Manifestation Of Primary Antiphospholipid Syndrome Research Articles

Chronic Budd-Chiari syndrome as an initial manifestation of primary antiphospholipid syndrome in a young patient.

Chronic Budd-Chiari syndrome as an initial manifestation of primary antiphospholipid syndrome in a young patient.

A Successful Management of 29 year old Female with Left Central Retinal Artery Occlusion due to Manifestation of Primary Antiphospholipid Syndrome

Ocular involvement in Anti Phospolipid Syndrome (APS) includes a broad spectrum of manifestations from the anterior and posterior segment or the presence of neuro-ophthalmologic features. A female, 29 years old, came to ER handled by ophthalmology department, with chief complaint left visual loss suddenly since 4 hours before admission. Investigations revealed stable vital signs, VOD 20/20, VOS 1/300, funduscopy showedpale and cherry red spot on left retina, OCT revealed hyperreflective of left inner retinal layer, IgG aCL 51.7 U/mL (50.8 U/mL in OPD 3 months later), and the other examinations were within normal limit. Patient was diagnosed with Central Retinal Artery Occlusion due toPrimary Antiphospolipid syndrome. She was performed occular massage and anterior chamber paracintesis procedure, and given O2 6-8 lpm NRBM, Timolol 0.5% eye drop left eye bid, acetazolamide 250 mg bid, Kalium Slow Release 1 tab qd, Levofloxacine eye drop 1 drop/hour post surgery. After the result of IgM aCL available, we added warfarin 2 mg qd and aspirin 320 mg qd. Patient was discharged 2 days later as visual acuity improved with VOD 20/20 and VOS 0.5/60. Key words: Central retina artery occlusion, primary anti phospolipid syndrome, anti cardiolipin antibody

Predictive value of endothel dysfunction, arterial stiffness and carotid intima media thickness in thrombotic manifestations of primary antiphospholipid syndrome

Aim: Primary antiphosphplipid sydrome (APS) is characterized by recurrent venous or/and arterial thrombosis and fetal loss in the presence of lupus antikoagulant, anti-cardiolipin and anti-beta2 glycoprotein I antibodies. Follow-up (2005-2015) study examined how the angiological , antiphospholipid antibodies and metabolic parameters have changed. The aim was to define if there is any assosiation with the latter thrombotic events and the changing of examined parameters.

The IgM isotype of anti-annexin A5 antibodies and multiple positivity of conventional antiphospholipid antibodies: increasing the number of clinical manifestations of primary antiphospholipid syndrome.

We evaluated the importance of anti-annexin A5 antibodies (aanxA5 Abs) for clinical (thrombosis and/or recurrent pregnancy loss) and serologic (presence of antiphospholipid antibodies: lupus anticoagulant (LA), anticardiolipin (aCL), and anti-β2 glycoprotein I (aβ2GPI) antibodies) features of patients with primary antiphospholipid syndrome (PAPS). Our study included 70 patients with PAPS according to the international consensus criteria for APS. The mean age of the analyzed patients was 45.97 ± 12.72. The disease duration above 5years was present in 31/70 of patients. Concentrations of analyzed antibodies were measured by ELISA. Cutoff values were set in accordance to the manufacturers' recommendations. History of recurrent pregnancy loss was associated with double positivity for aanxA5 IgM and LA (χ (2) = 4.000, P = 0.046) and triple positivity for aanxA5 IgM + LA + aβ2GPI IgM (χ (2) = 4.168, P = 0.041). Venous thromboses were associated with triple positivity for aanxA5 IgM + aCLIgG + aβ2GPI IgM (χ (2) = 3.965, P = 0.046). The IgG isotype of aanxA5 Abs was in positive correlation with aCL Abs of the IgG (r = 0.310, P = 0.009) and IgM (r = 0.254, P = 0.034) isotype. The presence of the clinical manifestations of PAPS is increasing with the number of positive conventional aPL and the IgM aanxA5 Abs tests. This new combination of Abs is beneficial even when the number of patients with positivity for aanxA5 Abs is low. This is important in further detection of patients prone to recurrence of thrombotic episodes.

Nonarteritic anterior ischemic optic neuropathy as the presenting manifestation of primary antiphospholipid syndrome

Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by vascular thrombosis or pregnancy morbidity. Ocular involvement in APS includes a broad spectrum of manifestations involving anterior and posterior segment or the presence of neuroophthalmologic features. Nonarteritic anterior ischemic optic neuropathy (NAION) is a very rare finding, and in this report a case having NAION as the prevailing sign of APS is presented. A middle-aged women who presented with visual disturbances in her left eye (LE) and turned out to have the diagnosis of primary APS with the help of rheumatological investigations is discussed. She was treated with oral steroids for NAION in her LE. With systemic and rheumatological work-up, primary APS was diagnosed, and hydroxychloroquine, coumadin, and aspirin were started, after which she remained stable under control. Due to the important diagnostic and therapeutic implications of APS, it should be considered in the differential diagnosis of NAION, particularly when the etiology is uncertain.

