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BackgroundCoronavirus disease 2019 (COVID-19) is a complex disease with many clinicopathological aspects, including abnormal immunothrombosis, and the full comprehension of its pathogenetic mechanisms is urgently required.Methods/ResultsBy means of a multidisciplinary approach, we here report a catastrophic COVID-19 in a 44-year-old Philippine male patient, discovered lupus anticoagulant (LAC)-positive shortly before death, occurred 8 days after hospitalization in a clinical scenario refractory to standard high acuity care recalling Asherson’s syndrome (catastrophic antiphospholipid syndrome).ConclusionA parallelism between this severe form of COVID-19 and Asherson’s syndrome can be so drawn. Both the diseases in fact exhibit hypercytokinemia, thrombotic microangiopathy, disseminated intravascular coagulation and multiple organ failure, they show a relationship with viral infections, and they are burdened by a high mortality rate. A genetic predisposition to develop these two overlapping conditions may be supposed.

Anti-phospholipid syndrome is an autoimmune disorder characterized by episodes of arterial and/or venous thrombosis and/or pregnancy morbidity in the presence of anti-phospholipid antibodies. Catastrophic anti-phospholipid syndrome is an accelerated form of the disease with rapid involvement of multiple organ systems often posing a diagnostic challenge. There is a paucity of literature on the myriad presentations of catastrophic anti-phospholipid syndrome owing to the orphan nature of the disease. We present three cases of catastrophic anti-phospholipid syndrome in patients with systemic lupus erythematosus that presented with episodes of thrombosis involving both arterial and venous systems and multisystem organ failure. Timely diagnoses were made based on a high index of suspicion and were managed with a combination of systemic glucocorticoids, cyclophosphamide, plasmapheresis, intravenous immunoglobulin and other supportive measures. However, despite providing the standard of care, we encountered a poor outcome in two of these patients, highlighting the high mortality associated with catastrophic anti-phospholipid syndrome.

Catastrophic antiphospholipid syndrome (CAPS) is a rare variant of antiphospholipid syndrome characterized by widespread thrombotic microangiopathy and multiorgan failure. Clinically, CAPS signs and symptoms can mimic vasculitis of systemic lupus erythematosus, disseminated intravascular coagulation, and thrombotic thrombocytopenic purpura. CAPS is burdened by high mortality, nearly 50 % in most series. However, patients surviving the acute phase rarely suffer of CAPS relapses. Moreover, concomitant pulmonary hemorrhagic alveolitis is a very rare complication warranting an ominous prognosis. Only few reports of relapsing CAPS are described in literature, and pathogenetic mechanisms are poorly understood and the optimal treatment is yet unknown. We report a case of a young man suffering from multiple relapses of CAPS and recurrent hemorrhagic pulmonary alveolitis refractory to aggressive combination treatment.

A review of 250 patients with the catastrophic antiphospholipid (Asherson's) syndrome (CAPS) taken from the web site organized by the Europhospholipid Group ( http://www.med.ub.es/MIMMUN/FORUM/CAPS.HTM) is presented in this paper. A short historical overview of the antiphospholipid syndrome (APS) is followed by a description of the “triggering” factors, associated autoimmune diseases, clinical presentation, presumed pathogenesis, prognosis, mode of death and suggested therapies. Triggering factors are present in approximately 50% of patients and consist predominantly of infections, trauma, including minor surgical procedures such as biopsies, obstetric-related multiorgan failure and malignancy-associated CAPS. The patients present mainly with multiorgan failure resulting from predominantly small vessel occlusions affecting mainly intra-abdominal organs such as bowel, liver, pancreas, and adrenals, although large vessel occlusions do occur and comprise mainly deep vein thromboses (DVT) of the veins of the lower limbs and arterial occlusions causing strokes and peripheral gangrene. They do not however dominate the clinical picture. The condition differs considerably from the simple/classic APS in several respects, viz. the rapid development of multiorgan failure following the above-mentioned identifiable precipitating factors, the involvement of unusual organs such as bowel, reproductive organs, and bone marrow, complicating features of disseminated intravascular coagulation in 20% of cases, the acute (adult) respiratory distress syndrome (ARDS) in one third of patients, and severe thrombocytopenia; these not being encountered in the simple/classic APS. Treatment consisting of regular and repeated plasma exchanges using fresh frozen plasma, and IV immunoglobulins in addition to parenteral steroids and anticoagulation are necessary to improve the survival in a condition where the mortality is still of the order of 50%. Treatment may have to be continued for several weeks. Parenteral antibiotics may be indicated where an underlying infection is suspected. Antifungal therapy may also be indicated with prolonged treatment and the use of the monoclonal anti-CD20 molecule, Rituximab, has proven useful in those patients where thrombocytopenia poses a major risk of hemorrhage.

