scholarly journals Catastrophic antiphospholipid syndrome: a narrative review

2019 ◽  
Vol 1 (2) ◽  
pp. 47-51
Author(s):  
Maria Giulia Tinti ◽  
Vincenzo Carnevale ◽  
Angela De Matthaeis ◽  
Antonio Varriale ◽  
Angelo De Cata
Keyword(s):  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Rapid Development ◽  
Clinical Manifestations ◽  
Intravenous Immunoglobulins ◽  
Multiple Organ ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Small Vessels ◽  
Multiple Organs ◽  
Vascular Occlusions

The catastrophic antiphospholipid syndrome (CAPS) is a lifethreatening disorder characterized by the rapid development of multiple organs/systems thrombosis, in patients with persistently detectable antiphospholipid antibodies. The vascular occlusions predominantly affect small vessels, leading to a disseminated thrombotic microangiopathic syndrome. Most CAPS episodes are related to the presence of a precipitating factor, such as infections and malignant diseases, usually ending up in multiple organ failure. Clinical manifestations may vary according to the extent of the thrombosis, predominantly affecting kidneys, lungs, brain, heart, and skin. Treatment is based on the administration of anticoagulants, corticosteroids, plasma exchange and/or intravenous immunoglobulins. Cyclophosphamide is recommended in CAPS associated with systemic lupus erythematosus. Additionally, rituximab and eculizumab have been used in refractory cases. Overall mortality is still 36.9%, despite recent progress in the therapeutic approach.

scholarly journals Catastrophic antiphospholipid syndrome (Asherson’s syndrome) caused by a suspected intestinal neuroendocrine tumor: a case report

2021 ◽  
Vol 55 (3) ◽  
pp. 206-210
Author(s):  
V.P. Shypulіn ◽  
V.V. Cherniavskyi ◽  
L.S. Hvozdecka ◽  
A.V. Neverovskyi ◽  
V.V. Tishchenko
Keyword(s):  
Small Intestine ◽  
Neuroendocrine Tumor ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Immunohistochemical Study ◽  
Blood Clot ◽  
Connective Tissue Diseases ◽  
Multiple Organ ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Small Vessels

Background. Every year, the number of people with rare forms of the disease is increasing worldwide. One of these is the catastrophic antiphospholipid syndrome — Asherson’s syndrome. To date, it is being actively studied, but the pathophysiological mechanisms of its development have not yet been fully investigated. Our work is the first attempt to describe Asherson’s syndrome on the example of a clinical case in Ukraine. Objective: to determine the factors and mechanisms that led to the death of a patient with Asherson’s syndrome in Ukraine. Materials and methods. The structural-logical analysis and the clinical-statistical method were used. Results. Based on clinical and laboratory criteria, our medical team established a clinical diagnosis of systemic lupus erythematosus complicated by a catastrophic antiphospholipid syndrome (CAPS). Despite treatment with glucocorticoids and anticoagulants according to international guidelines, the patient died. The autopsy results showed that the immediate cause of death was a large blood clot that blocked the pulmonary artery and its main branches. Histological examination revealed thrombosis of small vessels of the kidneys and brain; a neuroendocrine tumor (G2; pT3pNxpM1b) of the small intestine with metastases to the liver, brain, myocardium and kidneys was suspected. An additional immunohistochemical study was performed to clarify the histological diagnosis. The morphological picture and results of immunohistochemical study mostly correspond to the moderately differentiated (G2) non-keratinizing squamous cell carcinoma (ICD-O code: 8070/3) with damage to the walls of the small intestine, liver, lungs, kidneys, myocardium and brain. Conclusions. These data emphasize that despite the rarity of Asherson’s syndrome, it is always necessary to consider its probability in the presence of signs of multiple thrombosis and multiple organ failure. Because its development is the result of serious diseases, including connective tissue diseases, malignancies, infections, the etiotropic and pathogenetic treatment can prevent the development of CAPS and death.

