First Manifestation Of Antiphospholipid Syndrome Research Articles

Combined central retinal artery and vein occlusion as the first manifestation of antiphospholipid syndrome: case report of an infrequent presentation

Combined central retinal artery and vein occlusion as the first manifestation of antiphospholipid syndrome: case report of an infrequent presentation

Gastrointestinal symptoms as the first manifestation of antiphospholipid syndrome

BackgroundAntiphospholipid syndrome (APS) is an acquired pre-thrombotic autoimmune condition, which produces autoantibodies called antiphospholipid antibodies (APL) against phospholipid-binding plasma proteins. The diagnosis of APS requires at least one of Sapporo standard clinical manifestations and one laboratory criteria (persistently medium/high titer anticardiolipin antibodies, and/or medium/high titer anti-β2-glycoprotein I antibodies, and/or a positive lupus anticoagulant test). Gastrointestinal lesions are rarely reported in APS patients. APS cases with recurrent abdominal pain as the first clinical manifestation are even rarer.Case presentationThis report describes an APS case with recurrent abdominal pain as the first clinical manifestation of antiphospholipid syndrome. The patient has a history of two miscarriages. Computed tomography of the abdomen confirmed mesenteric thrombosis and intestinal obstruction while laboratory tests for serum antiphospholipid and anti-β2-glycoprotein I antibodies were positive. This led to the diagnosis of APS.ConclusionsThis paper provides useful information on gastrointestinal manifestations and APS, also including a brief literature review about possible gastrointestinal symptoms of APS.

Giant native aortic valve thrombus under non-vitamin K antagonist oral anticoagulant: first manifestation of antiphospholipid syndrome.

Giant native aortic valve thrombus under non-vitamin K antagonist oral anticoagulant: first manifestation of antiphospholipid syndrome.

Two effective cases of additional pedal artery angioplasty for severe lower limb ischemia following acute thrombotic artery occlusion with hypercoagulable state diseases

BackgroundAcute limb ischemia (ALI) and critical limb ischemia (CLI) following ALI are life-threatening diseases. The rare potential causes of ALI include hypercoagulable state diseases, such as antiphospholipid syndrome (APS) and essential thrombocythemia (ET). Hypercoagulability often make revascularization for arterial occlusion, especially associated with infrapopliteal lesions, difficult. This is because the vessels have poor run-off, and elevated peripheral vascular resistance associated with microcirculation failure, due to a high thrombus burden. There is no established treatment for this issue.Case presentationA 45 years-old and a 56 years-old male suffered from thrombotic arterial occlusion as a first manifestation of APS and ET, respectively. Combination therapy with aggressive anti-thrombotic therapy and revascularization, such as endovascular therapy and surgical thrombectomy based on the angiosome concept, was performed. However, the high thrombus burden caused a poor pedal outflow, and significant limb ischemia remained. Additional pedal artery angioplasty was performed to improve residual limb ischemia in each case and provided sufficient blood flow to the foot.ConclusionThe pedal artery angioplasty for thrombotic pedal artery occlusion cases, associated with hypercoagulable state diseases, seems to be a treatment option for relieving residual limb ischemia.

Cutaneous necrotizing vasculitis as a first manifestation of antiphospholipid syndrome with catastrophic development

We describe a case of anti-phospholipid syndrome, with catastrophic development and unusual brain involvement, in which the first manifestation was necrotizing cutaneous vasculitis. A 58-year-old woman presented with fever, gangrene of her lower limbs and ischemic lesions of her arms and hands. Laboratory results showed normal white cell count and normal procalcitonin value. The vascular ultrasound study of the lower limbs did not show any thrombosis or stenosis. A chest-abdomen computed tomography scan showed inflammatory thickening of the lung. A month after admission she developed confusion, weakness and aphasia. Magnetic resonance imaging of the brain showed hyperintense bilateral symmetrical lesions in the frontal and cerebellar regions. Anti-cardiolipin antibodies and lupus anticoagulant were negative, whereas anti-β 2 glycoprotein 1 were in high titer. She was started on steroids, and subsequently warfarin, acetylsalicylic acid and hydroxychloroquine, with progressive improvement of her clinical status.

