (2013) The estimated frequency of antiphospholipid antibodies in patients with pregnancy morbidity, stroke, myocardial infarction, and deep vein thrombosis: a critical review of the literature. Arthritis Care Res (Hoboken) 65(11):1869–1873
Cabiedes J et al (2002) Anti-cardiolipin, anti-cardiolipin plus bovine, or human beta(2)glycoprotein-I and anti-human beta(2)glycoprotein-I antibodies in a healthy infant population. Arch Med Res 33(2):175–179
Sene D, Piette JC, Cacoub P (2008) Antiphospholipid antibodies, antiphospholipid syndrome and infections. Autoimmun Rev 7(4):272–277
Harel M et al (2005) The infectious etiology of the antiphospholipid syndrome: links between infection and autoimmunity. Immunobiology 210(10):743–747
Zachou K et al (2003) Anti-cardiolipin antibodies in patients with chronic viral hepatitis are independent of beta2-glycoprotein I cofactor or features of antiphospholipid syndrome. Eur J Clin Invest 33(2):161–168
Palomo I et al (2003) Prevalence of antiphospholipid and antiplatelet antibodies in human immunodeficiency virus (HIV)-infected Chilean patients. J Clin Lab Anal 17(6):209–215
Asherson RA, Cervera R (2003) Antiphospholipid antibodies and infections. Ann Rheum Dis 62(5):388–393
Blank M, Shoenfeld Y (2004) Beta-2-glycoprotein-I, infections, antiphospholipid syndrome and therapeutic considerations. Clin Immunol 112(2):190–199
Hunt JE et al (1992) A phospholipid-beta 2-glycoprotein I complex is an antigen for anticardiolipin antibodies occurring in autoimmune disease but not with infection. Lupus 1(2):75–81
Uthman IW, Gharavi AE (2002) Viral infections and antiphospholipid antibodies. Semin Arthritis Rheum 31(4):256–263
Ramos-Casals M et al (2004) Clinical features related to antiphospholipid syndrome in patients with chronic viral infections (hepatitis C virus/HIV infection): description of 82 cases. Clin Infect Dis 38(7):1009–1016
Cervera R et al (2004) Antiphospholipid syndrome associated with infections: clinical and microbiological characteristics of 100 patients. Ann Rheum Dis 63(10):1312–1317
Rojas-Rodriguez J et al (2000) Catastrophic antiphospholipid syndrome: clinical description and triggering factors in 8 patients. J Rheumatol 27(1):238–240
Font C et al (2011) Solid cancer, antiphospholipid antibodies, and venous thromboembolism. Autoimmun Rev 10(4):222–227
Yoon KH et al (2003) High prevalence of antiphospholipid antibodies in Asian cancer patients with thrombosis. Lupus 12(2):112–116
Miesbach W, Scharrer I, Asherson R (2006) Thrombotic manifestations of the antiphospholipid syndrome in patients with malignancies. Clin Rheumatol 25(6):840–844
Armas JB et al (2000) Anticardiolipin and antinuclear antibodies in cancer patients–a case control study. Clin Exp Rheumatol 18(2):227–232
Turna H et al (2009) Is there any effect of tumor burden on hemostatic parameters in cancer patients? A case-control study of hemostatic abnormalities and anticardiolipin antibodies in solid tumors. Clin Appl Thromb Hemost 15(4):454–457
Schved JF et al (1994) A prospective epidemiological study on the occurrence of antiphospholipid antibody: the Montpellier Antiphospholipid (MAP) Study. Haemostasis 24(3):175–182
Ozguroglu M et al (1999) Serum cardiolipin antibodies in cancer patients with thromboembolic events. Clin Appl Thromb Hemost 5(3):181–184
Pusterla S et al (2004) Antiphospholipid antibodies in lymphoma: prevalence and clinical significance. Hematol J 5(4):341–346
Zuckerman E et al (1995) Increased thromboembolic incidence in anti-cardiolipin-positive patients with malignancy. Br J Cancer 72(2):447–451