Aortitis in the setting of catastrophic antiphospholipid syndrome in a patient with systemic lupus erythematosus

Lupus ◽  
2020 ◽  
Vol 29 (9) ◽  
pp. 1126-1129
Author(s):  
Andrés González-García ◽  
Mónica López-Rodríguez ◽  
Sandra Redondo ◽  
José Luis Patier ◽  
Pilar Masso ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Rare Condition ◽  
Intravenous Immunoglobulins ◽  
Systemic Lupus ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Small Vessels ◽  
Short Period ◽  
Blue Toe Syndrome

Catastrophic antiphospholipid syndrome (CAPS) is a rare condition characterized by multiple thromboses affecting mainly small vessels in a short period of time in patients with antiphospholipid antibodies. A high suspicion index is mandatory in order to initiate rapidly aggressive immunomodulatory therapy to avoid a very poor prognosis. Systemic lupus erythematosus (SLE) is often associated with antiphospholipid syndrome, with a worse outcome when the catastrophic features occur. We report the case of a 64-year-old woman with a clinical debut of SLE who presented concomitantly with CAPS with several thrombosis affecting the kidney, spleen and bilateral limbs with blue toe syndrome in both legs. Furthermore, she presented with aortitis, with a malaise and myalgias and general syndrome (asthenia, hyporexia and mild weight loss). Fortunately, she had a good response to multi-target combination therapy (anticoagulants, corticosteroids, hydroxychloroquine, intravenous immunoglobulins, plasma exchange and rituximab). Here, we discuss the association between aortitis and CAPS secondary to SLE, and review the literature regarding similar conditions.

Catastrophic antiphospholipid syndrome (Ronald Asherson syndrome) and obstetric pathology

2018 ◽  
Vol 46 (4) ◽  
pp. 387-400 ◽  
Author(s):  
Alexander D. Makatsariya ◽  
Jamilya Khizroeva ◽  
Viktoriya O. Bitsadze
Keyword(s):  
Mortality Rate ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Multiorgan Failure ◽  
Multiple Organ ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Small Vessels ◽  
Threatening Condition ◽  
Life Threatening ◽  
Large Vessels

Abstract Background: Catastrophic antiphospholipid syndrome (CAPS) is an uncommon, often fatal, variant of the antiphospholipid syndrome (APS) that results in a widespread coagulopathy and high titres of antiphospholipid antibodies (aPL) and affects predominantly small vessels supplying organs with the development of multiorgan failure. It remains unclear why some patients develop the typical clinical picture of APS (thrombosis of large vessels), whereas others show the development of progressive microthrombosis, which the authors called “thrombotic storm” and multiple organ failure, that is, CAPS. Materials and methods: Since 2001–2016, we discovered 17 patients with CAPS development. Conclusion: CAPS is life-threatening condition, but optimal treatment for CAPS is not developed yet and the mortality rate is as high as 30%–40%.

scholarly journals Microthrombotic renal involvement in an SLE patient with concomitant catastrophic antiphospholipid syndrome: the beneficial effect of rituximab treatment

Lupus ◽  
2018 ◽  
Vol 27 (9) ◽  
pp. 1552-1558 ◽  
Author(s):  
Á Diószegi ◽  
T Tarr ◽  
M Nagy-Vincze ◽  
M Nánásy-Vass ◽  
R Veisz ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Renal Function ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Clinical Findings ◽  
Severe Thrombocytopenia ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Laboratory Findings

