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ANCA-associated vasculitis (AAV) includes three disease subtypes with partly overlapping clinical manifestations: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). This Review article provides an update on the diagnosis and classification of AAV, discussing parameters for assessing disease activity and predicting outcomes towards a personalized medicine approach.

Immunosuppressive treatment of patients with idiopathic membranous nephropathy is controversial because of the toxicity of the therapy and the variable natural course of the disease. In this Review, the authors discuss the 2012 Kidney Disease: Improving Global Outcomes guideline for the treatment of patients with idiopathic membranous nephropathy and highlight the remaining areas of uncertainty.

As granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) share histopathologic and clinical features, including the presence of antineutrophil cytoplasmic autoantibodies (ANCAs), patients with these syndromes are commonly labelled as having ANCA-associated vasculitis (AAV) and are subjected to similar treatments. A recent study suggests that dividing AAV into five classes based on disease phenotype provides better prognostic information than the classic division into GPA and MPA.

Two recent studies have investigated the prevalence of anti-hLAMP-2 antibodies in patients with antineutrophil cytoplasmic antibody-associated vasculitides. Results from these studies suggest that more work is needed to standardize the assays used to detect anti-hLAMP-2 antibodies in patient sera before we can estimate their true prevalence in different patient populations and assess their potential clinical utility.

Knowledge of the pathogenesis of glomerular disease and approaches to therapy continued to advance in 2014. Key studies identified thrombospondin type-1 domain-containing protein 7A as an antigenic target in primary membranous nephropathy, and demonstrated efficacy of rituximab as maintenance therapy in relapsing or steroid-dependent nephrotic syndrome and antineutrophil cytoplasmic antibody-associated vasculitis.

Since the discovery of PLA2R, several target antigens associated with membranous nephropathy have been identified. This Review describes the distinct clinical associations, pathology and prognostic findings associated with each of the identified target antigens and implications for the reclassification of membranous nephropathy.

Membranous nephropathy (MN) is a rare autoimmune disease of podocyte-directed antibodies, such as anti-phospholipase A2 receptor. Here, the authors report a genome-wide association study for MN and identify two previously unreported loci encompassing the NFKB1 and IRF4 genes and additional ancestry-specific effects.

Membranous nephropathy is an immune-mediated disease and is the leading cause of nephrotic syndrome in adults. Here, the authors discuss the role of B cell-depleting regimens in the treatment of this disease and the potential use of rescue therapy with agents that target plasma cells, which might prevent antigen–antibody interactions and immune complex-mediated complement activation.

Focal segmental glomerulosclerosis is a histopathological lesion that has heterogeneous causes. In this Perspectives article, the authors outline a rationale for the stratification of focal segmental glomerulosclerosis based on current understanding of the pathophysiology of podocyte stress and damage and propose that such an approach will improve the rational design and success of therapeutic trials.

The type of anti-neutrophil cytoplasmic antibody (ANCA) seems to be a major determinant of clinical presentation in ANCA-associated vasculitides (AAV). The authors of this Review argue that ANCA specificity for PR3 or MPO provides clinically useful information and should be used in the classification of AAV.

Cryoglobulinaemia is characterized by the presence of precipitable immunoglobulins in the serum and can lead to a clinical syndrome called cryoglobulinaemic vasculitis. This Primer describes the underlying mechanisms, diagnosis and management of cryoglobulinaemia.

Membranous nephropathy is a glomerular disease that is the most frequent cause of nephrotic syndrome in adults. This Primer reviews the epidemiology, pathophysiology, diagnosis and management of this disease and discusses how to improve long-term renal prognosis and quality of life.

This Expert Consensus Document from the International Kidney and Monoclonal Gammopathy Research Group includes an updated definition of monoclonal gammopathy of renal significance (MGRS) and recommendations for the use of kidney biopsy and other modalities for evaluating suspected MGRS