Clinical spectrum of males with primary antiphospholipid syndrome and systemic lupus erythematosus: a comparative study of 73 patients

Abstract

The objective of this study was to compare the clinical findings, laboratory data, functional outcome and chronic damage in male patients with primary antiphospholipid syndrome (PAPS) and systemic lupus erythematosus (SLE). We studied 29 male patients with PAPS and 44 with SLE. Clinical findings, laboratory data, lupus damage index (SLICC/ACR DI), and functional outcome in PAPS, were analysed in each group. The mean age at diagnosiswas 29.8 + 10.4 years in patientswith PAPS and 26 + 10.1 years in SLE patients. The duration of disease was 4.5 + 2.6 versus 5.2 + 3.8 years in patients with PAPS and SLE, respectively(P NS). In patients with PAPS the most frequent clinical manifestations were venous thrombosis, thrombocytopenia, and pulmonary thromboembolism. Patients with SLE had joint, skin and renal involvement more frequently than those with PAPS (P 0.0001). All PAPS patients had anticardiolipin antibodies (aCL), and 14 patients (48%) had lupus anticoagulant (LA). All SLE patients had antinuclear antibodies (ANAs). Anti-dsDNA antibodies were positive in 39% of SLE patients. Five patients died: one with ‘catastrophic’ APS and four with SLE. SLICC/ACR-DI score in SLE patients was 1.9 (SD 1). In PAPS patients poor functional outcome was due to myocardial infarction, pulmonary thromboembolism, stroke and mesenteric thrombosis. Lupus nephritis was the principal organ damage in SLE. In conclusion, in male patients with PAPS and SLE, the clinical manifestations were significantly different. Arterial thrombosis was the major cause of functional impairment and permanent organ damage in PAPS. Renal involvement was the major cause of chronic damage in SLE.

Keywords

antiphospholipid syndrome damage index functional outcome males systemic lupus erythematosus

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References

Hughes GRV. Systemic lupus erythematosus . Postgrad Med J 1988; 64: 515-521 .

Bell D , Rigsby R , Stiller C , Clark W , Harth M , Ebers G. HLA antigens in systemic lupus erythematosus: Relationship to disease, severity, age at onset, and sex . J Rheumatol 1984; 11: 475-479 .

Hochberg MC , Boyd RE , Ahearn JM , Arnett FC , Bias WB , Provost TT et a1. Systemic lupus erythematosus. A review of clinic-laboratory features and immunogenetic markers in 150 patients with emphasis on demographic subsets . Medicine 1985; 64: 285-295 .

Cervera R , Khamashta MA , Font J , Sebastiani GD , Gil A , Lavilla P et al. Systemic lupus erythematosus: Clinical and immunologic patterns of disease expression in a cohort of 1000 patients . Medicine 1993; 72: 113-124 .

Lahita RG , Bradlow HL , Kunkel HG , Fishman J. Alterations of estrogen metabolism in systemic lupus erythematosus . Arthritis Rheum 1979; 22: 1195-1198 .

Walker SE , Jacobson JD. Roles of prolactin and gonadotropin-releasing hormone in rheumatic diseases . Rheum Dis Clin North Am 2000; 26: 713-736 .

Jara LJ , Gómez-Sánchez C , Silveira LH , Martinez-Osuna P , Vasey FB , EspinozaLR. Hyperprolactinemia in systemic lupus erythematosus(SLE): Association with disease activity . Am J Med Sci 1992; 303: 222-226 .

Kaufman LD , Gómez-Reino JJ , Heinicke MH , Gorevic PD. Male lupus: Retrospective analysis of the clinical and laboratory features of 52 patients with a review of the literature . Semin Arthritis Rheum 1989; 18: 189-197 .

Miller MIT , Urowitz MB , Gladman DD , Killinger DW. Systemic lupus erythematosus in males . Medicine 1983; 62: 327-334 .

10.

Sthoeger ZM , Geltner D , Rider A , Bentwitch Z. Systemic lupus erythematosus in 49 Israeli males: A retrospective study . Chin Exp Rheumatol 1987; 5: 233-240 .

11.

