Antiphospholipid Antibody-Associated Chorea

2008 ◽  
Vol 35 (11) ◽  
pp. 2165-2170 ◽  
Author(s):  
NICOLE M. ORZECHOWSKI ◽  
ALEXANDRA P. WOLANSKYJ ◽  
J. ERIC AHLSKOG ◽  
NEERAJ KUMAR ◽  
KEVIN G. MODER
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Age At Onset ◽  
Anticardiolipin Antibody ◽  
Bleeding Complications ◽  
Study Cohort ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus ◽  
Clinical Expression

ObjectiveTo describe the clinical features, treatment, and outcomes of patients with antiphospholipid antibody (aPL)-associated chorea.MethodsThe study cohort consisted of consecutive patients with chorea evaluated between 1990 and 2005 with documented aPL at time of their neurologic diagnosis.ResultsEighteen patients were identified, 4 with systemic lupus erythematosus (SLE). The 14 non- SLE patients experienced 1.6 vascular thromboses/pregnancy losses per person, while patients with SLE experienced 0.5 events/person. Four non-SLE patients (29%) and no SLE patients met criteria for antiphospholipid antibody syndrome (APS). None of these 4 tested positive for IgM anticardiolipin antibody (aCL). In contrast, 10 (71%) non-APS patients tested positive for IgM aCL. Chorea was most often bilateral, mild to moderate, and occurred once with a median age at onset of 44 and 33 years in non-SLE and SLE patients, respectively. Therapy included immunosuppression in 3 (21%) non-SLE patients and in all SLE patients. Antidopaminergic agents were used in 7 (39%). All patients responded to treatment. Five patients received anticoagulation for thrombosis and 2 died of bleeding complications, both non-SLE patients.ConclusionaPL-associated chorea occurs most often in women and severity is mild to moderate. Clinical expression of chorea does not differ between those with and without SLE. Anticoagulation should be reserved for thrombosis treatment and not simply for chorea in the presence of aPL, as 2 patients died of bleeding. The absence of IgM aCL in patients with APS supports prior evidence that IgG aCL and lupus anticoagulant may be the more clinically relevant antibodies for thrombosis. However, IgM aCL may be important in patients with chorea

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.

scholarly journals The majority of Swedish systemic lupus erythematosus patients are still affected by irreversible organ impairment: factors related to damage accrual in two regional cohorts

Lupus ◽  
2019 ◽  
Vol 28 (10) ◽  
pp. 1261-1272 ◽  
Author(s):  
M Frodlund ◽  
S Reid ◽  
J Wetterö ◽  
Ö Dahlström ◽  
C Sjöwall ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Causes Of Death ◽  
Age At Onset ◽  
Damage Index ◽  
Organ Damage ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus ◽  
Damage Accrual

Background Although the survival of patients with systemic lupus erythematosus (SLE) has improved, irreversible organ damage remains a critical concern. We aimed to characterize damage accrual and its clinical associations and causes of death in Swedish patients. Methods Accumulation of damage was evaluated in 543 consecutively recruited and well-characterized cases during 1998−2017. The Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology damage index (SDI) was used to estimate damage. Results Organ damage (SDI ≥ 1) was observed in 59%, and extensive damage (SDI ≥ 3) in 25% of cases. SDI ≥ 1 was significantly associated with higher age at onset, SLE duration, the number of fulfilled SLICC criteria, neurologic disorder, antiphospholipid antibody syndrome (APS), hypertension, hyperlipidemia, depression and secondary Sjögren's syndrome (SS). In addition, SDI ≥ 3 was associated with serositis, renal and haematological disorders and interstitial lung disease. A multiple regression model identified not only well-known risk factors like APS, antihypertensives and corticosteroids, but pericarditis, haemolytic anaemia, lymphopenia and myositis as being linked to SDI. Malignancy, infection and cardiovascular disease were the leading causes of death. Conclusions After a mean SLE duration of 17 years, the majority of today's Swedish SLE patients have accrued damage. We confirm previous observations and report some novel findings regarding disease phenotypes and damage accrual.

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.

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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