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Arteropathies, Microcirculation and Vasculitis
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
Small vessel occlusions are part of the vascular manifestations of APS and may affect glomerular, skin, retinal, bowel, hepatic or pulmonary vessels. The laboratory diagnosis of APS relies on the identification of lupus anticoagulant (LA) or antibodies against cardiolipin (aCL) and β2 glycoprotein I (anti-β2GPI) found in SLE. Since the original definitions of the syndrome several subsets have been identified. The catastrophic antiphospholipid syndrome (CAPS/Asherson's syndrome) is often fatal, with widespread small vessel occlusions dominating the clinical picture and with many distinguishing characteristics separating it from simple APS. Microvascular and microangiopathic antiphospholipid-associated syndromes (‘MAPS’) is an umbrella term relating to all these conditions.
Management of recurrent second-trimester missed abortion
Published in Minakshi Rohilla, Recurrent Pregnancy Loss and Adverse Natal Outcomes, 2020
Catastrophic antiphospholipid syndrome (CAPS) is an infrequent but most severe thrombotic presentation of APS. The international consensus statement for classification criteria includes the following: Clinically evident thrombosis of three or more organs or systemsRapid disease progression of disease pathology, over less than a weekHistopathological confirmation of thrombosis in at least one organPositive serology for aPL
Haemostasis and fibrinolysis
Published in Ken Myers, Paul Hannah, Marcus Cremonese, Lourens Bester, Phil Bekhor, Attilio Cavezzi, Marianne de Maeseneer, Greg Goodman, David Jenkins, Herman Lee, Adrian Lim, David Mitchell, Nick Morrison, Andrew Nicolaides, Hugo Partsch, Tony Penington, Neil Piller, Stefania Roberts, Greg Seeley, Paul Thibault, Steve Yelland, Manual of Venous and Lymphatic Diseases, 2017
Ken Myers, Paul Hannah, Marcus Cremonese, Lourens Bester, Phil Bekhor, Attilio Cavezzi, Marianne de Maeseneer, Greg Goodman, David Jenkins, Herman Lee, Adrian Lim, David Mitchell, Nick Morrison, Andrew Nicolaides, Hugo Partsch, Tony Penington, Neil Piller, Stefania Roberts, Greg Seeley, Paul Thibault, Steve Yelland
Catastrophic antiphospholipid syndrome is a life-threatening condition found in about 1% of these patients. Precipitating events include infection, surgery, medications and inconsistent anticoagulation. It has a 50% mortality associated with disseminated intravascular coagulation. The diagnosis is based on involvement of at least three organ systems or tissues, presence of antibodies and histological evidence of small vessel occlusion.
Thrombotic complications in adult patients with severe single coagulation factor or platelet defects – an overview
Published in Expert Review of Hematology, 2019
Hanne Skaadel, Øystein Bruserud
Catastrophic antiphospholipid syndrome. Antiphospholipid syndrome is characterized by venous or arterial thromboses, and/or recurrent pregnancy morbidity, associated with persistent antiphospholipid antibodies [50]. On the other hand, the catastrophic form of the syndrome is characterized by microvascular thrombotic events, venous thrombosis is also common whereas arterial thrombosis is less common [50]. Between 20% and 50% of patients with antiphospholipid syndrome have thrombocytopenia <100 x 109/L [59] and there seems to be a subset of patients that is characterized by high IgG anticardiolipid antibodies or lupus anticoagulant, neuropsychiatric manifestations, arterial or venous thrombosis, thrombocytopenia, and autoimmune hemolysis [60]. The mechanisms behind the thrombocytopenia can be immune mediated, microangiopathy, decreased production, or increased pooling [59]. The treatment of antiphospholipid syndrome is based on supportive care, anticoagulation, immunomodulation or immunosuppression [61].
Catastrophic Antiphospholipid Syndrome: Skin, Eye and Brain Involvement
Published in Ocular Immunology and Inflammation, 2022
Juan Martin Sanchez, Mauricio Davila, Radgonde Amer
A 17-year-old girl presented with fatigue, loss of appetite, arthralgia, and non-resolving lower limb skin ulcers. On admission, livedo reticularis on the face (Figure 1) and lower limbs, and skin ulcers in both legs (Figure 2) were noted. The diagnostic workup showed anemia of 6.6 gr% (normal 12.4–16.1), elevated ESR of 140 mm/hour (normal 1–20) and CRP 3.36 (normal<0.5 mg %), positive ANA, anti-DNA autoantibodies (titer at > 1000, positive if > 75 IU/ml) and positive antiphospholipid antibodies (anticardiolipin IgM and IgG, anti-B2-glycoprotein Ι IgM). Visual acuity was 6/6 in each eye. Anterior segments were normal. Left fundus was normal. Right eye funduscopy showed cotton-wool spots along the inferotemporal vascular arcades with arteriolar occlusion, perivascular retinal hemorrhages, and ischemia in the temporal peripheral retina (Figure 3). Fluorescein angiography showed the extent of non-perfusion and vascular and optic disc leakage (Figure 4). Brain MRI revealed several white matter lacunar infarcts supra and infratentorial, minute cortical/subcortical hemorrhages, and a subarachnoidal insular hemorrhage (Figure 5). The clinical picture was consistent with probable catastrophic antiphospholipid syndrome. No precipitating factor was identified. The patient was treated with high-dose intravenous methylprednisolone and cyclophosphamide, as well as an anticoagulant (Enoxaparin). Laser photocoagulation was applied to the ischemic retina. Within 2 months of treatment, the facial livedo reticularis resolved and the leg ulcers markedly improved (Figure 1, 2).
Response to letter to the editor: catastrophic antiphospholipid antibody syndrome and multiple organ dysfunctions in critically ill patients with COVID-19
Published in Expert Review of Respiratory Medicine, 2020
Chiara Robba, Denise Battaglini, Paolo Pelosi, Patricia R. M. Rocco
The antiphospholipid syndrome is a systemic autoimmune disorder that causes an increased risk of blood clots with recurrent arterial and/or venous thrombosis. The diagnosis is made on the basis of clinical manifestations and confirmed by the presence of any of the three antiphospholipid syndrome antibodies: lupus anticoagulant, anticardiolipin, and beta-2 glycoprotein I. Less frequently, the antiphospholipid syndrome is characterized by a reduction in platelets. A minority of patients might develop catastrophic antiphospholipid syndrome, characterized by occlusion of small vessels and multiple organ disease in the presence of antiphospholipid antibodies.