Eosinophilic granulomatosis with polyangiitis (Churg–Strauss)

Mahr, Alfred; Moosig, Frank; Neumann, Thomas; Szczeklik, Wojciech; Taillé, Camille; Vaglio, Augusto; Zwerina, Jochen

doi:10.1097/bor.0000000000000015

Cited by 124 publications

(50 citation statements)

References 59 publications

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“…[2] Steroids are the mainstay of treatment, often supplemented with adjuvant cyclophosphamide or azathioprine. [2,3,10] The response to steroids can be rapid, with improvement of symptoms in weeks, as seen in our patient. [10] TNF blockers and MTX do not appear to be preventative, and rheumatoid meningitis has been documented to recur when switching from cyclophosphamide to infliximab.…”

Section: Discussionmentioning

confidence: 71%

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Rheumatoid arthritis presenting as rheumatoid meningitis: A case report

Jessee

Keenan

2017

CRIM

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Rheumatoid meningitis typically presents as a late manifestation of rheumatoid arthritis, and its pathogenesis remains uncertain. We describe a patient with neurological symptoms, subsequently found to have a brain mass of unclear etiology. The findings from imaging and histological analysis led to a diagnosis of rheumatoid meningitis. Surprisingly, this case presented in the absence of a history of arthritis or other systemic signs typically seen in RA. This case highlights the fact that rheumatoid meningitis can be the initial presenting symptom of rheumatologic disease and should be kept in the differential in patients with or without a history of RA.

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Section: Discussionmentioning

confidence: 71%

“…[2][3][4] As seen in our patient, rheumatoid meningitis can develop irrespective of systemic inflammation and arthritis. [2,5] The rarity of diagnosis limits available information about the disease, with the largest case series using less than two dozen patients.…”

Section: Discussionmentioning

confidence: 89%

Rheumatoid arthritis presenting as rheumatoid meningitis: A case report

Jessee

Keenan

2017

CRIM

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“…The five factors score (FFS) may be a guide for clinicians, this score assigns one point to each of the following items, namely, gastrointestinal involvement, CNS involvement, cardiac involvement, proteinuria >1 g/24 h and serum creatinine >141 μmol/L (35). Patients with poor prognosis factors (FFS ≥1) are often treated with both glucocorticoids (classically prednisone at dosage of 1 mg/kg of total body weight/day with a maximum dosage of 75 mg/day, for 1 month and then tapered) and cyclophosphamide (CYC, 2 mg/kg of total body weight/day), while the typical approach for patients with a better prognosis (e.g., FFS of 0) is glucocorticoid therapy alone (54). Recently, a revised FFS has been proposed an age over 65 years, cardiac symptoms, gastrointestinal involvement, renal insufficiency (serum creatinine >150 μmol/L) and absence of ear, nose, and throat manifestations have been pointed out as predictors of 5-year mortality (55).…”

Section: Treatment and Outcomementioning

confidence: 99%

Eosinophilic Granulomatosis with Polyangiitis: An Overview

et al. 2014

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Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic disorder, belonging to the small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, defined as an eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium-sized vessels, associated with asthma and eosinophilia. EGPA pathogenesis is not well known: HLA-DRB1*04 and *07, HLA-DRB4 and IL10.2 haplotype of the IL-10 promoter gene are the most studied genetic determinants. Among the acquired pathogenetic factors, the exposure to different allergens, infections, vaccinations, drugs, and silica exposure have been involved. Eosinophils are the most characteristic cells in EGPA and different studies have demonstrated their role as effector and immunoregulatory cells. EGPA is considered as a disease with a prevalent activation of the Th-2 cellular-mediated inflammatory response and also humoral immunity plays an important role. A link between B and T inflammatory responses may explain different disease features. EGPA typically develops into three sequential phases: the allergic phase, distinguished by the occurrence of asthma, allergic rhinitis, and sinusitis, the eosinophilic phase, in which the main pathological finding is the eosinophilic organ infiltrations (e.g., lungs, heart, and gastrointestinal system), and the vasculitic phase, characterized by purpura, peripheral neuropathy, and constitutional symptoms. ANCA (especially pANCA anti-myeloperoxidase) are present in 40–60% of the patients. An elevation of IgG4 is frequently found. Corticosteroids and cyclophosphamide are classically used for remission induction, while azathioprine and methotrexate are the therapeutic options for remission maintenance. B-cell depletion with rituximab has shown promising results for remission induction.

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“…57,58 There are no known causal triggers of EGPA. 59 Disease onset typically occurs in people aged 40–60 years, and there is no known sex, familial, or ethnic predisposition. Candidate-gene association studies, including positive and negative disease associ ations with various HLA class II polymorphisms, 60 implicate potential immunogenetic factors.…”

Section: Egpamentioning

confidence: 99%

Eosinophils in vasculitis: characteristics and roles in pathogenesis

2014

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Eosinophils are multifunctional granular leukocytes that are implicated in the pathogenesis of a wide variety of disorders, including asthma, helminth infection, and rare hypereosinophilic syndromes. Although peripheral and tissue eosinophilia can be a feature of many types of small-vessel and medium-vessel vasculitis, the role of eosinophils has been best studied in eosinophilic granulomatosis with polyangiitis (EGPA), where eosinophils are a characteristic finding in all three clinical stages of the disorder. Whereas numerous studies have demonstrated an association between the presence of eosinophils and markers of eosinophil activation in the blood and tissues of patients with EGPA, the precise role of eosinophils in disease pathogenesis has been difficult to ascertain owing to the complexity of the disease process. In this regard, results of clinical trials using novel agents that specifically target eosinophils are providing the first direct evidence of a central role of eosinophils in EGPA. This Review focuses on the aspects of eosinophil biology most relevant to the pathogenesis of vasculitis and provides an update of current knowledge regarding the role of eosinophils in EGPA and other vasculitides.

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Eosinophilic granulomatosis with polyangiitis (Churg–Strauss)

Cited by 124 publications

References 59 publications

Rheumatoid arthritis presenting as rheumatoid meningitis: A case report

Rheumatoid arthritis presenting as rheumatoid meningitis: A case report

Eosinophilic Granulomatosis with Polyangiitis: An Overview

Eosinophils in vasculitis: characteristics and roles in pathogenesis

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