Clinical characteristics and risk factors of microvascular involvement in primary antiphospholipid syndrome: a longitudinal single-center study in China

Abstract

Objectives

The objective of this study was to investigate risk factors of microvascular involvement and survival in Chinese patients with primary antiphospholipid syndrome.

Methods

In this single-center, retrospective study, we enrolled 112 patients with a confirmed diagnosis of primary antiphospholipid syndrome who were admitted to Peking Union Medical College Hospital from January 2004 to December 2016. Demographic data, clinical characteristics, laboratory results, and follow-up records were collected.

Results

A total of 112 patients with primary antiphospholipid syndrome were studied. Microvascular involvement was identified in 21 patients (18.75%). Patients with microvascular involvement experienced fewer episodes of arterial or venous thrombosis (28.6% vs. 84.6%) and a higher incidence of thrombocytopenia (85.7% vs. 54.9%), respectively. Low complement and elevated high-sensitivity CRP levels were observed more frequently in the microvascular group compared with the non-microvascular group (complement 38.1% vs. 18.7%; high-sensitivity CRP 71.4% vs. 31.9%, respectively). Anti-β2-glycoprotein I antibodies were more prevalent in patients with microvascular involvement than in patients without (66.7% vs. 33.0%, respectively). Multivariate logistic regression analysis revealed that thrombocytopenia (odds ratio = 4.523, 95% confidence interval 1.139–17.962), elevated high-sensitivity CRP levels (odds ratio = 6.385, 95% confidence interval 1.969–20.704), and anti-β2-glycoprotein I antibody positivity (odds ratio = 5.042, 95% confidence interval 1.555–16.352) were independent risk factors for microvascular involvement. A Kaplan–Meier analysis revealed that survival was significantly poorer in patients with microvascular involvement compared with patients without (p = 0.0278).

Conclusions

In addition to arterial and venous thrombosis, antiphospholipid syndrome can affect the microvasculature of select organs. It is thus important for clinicians to be aware that antiphospholipid syndrome-associated microvascular involvement has a unique pathogenesis and can be a life-threatening condition.

Keywords

Antiphospholipid syndrome microangiopathy anti-β2GPI antibodies complement inflammation

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References

Asherson

Cervera

. Microvascular and microangiopathic antiphospholipid-associated syndromes (“MAPS”): Semantic or antisemantic?. Autoimmunity Rev 2008; 7: 164–167.

Asherson

Pierangeli

Cervera

. Microangiopathic antiphospholipid-associated syndromes revisited: New concepts relating to antiphospholipid antibodies and syndromes. J Rheumatol 2007; 34: 1793–1795.

Hughson

Nadasdy

McCarty

Sholer

Min

Silva

. Renal thrombotic microangiopathy in patients with systemic lupus erythematosus and the antiphospholipid syndrome. Am J Kidney Dis J Nat Kidney Found 1992; 20: 150–158.

Noda

Ogura

Tsutsumi

, et al. Thrombotic microangiopathy due to multiple autoantibodies related to antiphospholipid syndrome. Pediatr Nephrol 2012; 27: 681–685.

Suzumori

Obayashi

Kumagai

Goto

Yoshida

Sugiura-Ogasawara

. A case of microangiopathic antiphospholipid-associated syndromes during pregnancy: Review of the literature. Case Rep Med 2012; 2012: 827543.

Johnsen

Valborgland

Gudlaugsson

Bostad

Omdal

. Thrombotic microangiopathy and the antiphospholipid syndrome. Lupus 2010; 19: 1569–1572.

Miyakis

Lockshin

Atsumi

, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thrombosis Haemostasis: JTH 2006; 4: 295–306.

Asherson

Cervera

de Groot

, et al. Catastrophic antiphospholipid syndrome: International consensus statement on classification criteria and treatment guidelines. Lupus 2003; 12: 530–534.

Asherson

Pierangeli

Cervera

. Is there a microangiopathic antiphospholipid syndrome?. Ann Rheum Dis 2007; 66: 429–432.

10.

Cervera

Rodríguez-Pintó

Colafrancesco

, et al. 14th International Congress on Antiphospholipid Antibodies Task Force Report on Catastrophic Antiphospholipid Syndrome. Autoimmunity reviews 2014; 13: 699–707.

11.

Espinosa

Cervera

Asherson

. Catastrophic antiphospholipid syndrome and sepsis. A common link?. J Rheumatol 2007; 34: 923–926.

