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Renal involvement in antiphospholipid syndrome

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Abstract

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.

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Acknowledgements

Jozelio Freire de Carvalho received Grants from the Federico Foundation and from the CNPq (300665/2009-1).

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All authors were involved in drafting the article or revising it critically for important intellectual content, and all authors approved the final version to be submitted for publication. Dr. CEMR had full access to all study data and takes responsibility for the integrity of the data. Study conception and design: FVAdeA, DGM, CEMR. Acquisition of data: FVAdeA, CEMR. Analysis and interpretation of data: FVAdeA, JFdeC, CEMR.

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Correspondence to Carlos Ewerton Maia Rodrigues.

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de Azevedo, F.V.A., Maia, D.G., de Carvalho, J.F. et al. Renal involvement in antiphospholipid syndrome. Rheumatol Int 38, 1777–1789 (2018). https://doi.org/10.1007/s00296-018-4040-2

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