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.

scholarly journals Catastrophic Antiphospholipid Syndrome Triggered by Fulminant Chikungunya Infection in a Patient With Systemic Lupus Erythematosus

2016 ◽  
Vol 68 (4) ◽  
pp. 1044-1044 ◽  
Author(s):  
Juan-Felipe Betancur ◽  
Erika Paola Navarro ◽  
José Humberto Bravo Bonilla ◽  
Armando Daniel Cortés ◽  
Juan Diego Vélez ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Catastrophic Antiphospholipid Syndrome

scholarly journals Invasive Aspergillosis Masquerading as Catastrophic Antiphospholipid Syndrome

2013 ◽  
Vol 22 (5) ◽  
pp. 448-451 ◽  
Author(s):  
Kathryn S. Robinett ◽  
Bethany Weiler ◽  
Avelino C. Verceles
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Invasive Aspergillosis ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Signs And Symptoms ◽  
Subdural Hemorrhage ◽  
Systemic Lupus ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Oral Ulcers ◽  
History Of

A 25-year-old woman with a history of systemic lupus erythematosus who was taking steroids came to the hospital because of vague signs and symptoms of weight loss, constipation, and oral ulcers. Multiorgan dysfunction developed, and catastrophic antiphospholipid syndrome was suspected. She was treated with an intravenous infusion of heparin, but she experienced a subdural hemorrhage and died on day 10 of the hospitalization. An autopsy revealed disseminated invasive aspergillosis. This case illustrates that invasive aspergillosis is a frequently missed diagnosis and should be part of the differential diagnosis for any patient who is immunosuppressed, including patients with autoimmune diseases such as systemic lupus erythematosus.

Pediatric catastrophic antiphospholipid syndrome patient evolving to systemic lupus erythematosus

Lupus ◽  
2020 ◽  
Vol 30 (1) ◽  
pp. 155-157
Author(s):  
Jozélio Freire de Carvalho ◽  
Felipe Freire Silva ◽  
Yehuda Shoenfeld
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Lupus Anticoagulant ◽  
Systemic Lupus ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Thrombotic Occlusion ◽  
Acute Peritonitis ◽  
Intravenous Heparin

Objectives We aim to describe a rare case of a young patient with a catastrophic antiphospholipid syndrome (cAPS) who evolved to systemic lupus erythematosus (SLE). Methodology: Case report description. Case report A 15 years old girl came to the emergency department with acute peritonitis secondary to ischemic perforation of the sigmoid. Angiotomography was suggestive of a thrombotic occlusion of the upper mesenteric artery. Laboratory tests revealed a positive lupus anticoagulant and antinuclear antibodies. A cAPS diagnosis was determined based on more than three different sites of thrombosis (lung, kidney, spleen and sigmoid) in less than one week with a lupus anticoagulant. She was treated with intravenous methylprednisolone 1 mg/kg/day associated with intravenous heparin followed by intravenous immunoglobulin. She had a very good outcome with a total improvement and was discharged from the hospital after 2 months. After 12 weeks, the lupus anticoagulant remained positive. After two months, a systemic lupus erythematosus was diagnosed. She was treated with increasing dose of prednisone and azathioprine 100 mg/day and hydroxychloroquine were added to the treatment. After 2 weeks, she was asymptomatic. She is currently well, asymptomatic using hydroxychloroquine, azathioprine and warfarin. Conclusion This article reports a very unusual presentation which is rarely reported and clinicians should be alert to the possibility that CAPS may be the presenting event for SLE patients, condition known in adults and should also be remembered in pediatric scenario.

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.

Catastrophic antiphospholipid syndrome associated with systemic lupus erythematosus: a case-based review

2020 ◽  
Author(s):  
Jesus Garcia-Diaz ◽  
Mara Escudero-Salamanca ◽  
Ricardo Alvarez-Santana ◽  
Nilda Espinola-Zavaleta
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Underlying Disease ◽  
Rapid Progression ◽  
Systemic Lupus ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Good Clinical Condition ◽  
History Of

Antiphospholipid syndrome (APS) can occur as a primary disease or secondary to an underlying disease, such as systemic lupus erythematosus, or other systemic autoimmune diseases. Catastrophic APS refers to a rapid progression of the disease with the development of thrombotic events that affect three or more organs. This is the case of a 22-year-old woman without history of pregnancy. She developed a catastrophic APS associated with systemic lupus erythematosus, with kidney damage (focal lupus nephritis III), pulmonary embolism, and Libman–Sacks mitral valve endocarditis. Accurate diagnosis and optimal medical treatment (anticoagulants, corticosteroids, antimalarials, diuretics) improved her disease, and the patient was discharged in good clinical condition and continues her multidisciplinary follow-up in the outpatient clinic of our institution.

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