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      A Case of Adult-Onset IgA Vasculitis in a Cirrhotic Patient

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      Author(s): 1 , , 1 , 1 , 1 , 1
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      Publication date (Electronic, pub):
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      Journal: Cureus
      Publisher: Cureus
      Keywords: iga nephropathy, alcoholic liver cirrhosis, liver cirrhosis, henoch-schönlein purpura, immunoglobulin a vasculitis

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          Abstract

          Immunoglobulin A vasculitis (IgAV; formerly called Henoch-Schönlein purpura) is a disease commonly seen in children as an immune reaction after a viral infection. It is a small vessel vasculitis characterized by immune complex deposits in various organs throughout the body. It mainly affects the skin, joints, abdomen and kidneys. This presentation is less likely to be seen in adults. In adults, IgAV can be seen due to decreased clearance of immune complexes through the liver. A damaged liver due to alcoholic liver cirrhosis can hinder the clearance of IgA complexes. We present an unusual case of a 42-year-old female who presented with alcoholic liver cirrhosis and ascites and later developed a purpuric rash in her lower extremities.

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          New insights in the pathogenesis of immunoglobulin A vasculitis (Henoch-Schönlein purpura).

          Marieke Heineke, Aranka V Ballering, Agnès Jamin … (2017)
          Immunoglobulin A vasculitis (IgAV), also referred to as Henoch-Schönlein purpura, is the most common form of childhood vasculitis. The pathogenesis of IgAV is still largely unknown. The disease is characterized by IgA1-immune deposits, complement factors and neutrophil infiltration, which is accompanied with vascular inflammation. Incidence of IgAV is twice as high during fall and winter, suggesting an environmental trigger associated to climate. Symptoms can resolve without intervention, but some patients develop glomerulonephritis with features similar to IgA nephropathy that include hematuria, proteinuria and IgA deposition in the glomerulus. Ultimately, this can lead to end-stage renal disease. In IgA nephropathy immune complexes containing galactose-deficient (Gd-)IgA1 are found and thought to play a role in pathogenesis. Although Gd-IgA1 complexes are also present in patients with IgAV with nephritis, their role in IgAV is disputed. Alternatively, it has been proposed that in IgAV IgA1 antibodies are generated against endothelial cells. We anticipate that such IgA complexes can activate neutrophils via the IgA Fc receptor FcαRI (CD89), thereby inducing neutrophil migration and activation, which ultimately causes tissue damage in IgAV. In this Review, we discuss the putative role of IgA, IgA receptors, neutrophils and other factors such as infections, genetics and the complement system in the pathogenesis of IgA vasculitis.
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            Epidemiology of immunoglobulin A vasculitis (Henoch-Schönlein): current state of knowledge.

            Maryam Piram, Alfred Mahr (2013)
            To review the current knowledge of epidemiological features of immunoglobulin (Ig) A vasculitis (Henoch-Schönlein) and disease etiology.
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              Secondary IgA nephropathy

              Manish K. Saha, Bruce Julian, Jan Novak … (2018)
              IgA nephropathy is the most common primary glomerulonephritis worldwide. Its frequent coexistence with inflammatory, infectious, or malignant processes raises the possibility of a pathologic rather than coincidental association. Major strides have been made to elucidate the underlying pathophysiologic events that culminate in the development of primary IgA nephropathy. Whether secondary forms of the disease share common pathways triggered by underlying disorders or different mechanisms leading to similar pathologic findings remains to be determined. In this article, we describe the most frequent etiologies for secondary IgA nephropathy and review the available literature for the pathophysiology.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Cureus
                Journal ID (iso-abbrev): Cureus
                Journal ID (issn): 2168-8184
                Title: Cureus
                Publisher: Cureus (Palo Alto (CA) )
                ISSN (Electronic): 2168-8184
                Publication date (Electronic, pub): 9 August 2022
                Publication date (Electronic, collection): August 2022
                Volume: 14
                Issue: 8
                Electronic Location Identifier: e27812
                Affiliations
                [1 ] Internal Medicine, HCA Oak Hill Hospital, Spring Hill, USA
                Author notes
                Article
                DOI: 10.7759/cureus.27812
                PMC ID: 9454305
                SO-VID: 7bb59a24-9776-49d1-9df6-9b72b518028b
                Copyright © Copyright © 2022, Bazigh et al.
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                Date accepted : 9 August 2022
                Categories
                Subject: Internal Medicine
                Subject: Infectious Disease
                Subject: Rheumatology

                Keywords: iga nephropathy,alcoholic liver cirrhosis,liver cirrhosis,henoch-schönlein purpura,immunoglobulin a vasculitis
                Data availability:
                Keywords: iga nephropathy, alcoholic liver cirrhosis, liver cirrhosis, henoch-schönlein purpura, immunoglobulin a vasculitis

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