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IgA Nephropathy and Schöenlein-Henoch Purpura Nephritis

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Core Concepts in Parenchymal Kidney Disease

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Abstract

IgA nephropathy (IgAN) is the most common primary glomerulonephritis worldwide, even though its prevalence varies considerably among countries. It is characterized by predominantly immunoglobulin A (IgA) mesangial deposits in the glomerulus. Similar histological features are detected in nephritic patients with Schöenlein purpura. Although significant progress has been made in understanding the pathogenesis of IgAN, no single unifying pathological mechanism fully explains its development. A release of proinflammatory and pro-fibrotic mediators, engendering mesangial cell proliferation, is the key point of this process. The clinical course of IgAN is variable. Most often it progresses indolently to renal insufficiency. This variability is influenced by differing health policies. The broad clinical spectrum of IgAN includes isolated urinary abnormalities, acute nephritic syndrome, nephrotic syndrome, acute renal failure, chronic renal failure, and rapidly progressive renal failure. However, macroscopic hematuria (beginning 1–3 days after an upper respiratory tract infection) is typical at onset. A wide spectrum of histological lesions can be observed: focal or diffuse mesangial proliferation is the most common finding, but we may also see extracapillary or endocapillary proliferation and lesions suggestive of chronic damage (i.e., tubulointerstitial fibrosis, glomerular and vascular sclerosis). Therapy varies according to clinical features and pathological findings.

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Acknowledgments

The authors are indebted with Anna Maria Asunis, Pathology Unit, G. Brotzu Hospital, Cagliari, and Manuela Nebuloni, Antonella Tosoni (Pathology Unit), and Giovanni Belgiojoso (Nephrology Unit), L. Sacco Hospital, Milan, Italy, for providing the pictures of renal histology, and Maria Laura Lecca, Andrea Angioi, and Doloretta Piras (Renal Unit G. Brotzu Hospital Cagliari) for technical assistance.

Conflict of Interest. 

There are no conflicts of interest to declare.

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Authors and Affiliations

  1. Division of Nephrology (Renal Unit), Azienda Ospedaliera G. Brotzu, Cagliari, Italy

    Antonello Pani M.D.

  2. Department of Rare, Immunologic, Haemathologic, Immunohaemathologic Diseases, Giovanni Bosco Hospital and University of Turin, Turin, Italy

    Dario Roccatello M.D.

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  1. Antonello Pani M.D.
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Correspondence to Antonello Pani M.D. .

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Editors and Affiliations

  1. Div. Nephrology & Hypertension, Mayo Clinic Dept. Internal Medicine, Rochester, Minnesota, USA

    Fernando C. Fervenza

  2. Renal Division, Brigham and Women's Hospital, Boston, Massachusetts, USA

    Julie Lin

  3. Mayo Clinic, Rochester, Minnesota, USA

    Sanjeev Sethi

  4. Renal Division, Brigham and Women's Hospital Harvard Medical School, Boston, Massachusetts, USA

    Ajay K. Singh

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Pani, A., Roccatello, D. (2014). IgA Nephropathy and Schöenlein-Henoch Purpura Nephritis. In: Fervenza, F., Lin, J., Sethi, S., Singh, A. (eds) Core Concepts in Parenchymal Kidney Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-8166-9_8

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