Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a case of HELLP syndrome, multiple liver infarctions, and intrauterine fetal death in a woman in the 17th week of pregnancy with SLE and APS who had been in remission on a regimen of low-dose prednisolone and aspirin. An increase in the dosage of corticosteroid together with intravenous heparin infusion led to improvement of the clinical symptoms, laboratory parameters, and multifocal low-density liver lesions detected by computed tomography. Early onset and signs of severe organ involvement are the characteristic features of HELLP syndrome associated with APS, and patients that are at risk should be followed up carefully.
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PMID:HELLP syndrome, multiple liver infarctions, and intrauterine fetal death in a patient with systemic lupus erythematosus and antiphospholipid syndrome. 1972 3

Systemic lupus erythematosus (SLE) is a multisystem disease. This study was undertaken to assess the outcome of pregnancies in patients with inactive SLE. We prospectively studied 20 female patients with diagnosis of stable class IV Lupus nephritis followed up at King Abdul Aziz University Hospital, in Jeddah, Saudi Arabia between 1998 and 2008. Before each pregnancy all the patients had their blood pressure, serum creatinine, creatinine clearance, serology for SLE and 24-hour urine protein excretion measured and then repeated at monthly intervals during the pregnancy. Statistical analysis was performed using the Wilcoxon signed-rank test. Despite having negative antinuclear antibody (ANA) significant complications were observed during pregnancy. The daily proteinuria during 34-36 weeks' gestation was significantly higher (P< 0.05) than during 32 weeks. Two patients had abortions one stillbirth and 2 required termination of the pregnancy; one due to severe hypertension, and other due to renal impairment. One patient developed HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome. 14 patients had a successful pregnancy, including 4 requiring a cesarian section. In conclusion, although no clinical evidence of lupus disease activity was demonstrated pre-conception proteinuria significantly increased during pregnancy along with maternal and fetal complications. Pregnant females with diagnosis of SLE need a multidisciplinary care during the pregnancy and post-partum period.
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PMID:Outcome of pregnancy in patients with inactive systemic lupus erythromatosus and minimal proteinuria. 1973 77

Preeclampsia, eclampsia and HELLP syndrome are severe complications of pregnancy associated with a high morbidity and mortality for both mother and foetus. The issue of prognosis with regards to risk of relapse is therefore of upmost importance for both the patients and doctors managing their subsequent pregnancies. The overall risk of relapse is estimated to be less than 10%. The risk is especially increased in case of early onset, before the 28(th) week of amenorrhea. In such patients, an underlying renal disease, essential hypertension or secondary hypertension might be identified. A renal consultation must ensure the complete resolution of the hypertension and proteinuria, otherwise investigations need to be carried out looking for an underlying renal or vascular disease affecting the renal parenchyma or outflow. Screening for a hereditary thrombophilia is performed in the immediate post-partum period following and early onset PE, especially if associated with an IUGR, fetal death, or in an early and severe relapse. A renal biopsy is only rarely considered: it is either performed within the first post-partum days whenever a systemic disease flare-up is suspected (i.e. SLE), or late if the proteinuria failed to settle beyond 6 months post-part. Severe PE is a marker of a vascular disease of short duration, but must be considered a risk factor for later atherosclerosis with cardiovascular complications, justifying long term cardiovascular, renal and metabolic follow-up.
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PMID:[Late prognosis after preeclampsia]. 2048 49

During pregnancy, steroids are usually used in maternal diseases such as adrenal failure or other autoimmune diseases, e.g. idiopathic thrombocytopenic purpura (ITP), Crohn's disease, systemic lupus erythematosus, dermatomyositis, scleroderma, Addison's disease and hyperemesis gravidarum, HELLP syndrome. Endogenous or exogenousmaternal steroids are metabolized by the placental enzyme 11 beta-hydroxy steroid dehydrogenase type 2. Prednisolone and methylprednisolone are highly sensitive to this enzyme, while dexamethasone and betamethasone are less well metabolized. Steroids which can cross the placental barrier are administered in cases like fetal lupus, congenital adrenal hyperplasia and for enhancement of fetal lung maturation, whereas steroids used in maternal diseases are usually the ones with low affinity to the placenta; however, in case of long-term use or in high doses, placental enzyme saturation occurs and thus, resulting in fetal adrenal suppression. Antenatal steroids can lead to low birth weight, as observed in our patient. Here, we report a case with fetal adrenal suppression due to maternal methylprednisolone use presenting with early hypoglycaemia and late hyponatremia in neonatal period and requiring three-month replacement therapy.
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PMID:Fetal adrenal suppression due to maternal corticosteroid use: case report. 2191 31

