A case of catastrophic antiphospholipid antibody syndrome complicated with systemic lupus erythematosus, double positive for anti-cardiolipin/β2 glycoprotein I and anti-phosphatidylserine/prothrombin autoantibodies

2012 ◽  
Vol 22 (5) ◽  
pp. 769-773 ◽  
Author(s):  
Eri Hirakawa ◽  
Kazuyoshi Saito ◽  
Shintaro Hirata ◽  
Tatsuya Atsumi ◽  
Takao Koike ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus ◽  
Double Positive ◽  
Glycoprotein I

scholarly journals CLINICAL AND IMMUNOLOGICAL CORRELATIONS IN PATIENTS WITH SYSTEMICLUPUS ERYTHEMATOSUS WITH ANTIPHOSPHOLIPID ANTIBODY SYNDROME

2017 ◽  
Vol 9 (3) ◽  
pp. 7-11 ◽  
Author(s):  
E A Belolipetskaia ◽  
I B Beliaeva ◽  
V I Mazurov ◽  
E A Trofimov ◽  
S V Lapin
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Annexin V ◽  
Anticardiolipin Antibodies ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus ◽  
Glycoprotein I ◽  
Threefold Increase

Antiphospholipid antibodies (aPL): lupus anticoagulant (LA), anticardiolipin antibodies (aCL) and anti-β2-glycoprotein I antibodies (anti-β2GPI) are found in 12 to 44% of systemic lupus erythematosus (SLE) patients. On average, antiphospholipid antibody syndrome (APS) develops in50% of aPL-positive patients with SLE. The seronegative APS is characterized by the absence of the diagnostic levels of "classical" aPL and by the presence of non-criteria aPL: antibodies against pro- thrombin (aPT), antibodies against annexin V, antibodies against phosphatidylethanoamine (aPE), antibodies to phosphatidylserine/prothrombin complex (aPS-PT) and antibodies against negatively charged phospholipids. The presence of four antibodies (LA + aCT + anti-β2GPI + aPT) is associated with a threefold increase in the risk of thrombosis. The presence of aCL and anti-β2GPI in SLE patients with APS and recurrent thromboses is associated with the HLA dRB1 * 0402.

Missed hypereosinophilic syndrome in a critically ill patient with systemic lupus erythematosus

2021 ◽  
Vol 14 (1) ◽  
pp. e236592
Author(s):  
Ying Ling ◽  
Mary Jane Bell ◽  
Lisa Chodirker ◽  
Shirley Lake
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Hypereosinophilic Syndrome ◽  
Clinical Manifestations ◽  
Critically Ill Patient ◽  
Total Leucocyte Count ◽  
High Dose ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus

A high functioning 74-year-old man with systemic lupus erythematosus presented to the emergency department with acute anxiety. He was found to have elevated cardiac enzymes and admitted to the cardiology service for investigation. In hospital, he developed an erythematous papular rash, and deteriorated to being somnolent and bedridden. He was found to have new multiterritory ischaemic strokes. It was eventually noted that he had persistent eosinophilia, present even on admission, which had been overlooked as the total leucocyte count was normal. Serology for antiphospholipid antibody syndrome (APS) was positive. He was diagnosed with hypereosinophilic syndrome (HES) secondary to new APS, and responded to high-dose steroids. This case highlights the importance of fully evaluating a leucocyte differential to make a diagnosis of HES. We discuss the definition, clinical manifestations, diagnostic approach and management of this important condition.

Pyoderma Gangrenosum Mimicking Antiphospholipid Antibody Syndrome in a Child With Juvenile Systemic Lupus Erythematosus

2020 ◽  
Author(s):  
metin kaya gürgöze ◽  
Aslıhan Kara ◽  
Mehmet yusuf sarı ◽  
İlknur Çalık ◽  
Saadet Akarsu
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Skin Biopsy ◽  
Pyoderma Gangrenosum ◽  
Lupus Erythematosus ◽  
Anticoagulation Therapy ◽  
Anticardiolipin Antibody ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus ◽  
History Of

Abstract Background: Although pyoderma gangrenosum (PG) -like lesions have been rarely described in adults with the antiphospholipid antibody syndrome (APS) and systemic lupus erythematosus (SLE), the occurrence of PG as a preceding manifestation of APS in children with SLE has not been reported until. We present a young girl with SLE and APS who developed progressive extstensive ulcerations that were consistent with PG.Case presentation: A 14-year-old girl with a 2-year history of SLE was admitted to our department, complaining painful crusted ulcerations on her legs. Skin biopsy was reported as PG. However, she did not respond to immunosuppressive therapy administered. When her skin biopsy findings is reassessed in keeping with the positive anticardiolipin antibody results, superficial small vessel microthrombosis was observed. Diagnosis of APS and PG developing secondary to SLE were made. It was resulted in marked clinical improvement with anticoagulation therapy in addition to immunosuppressives as is recommended in APS. Conclusions: Based in clinical, pathological and response to proposed treatment, we can state that PG -like lesions in children with SLE could be considered as a secondary form of APS.

The antiphospholipid (antibody) syndrome

2011 ◽  
pp. 343-352
Author(s):  
Alan J. Hakim ◽  
Gavin P.R. Clunie ◽  
Inam Haq
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Autoimmune Diseases ◽  
Venous Thrombosis ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Lupus Anticoagulant ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus ◽  
Pregnancy Morbidity

Introduction 344 Epidemiology and pathology 345 Clinical features of antiphospholipid syndrome 346 Treatment of antiphospholipid syndrome 348 Catastrophic antiphospholipid syndrome 350 The antiphospholipid syndrome (APS) was first described in the 1980s and comprises arterial and venous thrombosis with or without pregnancy morbidity in the presence of anticardiolipin (ACL) antibodies or the lupus anticoagulant (LAC). It can be primary, or secondary to other autoimmune diseases, most commonly systemic lupus erythematosus (SLE) (...

scholarly journals Primary Coronary Sinus Thrombosis in Secondary Antiphospholipid Antibody Syndrome

2011 ◽  
Vol 2 (2) ◽  
pp. 102-104
Author(s):  
Joseph Theodore ◽  
P. Chitrambalam ◽  
K. Pradeep ◽  
S. Viswakumar
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Coronary Sinus ◽  
Lupus Erythematosus ◽  
Sinus Thrombosis ◽  
Rare Complication ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus ◽  
Inflammatory Autoimmune Disease ◽  
Intracardiac Thrombosis

Antiphospholipid antibody syndrome (APLA) is a non-inflammatory autoimmune disease characterised by spontaneous abortion, thrombocytopenia and thrombosis (arterial and venous). Intracardiac thrombosis is a rare complication of APLA, but coronary sinus thrombosis in APLA has hitherto not been reported. We recently treated a young woman with secondary APLA and systemic lupus erythematosus in whom coronary sinus thrombosis was detected in association with recurrent pulmonary embolism. Key Words: intracardiac thrombosis; antiphospholipid antibody syndrome; systemic lupus erythematosus; coronary sinus thrombosis DOI: http://dx.doi.org/10.3126/ajms.v2i2.3885 Asian Journal of Medical Sciences 2 (2011) 102-104

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