Catastrophic antiphospholipid antibody syndrome

Abstract

Antiphospholipid antibody syndrome (APS) is characterized by recurrent thrombosis with the presence of circulating antiphospholipid antibodies. A diagnosis of APS requires the presence of at least one clinical and one laboratory criteria (detection of aCL IgG or IgM antibodies or the presence of lupus anticoagulant on two or more consecutive occasions 6 weeks apart). A severe, rapidly progressive form characterized by clinical involvement of at least three different organ systems with histopathological evidence of small and large vessel occlusion is termed catastrophic antiphospholipid syndrome. Early recognition of APS is crucial since aggressive management can result in a favorable outcome. We present the case of a 12-year-old boy who presented with a devastating illness with multiple thrombotic episodes and rapidly progressive renal failure.

Renal Involvement in the Antiphospholipid Syndrome (APS)—APS Nephropathy

“…A possible explanation is that CAPS is a very rare disease, and furthermore, kidney biopsies are only rarely performed because of the catastrophic nature of the syndrome. In isolated cases with CAPS where a kidney biopsy was performed [98][99][100][101][102][103], as well as in autopsy material [96], fibrin thrombi have been detected in arterioles and glomerular tuft (TMA). The presence of chronic vascular lesions such as FIH and FCA has been only sporadically described [99,103].…”

Catastrophic antiphospholipid syndrome presented with sudden renal failure and history of long-lasting psychosis and hypertension in a 42 years old women

“…CAPS is the most severe form of APS with acute multiple organ involvement and small vessel thrombosis (4)(5)(6)(7)(8)(9). CAPS is an accelerated variant of APS, resulting in multi-organ failure due to extensive thrombosis formation (2)(3)(4).The diagnosis of this syndrome requires the presence of clinical evidence of involvement of three or more organs in a period of less than a week, histopathological evidence of small vessel occlusion in at least one organ, and finally laboratory confirmation of the presence of antiphospholipid antibodies (APAs), usually in high titer (2-6).…”

“…However, they are usually in a lifethreatening situation and its potentially lethal outcome emphasizes its importance in clinical medicine today. The rarity of this variant of APSN makes it extraordinarily difficult to study in any systematic way (4)(5)(6)(7)(8)(9). Early recognition of APS is crucial, while aggressive management can result in a favorable outcome (2)(3)(4)(5).…”

“…The recognized renal manifestations of this syndrome defined as APS nephropathy, are the renal artery thrombosis/stenosis, renal vein thrombosis, renal infarction, end-stage renal disease, increased allograft vascular thrombosis, some types of glomerular disease, hypertension and thrombotic microangiopathy (TMA) (1,2,(10)(11)(12). TMA is characterized by acute thrombotic lesions in glomeruli and/ or arterioles and chronic vascular lesions such as fibrous intimal hyperplasia of arterioles and interlobular arteries, organized thrombi with or without recanalization, and fibrous arterial and arteriolar occlusions or focal cortical atrophy (3)(4)(5)(6)(7)(8)(9). TMA is histological diagnosis and clinical differential diagnosis includes malignant hyper-tension, hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, disseminated intravascular coagulation, scleroderma, toxemia of pregnancy and APS.…”

Microthrombotic/Microangiopathic Manifestations of the Antiphospholipid Syndrome

“…In the published literature, microvascular thrombotic/ microangiopathic manifestations of APS in children have been described in several case reports or small case series [63][64][65][66][67][68][69], although there are very few reports with wellcharacterized histopathologic findings. Tsirpanlis et al [65] described a 14-year-old girl with persistently positive aPL who developed rapidly progressive renal, cardiac, and hepatic-biliary involvement.…”