Antiphospholipid syndrome is characterized by multiple arterial and/or venous thrombotic events, recurrent fetal losses in the presence of antiphospholipid antibodies (aPL). Catastrophic antiphospholipid syndrome is a life-threatening, rare subset of antiphospholipid syndrome when the thrombotic events affect at least three organs, and clinical manifestations develop simultaneously or within a week. Diagnostically, small vessel occlusions can be detected by histopathology in the presence of aPL. Our case report describes an 18-year-old man who has been treated for antiphospholipid syndrome associated with systemic lupus erythematosus (SLE) since 2011. The clinical findings were dominated by recurrent deep vein thrombosis, and severe proteinuria caused by lupus nephritis, accompanied by mild serological and laboratory findings. The patient was hospitalized in March 2014 because of severe thrombocytopenia and infective diarrhoea. At this time the renal functions deteriorated rapidly. Simultaneously, left upper extremity paresis was observed; computed tomography showed ischaemic lesions in the territory of the middle cerebral artery. Abdominal discomfort and pain occurred. On computed tomography scan ischaemic lesions were seen in the spleen, the right kidney and the coeliac trunk. Laboratory and serological findings verified the presence of aPL and anti-DNA antibodies, anaemia and thrombocytopenia. Based on the above-mentioned clinical and laboratory findings, the diagnosis of catastrophic antiphospholipid syndrome was established. Anticoagulation, corticosteroids and plasma exchange treatment, as well as haemodiafiltration were initiated. Although the thrombotic cascade decelerated following these interventions, we could not see an improvement in the renal function. Rituximab treatment was started, leading to a significant improvement in renal function. After 5 weeks of treatment the patient was discharged from hospital.

scholarly journals A probable case of catastrophic antiphospholipid syndrome: Should high-dose steroids be given in the setting of polymicrobial sepsis?

2019 ◽  
Vol 7 ◽  
pp. 2050313X1983953 ◽  
Author(s):  
Shanna Ariane Tucker ◽  
Justin Choi ◽  
Dhruv Khullar
Keyword(s):  
Diagnostic Criteria ◽  
Antiphospholipid Syndrome ◽  
Clinical Manifestations ◽  
Intravenous Immunoglobulins ◽  
Polymicrobial Sepsis ◽  
High Dose ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Probable Case ◽  
Best Management ◽  
Uremic Syndrome

In this clinical vignette, we present a case of a 59-year-old woman with catastrophic antiphospholipid syndrome likely triggered by polymicrobial sepsis. The diagnostic criteria and clinical manifestations of catastrophic antiphospholipid syndrome are reviewed. We also compare diagnostic criteria and clinical manifestations with other clinical entities in the differential diagnosis, including thrombotic thrombocytopenic purpura–hemolytic-uremic syndrome, disseminated intravascular coagulation, sepsis, and inflammatory bowel disease. Catastrophic antiphospholipid syndrome is a rare, but lethal condition, and treatment recommendations are based on expert consensus and analyses of the international Catastrophic Antiphospholipid Syndrome Registry. Current management guidelines recommend triple therapy, with anticoagulation, glucocorticoids, and plasma exchange or intravenous immunoglobulins. This case brings this rare clinical entity to the attention of clinicians and emphasizes the need for more research to understand the best management. It also raises the question of whether high-dose steroids should be continued for treatment of catastrophic antiphospholipid syndrome in the setting of a severe sepsis.

CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME: DIAGNOSTICS AND TREATMENT

Vestnik ◽  
2021 ◽  
pp. 341-344
Author(s):  
М.А. Жанузаков ◽  
М.К. Бапаева ◽  
Г.К. Джалилова ◽  
А.Ж. Шурина ◽  
Э.М. Утежанов
Keyword(s):  
Differential Diagnosis ◽  
Early Diagnosis ◽  
Multiple Organ Failure ◽  
Antiphospholipid Syndrome ◽  
Disseminated Intravascular Coagulation ◽  
Clinical Case ◽  
Clinical Manifestations ◽  
Multiple Organ ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Adequate Treatment