12.

Garcia-Carrasco

Mendoza-Pinto

Macias-Diaz

, et al. The role of infectious diseases in the catastrophic antiphospholipid syndrome. Autoimmunity Rev 2015; 14: 1066–1071.

13.

Rosario

Zandman-Goddard

Meyron-Holtz

D'Cruz

Shoenfeld

. The hyperferritinemic syndrome: Macrophage activation syndrome, Still's disease, septic shock and catastrophic antiphospholipid syndrome. BMC Med 2013; 11: 185.

14.

Berman

Rodriguez-Pinto

Cervera

, et al. Pediatric catastrophic antiphospholipid syndrome: Descriptive analysis of 45 patients from the “CAPS Registry”. Autoimmunity Rev 2014; 13: 157–162.

15.

Zheng

Chen

, et al. Antiphospholipid antibody profiles in lupus nephritis with glomerular microthrombosis: a prospective study of 124 cases. Arthritis Rest Ther 2009; 11: R93.

16.

Meroni

Raschi

Testoni

Parisio

Borghi

. Innate immunity in the antiphospholipid syndrome: Role of toll-like receptors in endothelial cell activation by antiphospholipid antibodies. Autoimmunity Rev 2004; 3: 510–515.

17.

Hulstein

Lenting

de Laat

Derksen

Fijnheer

de Groot

. beta2-Glycoprotein I inhibits von Willebrand factor dependent platelet adhesion and aggregation. Blood 2007; 110: 1483–1491.

18.

Oku

Atsumi

Bohgaki

, et al. Complement activation in patients with primary antiphospholipid syndrome. Ann Rheum Dis 2009; 68: 1030–1035.

19.

Shamonki

Salmon

Hyjek

Baergen

. Excessive complement activation is associated with placental injury in patients with antiphospholipid antibodies. Am J Obstet Gynecol 2007; 196: 167.e1–5.

20.

Tedesco

Borghi

Gerosa

, et al. Pathogenic role of complement in antiphospholipid syndrome and therapeutic implications. Frontiers Immunol 2018; 9: 1388.

21.

Cervera

Tektonidou

Espinosa

, et al. Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations (II): Thrombocytopenia and skin manifestations. Lupus 2011; 20: 174–181.

22.

Rodríguez-Pintó

Moitinho

Santacreu

, et al. Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of 500 patients from the International CAPS Registry. Autoimmunity Rev 2016; 15: 1120–1124.

23.

Le Thi Thuong

Tieulie

Costedoat

, et al. The HELLP syndrome in the antiphospholipid syndrome: Retrospective study of 16 cases in 15 women. Ann Rheum Dis 2005; 64: 273–278.

24.

Pontara

Banzato

Bison

, et al. Thrombocytopenia in high-risk patients with antiphospholipid syndrome. J Thrombosis Haemostasis: JTH 2018; 16: 529–532.

25.

Vlachoyiannopoulos

Routsias

. A novel mechanism of thrombosis in antiphospholipid antibody syndrome. J Autoimmunity 2010; 35: 248–255.

26.

Raschi

Testoni

Bosisio

, et al. Role of the MyD88 transduction signaling pathway in endothelial activation by antiphospholipid antibodies. Blood 2003; 101: 3495–3500.

27.

Perez

Stojanovich

Naranjo

, et al. Presence of immune complexes of IgG/IgM Bound to B2-glycoprotein I is associated with non-criteria clinical manifestations in patients with antiphospholipid syndrome. Frontiers Immunol 2018; 9: 2644.

28.

Hughes

Harris

Gharavi

. The anticardiolipin syndrome. J Rheumatol 1986; 13: 486–489.

29.

Shoenfeld

. Systemic antiphospholipid syndrome. Lupus 2003; 12: 497–498.

30.

Asherson

Cervera

Piette

, et al. Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. Med 1998; 77: 195–207.

31.

Tektonidou

. Antiphospholipid syndrome nephropathy: From pathogenesis to treatment. Front Immunol 2018; 9: 1181.

32.

Praprotnik

Ferluga

Vizjak

Hvala

Avcin

Rozman

. Microthrombotic/microangiopathic manifestations of the antiphospholipid syndrome. Clin Rev Allergy Immunol 2009; 36: 109–125.

33.

Levine

Branch

Rauch

. The antiphospholipid syndrome. New England J Medicine 2002; 346: 752–763.

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