Bazzan M et al (2009) Presence of low titre of antiphospholipid antibodies in cancer patients: a prospective study. Intern Emerg Med 4(6):491–495
de Meis E, Monteiro RQ, Levy RA (2009) Lung adenocarcinoma and antiphospholipid antibodies. Autoimmun Rev 8(6):529–532
Biggioggero M, Meroni PL (2010) The geoepidemiology of the antiphospholipid antibody syndrome. Autoimmun Rev 9(5):A299–A304
Mahaum N, Prete PE (2011) Antiphospholipid antibodies in malignancy: are these pathogenic or epiphenomena? J Clin Rheumatol 17(8):429–431
Atzeni F et al (2005) Autoimmunity and anti-TNF-alpha agents. Ann N Y Acad Sci 1051:559–569
Ferraccioli G et al (2002) Anticardiolipin antibodies in rheumatoid patients treated with etanercept or conventional combination therapy: direct and indirect evidence for a possible association with infections. Ann Rheum Dis 61(4):358–361
Bobbio-Pallavicini F et al (2004) Autoantibody profile in rheumatoid arthritis during long-term infliximab treatment. Arthritis Res Ther 6(3):R264–R272
Hemmati I, Kur J (2013) Adalimumab-associated antiphospholipid syndrome: a case report and review of the literature. Clin Rheumatol 32(7):1095–1098
Vereckei E et al (2010) Anti-TNF-alpha-induced anti-phospholipid syndrome manifested as necrotizing vasculitis. Scand J Rheumatol 39(2):175–177
Hughes GR (1983) Thrombosis, abortion, cerebral disease, and the lupus anticoagulant. Br Med J (Clin Res Ed) 287(6399):1088–1089
Avcin T et al (2008) Pediatric antiphospholipid syndrome: clinical and immunologic features of 121 patients in an international registry. Pediatrics 122(5):e1100–e1107
Petri M (2000) Epidemiology of the antiphospholipid antibody syndrome. J Autoimmun 15(2):145–151
Alarcon-Segovia D et al (1992) Preliminary classification criteria for the antiphospholipid syndrome within systemic lupus erythematosus. Semin Arthritis Rheum 21(5):275–286
Gomez-Puerta JA et al (2005) Long-term follow-up in 128 patients with primary antiphospholipid syndrome: do they develop lupus? Medicine (Baltimore) 84(4):225–230
Freire PV et al (2014) Distinct antibody profile: a clue to primary antiphospholipid syndrome evolving into systemic lupus erythematosus? Clin Rheumatol 33(3):349–353
Tarr T et al (2007) Primary antiphospholipid syndrome as the forerunner of systemic lupus erythematosus. Lupus 16(5):324–328
Cervera R et al (2002) Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 46(4):1019–1027
Gattorno M et al (2003) Outcome of primary antiphospholipid syndrome in childhood. Lupus 12(6):449–453
Uthman I, Khamashta M (2005) Ethnic and geographical variation in antiphospholipid (Hughes) syndrome. Ann Rheum Dis 64(12):1671–1676
Domenico Sebastiani G, Minisola G, Galeazzi M (2003) HLA class II alleles and genetic predisposition to the antiphospholipid syndrome. Autoimmun Rev 2(6):387–394
Freitas MV et al (2004) Is HLA class II susceptibility to primary antiphospholipid syndrome different from susceptibility to secondary antiphospholipid syndrome? Lupus 13(2):125–131
Caliz R et al (2001) HLA class II gene polymorphisms in antiphospholipid syndrome: haplotype analysis in 83 Caucasoid patients. Rheumatology (Oxford) 40(1):31–36
Tincani A et al (2009) The interplay between the antiphospholipid syndrome and systemic lupus erythematosus. Autoimmunity 42(4):257–259
Vianna JL et al (1994) Comparison of the primary and secondary antiphospholipid syndrome: a European Multicenter Study of 114 patients. Am J Med 96(1):3–9
Soltesz P et al (2003) Evaluation of clinical and laboratory features of antiphospholipid syndrome: a retrospective study of 637 patients. Lupus 12(4):302–307