Клинические проявления катастрофического антифосфолипидного синдрома многообразны, что затрудняет своевременную диагностику и соответственно адекватное лечение. Цель: устранить трудности, возникающие у практических врачей при постановке диагноза, дифференциальной диагностике и лечении катастрофического антифосфолипидного синдрома, основываясь на анализе данных литературы и на примере клинического случая. Материалы и методы: исследований по данной проблеме в отечественных источниках мы не встретили, что привело к необходимости остановиться на литературных данных и клиническом примере. Результаты: на основании анамнеза заболевания, результатов клинического и дополнительных методов исследования пациента, установлен диагноз катастрофического антифосфолипидного синдрома. Выводы: для ранней диагностики катастрофического антифосфолипидного синдрома необходима настороженность при возникновении признаков ДВС-синдрома и быстро развивающейся полиорганной недостаточности. Clinical manifestations of the catastrophic antiphospholipid syndrome are diverse, which complicates timely diagnosis and, accordingly, adequate treatment. Goal: eliminate the difficulties encountered by practitioners in the diagnosis, differential diagnosis and treatment of catastrophic antiphospholipid syndrome, based on the analysis of literature data and on the example of a clinical case. Materials and methods: we did not find any studies on this issue in domestic sources, which led to the need to focus on literature data and a clinical example. Results: on the basis of the anamnesis of the disease, the results of clinical and additional research methods of the patient, a diagnosis of catastrophic antiphospholipid syndrome was established. Conclusions: for early diagnosis of catastrophic antiphospholipid syndrome, alertness is required when signs of disseminated intravascular coagulation and rapidly developing multiple organ failure occur.

scholarly journals Case of an unusual diagnosis of primary antiphospholipid syndrome with multiple clinical complications

2020 ◽  
Vol 2020 (12) ◽  
Author(s):  
Stathis Tsiakas ◽  
Chrysanthi Skalioti ◽  
Paraskevi Kotsi ◽  
Ioannis Boletis ◽  
Smaragdi Marinaki
Keyword(s):  
Antiphospholipid Syndrome ◽  
Thrombotic Event ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Young Male ◽  
Multiple Organ ◽  
Systemic Autoimmune Disease ◽  
Antiphospholipid Antibody ◽  
Primary Antiphospholipid Syndrome ◽  
Wide Range

ABSTRACT Antiphospholipid syndrome (APS) is a systemic autoimmune disease defined by the presence of antiphospholipid antibodies in association with thrombotic events and/or obstetric complications. Renal involvement is not infrequent in both primary and secondary APS. Kidney manifestations comprise a wide range of clinical features, including hypertension, major renal vessel thrombosis or microvascular endothelial injury, also described as APS nephropathy. In the absence of a thrombotic event, clinical manifestations of APS are often non-specific. We recently encountered a case of primary APS in a young male with newly diagnosed hypertension and renal impairment. The diagnosis of APS was initially suspected by his kidney biopsy findings, when electron microscopy examination showed the features of chronic microangiopathy, and was later confirmed by a triple positive antiphospholipid antibody profile and multiple organ involvement.

Molecular mechanisms of vasculopathy and coagulopathy in COVID-19

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Suzan Al-Gburi ◽  
Stefan Beissert ◽  
Claudia Günther
Keyword(s):  
Angiotensin Ii ◽  
Disease Control ◽  
Lupus Erythematosus ◽  
Molecular Mechanisms ◽  
Multiple Organ ◽  
Type I ◽  
Type I Ifn ◽  
Systemic Complications ◽  
Small Vessels ◽  
Specific Regulation

Abstract COVID-19 primarily affects the respiratory system and may lead to severe systemic complications, such as acute respiratory distress syndrome (ARDS), multiple organ failure, cytokine storm, and thromboembolic events. Depending on the immune status of the affected individual early disease control can be reached by a robust type-I-interferon (type-I-IFN) response restricting viral replication. If type-I-IFN upregulation is impaired, patients develop severe COVID-19 that involves profound alveolitis, endothelitis, complement activation, recruitment of immune cells, as well as immunothrombosis. In patients with proper initial disease control there can be a second flare of type-I-IFN release leading to post-COVID manifestation such as chilblain-like lesions that are characterized by thrombosis of small vessels in addition to an inflammatory infiltrate resembling lupus erythematosus (LE). Mechanistically, SARS-CoV-2 invades pneumocytes and endothelial cells by acting on angiotensin-II-converting enzyme 2 (ACE2). It is hypothesized, that viral uptake might downregulate ACE2 bioavailability and enhance angiotensin-II-derived pro-inflammatory and pro-thrombotic state. Since ACE2 is encoded on the X chromosome these conditions might also be influenced by gender-specific regulation. Taken together, SARS-CoV-2 infection affects the vascular compartment leading to variable thrombogenic or inflammatory response depending on the individual immune response status.

scholarly journals Leukoencephalopathy and cerebral edema as the presenting manifestations of SLE in an ANA-negative adolescent female: A case report and review of literature.