Antiphospholipid syndrome (APS) is associated with a risk of obstetrical complications, affecting both the mother and the fetus. Obstetrical APS is defined by a history of three consecutive spontaneous miscarriages before 10 weeks of gestation (WG), an intra-uterine fetal death after 10 WG, or a premature birth before 34 WG because of severe pre-eclampsia, eclampsia or placental adverse outcomes (intrauterine growth retardation, oligohydramnios). Pregnancy in women with a diagnosis of obstetric APS is at increased risk for placental abruption, HELLP (Hemolysis, Elevated Liver enzymes, Low Platelet count) syndrome and thrombosis that may be part of a catastrophic antiphospholipid syndrome (CAPS). A previous thrombosis and the presence of a lupus anticoagulant are risk factors for pregnancy failure. A multidisciplinary approach, associating the internist, the anesthesiologist and the obstetrician, is recommended for these high-risk pregnancies. Preconception counseling is proposed to identify pregnancy contraindications, and to define and adapt the treatment prior and during the upcoming pregnancy. Heparin and low-dose aspirin are the main treatments. The choice between therapeutic or prophylactic doses of heparin will depend on the patient's medical history. The anticoagulant therapeutic window for delivery should be as narrow as possible and adapted to maternal thrombotic risk. There is a persistent maternal risk in the postpartum period (thrombosis, HELLP syndrome, CAPS) justifying an antithrombotic coverage during this period. We suggest a monthly clinical and biological monitoring which can be more frequent towards the end of pregnancy. The persistence of notches at the Doppler-ultrasound evaluation seems to be the best predictor for a higher risk of placental vascular complications. Treatment optimization and multidisciplinary antenatal care improve the prognosis of pregnancies in women with obstetric APS, leading to a favorable outcome most of the time.
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PMID:[Pregnancy and antiphospholipid syndrome]. 2234 91

Microangiopathic antiphospholipid-associated syndromes (MAPSs) are reported as encompassing several conditions mainly affecting the microvasculature of selected organs: the liver in HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet); kidney, brain, and skin in TTP (thrombotic thrombocytopenic purpura). It is predominant in patients with catastrophic antiphospholipid syndrome (APS). A recent report suggests that APS is not only a thrombotic disease but also associated with microangiopathic features, and it can explain the greater prevalence of HELLP syndrome in these patients. We here report a case of MAPS during pregnancy associated with systemic lupus erythematosus (SLE) in early second trimester.
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PMID:A Case of Microangiopathic Antiphospholipid-Associated Syndromes during Pregnancy: Review of the Literature. 2281 28

Purtscher's retinopathy is usually associated with trauma, acute pancreatitis, vasculitis, lupus, and bone fractures. It was rarely described postpartum in patients with preeclampsia as well as associated with HELLP syndrome. We present a case of a multiparous patient aged 44 with severe preeclampsia and postpartum HELLP syndrome complicated with Purtscher-like retinopathy and large ruptured subcapsular liver hematoma that required emergency abdominal surgery after premature delivery of a dead fetus. Postsurgical outcome was favorable regarding both liver function and visual acuity.
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PMID:HELLP Syndrome Complicated with Postpartum Subcapsular Ruptured Liver Hematoma and Purtscher-Like Retinopathy. 2285 4