Molina JF , Drenkard C , Molina J , Cardiel MH , Uribe O , Anaya JM et al. Systemic lupus erythematosus in males. A study of 107 Latin-American patients . Medicine 1996; 75: 124-130 .

12.

Massardo L , Martinez ME , Jacobelli S , Villarroel L , Rosenberg H , Rivero S. Survival of Chilean patients with systemic lupus erythematosus . Semin Arthritis Rheum 1994; 24: 1-11 .

13.

Lahita RG , Chiorazzi N , Gibofsky A , Winchester RJ , Kunkel HG. Familial systemic lupus erythematosus in males . Arthritis Rheum 1983; 26: 39-44 .

14.

Ward MM , Studenski S. Systemic lupus erythematosus in men: A multivariate analysis of gender differences in clinical manifestations . J Rheumatol 1990; 17: 220-224 .

15.

Wallace DJ. The clinical presentation of systemic lupus erythematosus. In: Wallace DJ , Hahn BH (eds) Dubois’ Lupus Erythematosus, 5th ed. William&Wilkins: Baltimore , 1997, 627-633.

16.

Hughes GRV. Thrombosis, abortion, cerebral disease and the lupus anticoagulant . Br Med J 1983; 287: 1088-1089 .

17.

Harris EN , Gharavi AE , Boey ML , Patel BM , Mackworth-Young CG , Loizou S et al. Anticardiolipin antibodies: detection by radioimmuno-assay and association with thrombosis in systemic lupus erythematosus . Lancet 1983; 2: 1211-1214 .

18.

McNeil HP , Simpson RJ , Chesterman CN , Krilis SA. Antiphospholipid antibodies are directed against a complex antigen that includes a lipid-binding inhibitor of coagulation: beta-2-glycoprotein I (apolipoprotein H) . Proc Natl Acad Sci USA 1990; 87: 4120-4124 .

19.

Bevers EM , Galli M , Barbui T , Comfurius P , Zwaal RF. Lupus anticoagulant IgG are not directed to phospholipid only, but to a complex of lipid-bound human prothrombin . Thromb Haemost 1991; 66: 629-632 .

20.

Alarcón-Segovia D , Sánchez Guerrero J. Primary antiphospholipid syndrome . J Rheumatol 1989; 16: 482-488 .

21.

Asherson RA , Khamashta MA , Ordi-Ros J , Derksen RH , Machin SJ , Barquinero J et al. The ‘Primary’ antiphospholipid syndrome: Major clinical and serological features . Medicine 1989; 68: 366-374 .

22.

Mujic F , Cuadrado MJ , Lloyd M , Khamashta MA , Page G , Hughes GRV. Primary antiphospholipid syndrome evolving into systemic lupus erythematosus . J Rheumatol 1995; 22: 1589-1592 .

23.

Derksen RHWM , Gmeling-Meijling FHJ , De Groot PhG. Primary antiphospholipidsyndrome evolving into systemic lupus erythematosus . Lupus 1996; 5: 77-80 .

24.

Blanco F , Ramos-Casals M , García-Carrasco M , Cervera R , Font J , Ingelmo M. Primary antiphospholipid syndrome evolving into systemic lupus erythematosus: a report of 3 new cases and a review of the literature . Rev Clin Esp 1999; 199: 586-588 .

25.

Austin HA 3rd , Muenz LR , Joyce KM , Antonovich TA , Kullick ME , Klippel JH et al. Prognostic factors in lupus nephritis: contributions of renal histologic data . Am J Med 1983; 75: 382-391 .

26.

Blum A , Rubinow A , Galun E. Predominance of renal involvement in male patients with systemic lupus erythematosus . Clin Exp Rheumatol 1991; 9: 206-207 .

27.

Kaufman LD , Gomez-Reino JJ , Heinicke MH , Gorevic PD. Male lupus: Retrospective analysis of the clinical and laboratory features of 52 patients, with a review of the literature . Semin Arthritis Rheum 1989; 18: 189-197 .

28.

Chang D-M , Chang C-C , Koo S-Y , Chu S-J , Chang M-L. The clinical features and prognosis of male lupus in Taiwan . Lupus 1998; 7: 462-468 .

