Primary Antiphospholipid Antibody Syndrome and Microscopic Polyarteritis in the Puerperium: A Case Report

Abstract

The primary antiphospholipid antibody syndrome (PAPS) and systemic lupus erythematosus (SLE) may occur in women of reproductive age, and both syndromes have been reported to exacerbate during periods of hormonal flux such as oral contraceptive administration, pregnancy, or the puerperium. These disorders have features which cause them to resemble other multisystem diseases. PAPS and SLE must be differentiated from preeclampsia with HELLP syndrome and thrombotic thrombocytopenic purpura occurring during pregnancy. These four disorders differ in prognosis and required therapies. We report an instructive case of PAPS in a patient who developed microscopic polyarteritis with significant glomerulonephritis following prostaglandin-induced delivery of a stillborn fetus. We compare the manifestations of PAPS, SLE, HELLP, and thrombocytopenic purpura, and discuss the possible role of hormonal imbalance as a precipitating event.

Prolactin, growth hormone and the immune system in humans

“…In all the conditions examined in the present review, immunoregulatory mechanisms might be operational, and the redundancy of the system allows correct immune function. The increased incidence of autoimmune diseases and of exacerbations in women, in particular during pregnancy or the postpartum, is strong but still circumstantial evidence for the role of hormones, in particular for SLE, the antiphospholipid syndrome or lymphocytic hypophysitis [66,73]. The complexity of the endocrine network makes it difficult to estimate the contribution of different hormones in increasing the severity of the disease.…”

“…The data suggest that sustained hyperprolactinaemia plays a deleterious role in both development and progression of the disease. In the primary antiphospholipid syndrome, an autoimmune disease that shares several features with SLE, the hormonal context is also compatible with a precipitating or aggravating role for PRL, as disease occurrence is often related to pregnancy or the puerperium [66].…”

Hemolysis, Elevated Liver Enzymes and Low Platelet (HELLP) Syndrome Associated with Systemic Lupus Erythematosus

“…WhenSLE patients are pregnant, especially those with positive anti-phospholipid antibodies, careful observation is necessary to detect development of pre-eclampsia, including HELLP syndrome, as early as possible. Low-dose aspirin is thought to reduce the frequency and severity of pre-eclampsia because of its inhibitory effects on thromboxan synthesis by platelets (2,3,8), and it is now administered to high-risk women as a prophylactic agent as it is in the case of primary anti-phospholipid antibody syndrome (3). Low-dose aspirin was also prescribed for the present patient in an effort to avoid unfavorable events induced by anti-phospholipid antibodies.…”

Book Review: Systemic lupus erythematosus and thrombotic thrombocytopenic purpura. Report of three cases and review of the literature

“…4 The differential diagnosis between these two conditions may be dif®cult, even if distinctive clinical and laboratory features are present. 5 The treatment of the two diseases is different; nevertheless, plasma exchange and steroids can be useful in both disorders.…”