The complement cascade comprises soluble and cell surface proteins and is an important arm of the innate immune system. Once activated, the complement system rapidly generates large quantities of protein fragments that are potent mediators of inflammatory, vasoactive and metabolic responses. Although complement is crucial to host defence and homeostasis, its inappropriate or uncontrolled activation can also drive tissue injury. For example, the complement system has been known for more than 50 years to be activated by glomerular immune complexes and to contribute to autoimmune kidney disease. Notably, the latest research shows that complement is also activated in kidney diseases that are not traditionally thought of as immune-mediated, including haemolytic-uraemic syndrome, diabetic kidney disease and focal segmental glomerulosclerosis. Several complement-targeted drugs have been approved for the treatment of kidney disease, and additional anti-complement agents are being investigated in clinical trials. These drugs are categorically different from other immunosuppressive agents and target pathological processes that are not effectively inhibited by other classes of immunosuppressants. The development of these new drugs might therefore have considerable benefits in the treatment of kidney disease.

• MeSH
• Complement Activation MeSH
• Autoimmune Diseases * MeSH
• Kidney Glomerulus pathology   MeSH
• Complement System Proteins metabolism   MeSH
• Kidney metabolism   MeSH
• Humans MeSH
• Kidney Diseases * metabolism   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Review MeSH
• Names of Substances
• Complement System Proteins MeSH

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are a group of diseases characterised by necrotizing inflammation of small vessels such as arterioles, venules, and capillaries. ANCA-associated vasculitides (AAV) are referred to as small vessel vasculitides. Three AAV subgroups, namely granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA), are defined according to clinical features. The most common disease with renal involvement in AAV is MPA Approximately 90% of patients with MPA have renal involvement. While this rate is 70-80% in GPA, less than half of EGPA patients have renal involvement. Untreated survival in AAVs is less than one year. With appropriate immunosuppressive therapy, the 5-year renal survival rate is 70-75%. Without therapy, the prognosis is poor but treatments, typically immunosuppressants, have improved survival, albeit with considerable morbidity from glucocorticoids and other immunosuppressive medications. Current challenges include improving the measures of disease activity and risk of relapse, uncertainty about optimal therapy duration and a need for targeted therapies with fewer adverse effects. In this review, we described the treatment of renal involvement in AAV in line with current studies.

• Keywords
• Anti-neutrophil cytoplasmic antibody, Eosinophilic GPA, Granulomatosis with polyangiitis, Microscopic polyangiitis,
• MeSH
• Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis * complications   drug therapy   MeSH
• Churg-Strauss Syndrome * drug therapy   MeSH
• Granulomatosis with Polyangiitis * complications   drug therapy   MeSH
• Immunosuppressive Agents therapeutic use   MeSH
• Humans MeSH
• Microscopic Polyangiitis * drug therapy   MeSH
• Kidney Diseases * drug therapy   MeSH
• Antibodies, Antineutrophil Cytoplasmic MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Review MeSH
• Names of Substances
• Immunosuppressive Agents MeSH
• Antibodies, Antineutrophil Cytoplasmic MeSH

Efficacy of immunosuppressive treatment of Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is complicated by its toxicity. With the replacement of cyclophosphamide with rituximab, serious adverse events seem to be associated especially with high-dose corticosteroids. Activation of alternative complement pathway plays an important role in the pathogenesis of AAV. Avacopan (C5a receptor inhibitor) was demonstrated to have at least similar efficacy and better safety (in terms of corticosteroid-related adverse events) compared with high-dose corticosteroids in the induction treatment of AAV. Other modes of the inhibition of alternative complement pathway are currently tested in AAV or could be considered on the basis of the experience in other glomerular diseases.

• Keywords
• ANCA, ANCA - associated vasculitis, C5a, avacopan, complement,
• MeSH
• Complement Pathway, Alternative MeSH
• Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis * MeSH
• Cyclophosphamide adverse effects   MeSH
• Humans MeSH
• Antibodies, Antineutrophil Cytoplasmic MeSH
• Rituximab therapeutic use   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Research Support, Non-U.S. Gov't MeSH
• Review MeSH
• Names of Substances
• Cyclophosphamide MeSH
• Antibodies, Antineutrophil Cytoplasmic MeSH
• Rituximab MeSH

IgA nephropathy is the most common primary glomerulonephritis with potentially serious outcome leading to end stage renal disease in 30 to 50% of patients within 20 to 30 years. Renal biopsy, which might be associated with risks of complications (bleeding and others), still remains the only reliable diagnostic tool for IgA nephropathy. Therefore, the search for non-invasive diagnostic and prognostic markers for detection of subclinical types of IgA nephropathy, evaluation of disease activity, and assessment of treatment effectiveness, is of utmost importance. In this review, we summarize treatment options for patients with IgA nephropathy including the drugs currently under evaluation in randomized control trials. An early initiation of immunosupressive regimens in patients with IgA nephropathy at risk of progression should result in the slowing down of the progression of renal function to end stage renal disease.

• Keywords
• ACEI, CKD, IgAN, corticosteroids, progression, proteinuria,
• MeSH
• Models, Biological MeSH
• Glomerulonephritis, IGA therapy   MeSH
• Clinical Trials as Topic MeSH
• Humans MeSH
• Risk Factors MeSH
• Practice Guidelines as Topic MeSH
• Animals MeSH
• Check Tag
• Humans MeSH
• Animals MeSH
• Publication type
• Journal Article MeSH
• Review MeSH