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      Can cryoglobulinemia trigger ANCA vasculitis?

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          Abstract

          Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a rare and heterogeneous group of autoimmune diseases. These pauciimmune vasculitis differ from the cryoglobulinemic vasculitis mediated by immune complexes. We report the case of a 79-year-old male with silicosis and chronic alcoholic liver disease, presenting with a rapidly progressive glomerulonephritis and pancytopenia. Work-up revealed hypocomplementemia, polyclonal hypergammaglobulinemia, type 3 cryoglobulinemia, p-ANCA positivity and elevated anti-MPO. Renal biopsy showed a pattern of chronic interstitial nephritis with fibrocellular crescents, and immunofluorescence staining was negative. Treatment was started with corticosteroids and rituximab with improvement of the renal function, decrease of the anti-MPO titer and disappearance of the cryoglobulinemia. In this case, renal injury was caused by ANCA vasculitis, whose etiology remains unknown despite the recognizable risk factor for ANCA formation (silicosis). The importance of the cryoglobulinemia is not clear, as it could be part of the pathogenesis or just an epiphenomenon secondary to the autoimmune and the chronic liver diseases.

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          ANCA-associated vasculitis.

          Max Yates, Richard Watts (2017)
          The vasculitides are a heterogeneous group of conditions typified by their ability to cause vessel inflammation with or without necrosis. They present with a wide variety of signs and symptoms and, if left untreated, carry a significant burden of mortality and morbidity. The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are three separate conditions - granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA; previously known as Churg-Strauss syndrome). This review examines recent developments in the pathogenesis and treatment of AAV, focusing on developments in treatment following the introduction of rituximab, in particular.
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            How I treat cryoglobulinemia.

            Eli Muchtar, Hila Magen, Morie Gertz (2017)
            Cryoglobulinemia is a distinct entity characterized by the presence of cryoglobulins in the serum. Cryoglobulins differ in their composition, which has an impact on the clinical presentation and the underlying disease that triggers cryoglobulin formation. Cryoglobulinemia is categorized into two main subgroups: type I, which is seen exclusively in clonal hematologic diseases, and type II/III, which is called mixed cryoglobulinemia and is seen in hepatitis C virus infection and systemic diseases such as B-cell lineage hematologic malignancies and connective tissue disorders. Clinical presentation is broad and varies between types but includes arthralgia, purpura, skin ulcers, glomerulonephritis, and peripheral neuropathy. Life-threatening manifestations can develop in a small proportion of patients. A full evaluation for the underlying cause is required, because each type requires a different kind of treatment, which should be tailored on the basis of disease severity, underlying disease, and prior therapies. Relapses can be frequent and can result in significant morbidity and cumulative organ impairment. We explore the spectrum of this heterogeneous disease by discussing the disease characteristics of 5 different patients.
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              Updates in ANCA-associated vasculitis.

              Christian Pagnoux (2016)
              Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides are small-vessel vasculitides that include granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Renal-limited ANCA-associated vasculitides can be considered the fourth entity. Despite their rarity and still unknown cause(s), research pertaining to ANCA-associated vasculitides has been very active over the past decades. The pathogenic role of antimyeloperoxidase ANCA (MPO-ANCA) has been supported using several animal models, but that of antiproteinase 3 ANCA (PR3-ANCA) has not been as strongly demonstrated. Moreover, some MPO-ANCA subsets, which are directed against a few specific MPO epitopes, have recently been found to be better associated with disease activity, but a different method than the one presently used in routine detection is required to detect them. B cells possibly play a major role in the pathogenesis because they produce ANCAs, as well as neutrophil abnormalities and imbalances in different T-cell subtypes [T helper (Th)1, Th2, Th17, regulatory cluster of differentiation (CD)4+ CD25+ forkhead box P3 (FoxP3)+ T cells] and/or cytokine-chemokine networks. The alternative complement pathway is also involved, and its blockade has been shown to prevent renal disease in an MPO-ANCA murine model. Other recent studies suggested strongest genetic associations by ANCA type rather than by clinical diagnosis. The induction treatment for severe granulomatosis with polyangiitis and microscopic polyangiitis is relatively well codified but does not (yet) really differ by precise diagnosis or ANCA type. It comprises glucocorticoids combined with another immunosuppressant, cyclophosphamide or rituximab. The choice between the two immunosuppressants must consider the comorbidities, past exposure to cyclophosphamide for relapsers, plans for pregnancy, and also the cost of rituximab. Once remission is achieved, maintenance strategy following cyclophosphamide-based induction relies on less toxic agents such as azathioprine or methotrexate. The optimal maintenance strategy following rituximab-based induction therapy remains to be determined. Preliminary results on rituximab for maintenance therapy appear promising. Efforts are still under way to determine the optimal duration of maintenance therapy, ideally tailored according to the characteristics of each patient and the previous treatment received.
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                Author and article information

                Journal
                Journal ID (publisher-id): nep
                Title: Portuguese Journal of Nephrology & Hypertension
                Abbreviated Title: Port J Nephrol Hypert
                Publisher: Sociedade Portuguesa de Nefrologia (Lisboa, , Portugal )
                ISSN (Print): 0872-0169
                Publication date (Print and electronic): March 2020
                Volume: 34
                Issue: 1
                Pages: 51-54
                Affiliations
                [1] orgnameHospital Pedro Hispano orgdiv1Internal Medicine Department
                [2] orgnameHospital Pedro Hispano orgdiv1Nephrology Department
                Article
                Publisher ID: S0872-01692020000100009 Publisher ID: S0872-0169(20)03400100009
                DOI: 10.32932/pjnh.2020.04.063
                SO-VID: 3edaec49-eeb5-4e32-92de-b4300ddd41e0
                License:

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                Date received : 20 October 2019
                Date accepted : 08 February 2020
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 15, Pages: 4
                Product

                SciELO Portugal

                Categories
                Subject: Case Reports

                Keywords: cryoglobulinemia,Crescents,interstitial nephritis,rapidly progressive glomerulonephritis,vasculitis
                Data availability:
                Keywords: cryoglobulinemia, Crescents, interstitial nephritis, rapidly progressive glomerulonephritis, vasculitis

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