Background The antiphospholipid-antibody syndrome is a thrombophilic disorder in which venous or arterial thrombosis, or both, may occur in patients with antiphospholipid antibodies. The optimal treatment of these patients is unclear. We assessed the efficacy of warfarin, low-dose aspirin, or both in the secondary prevention of thrombosis in patients with the syndrome. Methods One hundred forty-seven patients (124 [84 percent] of whom were female) with the antiphospholipid-antibody syndrome and a history of thrombosis were studied retrospectively. The syndrome was primary in 62 patients and was associated with systemic lupus erythematosus in 66 patients and lupus-like disease in 19. Each patient's history was reviewed. Results One hundred one patients (69 percent) had a total of 186 recurrences of thrombosis. The median time between the initial thrombosis and the first recurrence was 12 months (range, 0.5 to 144 months). Treatment with high-intensity warfarin (producing an international normalized ratio o...

The Management of Thrombosis in the Antiphospholipid-Antibody Syndrome

The antiphospholipid syndrome: ten years on

https://www.amjmed.com/article/S0002-9343(98)00060-6/pdf

Anticardiolipin Antibodies Predict Early Recurrence of Thromboembolism and Death Among Patients with Venous Thromboembolism Following Anticoagulant Therapy

Even 10 yr after the identification of the antiphospholipid syndrome (APS), renal involvement in the course of APS is still relatively unrecognized, and is probably underestimated. The association of anticardiolipin antibodies and/or lupus anticoagulant with the development of a vaso-occlusive process involving numerous organs is now confirmed. In a multicenter study, 16 cases of "primary" APS (PAPS) were found and followed for 5 yr or more, all with renal biopsy. In all 16 cases of PAPS, there was a vascular nephropathy characterized by small vessel vaso-occlusive lesions associated with fibrous intimal hyperplasia of interlobular arteries (12 patients), recanalizing thrombi in arteries and arterioles (six patients), and focal cortical atrophy (10 patients). In combination, these led to progressive destruction of the kidney, accelerated by acute glomerular and arteriolar microangiopathy in five patients. Focal cortical atrophy is a distinctive lesion, present in 10 biopsies, and likely represents the histologic and functional renal analogue to the multiple cerebral infarcts detected on imaging studies. The clinical hallmark of this vascular nephropathy in PAPS is systemic hypertension, only variably associated with renal insufficiency, proteinuria, or hematuria. The ensemble of histologic renal lesions defined in this study should aid in the separation of the lesions found in cases of secondary APS, especially systemic lupus erythematosus, into those lesions related to APS and those related to the underlying disease.

/pdf/the-intrarenal-vascular-lesions-associated-with-primary-51hxf1eu2l.pdf

The Intrarenal Vascular Lesions Associated with Primary Antiphospholipid Syndrome

OBJECTIVE--To determine whether the finding of antiphospholipid antibodies in patients with venous thromboembolic episodes should influence the duration of treatment with anticoagulant drugs by mouth. METHODS--A retrospective study was carried out in 19 patients with antiphospholipid antibodies and a history of venous thromboembolic episodes. The median follow up from the first venous thromboembolic episode was 93 months and the median age at this episode was 26 years. The patients had in total 34 venous thromboembolic episodes. The total follow up period comprised 32 periods with and 23 periods without anticoagulant drugs. RESULTS--The probability of being free of recurrent venous thromboembolic episodes, calculated by the Kaplan-Meier method, was significantly influenced by the use of anticoagulant drugs. Patients receiving oral anticoagulants had at eight years a 100% probability of survival without recurrence, whereas patients in whom anticoagulant drugs were stopped had a 50% probability of a recurrent venous thromboembolic episode at two years, and a 78% probability of recurrence at eight years. CONCLUSION--Patients with venous thromboembolic episodes and antiphospholipid antibodies have a high risk for recurrent venous thromboembolic episodes and long term treatment with anticoagulant drugs by mouth is an effective prophylaxis.

https://ard.bmj.com/content/annrheumdis/52/9/689.full.pdf

Patients with antiphospholipid antibodies and venous thrombosis should receive long term anticoagulant treatment.

Nineteen patients out of 250 subjects with antiphospholipid antibodies, who had initially presented to the lupus clinic at St Thomas's Hospital, London five or more years ago with a history of venous/arterial occlusions, were entered into the study. The patients were divided into two main groups: I those who remained well without any further thromboembolic complications (n = 10); II those who developed recurrent thrombotic events in the five year period (n = 9). The patients were followed up to determine the relation between the level or the isotype of the anticardiolipin antibodies, or both, to the recurrent thromboembolic events, and the effect of a variety of treatments (corticosteroids, immunosuppression, anticoagulation) in the prevention of further vascular occlusions. Lupus activity over the five year period varied considerably between the two groups--those in group I tending to be relatively inactive compared with those in group II. For some patients in group II thromboembolic events seemed to occur at the time of lupus activity. Antiphospholipid antibodies remained positive in all patients, the levels remaining fairly constant. Levels fell in only one patient in group I and in two in group II. Patients in group II had more systemic lupus erythematosus related disease than those in group I; most were receiving concomitant steroid and immunosuppressive therapy, but this did not seem to protect against the development of further occlusions. All patients were given anticoagulation treatment (warfarin/heparin) or salicylates (low dose aspirin 75 mg daily), or both. Patients with deep vein thromboses developed more complications during anticoagulation therapy than those with cerebrovascular symptoms. Problems in anticoagulation control and recurrent thromboses consequent on warfarin withdrawal despite the administration of subcutaneous heparin were responsible for complications in most patients in group II.

https://ard.bmj.com/content/annrheumdis/50/11/805.full.pdf

C. Pal

Papers

Antiphospholipid syndrome: five year follow up.