Cerebral venous sinus thrombosis as an initial manifestation of primary antiphospholipid syndrome

Cerebral venous sinus thrombosis is a rare neurologic manifestation of antiphospholipid syndrome. We report a case of a 49-year-old woman who presented to the emergency department with recurrent episodes of transient clumsiness of the left upper extremity. The results of unenhanced brain computed tomography and electroencephalography were unremarkable. Serial neuroimages revealed superior sagittal sinus thrombosis. Thrombophilia screen disclosed positive lupus anticoagulant. In this case report, we aim to emphasize the significance of recognizing an unusual presentation of antiphospholipid syndrome.

Life-threatening cardiac manifestations of primary antiphospholipid syndrome

We report a rare case of primary antiphospholipid syndrome (APS) in a 43-year-old man presenting as recurrent acute coronary stent thrombosis and complicated by three myocardial infarctions. As illustrated in this report, in APS patients recurrent life-threatening arterial thrombotic events may occur in spite of recommended anticoagulant therapy. We conclude that the APS should be considered as a potential cause of acute coronary syndrome, particularly in young individuals with a history of recurrent thrombotic events and/or with abnormal coagulation test results. Further studies are needed to determine the best therapeutic strategy for APS patients with acute coronary syndrome.

Thrombotic renal and adrenal manifestations of primary antiphospholipid syndrome

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L’hémorragie intra-alvéolaire, une complication rare du syndrome primaire des antiphospholipides

Pulmonary embolism is the main pulmonary manifestation of primary antiphospholipid syndrome. Other pulmonary manifestations including intra-alveolar haemorrhage are less common. We report a 36-year-old man with a primary antiphospholipid syndrome who presented with an acute respiratory failure due to the association of pulmonary embolism and intra-alveolar haemorrhage. This diagnosis should be systematically considered as it is life threatening and requires a specific therapy.

Cardiac Tamponade in a Patient with Predominantly Cutaneous Manifestations of Primary Antiphospholipid Syndrome

Cardiac Tamponade in a Patient with Predominantly Cutaneous Manifestations of Primary Antiphospholipid Syndrome

Localized retinal ischemia as an ocular manifestation of primary antiphospholipid syndrome

Case report: Herein, a case of an unusual ocular presentation of primary antiphospholipid syndrome. The patient disclosed localized retinal ischemia due to branch retinal artery occlusion, confirmed by fluorescein angiography and manual perimetry.Comments: The ocular manifestation led to the diagnosis and successful treatment of the systemic disease.

Localized retinal ischemia as an ocular manifestation of primary antiphospholipid syndrome

Localized retinal ischemia as an ocular manifestation of primary antiphospholipid syndrome

The impact of gender on clinical manifestations of primary antiphospholipid syndrome

The objective of the study was to determine the clinical differences at diagnosis and during follow-up between male and female patients with primary antiphospholipid syndrome (PAPS). We analysed 68 patients, 30 males and 38 females diagnosed and followed between 1990 and 2003. Patients with antiphospholipid syndrome associated with systemic lupus erythematosus at onset and during follow-up were excluded. The mean age at diagnosis was 31.4 +/- 11 years in males and 35.7 +/- 11 years in females (NS). The follow-up after diagnosis was 8.7 +/- 3.1 years in males and 9.2 +/- 2.9 years in females (NS). We did not find significant differences between the two groups with respect to venous and arterial thrombosis. However, in female patients, stroke was more prevalent than in male patients (12/38 versus 3/30, P = 0.03). In contrast, we found a significant prevalence of severe gastrointestinal complications in male compared to female patients (7/30 versus 1/38, P = 0.009). One male patient died due to catastrophic antiphospholipid syndrome. This study suggests that clinical course in patients with PAPS may be different with significant prevalence of central nervous system involvement in females and gastrointestinal involvement in males. Factors such as accelerated atherosclerosis, hormones, related to gender could be the explanation of these findings.

Clinical manifestations of chronic inflammatory demyelinating polyneuropathy with anti-cardiolipin antibodies

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune syndrome where certain autoantibodies define clinicopathologic subgroups. In the present study, serum anti-cardiolipin antibodies (aCL) were evaluated. We investigated aCL in sera from 21 patients diagnosed with CIDP in our hospital between 1991 and 2001. The four CIDP patients with aCL (aCL+) were compared with 17 patients without aCL (aCL-). All aCL+ patients displayed sensory-motor polyneuropathy, with severity and distribution of weakness resembling those in aCL- patients. Anti-nuclear antibody titer of aCL+ patients were significantly higher than those in aCL- patients. None of aCL+ patients presented clinical manifestations of primary anti-phospholipid syndrome (APS), such as thromboses or recurrent abortion. Although the aCL+ patients were older and had more complications and more severe pathologic features than aCL- patients, they responded well to steroid pulse or intravenous immunoglobulin. The aCL in CIDP apparently differ from 'autoimmune' aCL in APS, instead being among the autoantibodies pathologically involved in CIDP subgroups.

