Factor V Leiden, antiphospholipid antibodies and thrombosis in systemic lupus erythematosus.

Thromboembolic complications are frequently observed in patients with systemic lupus erythematosus (SLE). Significant associations have been reported between these complications and the presence of antiphospholipid antibodies, notably the lupus anticoagulant and anticardiolipin antibodies. Factor V Leiden is a genetic disorder associated with an increased risk of venous thrombosis. We studied these factors in 173 patients with SLE in relation to both arterial and venous thrombosis. The frequency of factor V Leiden in SLE patients in comparable to that in the Dutch population (5%) and a risk factor for venous thrombosis (odds ratio 4.9; CI 1.2-19.6), but not for arterial thrombosis. The lupus anticoagulant is a risk factor for both arterial thrombosis (odds ratio 7.1: CI 2.9-17.4) and venous thrombosis (odds ratio 6.4; CI 2.7-15.4). From multivariate analysis, both the lupus anticoagulant and factor V Leiden appeared independent risk factors for venous thrombosis.

Primary antiphospholipid syndrome evolving into systemic lupus erythematosus.

A young woman had a history of spontaneous venous thromboembolic disease which recurred on several occasions after cessation of treatment with oral anticoagulants. The presence of antiphospholipid antibodies (lupus anticoagulant and a high titre of lgG class anticardiolipin antibodies) in the absence of other clinical and serological features of systemic lupus erythematosus (SLE) confirmed a diagnosis of primary antiphospholipid syndrome (PAPS). Antinuclear antibodies (ANA) were positive (1:1280; speckled pattern). Twelve years after the first thrombotic episode she fulfilled criteria for the classification of SLE (antinuclear antibodies, platelet count < 100 x 10(9)/l, anti-dsDNA antibodies, Coombs' positive haemolytic anaemia). She suffered a myocardial infarction while adequately anticoagulated and developed polyarthritis and immune complex-mediated nephritis over the next 3 years. This case history supports suggestions made by others that a strongly positive ANA test in a patient diagnosed with PAPS may be a harbinger for the development of SLE. Such evolution can take place over more than 10 years.

Lymphocyte status of lymph node and blood in acquired immunodeficiency syndrome (AIDS) and AIDS-related complex disease.

Blood lymphocytes and lymph nodes from three patients with acquired immunodeficiency syndrome (AIDS) and two cases with AIDS-related complex (ARC) were studied during the course of the disease. The lymph node histomorphology could be grossly categorized into three stages: follicle hyperplasia, follicle degeneration and the depleted stage. In the first stage, follicles show large and sometimes irregular-sized germinal centres and narrow B-cell mantles. Dendritic reticulum cells in the germinal centres reveal disruption of their fine dendritic processes; often indentations are visible, filled with small B- and T-lymphocytes of follicle mantle and paracortical origin. These lesions may be the first signs of follicle degeneration, in which only hyalinized remnants of follicle centres are found. The changes in the paracortex are less prominent than those in the follicles. The disturbance in the numerical balance of T-cell subsets of helper-induced and suppressor-cytotoxic phenotypes is less than that found in the blood. In some cases the paracortex reveals a high density of T6-positive elements resembling interdigitating cells, suggestive of continuous antigen exposure. We found follicle hyperplasia in ARC, follicle degeneration in one case of ARC and in full-blown AIDS, while the depleted stage was found in lymph nodes at autopsy of AIDS patients. Lymph node changes do not completely correlate with the changes in the blood lymphocyte pool. Investigations on lymph nodes may be of value in the assessment of the prognosis of the disease.