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      Concurrent Use of Mepolizumab and Rituximab for Eosinophilic Granulomatosis With Polyangiitis and Multisystem Involvement

      case-report
      1 , , 2 , 3
      ,
      Cureus
      Cureus
      mepolizumab, egpa, rituximab, glomerulonephritis, vasculitis

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          Abstract

          Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss, is an anti-neutrophil cytoplasmic antibody (ANCA)-associated autoimmune vasculitis, involving small- and medium-sized arteries, which could involve several organs. This rare syndrome can present with a myriad of symptoms, which may make diagnosis challenging. It has been suggested that there are variants of EGPA, which may respond differently to available modes of treatment. Multiple and different mechanisms may be at play in each case of EGPA. This may influence the decision of clinicians to combine treatment strategies as done in this case. The addition of immunosuppressive agents other than high-dose steroids may mitigate end-organ damage, facilitate faster recovery, and prevent relapse. Rituximab among others has been seen to provide better outcomes, including a lower incidence of relapse. Mepolizumab was approved by the Food and Drug Administration (FDA) in 2017 for the treatment of EGPA. Administered at a higher dose than approved for severe eosinophilic asthma, it has been shown to lengthen remission in EGPA. The optimal dose and duration of therapy with mepolizumab remain unclear. The rarity alone of EGPA creates room for further investigation regarding pathogenesis, outcome over time, and treatment strategies, which may vary depending on how an individual case presents.

          This case describes the course of a 55-year-old woman who presented with respiratory symptoms, pauci-immune necrotizing granulomatous nephropathy, and neuropathy secondary to P-ANCA-positive EGPA who was successfully treated with rituximab and mepolizumab, in addition to glucocorticoids.

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          Most cited references11

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          The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis).

          Criteria for the classification of Churg-Strauss syndrome (CSS) were developed by comparing 20 patients who had this diagnosis with 787 control patients with other forms of vasculitis. For the traditional format classification, 6 criteria were selected: asthma, eosinophilia greater than 10% on differential white blood cell count, mononeuropathy (including multiplex) or polyneuropathy, non-fixed pulmonary infiltrates on roentgenography, paranasal sinus abnormality, and biopsy containing a blood vessel with extravascular eosinophils. The presence of 4 or more of these 6 criteria yielded a sensitivity of 85% and a specificity of 99.7%. A classification tree was also constructed with 3 selected criteria: asthma, eosinophilia greater than 10% on differential white blood cell count, and history of documented allergy other than asthma or drug sensitivity. If a subject has eosinophilia and a documented history of either asthma or allergy, then that subject is classified as having CSS. For the tree classification, the sensitivity was 95% and the specificity was 99.2%. Advantages of the traditional format compared with the classification tree format, when applied to patients with systemic vasculitis, and their comparison with earlier work on CSS are discussed.
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            Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome.

            Since testing for antineutrophil cytoplasmic antibodies (ANCA) became available for routine evaluation, no large homogeneous cohort of patients with the Churg-Strauss syndrome has been studied. To define the clinical and biological characteristics of newly diagnosed Churg-Strauss syndrome, according to the presence or absence of ANCA. Cross-sectional analysis of manifestations of participants who were enrolled in treatment trials between December 1995 and December 2002. Multicenter study in 63 clinical centers in France, Belgium, Latvia, and the United Kingdom, coordinated by the French Vasculitis Study Group. 112 patients with Churg-Strauss syndrome that was recently diagnosed on the basis of current classifications. The authors compared principal demographic, clinical, and laboratory features according to ANCA status at diagnosis. The authors detected ANCA in 43 (38%) patients. Positive ANCA status at diagnosis was associated with renal involvement, peripheral neuropathy, and biopsy-proven vasculitis, whereas negative ANCA status was associated with heart disease and fever. The authors assessed ANCA by immunofluorescence, but they did not assess ANCA centrally or systematically retest if ANCA was undetected at diagnosis. Phenotypically, ANCA-positive and ANCA-negative Churg-Strauss syndrome might differ. The association of ANCA positivity with clinical symptoms that indicate inflammation and necrosis of small vessels might characterize a predominantly vasculitic pattern of the Churg-Strauss syndrome.
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              Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome.

              Drawing on our experience of 16 cases and a review of the English literature, we propose that CSS is under-diagnosed because of exclusive emphasis upon pathologic recognition of the disorder. The classical histological picture comprises a necrotizing vasculitis, eosinophilic tissue infiltration and extravascular granulomas, but it is only found in a minority of cases, and is not pathognomonic of the condition (69, 108). On the other hand, the clinical pattern of the disorder is most distinctive, and CSS can be readily identified on clinical grounds. Typically, it begins with allergic rhinitis, which is often complicated by nasal polyposis and sinusitis. Asthma and peripheral blood eosinophilia are essential features, often accompanied by pulmonary infiltrates. The systemic vasculitis of CSS resembles that of PAN, but severe renal disease is uncommon (the typical renal lesion is a focal segmental glomerulonephritis), and cardiac involvement accounts for 50% of deaths. Diagnostic difficulties arise from the close relationship of CSS to other granulomatous, vasculitic and eosinophilic disorders. CSS is usefully regarded as a point of overlap between these three disease spectrums (Fig. 5). Individual components of each spectrum can occur in the course of CSS; hence cases may be reported as PAN developing as a complication of Löffler syndrome or eosinophilic gastroenteritis (37, 57, 66). The hypereosinophilia of CSS tends to be less severe and more steroid-responsive than in HES, and evidence of eosinophil degranulation was not found in the patients we studied. Complement abnormalities are not a prominent feature of the disorder, and circulating immune complexes were detected in only two cases; both contained IgM. This may be of pathogenetic significance as IgM deposition was a dominant feature in four of the five cases with positive renal immunofluorescence. IgE levels were elevated in all patients studied during the vasculitic phase, and skin-prick tests were positive in 8 of 10 patients tested. CSS responds well to treatment with steroids, although some patients benefit from the addition of immunosuppressive agents. The vasculitic illness is usually of limited duration, but relapses can occur, and should be detected and treated early. Major problems in the post-vasculitic phase stem from hypertension and persisting peripheral nerve damage. Allergic upper and lower respiratory tract disease is an important cause of morbidity in the pre- and post-vasculitic periods.
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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                17 July 2020
                July 2020
                : 12
                : 7
                : e9242
                Affiliations
                [1 ] Internal Medicine, University of Connecticut (UCONN) School of Medicine, Hartford, USA
                [2 ] Internal Medicine, University of Connecticut (UCONN) Health, Farmington, USA
                [3 ] Nephrology, Saint Francis Hospital and Medical Center, Hartford, USA
                Author notes
                Article
                10.7759/cureus.9242
                7430662
                32821588
                83ea5e2a-feff-4464-9996-9a5f8acdfbab
                Copyright © 2020, Afiari et al.

                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
                : 22 June 2020
                : 17 July 2020
                Categories
                Internal Medicine
                Allergy/Immunology
                Nephrology

                mepolizumab,egpa,rituximab,glomerulonephritis,vasculitis
                mepolizumab, egpa, rituximab, glomerulonephritis, vasculitis

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