Paroxysmal non-kinesigenic dyskinesia in antiphospholipid syndrome.

We report on a patient with a mixed movement disorder classifiable as a paroxysmal nonkinesigenic dyskinesia, occurring as the first manifestation of primary antiphospholipid syndrome (PAPS). Possible pathophysiology is discussed based on recent literature, and we stress that PAPS must be considered in movement disorders of a paroxysmal nature.

Avascular Necrosis of Bone Associated With Primary Antiphospholipid Syndrome

We describe, the case of a 34-year-old mestizo Mexican woman with previous history of fetal loss, deep vein thrombosis that developed avascular necrosis (AVN) of the right knee with strong positive levels of anticardiolipin antibodies (aCL) IgG and IgM. AVN of bone in association with aCL has been seen principally in systemic lupus erythematosus patients. However, few cases have been reported of AVN as a clinical manifestation of primary antiphospholipid syndrome. A review of the association of aCL and AVN in patients with systemic lupus erythematosus, primary antiphospholipid syndrome, and idiopathic osteonecrosis is presented.

Intracardiac thrombi in primary antiphospholipid syndrome: two case reports

We describe two patients who developed stroke and ischemia of the left lower limb, related to intracardiac thrombi, as the first manifestation of primary antiphospholipid syndrome (PAPS). Transesophageal echocardiography (TEE) revealed intracardiac thrombi as abnormal hyperechogenic, nonmobile masses, firmly attached to the left atrial appendage and the anterior part of the mitral annulus, respectively. Our patients received high-intensity oral anticoagulant therapy (INR 3.0–4.0), which resulted in the rapid disappearance of clinical symptoms, without subsequent recurrence of thromboembolic manifestations, and the disappearance of intracardiac thrombi on TEE. Our findings underscore that PAPS should be suspected in patients presenting with intracardiac thrombi, even if they have no previous history of thromboembolic disorders. Moreover, because intracardiac thrombi may precede other manifestations of PAPS, this finding in young patients without underlying heart disease should invoke a search for both antiphospholipid and anticardiolipin antibodies.

Chorea can Present Long before Other Manifestations of Primary Antiphospholipid Syndrome

Chorea can Present Long before Other Manifestations of Primary Antiphospholipid Syndrome

Isolated fascicular oculomotor nerve palsy as the initial presentation of the antiphospholipid syndrome

This case report describes a 24-year-old female who presented with sudden onset of painless diplopia and ptosis in her left eye. Examination identified an isolated incomplete pupil-sparing left oculomotor nerve palsy. Magnetic resonance imaging demonstrated focal hyperintensity in the left midbrain with infarction suggested by diffusion-weighted imaging. A diagnosis of primary antiphospholipid syndrome was made with the demonstration of a positive lupus anticoagulant. Other autoimmune markers were present on initial assessment, but did not fulfil diagnostic criteria for systemic lupus erythematosus. Anticoagulation with warfarin was commenced, with gradual resolution of neurological deficits. This case illustrates an unusual initial manifestation of primary antiphospholipid syndrome causing midbrain stroke in a young woman.

CT features of abdominal manifestations of primary antiphospholipid syndrome.

The purpose of this work was to evaluate the CT features of the abdominal manifestations of primary antiphospholipid syndrome (PAPS). Of the 32 patients who were confirmed to have PAPS among 751 patients with elevated antiphospholipid antibodies during a 2 year period, we retrospectively reviewed the 14 patients who underwent abdominal CT. The clinical indications for abdominal CT included abdominal pain, abdominal distension, or lower leg swelling. CT findings were analyzed with regard to the abdominal vascular system and abdominal organ involvement patterns as well as ancillary findings. Of the 14 patients with PAPS, 10 had involvement of the venous system (72%), 2 of the arterial system (14%), and 2 of both systems (14%). Of the 12 patients who had venous system involvement, 4 had thrombosis in the inferior vena cava (IVC), 2 in both the IVC and the hepatic vein, 1 in the IVC and splenic and portal veins, 1 in the IVC and hepatic and adrenal veins, 1 in the hepatic, portal, and renal veins, and 3 in the portal and superior mesenteric veins. Budd-Chiari syndrome developed in five of the nine patients who had thrombosis of the IVC or hepatic vein. Arterial thrombosis was noted in four patients, hepatic artery in two, aorta in one, renal artery in one, pancreatic arcade in one, and splenic artery in one, with infarct of multiple organs including the liver, jejunum, colon, kidney, and adrenal gland. Seven of the 14 patients (50%) manifested thrombosis or infarct of multiple extra-abdominal organs. PAPS should be included in the differential diagnosis when CT demonstrates infarcts in multiple organs or patients have recurrent episodes of venous or arterial thrombosis.