ANCA-associated vasculitis

Abstract

The anti-neutrophil cytoplasmic antibody (ANCA)associated vasculitides (AAVs) are diseases characterized by inflammation of blood vessels, endothelial injury and tissue damage. The three types of small-vessel vasculitis, namely granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA; previously known as Churg-Strauss syndrome), feature a loss of tolerance to neutrophil primary granule proteins, most often leukocyte proteinase 3 (PR3; also known as myeloblastin) or myeloperoxidase (MPO) (Table 1). The vessels involved in AAV are typically capillaries, arterioles and venules but small arteries and veins may also be affected. Autoimmunity is documented clinically by serum ANCAs to PR3 (PR3-ANCA) or MPO (MPO-ANCA), which are generally associated with the main syndromic AAV presentations (box 1). AAVs collectively represent one of several types of autoimmune vasculitis (Fig. 1). GPA and MPA can involve small blood vessels in any organ or tissue but commonly affect the upper and lower respiratory tract and the kidneys (box 2). Patients with AAV typically present with severe organ-threatening or life-threatening disease, although less severe presentations also occur. GPA is predominantly associated with PR3-ANCA and its clinical features typically include sinonasal disease, lower respiratory tract involvement with pulmonary haemorrhage and granulomatous inflammation, and glomerulonephritis. MPA is usually associated with MPO-ANCA and clinical features include more severe renal disease and some of the manifestations of GPA but without granulomatous inflammation. EGPA is characterized by asthma, eosinophilia and, in many (but not all) cases, vasculitis. EGPA is less common than GPA or MPA and, in some cases, is associated with ANCAs, mainly MPO-ANCA (Table 1). Although categorized as a form of AAV, EGPA has less overlap with the other AAVs than that between GPA and MPA with regard to its genetic, pathogenetic, and clinical features and its management and is typically considered a separate entity. Improvements in treatment and prognosis for patients with AAV have resulted from the translation of both preclinical and clinical research findings. Here, we provide an updated overview of the clinical and

What comes after the lockdown? Clustering of ANCA-associated vasculitis: single-centre observation of a spatiotemporal pattern

“…Clustering of ANCA-associated vasculitis: single-centre observation of a spatiotemporal pattern Antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides (AAV) are characterised by a heterogeneous clinical phenotype. 1 We report a cluster of 15 patients diagnosed with AAV either de novo (n=11) or with relapsing disease (n=4) during COVID-19 pandemic between February and August 2020. During this period, we observed two major phenomena: (1) an incidence-shift with a 'COVID-gap' of no diagnosed AAV cases during the lockdown period (March and April), followed by a 'postlockdown cluster' of 14 active patients (8 myeloperoxidase-ANCA, 6 proteinase 3-ANCA vasculitis) in the subsequent 4 months and (2) an increased incidence rate (figure 1 and online supplemental figure S3).…”

Eosinophile Granulomatose mit Polyangiitis (eGPA) in der Niere: Glomerulonephritis und interstitielle Nephritis

“…In der hier vorgestellten Arbeit wurden Fälle mit biopsiegesicherter Nierenbeteiligung zusammengetragen, um die Formen der Nierenbeteiligung besser zu definieren. Die Erkrankung ist auch in der an sich schon zu den seltenen Erkrankungen zählenden Gruppe der ANCA-assoziierten Vaskulitiden mit einer Inzidenz von 0,5–2,3 pro 1 Million Personenjahren und einer Prävalenz von 2–22,3 pro 1 Million die seltenste [1]. Die Autoren haben deswegen Register aus großen Zentren in Italien, Belgien, Großbritannien und Frankreich mit Unterstützung der französischen Vaskulitis-Studiengruppe zusammengefasst.…”

Microscopic Polyangiitis (MPO-ANCA) and Henoch-Schoenlein purpura overlap in a Child

“…Clinical features vary, but nephritis and pulmonary bleeding and peripheral nerve neuropathy, abdominal pain, joint pain, and skin eruptions are common, whereas central nervous system manifestations are rare. It was stipulated that MPO-ANCA has complex and unique pathogenesis, with evidence for a loss of tolerance to neutrophil proteins, leading to ANCA-mediated neutrophil activation, recruitment, and injury effector T cells also involved [2]. The prognosis often remains guarded with frequent relapses and high cumulative morbidity [3].…”