Churg-Strauss syndrome: Localization of eosinophil major basic protein in damaged tissues

Peen, Elisabeth; Hahn, Paulette; Lauwers, Gregory; Williams, Ralph C.; Gleich, Gerald J.; Kephart, Gail M.

doi:10.1002/1529-0131(200008)43:8<1897::aid-anr29>3.0.co;2-i

Cited by 42 publications

(20 citation statements)

References 11 publications

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“…18 Additionally, the cells themselves have been noted to be in an activated state, degranulating and releasing cytotoxic enzymes, resulting in local and systemic damage. 3,19 Patients with progressive disease are noted to have significantly increased eosinophil counts in bronchoalveolar lavage fluid compared with those seen in patients with CSS in partial remission. 3 This appeared to be reinforced in the cases of peripheral blood eosinophilia in our trial: patients tolerated lower steroid doses to control symptoms when eosinophil concentrations were concurrently low.…”

Section: Discussionmentioning

confidence: 99%

Mepolizumab as a steroid-sparing treatment option in patients with Churg-Strauss syndrome

Kim

Marigowda

Oren³

et al. 2010

Journal of Allergy and Clinical Immunology

256

126

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Section: Discussionmentioning

confidence: 99%

Mepolizumab as a steroid-sparing treatment option in patients with Churg-Strauss syndrome

Kim

Marigowda

Oren³

et al. 2010

Journal of Allergy and Clinical Immunology

256

126

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“…Serum markers of eosinophil activation, such as eosinophil cationic protein, correlates disease activity may even predict relapse [2,3]. Histopathological findings suggest a direct link between degranulation of activated eosinophils and tissue injury [4,5]. The American College of Rheumatology has proposed six criteria for CSS classification, four being necessary for CSS to be diagnosed with 85% sensitivity and 99.7% specificity.…”

Section: Discussionmentioning

confidence: 99%

An aggressive and lethal course of Churg–Strauss syndrome with alveolar hemorrhage, intestinal perforation, cardiac failure and peripheral neuropathy

et al. 2009

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Churg-Strauss syndrome (CSS) is a rare type of necrotizing vasculitis affecting small to medium-sized vessels typically characterized by asthma, lung infiltrates, necrotizing granulomas and hypereosinophilia. Herein, we describe a case of CSS presenting severe and aggressive course. A 35-year-old male patient with weight loss, dyspepsia, dyspnea and hemoptysis was admitted. The laboratory analyses indicated a remarkable eosinophilia, elevated levels of serum total IgE and positive cANCA. Thorax CT findings were suggestive of alveolar hemorrhage. Bronchoalveolar lavage revealed alveolar hemorrhage with eosinophilia and transbronchial lung biopsy showed eosinophilic vasculitis. Cardiac enzymes were increased and murmurs were audible revealing cardiomyopathy proven by echocardiography. Pulse cyclophosphamide and methyl prednisolone was immediately started. On the 21st day, intestinal perforation developed and urgent surgery was performed. During a follow-up, although a radiological improvement was observed in the chest X-ray, cardiac failure, peripheral neuropathy and skin lesions developed and high-dose intravenous immunoglobulin and anti-TNF therapy (adalimumab) were applied. Despite the therapy, he died from heart failure and septicemia at 68th day of therapy.

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“…13 Other impaired apoptosis-related molecules (eg, soluble CD95, 49 tumor necrosis factor-related apoptosisinducing ligand receptor 3, 50 and discoidin domain receptor 51 ) may contribute to the delayed eosinophil clearance seen in CSS. Infiltrating eosinophils release cytotoxic enzymes (eg, major basic protein, 52 eosinophil peroxidase, 53 and eosinophilic cationic protein 54 ) and, ultimately, cause tissue injury.…”

Section: Role Of Effector T Cellsmentioning

confidence: 99%

Anti-Neutrophil Cytoplasmic Antibody Pathogenesis in Small-Vessel Vasculitis

Gómez-Puerta

Bosch

2009

The American Journal of Pathology

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Vasculitides associated with serum positivity for antineutrophil cytoplasmic antibodies (ANCAs) that affect small-to medium-sized vessels are commonly known as ANCA-associated vasculitis (AAV) and include Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. Evidence derived from both in vitro studies and recent animal models points to a pathogenic role of ANCAs in AAV. In 2002, the first in vivo breakthrough in the pathogenesis of ANCAs showed that mouse ANCAs against myeloperoxidase (MPO) led to intrinsic pauci-immune renal vasculitis in mice. In 2004, a report using both in vitro and in vivo studies proposed that proteinase 3 (PR3)-directed autoimmunity involved the complementary peptide of PR3 (cPR3), which is encoded by the antisense strand of the PR3 gene. The last breakthrough came in October 2008 with a previously undescribed molecular explanation for the origin and development of injury in pauci-immune renal vasculitis, with potential clinical implications. This report showed that infection by fimbriated bacteria may trigger cross-reactive autoimmunity to a previously characterized ANCA antigen, lysosomal membrane protein-2, which is contained in the same vesicles that harbor MPO and PR3. Infection by fimbriated bacteria resulted in the production of autoantibodies, which activated neutrophils and killed human microvascular endothelium in vitro and caused renal vasculitis in rats. Although the evidence for a pathogenic role of ANCAs, mainly MPO-ANCAs, is striking, various questions remain unanswered. Understanding the key pathogenic mechanisms of AAV may provide a safer, more rational therapeutic approach than the traditional (ie, corticosteroids and immunosuppressants) treatment strategy.

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Churg-Strauss syndrome: Localization of eosinophil major basic protein in damaged tissues

Cited by 42 publications

References 11 publications

Mepolizumab as a steroid-sparing treatment option in patients with Churg-Strauss syndrome

Mepolizumab as a steroid-sparing treatment option in patients with Churg-Strauss syndrome

An aggressive and lethal course of Churg–Strauss syndrome with alveolar hemorrhage, intestinal perforation, cardiac failure and peripheral neuropathy

Anti-Neutrophil Cytoplasmic Antibody Pathogenesis in Small-Vessel Vasculitis

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