129 Catastrophic antiphospholipid syndrome and pregnancy: A case report

Introduction Association between antiphospholipid antibodies and poor gestational outcomes is recognized for decades. For the diagnosis of Catastrophic Antiphospholipid Syndrome (CAPS) is necessary high clinical suspicion and intensive care support is essential to save the patients in this really catastrophic disorder. Objectives Description of a case report and literature review of CAPS in gestation, looking updates in relation to the early diagnosis and appropriate management of these patients. Methods Case report of CAPS during pregnancy and literature review on Pubmed. Results Z.M.S.S, 33, G5P2, previously diagnosed with primary APS sought care at Hospital Sao Paulo on February 7, 2014, with a 34 weeks pregnancy, presenting hypertension, lower limb edema associated with laboratory findings of proteinuria and thrombocytopenia - Hellp syndrome class II (platelet 57.000). After pressure control, anticonvulsant therapy with magnesium sulfate, she was submitted to an emergency C/S with general anestesia, giving birth to a newborn female, weighing 2130 grams, Apgar 8/8. Evolved rapidly with coagulation disorder, severe epigastric pain radiating to the lower back, in association with sudden onset of pain in the left upper limb, having been referred to intensive care unit where she stayed until February 26. During research, we identified multiple vascular occlusion sites (hepatic microcirculation thrombosis, renal cortical and left brachial vein). The paciente underwent to immunosuppressive therapy with methylprednisolone, immunoglobulin and anticoagulation, with progressive improvement, and was home discharged on March 4, 2014 in good health. Catastrophic Antiphospholipid Syndrome (CAPS), also known as Asherson Syndrome, it’s a potentially fatal variant, that affects less than 1% of patients with antiphospholipid syndrome (APS). It was first described in 1992 by Asherson, is characterized by multiple vascular occlusive events, mainly affecting small vessels. Affects concomitantly different tissues, with progressive and rapid deterioration, leading to multiple organ failure and high mortality. Usually presents thrombocytopenia, hemolytic anemia associated with antiphospholipid antibodies in high titers. It may be the first manifestation of APS in a large percentage of cases. Among the triggers are infections, surgery, trauma, anticoagulation interruption and pregnancy. Clinical support is essential and includes removal of provocative factor, corticosteroid therapy, anticoagulation, intravenous immunoglobulin and plasmapheresis. Conclusion Although less than 1% of patients with APS develop this condition and despite all of the recommended therapies, this potentially fatal outcome (near 50%) shows their importance in modern medicine. Finally, we would like to thank the support of our Intensive Care Unit and Reumatology teams at UNIFESP.

A male patient with myocardial infarction as a first manifestation of antiphospholipid syndrome — treatment with rivaroxaban

Data on the efficacy of non-vitamin K antagonist oral anticoagulants (NOAC) in the antiphospholipid syndrome (APS) are not clear. We present a case of a male patient aged 34 years with a history of myocardial infarction at the age of 25 treated conservatively, at that time associated with the risk factors including arterial hypertension, cigarette smoking, hypercholesterolaemia, and overweight. The notable laboratory finding observed for the first time was prolonged activated partial thromboplastin time (aPTT). At the age of 32 the patient experienced left-sided distal thrombosis of the lower limb and ischaemic stroke. The prolonged aPTT was further evaluated. APS testing was performed revealing the positive lupus anticoagulant, elevated anticardiolipin IgG antibodies and b-2-glicoprotein I IgG antibodies. Anticoagulant therapy with warfarin was initiated. Due to the disadvantages of the need for international normalised ratio monitoring, the treatment with rivaroxaban 20 mg daily was implemented according to the patient’s preferences. The patient has been taking the rivaroxaban for 15 months and has not experienced the recurrent venous and arterial thromboembolism. This case confirms that patients with APS who experienced arterial thrombosis may benefit from treatment with NOAC. Moreover, it emphasises the need for thrombophilia screening in patients aged 50 years and younger following the myocardial infarction, especially below 30 years even in the presence of cardiovascular risk factors. Recurrent thrombotic events following myocardial infarction, especially in the absence of coronary artery stenosis, should prompt search for acquired thrombophilia, especially APS, which predisposes to venous thromboembolism and ischaemic stroke.