29.

Aydintug AO , Domenech I , Cervera R , Khamashta MA , Jedryka-Goral A , Vianna JL et al. Systemic lupus erythematosus in males: Analysis of clinical and laboratory features . Lupus 1992; 1: 295-298 .

30.

May KP , West SG , Moulds J et al. Different manifestations of the antiphospholipid antibody syndrome in a family with systemic lupus erythematosus . Arthritis Rheum 1993; 36: 528-533 .

31.

Wilson WA , Gharavi AE , Koike T , Lockshin MD , Branch DW , Piette JC et al. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome . Arthritis Rheum 1999; 42: 1309-1311 .

32.

Tan EM , Cohen AS , Fries JF , Masi AT , McShane DJ , Rothfield NF et al. The 1982 revised criteria for the classification of systemic lupus erythematosus . Arthritis Rheum 1982; 25: 1271-1277 .

33.

Gladman D , Ginzler E , Goldsmith C , Fortin P , Liang M , Urowitz M et al. The development and initial validation of the systemic lupus International Collaborating Clinics/American College of Rheumatology damage index for systemic lupus erythematosus . Arthritis Rheum 1996; 39: 363-369 .

34.

Gladman D , Urowitz MB , Goldsmith CH , Fortin P , Ginzler E , Gordon C et al. The reliability of the systemic lupus International Collaborating Clinics/American College of Rheumatology damage index in patients with systemic lupus erythematosus . Arthritis Rheum 1997; 40: 809-813 .

35.

Erkan D , Yazici Y , Sobel R , Lockshin MD. Primary antiphospholipid syndrome: functional outcome after 10 years . J Rheumatol 2000; 27: 2817-2821 .

36.

Harris EN , Pierangeli SS , Birch D. Anticardiolipin wet workshop report: 5th International Symposium on AntiphopholipidAntibodies . Am J Clin Pathol 1994; 101: 616-624 .

37.

Weber M , Hayem G , De Bandt M , Seifert B , Palazzo E , Roux S et al.

Classification of an intermediate group of patients with antiphospholipid syndrome and lupus-likedisease: primary or secondary antiphospholipid syndrome?

J Rheumatol 1999; 26: 2131-2136 .

38.

Amigo MC , García-Torres R , Robles M , Bochicchio T , Reyes PA. Renal involvement in primary antiphospholipidsyndrome . J Rheumatol 1992; 19: 1181-1185 .

39.

Sokunbi DO , Miller F , Wadhwa NK , Nord EP. Reversible renal failure in the primary antiphospholipidsyndrome—a report of two cases . J Am Soc Nephrol 1993; 4: 28-35 .

40.

Sakaguchi S , Kitasawa K , Watanabe M , Mukai K , Totsuka D , Shibata T et al. A case of primary antiphospholipidantibody syndrome with acute renal failure showing thrombotic microangiopathy . Am J Nephrol 1999; 19: 594-598 .

41.

Fakhouri F , Noel LH , Zuber J , Beafulis H , Martinez F , Lebon P et al. The expanding spectrum of renal diseases associated with antiphospholipid syndrome . Am J Kidney Dis 2003; 41: 1205-1211 .

42.

Galli M , Finazzi G , Barbui T. Thrombocytopeniain the antiphospholipid syndrome . Br J Haematol 1996; 93: 1-5 .

43.

Galindo M , Khamashta MA , Hughes GRV. Splenectomy for refractory thrombocytopenia in the antiphospholipid syndrome . Rheumatology (Oxford) 1999; 38: 848-853 .

44.

Zonana-Nacach A , Camargo-Coronel A , Yanez P , de Lourdes-Sanchez M , Jimenez-Balderas FJ , Aceves-Avila J et al. Measurement of damage in 210 Mexican patients with systemic lupus erythematosus: relationship with disease duration . Lupus 1998; 7: 119-123 .

45.

Asherson RA , Cervera R , Piette JC , Font J , Lie JT , Burcoglu A et al. Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients . Medicine 1998; 77: 195-207 .