Catastrophic antiphospholipid (Asherson's) syndrome (CAPS) is known to be a severe variant (1%) of antiphospholipid syndrome, with a high rate of mortality (50%). The distinguishing feature of CAPS is microvascular thromboses in the presence of antiphospholipid antibodies. Molecular mimicry between β2-glycoprotein I and infectious agents, endothelial cell activation and reduced fibrinolysis are the most frequently described pathophysiological mechanisms. Genetic risk factors have also been implicated in the onset of CAPS, although these have not yet been identified. There have been no randomized, controlled trials evaluating the efficacy of any medication on CAPS; however, when CAPS is suspected, aggressive multimodal treatment is required. Patients who receive a combination of anticoagulation therapy, glucocorticosteroids and plasma exchange with or without intravenous immunoglobulin show the best survival rates. Herein, we review the clinical and laboratory findings, diagnostic criteria, pathophysiology, treatment and prognosis of CAPS.

Catastrophic antiphospholipid syndrome (CAPS, Asherson’s syndrome ) is an unusual form of antiphospholipid syndrome (APS) characterized by multi-organ failure and high mortality. Fortunately, CAPS accounts for less than 1% of APS cases. Due to the rarity of the condition, an international registry of CAPS patients was created in 2000 supported by the European Forum on Antiphospholipid Antibodies held in Taormina, Italy at the Tenth International Congress on Antiphospholipid Antibodies. Clinical and laboratory features are the most important in the criteria for the diagnosis of this syndrome and can affect many organ systems. The majority of patients presented with multiple organ involvement at the time of CAPS. The combination of pulmonary, cardiac, and renal involvement was most commonly seen. The organ systems most commonly involved at the onset include the cardiopulmonary system, primarily characterized by dyspnea and respiratory failure, the central nervous system, and the renal system. Laboratory criteria for the classification of CAPS include the presence of antiphospholipid antibodies—LA and/or aCL and/or β 2 -GPI antibodies.

Antiphospholipid antibodies (aPL) are a common cause of acquired thrombophilia, termed antiphospholipid syndrome (APS, Huges syndrome ). Catastrophic antiphospholipid syndrome (CAPS, Asherson’s syndrome ) is an unusual form of APS characterized with multi-organ failure and high mortality. Fortunately, CAPS accounts for less than 1% of APS cases. The recurrence rate is low with a stable clinical course if these patients are treated with adequate anticoagulation therapy. Due to the rarity of the condition, an international registry of CAPS patients was created in 2000 supported by the European Forum on Antiphospholipid Antibodies held in Taormina, Italy at the Tenth International Congress on aPL. The objective of our study is to describe characteristics of 12 Serbian patients with CAPS included in the international CAPS registry.

The catastrophic anti-phospholipid (Asherson’s) syndrome (CAPS) is characterised by the rapid chronological development of fulminant thrombotic complications that predominantly affect small vessels and differs from the anti-phospholipid syndrome in its accelerated systemic involvement leading to multi-organic failure. Malignancy may play a pathogenic role in patients with CAPS, whereas infections are more important as triggering factors in patients without malignancies. CAPS patients with malignancies are generally older than CAPS patients without malignancies; they generally have the worst prognosis of the entire CAPS cohort.

The catastrophic antiphospholipid syndrome is a potentially life-threatening condition with a high mortality rate, the diagnosis of which requires a high degree of clinical awareness on the part of attending physicians. Patients with this syndrome have various symptoms in common: clinical evidence of multiple organ involvement developed over a very short time period, histopathological evidence of multiple small-vessel occlusions and laboratory confirmation of the presence of antiphospholipid antibodies, usually in high titers. The combination of high doses of intravenous heparin, steroids, γ-globulins and/or repeated plasma exchanges are the basic treatment of choice for all patients with this severe condition.