Rheumatology articles within Nature Reviews Nephrology

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  • Review Article |

    Here, the authors review traditional and disease-specific risk factors for vascular damage and the cellular and molecular mechanisms that drive vascular injury in systemic lupus erythematosus. They also discuss cardiovascular risk assessment, primary prevention strategies and current and future treatment approaches to cardiovascular disease in systemic lupus erythematosus.

    • William G. Ambler
    •  & Mariana J. Kaplan

  • Review Article |

    Autoimmune diseases are a diverse group of conditions characterized by aberrant B cell and T cell reactivity to normal host constituents. This Review provides an overview of the basis of autoimmune disease with a focus on autoantibodies, given their valuable role as markers for diagnosis, classification and of disease activity.

    • David S. Pisetsky

  • News & Views |

    Targeting type I interferon immune responses is a potential strategy for the treatment of systemic lupus erythematosus. Although a phase 2 clinical trial of anifrolumab did not meet its primary end point, further studies are needed to assess the effects of interferon blockade on flare rates of lupus nephritis. However, the observed higher risk of herpes zoster associated with anifrolumab use suggests that caution is warranted with this strategy.

    • Stefanie Steiger
    •  & Hans-Joachim Anders

  • News & Views |

    Glucocorticoids have, for decades, been an enduring component of treatment regimens for anti-neutrophil cytoplasm autoantibody (ANCA)-associated vasculitis. However, a growing number of studies have encouraged a move towards reduced glucocorticoid usage. Findings of the ADVOCATE trial suggest that complement blockade may pave the way for complete glucocorticoid avoidance.

    • Maria Prendecki
    •  & Stephen P. McAdoo

  • Year in Review |

    In 2020 a number of clinical trials have provided insights into therapeutic approaches for the treatment of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis and lupus nephritis. Moreover, mechanistic insights have potential to open new therapeutic strategies in the future.

    • Isabelle Ayoub
    •  & Patrick H. Nachman

  • News & Views |

    The PEXIVAS clinical trial demonstrated that, in patients with anti-neutrophil cytoplasmic autoantibody-associated vasculitis (AAV), adjuvant plasma exchange did not reduce the risk of all-cause mortality or end-stage kidney disease and a reduced dose of glucocorticoids was not inferior to standard dosing. These findings might warrant a change in standard AAV therapy.

    • Adam Morris
    •  & Duvuru Geetha

  • Review Article |

    Various cell types release extracellular DNA traps that protect the host against microbial infections. In this Review, the authors discuss how DNA traps not only participate in pathogen clearance but can also promote vascular disease and autoimmunity.

    • Christoph Daniel
    • , Moritz Leppkes
    •  & Martin Herrmann

  • Year in Review |

    In 2017, progress was made in several aspects of immune-mediated kidney disease. Mechanistic studies provided new insights into the underlying signals that confer risk to, or protection from, immune pathways, whereas new approaches to the treatment of immunological kidney disease will hopefully translate into a move away from the use of toxic corticosteroids.

    • Stephen R. Holdsworth
    •  & A. Richard Kitching

  • Review Article |

    Current treatment recommendations for lupus nephritis are largely guided by the 2003 International Society of Nephrology/Renal Pathology Society classification system. Here, the authors discuss potential approaches by which this classification system could be improved through consideration of underlying disease processes characterized by the presence of glomerular crescents, podocyte injury, tubulointerstitial lesions and vascular injury.

    • Feng Yu
    • , Mark Haas
    •  & Ming-Hui Zhao

  • News & Views |

    Mechanistic studies have implicated the alternative complement pathway, specifically C5a receptor activation, in the pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis. Results from a phase 2 trial suggest that C5a receptor blockade could enable the reduced use or complete withdrawal of steroids from induction protocols.

    • Ralph Kettritz

  • Review Article |

    T cells are critical drivers of autoimmunity and related organ damage, by supporting B-cell differentiation and antibody production or by directly promoting inflammation and cytotoxicity. This Review discusses the immune features of autoimmune nephropathies, with a focus on systemic lupus erythematosus and the role of T cells.

    • Abel Suárez-Fueyo
    • , Sean J. Bradley
    •  & George C. Tsokos

  • Review Article |

    Scleroderma renal crisis is a rare, potentially life-threatening complication of systemic sclerosis. Here, the authors discuss advances made in the detection, management and prognosis of scleroderma renal crisis, which can limit the progression of affected patients to chronic kidney disease.

    • Thasia G. Woodworth
    • , Yossra A. Suliman
    •  & Philip Clements

  • Review Article |

    The development of novel immunotherapies has improved treatment options for several immune-mediated diseases; however, outcomes for patients with immune-mediated kidney disease remain poor. In this Review, Hans-Joachim Anders, David Jayne and Brad Rovin suggest that this lack of progress relates to shortcomings in the approaches used to identify targets and measure outcomes. They discuss seven hurdles to the validation of therapeutic targets in immune-mediated kidney diseases and describe how these hurdles can be overcome to appropriately assess and introduce immunologic therapies for immune-mediated kidney disease.

    • Hans-Joachim Anders
    • , David R. W. Jayne
    •  & Brad H. Rovin

  • Review Article |

    Renal involvement in primary Sjögren syndrome (pSS) is a rare complication, but regular screening is required for early detection and prevention of progression to chronic kidney disease. In this Review, Hélène François and Xavier Mariette discuss the most frequent renal complications that can occur in pSS, namely tubulointerstitial nephritis and membranoproliferative glomerular nephritis. They outline the pathophysiology of these complications, the differential diagnoses, and current treatment options.

    • Hélène François
    •  & Xavier Mariette

  • Review Article |

    IgG4-related disease can affect any organ system, including the kidney. Renal involvement is an indication for prompt treatment, owing to an increased risk of chronic kidney disease. In this Review, Frank Cortazar and John Stone provide an overview of systemic IgG4-related disease before describing in detail the renal manifestations of IgG4-related tubulointerstitial nephritis and membranous glomerulonephropathy secondary to IgG4-related disease. They consider the pathophysiology, as well as current and future treatment options.

    • Frank B. Cortazar
    •  & John H. Stone

  • News & Views |

    Despite aggressive therapy, lupus nephritis (LN) remains an important predictor of morbidity in patients with systemic lupus erythematosus. Clinical trials of novel drugs have not improved LN outcomes; however, re-analysis of well-characterized cohorts has identified surrogate end points of long-term renal survival, which will facilitate testing and qualification of novel treatments.

    • Brad H. Rovin
    •  & Isabelle Ayoub

  • Review Article |

    Systemic lupus erythematosus (SLE) is a complex, autoimmune disorder that can have debilitating effects on various organs, including the kidneys. Here, Mohan and Putterman discuss the genetic candidates associated with SLE and lupus nephritis, and highlight possible molecular mechanisms that might contribute to disease pathology. Given the highly variable clinical presentation of SLE, the authors propose that greater understanding of the molecular basis of SLE and lupus nephritis is required to facilitate the generation of specific tailored therapies in the future.

    • Chandra Mohan
    •  & Chaim Putterman

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