Clinical Manifestations and Treatment of Churg-Strauss Syndrome

Baldini, Chiara; Talarico, Rosaria; Rossa, Alessandra Della; Bombardieri, Stefano

doi:10.1016/j.rdc.2010.05.003

Cited by 91 publications

(96 citation statements)

References 61 publications

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“…[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: Discussionsupporting

confidence: 68%

“…[8] Brain imaging traditionally shows meningeal thickening and localized enhancement. [4] MRI is the preferred imaging modality, although contrast CT can be used for diagnosis, as seen in our patient who could not undergo MRI due to pacemaker placement. [2,9] CSF studies often show elevated protein, with potential CSF biomarkers of disease activity including IL-6 and RF.…”

Section: Discussionmentioning

confidence: 90%

“…[9] Rheumatoid nodules are not required for diagnosis, and necrotizing granulomas without giant cells or epithelioid cells have previously been seen in rheumatoid meningitis, similar to our own patient. [4] Ultimately, the diagnosis of rheumatoid meningitis is one of exclusion, requiring ruling out more common infectious and inflammatory causes of meningitis.…”

Section: Discussionmentioning

confidence: 99%

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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: Discussionsupporting

confidence: 68%

Section: Discussionmentioning

confidence: 90%

Section: Discussionmentioning

confidence: 99%

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Rheumatoid arthritis presenting as rheumatoid meningitis: A case report

Jessee

Keenan

2017

CRIM

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“…Two case reports have described success with methotrexate as maintenance therapy [105,107]. Various biologic therapies have also been used with good effect in isolated cases of refractory, systemic CSS, including etanercept, infliximab, rituximab, and omalizumab [93,111]. Although these cases did not specifically address orbital disease, the aforementioned biologics may also be useful in refractory orbital CSS.…”

Section: Churg-strauss Syndromementioning

confidence: 99%

Orbital inflammatory disease management

Moore

Rootman

2016

Expert Review of Ophthalmology

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“…[1,2] Specifically, organ damage secondary to eosinophil infiltration underlies airway and lung parenchymal, cardiac and gut involvement, while mononeuritis multiplex, glomerulonephritis and purpura characterize the vasculitic phase. [3][4][5] We report the clinical disease course of a gentleman with steroid-resistant EGPA characterized by recalcitrant peripheral eosinophilia, central and peripheral nervous system involvement, myocarditis, diffuse alveolar hemorrhage (DAH), necrotizing myopathy, and extensive eosinophilic degranulation in gastrointestinal tissues; the latter only apparent after staining with targeted monoclonal antibodies to eosinophil peroxidase (EPX). This case highlights the synchronous presentation of multiple rare manifestations of EGPA, highlighting the multisystem complexity and clinical heterogeneity of this disease entity.…”

Section: Introductionmentioning

confidence: 99%

Recalcitrant steroid-resistant EGPA with multisystem involvement

Cervi

Cox

Khalidi

et al. 2017

CRIM

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Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem disorder classically characterized by sequential histopathologic and clinical disease phases. An eosinophilic tissue infiltration phase typically precedes the development of a systemic small-vessel vasculitic phase. The contribution of these two pathogenic mechanisms to disease course and the boundaries between them are often blurred. We report the case of a 49-year-old man with established p-ANCA positive EGPA presenting acutely with central and peripheral nervous system dysfunction, diffuse alveolar hemorrhage, biopsy-proven myositis, and myocarditis resistant to steroids. The patient's clinical course was marked by unremitting abdominal pain associated with significant weight loss. Although eosinophilic infiltrates were not observed on gastrointestinal mucosal biopsy, samples stained heavily for eosinophil peroxidase, indicating prior degranulation of eosinophils. Complete remission of eosinophil-mediated tissue infiltration and vasculitic phases was achieved only after the addition of intravenous cyclophosphamide to corticosteroid therapy. This case report demonstrates both: a) the importance of recognizing eosinophilic degranulation in addition to intact eosinophils in tissue and b) a unique presentation of a rare disease, underscoring the multifaceted and heterogeneous nature of EGPA and its overlapping disease phases.

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Clinical Manifestations and Treatment of Churg-Strauss Syndrome

Cited by 91 publications

References 61 publications

Rheumatoid arthritis presenting as rheumatoid meningitis: A case report

Rheumatoid arthritis presenting as rheumatoid meningitis: A case report

Orbital inflammatory disease management

Recalcitrant steroid-resistant EGPA with multisystem involvement

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