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

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.

Vestnik ◽  
2021 ◽  
pp. 345-349
Author(s):  
М.А. Жанузаков ◽  
М.К. Бапаева ◽  
А.Ж. Шурина ◽  
Э.М. Утежанов

Клинические проявления микроскопического полиангиита многообразны, что затрудняет своевременную диагностику и соответственно адекватное лечение. Цель: привлечь внимание терапевтов, пульмонологов, нефрологов к своевременному выявлению легочно-почечного синдрома и установлению его нозологической основы, в частности микроскопического полиангиита на примере клинического случая. Материалы и методы: проведены обследование пациента с микроскопическим полиангиитом и дифференциальная диагностика в рамках легочно-почечного синдрома. Результаты: на основании анамнеза заболевания, результатов клинического и дополнительных методов исследования пациента, установлен диагноз микроскопического полиангиита. Выводы: для ранней диагностики микроскопического полиангиита необходима настороженность при возникновении легочно-почечного синдрома в виде сочетания гломерулонефрита и геморрагического альвеолита на фоне сосудистой пурпуры. Clinical manifestations of microscopic polyangiitis are diverse, which complicates timely diagnosis and, accordingly, adequate treatment. Goal: to draw the attention of therapists, pulmonologists, nephrologists to the timely detection of pulmonary-renal syndrome and the establishment of its nosological basis, in particular microscopic polyangiitis by the example of a clinical case. Materials and methods: an examination of a patient with microscopic polyangiitis and differential diagnosis within the framework of pulmonary-renal syndrome were carried out. Results: based on the anamnesis of the disease, the results of clinical and additional research methods of the patient, the diagnosis of microscopic polyangiitis was established. Conclusions: for early diagnosis of microscopic polyangiitis, alertness is necessary in the event of pulmonary-renal syndrome in the form of a combination of glomerulonephritis and hemorrhagic alveolitis against the background of vascular purpura.


2020 ◽  
Vol 8 ◽  
pp. 2050313X2097222
Author(s):  
E M W Mintjens-Jager ◽  
M E Vos ◽  
G Kats-Ugurlu ◽  
G A P Hospers ◽  
A Rutgers ◽  
...  

Immune checkpoint inhibitors are used in the treatment of different types of tumors including melanoma and non-small cell lung carcinoma. The use of these inhibitors is associated with a broad spectrum of immune-related adverse effects. Here we report a case of a patient admitted to the intensive care unit with multiple organ failure due to catastrophic antiphospholipid syndrome following treatment with pembrolizumab, an immune checkpoint inhibitor, because of metastatic melanoma. The presented patient had multiple organ failure of lung, gastro-intestinal, renal, and the liver. Vascular thrombosis was confirmed by both imaging (pulmonary embolism on computed tomography–thorax) and histopathological examination of the intestines. In combination with the presence of IgA anti-cardiolipin antibodies and initially IgM anti-cardiolipin antibodies, catastrophic antiphospholipid syndrome was suspected. Despite treatment with plasmapheresis and corticosteroids, the patient died due to multiple organ failure. Catastrophic antiphospholipid syndrome is difficult to recognize and has high mortality rates despite supportive treatment. In this case report, discussion is provided regarding the possible immunological mechanism behind catastrophic antiphospholipid syndrome during or after treatment with immune checkpoint inhibitors. It is important to realize that in modern intensive care unit, more patients with immune-related adverse effects of the treatment with immune checkpoint inhibitors will be admitted, because of an increase in the number of patients treated with these checkpoint inhibitors. When these patients are admitted on the intensive care unit, multi-disciplinary consultation is important because of the difficulty of early recognition and optimal treatment of these possible lethal side effects.


2019 ◽  
Vol 1 (2) ◽  
pp. 47-51
Author(s):  
Maria Giulia Tinti ◽  
Vincenzo Carnevale ◽  
Angela De Matthaeis ◽  
Antonio Varriale ◽  
Angelo De Cata

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.


2020 ◽  
pp. 61-63
Author(s):  
S. Sh. Kakvaeva ◽  
M. A. Magomedova ◽  
A. N. Dzhalilova

One of the most serious problems of modern medicine is sepsis. The number of patients undergoing this complication is 20–30 million (WHO) annually and has no tendency to decrease. Sepsis is characterized by severe multiple organ failure due to a violation of the response of the macroorganism to an infectious agent. Moreover, it is dangerous with high mortality. Sepsis often develops in patients with immunodeficiency conditions, which primarily include pregnant women. The article presents a clinical observation of a case of periostitis in a pregnant woman complicated by a septic state.


2020 ◽  
Vol 2020 (12) ◽  
Author(s):  
Stathis Tsiakas ◽  
Chrysanthi Skalioti ◽  
Paraskevi Kotsi ◽  
Ioannis Boletis ◽  
Smaragdi Marinaki

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.


2022 ◽  
Vol 1 ◽  
pp. 21-23
Author(s):  
K. S. Lakshmi Srividya ◽  
Vidyasagar P

Angioedema is a transient, non-pitting oedema that involves subcutaneous or submucosal tissue. Angioedema in children can have varied aetiology and clinical manifestations, unlike that in adults. We report a case of angioedema of penis in a child resulting from insect bite and treated successfully with anti-histamine and leukotriene inhibitor. Penile angioedema should be kept in mind as a differential diagnosis of penile swelling, as early diagnosis and management may prevent fatal complications.


2014 ◽  
Vol 10 (6) ◽  
pp. 28-30
Author(s):  
А. Будаев ◽  
A. Budaev ◽  
А. Громов ◽  
A. Gromov ◽  
А. Зыкин ◽  
...  

<p>This work was devoted to improve the efficiency of diagnosis and treatment of patients with odontogenic phlegmons by studying predictive capability of different integral scales. The results of the use of methods for early differential diagnosis of various forms of odontogenic sepsis (development departments Kursk State Medical University and the Voronezh State Medical Academy. NNBurdenko): more than 30 points (the presence of multiple organ failure) – 22 patients (73,3%); less than 30 points (no organ failure) – 8 patients (26,7%). </p>


2008 ◽  
Vol 248 (6) ◽  
pp. 519-524
Author(s):  
E. G. Zakynthinos ◽  
T. Vassilakopoulos ◽  
D. D. Kontogianni ◽  
C. Roussos ◽  
S. G. Zakynthinos

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