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Autoimmun Rev. 2019 May;18(5):510-518. doi: 10.1016/j.autrev.2019.03.004. Epub 2019 Mar 4.

Refractory lupus nephritis: When, why and how to treat.

Author information

1
Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Hills Road, CB2 0QQ, Cambridge, Cambridge University Hospitals, United Kingdom; Department of Internal Medicine IV (Nephrology and Hypertension), Medical University of Innsbruck, Anichstraße 35, 6020 Innsbruck, Austria.
2
Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Hills Road, CB2 0QQ, Cambridge, Cambridge University Hospitals, United Kingdom.
3
Department of Internal Medicine IV (Nephrology and Hypertension), Medical University of Innsbruck, Anichstraße 35, 6020 Innsbruck, Austria.
4
Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Hills Road, CB2 0QQ, Cambridge, Cambridge University Hospitals, United Kingdom; Servicio de Nefrología y Trasplante Renal, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Universidad de Barcelona, Barcelona, Spain.
5
Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Hills Road, CB2 0QQ, Cambridge, Cambridge University Hospitals, United Kingdom; Department of Medicine, University of Cambridge, CB2 0QQ Cambridge, United Kingdom. Electronic address: dj106@cam.ac.uk.

Abstract

Refractory lupus nephritis indicates an inadequate response to lupus nephritis therapy. It implies persisting or worsening disease activity despite therapy, but the definition is complicated by the parameters of response, proteinuria and renal function, that do not discriminate clearly between activity and irreversible damage. Understanding the causes of refractory disease and developing treatment strategies is important because these patients are more likely to develop poor outcomes, especially end stage renal disease. This review explores current concepts and definitions of refractory disease and summarises treatment approaches that have been used in observational cohort studies and case series. We highlight the importance of optimising adherence to the prescribed immunosuppressive and supportive measures and avoidance of diagnostic delay. Treatment options include higher dose glucocorticoid, switching between cyclophosphamide and mycophenolate acid derivates, or addition of rituximab, the latter potentially in combination with belimumab. Less evidence supports extracorporeal treatment (plasma exchange or immunoadsorption), calcineurin inhibitors (cyclosporine A or tacrolimus), intravenous immunoglobulin and stem cell transplantation. Improvements in understanding what refractory disease is and how definitions can be integrated into treatment pathways has the potential to enhance lupus nephritis outcomes.

KEYWORDS:

Bortezomib; Immunoadsorption; Lupus nephritis; Multi-target; Refractory; Rituximab; Transplantation; Treatment

PMID:
30844548
DOI:
10.1016/j.autrev.2019.03.004
[Indexed for MEDLINE]

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