In a previous systematic literature search, we demonstrated that the frequencies of antiphospholipid antibodies (aPL) in general-population patients with pregnancy morbidity (PM), deep vein thrombosis (DVT), myocardial infarction (MI), and stroke (ST) are 6%, 10%, 11%, and 14%. To determine the association between aPL and clinical outcomes, we conducted a follow-up analysis of the 120 studies included in the original paper. Based on the analysis of 81 studies, a significant difference in the frequency of aPL criteria tests between patients and controls emerged considering all the outcomes together (10% versus 3%). In particular, a significant difference was reported for overall PM, pregnancy loss (PrL), late PrL, severe preeclampsia (PEC), ST, MI, and DVT. No difference emerged for early PrL, intrauterine growth restriction (IUGR), PEC, eclampsia (EC), and HELLP. A positive association was found in more than half of the studies for overall PrL, severe PEC, HELLP, ST, MI, and DVT and in less than half for early and late PrL, PEC, EC, and IUGR. The positive association between aPL and clinical outcomes included in the antiphospholipid syndrome classification criteria is not supported by every study, being particularly inconsistent for early PL, IUGR, PEC, EC, and HELLP.
Lupus 2015 Aug
PMID:The association between antiphospholipid antibodies and pregnancy morbidity, stroke, myocardial infarction, and deep vein thrombosis: a critical review of the literature. 2569 69

HELLP syndrome is a collection of symptoms described as hemolysis, elevated liver enzymes and low platelets. HELLP syndrome complicates 0.01-0.6% of pregnancies and can be considered a severe variant of preeclampsia. The occurrence of HELLP syndrome diagnosed before the 20th week of gestation has been most commonly reported in association with antiphospholipid antibody syndrome (APS) or triploid chromosomal anomalies. A 41-year-old primigravida was admitted at 17 weeks and 6 days gestation with hypertension, proteinuria, hemolytic anemia and acute renal injury. She was diagnosed with HELLP syndrome, and subsequently suffered from an intrauterine fetal demise. After delivery, the clinical manifestations of HELLP syndrome resolved within 7 days with the exception of her acute renal failure. Interdisciplinary teams of physicians were able to exclude other imitators of preeclampsia, such as hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP), APS, lupus and acute fatty liver of pregnancy. This case is difficult to diagnose, given the similar presentation of several microangiopathic hemolytic anemias. The clinical manifestations and laboratory findings of HELLP and its mimicking conditions seem as if they are mirror images of each other. However, the discrete differences in our patient presentation, clinical findings, laboratory results and overall postpartum course leave HELLP syndrome as the most consistent diagnosis. It is imperative to investigate for all possible etiologies as HELLP syndrome at 17 weeks gestation is extremely rare and mimicking conditions may require alternative management strategies.
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PMID:HELLP Syndrome at 17 Weeks Gestation: A Rare and Catastrophic Phenomenon. 2580 1

Pre-eclampsia complicated by severe HELLP (hemolysis, elevated liver enzymes and low platelet count) syndrome is a multi-organ disease, and can be difficult to differentiate from thrombotic microangiopathy (appearing as thrombotic thrombocytopenic purpura or hemolytic uremic syndrome), acute fatty liver, systemic erythematous lupus, antiphospholipid syndrome and severe sepsis. Many papers have highlighted the risks of misdiagnosis resulting in severe consequences for maternal health, and this can be fatal when thrombotic thrombocytopenic purpura is misdiagnosed as severe HELLP syndrome. The aim of this paper is to propose relevant markers to differentiate pre-eclampsia complicated by severe HELLP syndrome from its imitators, even in the worrying situation of apparently indistinguishable conditions, and thereby assist clinical decision-making regarding whether or not to commence plasma exchange. Relevant identifiers to establish the most accurate diagnosis include the frequency of each disease and anamnestic data. Frank hemolysis, need for dialysis, neurological involvement and absence of disseminated intravascular coagulation are indicative of thrombotic microangiopathy. The definitive marker for thrombotic thrombocytopenic purpura is undetectable ADAMTS 13 activity.
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PMID:Differentiation between severe HELLP syndrome and thrombotic microangiopathy, thrombotic thrombocytopenic purpura and other imitators. 2587 92


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