Renal involvement in eosinophilic granulomatosis with polyangiitis

Frontiers in Medicine

Medicine

Francesco Reggiani¹  Marta Calatroni¹  Vincenzo L’Imperio²  Fabio Pagni²  Renato Alberto Sinico³ 

[1] Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy;Nephrology and Dialysis Unit, IRCCS Humanitas Research Hospital, Rozzano, Italy;Department of Medicine and Surgery, Pathology, University of Milano-Bicocca, IRCCS (Scientific Institute for Research, Hospitalization and Healthcare) Fondazione San Gerardo dei Tintori, Monza, Italy;Nephrology and Dialysis Unit, IRCCS Humanitas Research Hospital, Rozzano, Italy;

关键词: EGPA;    rapidly progressive glomerulonephritis;    ANCA antibodies;    immunosuppressive therapies;    glucocorticoids;    necrotizing vasculitis;    interstitial infiltration;   

DOI  :  10.3389/fmed.2023.1244651

 received in 2023-06-22, accepted in 2023-09-05,  发布年份 2023

来源: Frontiers

【 摘 要 】

Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing vasculitis, which typically affects small-to medium-sized blood vessels. It is characterized by the presence of tissue infiltrates rich in eosinophils, along with the formation of granulomatous lesions. About 40% of cases have positive anti-neutrophil cytoplasm antibodies (ANCA), with predominant perinuclear staining, and anti-myeloperoxidase (anti-MPO) specificity in about 65% of cases. Typical manifestations of EGPA include the late onset of asthma, nasal and sinus-related symptoms, peripheral neuropathy, and significant eosinophilia observed in the peripheral blood. In contrast to granulomatosis with polyangiitis and microscopic polyangiitis, renal involvement in EGPA is less frequent (about 25%) and poorly studied. Necrotizing pauci-immune crescentic glomerulonephritis is the most common renal presentation in patients with ANCA-positive EGPA. Although rarely, other forms of renal involvement may also be observed, such as eosinophilic interstitial nephritis, mesangial glomerulonephritis, membranous nephropathy, or focal sclerosis. A standardized treatment for EGPA with renal involvement has not been defined, however the survival and the renal outcomes are usually better than in the other ANCA-associated vasculitides. Nonetheless, kidney disease is an adverse prognostic factor for EGPA patients. Larger studies are required to better describe the renal involvement, in particular for patterns different from crescentic glomerulonephritis, and to favor the development of a consensual therapeutic approach. In this article, in addition to personal data, we will review recent findings on patient clinical phenotypes based on ANCA, genetics and the impact of biological drugs on disease management.

【 授权许可】

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Copyright © 2023 Reggiani, L’Imperio, Calatroni, Pagni and Sinico.