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Rheumatology International
Erschienen in: Rheumatology International 10/2018

05.05.2018 | Review

Renal involvement in antiphospholipid syndrome

verfasst von: Francisco Vileimar Andrade de Azevedo, Diego Germano Maia, Jozelio Freire de Carvalho, Carlos Ewerton Maia Rodrigues

Erschienen in: Rheumatology International | Ausgabe 10/2018

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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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Metadaten
Titel
Renal involvement in antiphospholipid syndrome
verfasst von
Francisco Vileimar Andrade de Azevedo
Diego Germano Maia
Jozelio Freire de Carvalho
Carlos Ewerton Maia Rodrigues
Publikationsdatum
05.05.2018
Verlag
Springer Berlin Heidelberg
Erschienen in
Rheumatology International / Ausgabe 10/2018
Print ISSN: 0172-8172
Elektronische ISSN: 1437-160X
DOI
https://doi.org/10.1007/s00296-018-4040-2

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