Complement in ANCA-associated vasculitis: mechanisms and implications for management

Nature Reviews Nephrology 13, 359 (2017). doi:10.1038/nrneph.2017.37 Authors: Min Chen, David R. W. Jayne &Ming-Hui Zhao Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of potentially life-threatening autoimmune diseases. The main histological feature in the kidneys of patients with AAV is pauci-immune necrotizing crescentic glomerulonephritis with little immunoglobulin and complement deposition in the glomerular capillary walls. The complement system was
Source: Nature Reviews Nephrology - Category: Urology & Nephrology Authors: Tags: Review Source Type: research

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Authors: Hamano Y, Ito F, Suzuki O, Koura M, Matsuoka S, Kobayashi T, Sugitani Y, Wali N, Koyanagi A, Hino O, Suzuki S, Sugamata R, Yoshizawa H, Yumura W, Maruyama N, Kameoka Y, Noda Y, Hasegawa Y, Arai T, Suzuki K Abstract Lupus nephritis (LN) is the secondary glomerulonephritis (GN) involved in systemic lupus erythematosus (SLE) and a typical immune complex-type GN. Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is an autoimmune disease characterized by systemic vasculitis and pauci-immune-type crescentic glomerulonephritis (CrGN) with ANCA production. Human AAV causes death due to lun...
Source: Autoimmunity - Category: Allergy & Immunology Tags: Autoimmunity Source Type: research
Multisystem autoimmune diseases, including systemic lupus erythematosus (SLE) and vasculitis, are inflammatory conditions of unknown cause. Renal involvement can occur in a variety of forms and usually represents a severe disease manifestation. SLE is complicated by renal involvement (lupus nephritis) in over one-third of patients. Small vessel vasculitides, including antineutrophil cytoplasmic antibody-associated and antiglomerular basement membrane disease, also frequently affect the kidneys, causing a rapidly progressive glomerulonephritis.
Source: Medicine - Category: Internal Medicine Authors: Tags: Systemic disease and the kidney Source Type: research
Predictive markers of lymphomagenesis in Sjögren's syndrome: From clinical data to molecular stratification. J Autoimmun. 2019 Aug 17;:102316 Authors: Kapsogeorgou EK, Voulgarelis M, Tzioufas AG Abstract Sjögren's syndrome (SS) is a chronic systemic autoimmune disease, affecting predominantly the exocrine glands, a large array of systemic manifestations and high risk of lymphoma development. The latter constitutes the major adverse outcome of SS contributing in the increased morbidity and mortality of the disease. The vast majority of lymphomas in SS are B-cell non-Hodgkin's lymphomas (NHL),...
Source: Journal of Autoimmunity - Category: Allergy & Immunology Authors: Tags: J Autoimmun Source Type: research
Abstract Cryoglobulins are antibodies that precipitate at low temperatures and dissolve after rewarming. Cryoglobulinemia refers to the presence of circulating cryoglobulins and generally leads to a systemic inflammatory syndrome characterized by fatigue, arthralgia, purpura, ulcers, neuropathy and/or glomerulonephritis. The disease mainly involves small to medium-sized blood vessels and causes vasculitis due to cryoglobulin-containing immune complexes. Cryoglobulinemia is classified into three types (I, II and III) on the basis of immunoglobulin composition. Predisposing conditions include lymphoproliferative, au...
Source: Journal of Autoimmunity - Category: Allergy & Immunology Authors: Tags: J Autoimmun Source Type: research
This article reports a case of coexistence of recurrent anti-GBM disease and T-cell large granular lymphocytic (T-LGL) leukemia. Patient concerns: A 37-year-old man presented with hematuria, edema, and acute kidney injury for 2 months. Diagnosis: Anti-GBM disease was diagnosed by renal biopsy, in which crescentic glomerulonephritis was observed with light microscopy, strong linear immunofluorescent staining for immunoglobulin G on the GBM and positive serum anti-GBM antibody. Given this diagnosis, the patient was treated with plasmapheresis, steroids, and cyclophosphamide for 4 months. The anti-GBM antibody titer was...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
Crescentic glomerulonephritis (GN) without immune reactants or deposits (referred to as pauci-immune) is typically characterized by the presence of anti-neutrophilic cytoplasmic antibodies (ANCA). While ANCA-negative patients might be expected to have a more benign course, they often have poor renal outcomes, especially without treatment with steroids and immune-modulating therapy. Pauci-immune crescentic GN can also co-exist with other autoimmune conditions, including rheumatoid arthritis (RA). Here, we describe an ANCA-negative patient with RA who developed dialysis-requiring acute kidney injury (AKI) with findings consi...
Source: Case Reports in Nephrology and Dialysis - Category: Urology & Nephrology Source Type: research
Conclusion: Our data indicate reduced P2X7R expression and function in SLE patients compared with HC and, conversely, increased IL-6 signaling. The possible consequences of reduced P2X7R, mainly on cytokines network deregulation and lymphocyte proliferation, will be further investigated as well as the role of IL-6 as a possible therapeutic target especially in lupus serositis. Introduction With the first reports of Burnstock in 1970s, adenosine triphosphate (ATP) has passed from a simple molecule devoted to energy reserve, to a relevant extracellular signaling molecule able to mediate numerous physiological and pat...
Source: Frontiers in Pharmacology - Category: Drugs & Pharmacology Source Type: research
Shanshan Zhang1, Dongli Yuan2 and Ge Tan1* 1Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China 2The Institute of Medical Information, Chongqing Medical University, Chongqing, China Primary systemic vasculitis can affect every structure in both the central and peripheral nervous system, causing varied neurological manifestations of neurological dysfunction. Early recognition of the underlying causes of the neurological symptoms can facilitate timely treatment and improve the prognosis. This review highlights the clinical manifestations of primary systemic vasc...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
Rationale: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and anti-glomerular basement membrane (GBM) antibody disease are both rare autoimmune diseases. Monoclonal gammopathy of undetermined significance (MGUS) is one of the most common causes of plasma cell dyscrasias (PCD). The three entities can cause renal lesions via different mechanisms and, however, they have not been reported in a single patient with renal lesion. Patient concerns: Here, we describe a patient with half-year fatigue and 40-day nausea and vomiting. Laboratory workup displayed increased serum creatinine, proteinuria, and...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
​BY GREGORY TAYLOR, DO, &SCOTT LAGERVELD, DOA 54-year-old man with a significant past medical history of atrial fibrillation and congestive heart failure presented to the ED with a chief complaint of difficulty walking. He was evaluated at an outside hospital two months earlier, and was found to have methicillin-resistant Staphylococcus aureus bacteremia, lower extremity cellulitis, and acute decompensated congestive heart failure (CHF). He clinically improved over one week, his autoimmune workup was negative, and he was discharged. He failed to follow up with nephrology, cardiology, and dermatology, and st...
Source: The Case Files - Category: Emergency Medicine Tags: Blog Posts Source Type: research
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