Thrombotic microangiopathy due to multiple autoantibodies related to antiphospholipid syndrome

Section: Casementioning

confidence: 86%

Systemic lupus erythematosus with ADAMTS13 inhibitor-negative thrombotic microangiopathy treated with combination of mycophenolate mofetil, plasma exchange and steroid

Okubo

Shoda

Bannaĭ

et al. 2016

“…TMA changes in APS patients may be isolated and clinically silent at the onset of the disease, which is concordant with the current case. 11–14 Thrombi of varying ages or degrees of organization, although not specific, could be suggestive of APS. In our patient, no drugs including chemotherapeutic agents or calcineurin inhibitors, which are associated with TMA, were administered.…”

Section: Discussionmentioning

confidence: 96%

A 3-year follow-up of a patient with acute renal failure caused by thrombotic microangiopathy related to antiphospholipid syndrome: case report

Chen

et al. 2016

Background Microvascular manifestations of antiphospholipid antibody syndrome in the kidneys include acute renal failure, thrombotic microangiopathy and hypertension. Therapy has been largely empiric. Case report A 49-year-old Chinese man presented with anuric acute renal failure without abundant proteinuria and heavy haematuria, but markedly low levels of urinary sodium, potassium and chlorine upon admission. On day 1 of hospitalization, his thrombocytopenia, anaemia and renal failure showed rapid progression. The presence of lupus anticoagulant and vascular ischaemia of the small vessels in renal arteriography were also observed. Anticoagulants, continuous renal replacement therapy, glucocorticoids and six sessions of plasma exchange were started. After the fourth plasma exchange (on day 20), his urine output increased and began to normalize. On day 25, haemodialysis was stopped and his general condition gradually improved. A renal biopsy was subsequently performed, and the histopathological diagnosis was thrombotic microangiopathy due to antiphospholipid antibody syndrome. A further 3-year follow-up showed that his haemoglobin level, platelet count and serum creatinine were within the normal range, with stable blood pressure. Conclusion Treatment modalities such as anticoagulation, immunosuppression and plasma exchange are likely to be necessary when severe acute renal failure combined with thrombotic microangiopathy present in nephropathy of antiphospholipid antibody syndrome.

“…30 Since then, an increasing number of cases have been characterized by TMA-like small-vessel occlusions, often affecting the kidney. 4,5,9 Although the pathogenesis of APS nephropathy is not yet fully understood, antiphospholipid antibodies and complement activation have been recognized as important mechanisms in TMA development. 31 Other organs, such as the skin, central nervous system, lungs, heart, and eyes, may also be affected.…”

Section: Discussionmentioning

confidence: 99%

“…Only a few case reports of APS with microangiopathy are available in the literature. 4–6 The aim of this cohort study was to compare the clinical manifestations, laboratory features, and outcomes of primary APS patients with and without microvascular involvement.…”

Section: Introductionmentioning

confidence: 99%

Clinical characteristics and risk factors of microvascular involvement in primary antiphospholipid syndrome: a longitudinal single-center study in China

Sun¹,

Zhao²,

Zhang³

et al. 2019

Objectives The objective of this study was to investigate risk factors of microvascular involvement and survival in Chinese patients with primary antiphospholipid syndrome. Methods In this single-center, retrospective study, we enrolled 112 patients with a confirmed diagnosis of primary antiphospholipid syndrome who were admitted to Peking Union Medical College Hospital from January 2004 to December 2016. Demographic data, clinical characteristics, laboratory results, and follow-up records were collected. Results A total of 112 patients with primary antiphospholipid syndrome were studied. Microvascular involvement was identified in 21 patients (18.75%). Patients with microvascular involvement experienced fewer episodes of arterial or venous thrombosis (28.6% vs. 84.6%) and a higher incidence of thrombocytopenia (85.7% vs. 54.9%), respectively. Low complement and elevated high-sensitivity CRP levels were observed more frequently in the microvascular group compared with the non-microvascular group (complement 38.1% vs. 18.7%; high-sensitivity CRP 71.4% vs. 31.9%, respectively). Anti-β2-glycoprotein I antibodies were more prevalent in patients with microvascular involvement than in patients without (66.7% vs. 33.0%, respectively). Multivariate logistic regression analysis revealed that thrombocytopenia (odds ratio = 4.523, 95% confidence interval 1.139–17.962), elevated high-sensitivity CRP levels (odds ratio = 6.385, 95% confidence interval 1.969–20.704), and anti-β2-glycoprotein I antibody positivity (odds ratio = 5.042, 95% confidence interval 1.555–16.352) were independent risk factors for microvascular involvement. A Kaplan–Meier analysis revealed that survival was significantly poorer in patients with microvascular involvement compared with patients without ( p = 0.0278). Conclusions In addition to arterial and venous thrombosis, antiphospholipid syndrome can affect the microvasculature of select organs. It is thus important for clinicians to be aware that antiphospholipid syndrome-associated microvascular involvement has a unique pathogenesis and can be a life-threatening condition.