PM166 Coronary Thrombosis as a First Manifestation of Antiphospholipid Syndrome

PM166 Coronary Thrombosis as a First Manifestation of Antiphospholipid Syndrome

Non-arteritic anterior ischemic optic neuropathy as first manifestation of antiphospholipid syndrome in a young patient

Case reportWe report the case of a young patient with unilateral anterior ischemic optic neuropathy, with no known cardiovascular risk factors and visual acuity, preserved with positive anticardiolipin antibodies as a unique find. DiscussionNon-arteritic anterior ischemic optic neuropathy in the context of antiphospholipid syndrome is an uncommon finding, but it must be considered in the diagnosis of the atypical anterior ischemic optic neuropathy.

Manifestations dermatologiques du syndrome des antiphospholipides

A wide variety of dermatologic manifestations has been described in the antiphospholipid syndrome (APS). The most frequent skin lesion is livedo reticularis, present not only on the limbs but also on the trunk, with a fine irregular pattern. It belongs to the arterial subset of APS. Circumscribed ulcerations, resembling livedoid vasculitis, may be the first manifestation of APS. Ulcerations may also occur as a late complication of recurrent venous thrombosis. Extensive skin necrosis is a classic manifestation of catastrophic APS. Pseudo-vasculitis lesions are misdiagnosed if a skin biopsy is not performed, especially in the context of systemic lupus erythematosus. In systemic lupus erythematosus, primary anetoderma is always associated with antiphospholipid antibodies.

Ocular disorders as the prevailing manifestations of antiphospholipid syndrome: a case series

IntroductionAntiphospholipid syndrome is an autoimmune disorder characterized by either a history of vascular thrombosis (one or more clinical episodes of arterial, venous, or small vessel thrombosis in any tissue or organ) or pregnancy morbidity in association with the presence of antiphospholipid antibodies. The systemic features of the syndrome are characterized by large variability depending on the affected organ(s). Among them, neurological and behavioural disturbances, dermatological features as livedo reticularis and renal, ocular, liver or valvular heart manifestations have been reported in antiphospholipid syndrome patients. However, studies on the frequency and clinical presentation of the ocular manifestations as the prevailing (first) sign of antiphospholipid syndrome in patients suffering from "unexplained" ocular disease are missing. Herein, we present three cases suffering from unexplained ocular disease as first manifestation of antiphospholipid syndrome.Case presentationAll the three patients were referred to our department because of unexplained ocular features from the anterior or posterior segment and unexplained neuro-ophthalmologic symptoms. The first patient had bilateral retinal occlusive disease, the second and the third patient had unilateral nonarteritic anterior ischemic optic neuropathy with macular oedema. Moderate to high levels of antiphospholipid antibodies were detected in all of them at baseline as well as 6 to 12 weeks after initial testing confirming the presence of antiphospholipid antibodies. Anticoagulant treatment with acenocoumarol was instituted resulting in stabilization and/or improvement of ocular signs in all of them.ConclusionDue to the important diagnostic and therapeutic implications of antiphospholipid syndrome, the possibility of ocular features as the first clinical manifestation of antiphospholipid syndrome should be kept in mind of the physicians particularly in patients with no evident risk factors for ocular disease. In this case, prompt anticoagulant treatment and close follow-up seem to be essential for vision salvation and stabilization.