Danowski A et al (2009) Determinants of risk for venous and arterial thrombosis in primary antiphospholipid syndrome and in antiphospholipid syndrome with systemic lupus erythematosus. J Rheumatol 36(6):1195–1199
McClain MT et al (2004) The prevalence, onset, and clinical significance of antiphospholipid antibodies prior to diagnosis of systemic lupus erythematosus. Arthritis Rheum 50(4):1226–1232
Wahl DG et al (1997) Risk for venous thrombosis related to antiphospholipid antibodies in systemic lupus erythematosus–a meta-analysis. Lupus 6(5):467–473
Somers E, Magder LS, Petri M (2002) Antiphospholipid antibodies and incidence of venous thrombosis in a cohort of patients with systemic lupus erythematosus. J Rheumatol 29(12):2531–2536
Clowse ME et al (2006) Early risk factors for pregnancy loss in lupus. Obstet Gynecol 107(2 Pt 1):293–299
Cervera R et al (2009) Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis 68(9):1428–1432
van Vollenhoven RF et al (2014) Treat-to-target in systemic lupus erythematosus: recommendations from an international task force. Ann Rheum Dis 73(6):958–967
Khamashta MA et al (1995) The management of thrombosis in the antiphospholipid-antibody syndrome. N Engl J Med 332(15):993–997
Crowther MA et al (2003) A comparison of two intensities of warfarin for the prevention of recurrent thrombosis in patients with the antiphospholipid antibody syndrome. N Engl J Med 349(12):1133–1138
Finazzi G et al (2005) A randomized clinical trial of high-intensity warfarin vs. conventional antithrombotic therapy for the prevention of recurrent thrombosis in patients with the antiphospholipid syndrome (WAPS). J Thromb Haemost 3(5):848–853
Lim W, Crowther MA, Eikelboom JW (2006) Management of antiphospholipid antibody syndrome: a systematic review. JAMA 295(9):1050–1057
Ruiz-Irastorza G, Hunt BJ, Khamashta MA (2007) A systematic review of secondary thromboprophylaxis in patients with antiphospholipid antibodies. Arthritis Rheum 57(8):1487–1495
Kleinknecht D et al (1989) Recurrent thrombosis and renal vascular disease in patients with a lupus anticoagulant. Nephrol Dial Transplant 4(10):854–858
Leaker B et al (1991) Insidious loss of renal function in patients with anticardiolipin antibodies and absence of overt nephritis. Br J Rheumatol 30(6):422–425
Isenberg DA, Griffiths M, Neild GH (1995) Woman with livedo reticularis, renal failure, and benign urinary sediment. Nephrol Dial Transplant 10(2):295–297
Hanly JG et al (1999) A prospective analysis of cognitive function and anticardiolipin antibodies in systemic lupus erythematosus. Arthritis Rheum 42(4):728–734
Hanly JG et al (2010) Prospective analysis of neuropsychiatric events in an international disease inception cohort of patients with systemic lupus erythematosus. Ann Rheum Dis 69(3):529–535
Merkel PA et al (1996) The prevalence and clinical associations of anticardiolipin antibodies in a large inception cohort of patients with connective tissue diseases. Am J Med 101(6):576–583
Sanna G et al (2005) Antiphospholipid antibodies in patients with scleroderma: prevalence and clinical significance. Ann Rheum Dis 64(12):1795–1796
Author information
Authors and Affiliations
Corresponding authors
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer International Publishing Switzerland
About this chapter
Cite this chapter
Rahman, A., Raimondo, M.G. (2015). Secondary Antiphospholipid Syndrome. In: Meroni, P. (eds) Antiphospholipid Antibody Syndrome. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-319-11044-8_19
Download citation
DOI: https://doi.org/10.1007/978-3-319-11044-8_19
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-11043-1
Online ISBN: 978-3-319-11044-8
eBook Packages: MedicineMedicine (R0)