This is a review of scientific publications on renal involvement in antiphospholipid syndrome (APS), with focus on clinical and histopathological findings and treatment. A search for English-language articles on renal involvement in APS covering the period 1980-2017 was conducted in Medline/PubMed and Scopus databases using the MeSH terms "antiphospholipid syndrome", "antiphospholipid antibodies", "glomerulonephritis" and "thrombotic microangiopathy" (TMA). APS nephropathy is primarily the result of thromboses in renal arteries or veins, intraparenchymatous arteries and glomerular capillaries. On histology, APS nephropathy is characterized by TMA, but chronic vaso-occlusive lesions are also commonly observed (fibrous intimal hyperplasia, focal cortical atrophy, fibrous occlusions of arteries). Anticardiolipin and lupus anticoagulant are the most prevalent antibodies in patients with APS nephropathy. The spectrum of renal manifestations includes renal vein thrombosis, renal artery thrombosis/stenosis, TMA, increased allograft vascular thrombosis and malignant hypertension. Anticoagulation is the standard treatment of thrombotic events. In systemic lupus erythematosus (SLE) patients with antiphospholipid antibodies (aPL), kidney failure due to SLE nephritis (immune-complex disease) should be clearly distinguished from kidney failure due to APS-related TMA. In such cases, renal biopsy is mandatory. SLE nephritis requires immunosuppressive therapy, whereas APS nephropathy is usually treated with anticoagulants. Recently, eculizumab and sirolimus have been proposed as a rescue therapy. Based on our review, APS nephropathy appears to be a distinct clinical condition. TMA is a characteristic histopathological finding in APS and is strongly associated with the presence of aPL. This has important therapeutic implications and allows distinguishing APS nephropathy from lupus nephritis.
Background: Little has been published about the epidemiology of Granulomatosis with polyangiitis (GPA) in South America, especially in the intertropical zone, and no epidemiological data from Brazil are available. The purpose of the present study was to draw a clinical and demographic profile of GPA patients living in Northeastern Brazil based on laboratory, histological and imaging findings, and evaluate the frequency of organic involvement.Methods: Clinical, epidemiological and treatment data of GPA patients were collected retrospectively and compared with the literature.Results: The cohort included 25 GPA patients (84% female) aged 45.8 ± 16.1 years. Renal and ear-nose-throat (ENT) manifestations were the most common (both 64%). One third (32%) of the patients had 24-h proteinuria > 1 g, 50% had creatinine clearance < 50 mL/min at the time of diagnosis, and 33% had recurrent kidney damage during disease progress. The affected organs included lungs (60%), joints (44%), skin (32%), peripheral nervous system (28%), eyes (28%) and heart (16%). ENT involvement (n = 16/64%) was less frequent in our region than in São Paulo (n = 115/85.8%). Renal (n = 16/64%) and pulmonary (n = 15/60%) involvement was less frequent in our region than in the U.K. (renal n = 30/90%; pulmonary n = 28/84.8%).
Libman-Sacks endocarditis consists of aseptic valvular abnormalities, associated with systemic lupus erythematosus and antiphospholipid syndrome. Embolic ischaemic stroke is a possible clinical presentation. The authors present the case of a woman in her fourth decade who developed central facial palsy after several transient ischaemic attacks with visual loss. Cerebral imaging revealed multiple small ischaemic lesions in the right hemisphere. The transoesophagic echocardiogram showed mitral vegetations and she tested positive for antiphospholipid antibodies. She underwent mitral valve replacement for a mechanical prosthesis due to extensive valvular damage and started anticoagulation. The valve's microbiological exams were negative establishing the diagnosis of libman-sacks endocarditis. Endocarditis should be suspected with sequential cerebral ischaemia in patients of all ages. Valvular surgery is the mainstay of treatment in recurrent embolic events. Association with antiphospholipid antibodies is common yet a poor-known fact. The patient is currently asymptomatic on warfarin and aspirin, with a normal functioning mitral prosthesis.
To evaluate PBS ® MCIMMO cement in the filling of bone defects. Methods: Thirty-six adult male Wistar rats were divided into three groups of twelve individuals each (group 1, group 2 and group 3). In all groups, a bone failure in the femur was induced, 2.0 mm wide and 7.0 mm deep. In group 1, the PBS ® MCIMMO cement was applied to the bone defect produced and a titanium implant (CONNECTION ®) 1.5 mm thick and 6 mm long was installed. In group 2, only the PBS ® CIMMO cement was installed. In group 3, only bone failure was performed. Kruskal Wallis tests were performed to compare the mean area among the three groups. Results: In all comparisons, significance was observed for group 2 (p = 0.0014-0.0026). Conclusion: The PBS ® CIMMO cement induced bone neoformation, and integration between the newly formed bone, cement, and implant was observed.
Boiling-temperature measurements have been used to study the kinetics of the decomposition of urea. Values for the rate constants in neutral, acid, and basic media have been obtained and compared with literature data. The effect of the reverse reaction on the value of the experimental rate constants is discussed. The profile of the ebulliometric curves is different for the three media and is correlated with the number of various species present in solution. It is concluded that ebulliometric measurements can be useful for kinetic studies.
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