2020 ◽  
Author(s):  
Alexandra Theisen ◽  
Paroma Bose ◽  
Christina Knight ◽  
Melissa Oliver
Keyword(s):  
Cerebral Edema ◽  
Lupus Erythematosus ◽  
Primary Care Physicians ◽  
Neuropsychiatric Symptoms ◽  
Clinical Manifestations ◽  
Central Nervous System Involvement ◽  
Multiple Organ ◽  
Small Subset ◽  
Adolescent Female ◽  
Organ Systems

Abstract Background: Systemic lupus erythematosus (SLE) is an autoimmune disease with various clinical manifestations involving multiple organ systems. Neuropsychiatric manifestations of SLE has been associated with increased morbidity and mortality, thus it is important to recognize and diagnosis the disease entity and treat early. When neuropsychiatric symptoms are involved, typically there are many other systemic features to aid in the diagnosis of SLE. Many autoantibodies have been discovered and are used to help diagnose SLE. The antibody present in most cases of pediatric SLE, as well as in many other rheumatic diseases, is the nonspecific antinuclear antibody (ANA), making it a commonly used screening tool by primary care physicians when evaluating a patient with a possible rheumatic disorder. However, a small subset of SLE patients, 1-5%, present with a negative ANA, and it is important to keep SLE on the differential diagnosis in specific instances when a thorough infectious and neurological workup has been completed and proven to be inconclusive. Case Presentation: This case involves a Hispanic adolescent female with a negative ANA who presented with diffuse cerebral edema secondary to leukoencephalopathy due to SLE with central nervous system involvement. She had an extensive workup while inpatient involving metabolism, infectious disease, rheumatology, and neurology prior to obtaining the diagnosis of SLE. She was treated with both cyclophosphamide and rituximab and showed improvement. Conclusions: A review of the literature revealed 8 cases with SLE presenting with or developing diffuse cerebral edema and/or leukoencephalopathy. Our patient’s case differs in that she was also ANA negative despite other autoantibody positivity. While she did have low complements and transient leukopenia, she did not present with other signs of organ involvement, which made the diagnosis of SLE with neuropsychiatric involvement quite challenging. We discuss the importance of keeping SLE on the differential despite a negative ANA in complex cases without any other cause and to consider initial screening with not only the ANA but also dsDNA and complements to avoid missed diagnoses.

New targeted therapies for treatment of thrombosis in antiphospholipid syndrome

2007 ◽  
Vol 9 (30) ◽  
pp. 1-15 ◽  
Author(s):  
Silvia S. Pierangeli ◽  
Mariano E. Vega-Ostertag ◽  
Emilio B. González
Keyword(s):  
Antiphospholipid Syndrome ◽  
Targeted Therapies ◽  
Lupus Erythematosus ◽  
Pregnancy Loss ◽  
Cell Activation ◽  
Clinical Manifestations ◽  
Target Cells ◽  
Endothelial Cell Activation ◽  
In Vivo Models

Antiphospholipid (aPL) antibodies (Abs) are associated with thrombosis and pregnancy loss in antiphospholipid syndrome (APS), a disorder initially characterised in patients with systemic lupus erythematosus (SLE) but now known to occur in the absence of other autoimmune disease. There is strong evidence that aPL Abs are pathogenic in vivo, from studies of animal models of thrombosis, endothelial cell activation and pregnancy loss. In recent years, progress has been made in characterising the molecular basis of this pathogenicity, which includes direct effects on platelets, endothelial cells and monocytes as well as activation of complement. This review summarises the clinical manifestations of APS and current modalities of treatment, and explains recent advances in understanding the molecular events triggered by aPL Abs on target cells in coagulation pathways as well as effects of aPL Abs on complement activation. Based on this information and on additional scientific evidence using in vitro and in vivo models, new potential targeted therapies for treatment and/or prevention of thrombosis in APS are proposed and discussed.

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