Catastrophic antiphospholipid syndrome presented with coronary thrombosis, renal impairment, and suspected diffuse alveolar hemorrhage treated with rituximab biosimilar (CT-P10)

Catastrophic antiphospholipid syndrome (CAPS) is a lethal disease that occurs suddenly and progresses to multi-organ failure. We present a case of CAPS successfully treated with the rituximab biosimilar CT-P10. A 38-year-old man was referred with a sustained fever and unexplained elevated creatinine levels. Cardiac arrest by ventricular fibrillation occurred upon arrival at the hospital. We diagnosed probable CAPS because of coronary thrombus, renal impairment, suspected diffuse alveolar hemorrhage, and positive anticardiolipin antibody immunoglobulin G. We performed percutaneous coronary intervention for the cardiac arrest, and treated him with extracorporeal membrane oxygenation, mechanical ventilation, and continuous renal replacement therapy. When CAPS was diagnosed, we administered CT-P10 after administering high-dose glucocorticoid. Our case suggests that the use of a rituximab biosimilar is economically efficient in the treatment of CAPS, as in other rheumatic diseases. The patient was cured without recurrence at the 2-year follow-up.

Rare yet catastrophic presentation of undiagnosed antiphospholipid syndrome

A previously well 31-year-old woman initially presented to the emergency department with pneumonia, however, was found to be hypertensive and have new-onset cardiomegaly. She was admitted for intravenous antibiotics and concurrently a series of investigations were conducted to investigate hypertension and cardiomegaly. During the course of admission, she developed acute kidney injury and was found to have acute chronic occlusion in the abdominal aorta. She was diagnosed with catastrophic antiphospholipid syndrome. This is a rare form of antiphospholipid syndrome with a high mortality rate. Thus, it is important that clinicians are aware of this syndrome to facilitate early diagnosis and initiation of treatment.

Fatal catastrophic antiphospholipid syndrome at presentation of juvenile lupus

Catastrophic antiphospholipid syndrome (CAPS) is a severe and rare form of antiphospholipid syndrome, extremely uncommon inthe pediatric age group, characterized by multiple site thrombosis involving small, medium, and large blood vessels occurring over a short period of time (usually 1 week) causing multiorgan failure. We report the case of an 8-year-old girl presenting with fever, lymphadenopathy, and pain abdomen with refractory mitral regurgitation diagnosed as systemic lupus erythematosus with CAPS. All three antiphospholipid antibodies positivity in high titers further confirmed the diagnosis. In spite of the early initiation of adequate triple therapy (anticoagulation, steroids, and cyclophosphamide), our patient succumbed. The report aims to incorporate a greater awareness among clinicians for timely diagnosis and treatment of this condition and throws light on the varying ways in which lupus can present in children.

CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME: DIAGNOSTICS AND 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.

COVID-19 and Catastrophic Antiphospholipid Syndrome

One year after the beginning of the epidemic, mortality continues to be high despite several different protocols being tried. Critical patients with Covid 19 in some degree of organ failure and thrombotic events meet the diagnostic criteria of a complete or incomplete catastrophic antiphospholipid syndrome (CAPS) or at least we may need to consider a partial form of it. The findings of autopsies and the involvement of different organs and systems are similar to those of CAPS. Currently the only therapy that has been shown to reduce mortality include steroids, anticoagulation and an antinuclear antibody. The same therapy has been shown to be effective for CAPS.

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

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.