Churg–Strauss syndrome

Greco, Antonio; Rizzo, Maria Ida; Virgilio, Armando De; Gallo, Andrea; Fusconi, Massimo; Ruoppolo, Giovanni; Altissimi, G; Vincentiis, Marco de

doi:10.1016/j.autrev.2014.12.004

Cited by 250 publications

(218 citation statements)

References 77 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: 69%

“…[1] Rheumatoid meningitis is an uncommon and frequently difficult to diagnose manifestation of RA, capable of presenting with a variety of neurological symptoms. [2] However, rheumatoid meningitis is typically a late manifestation of disease, presenting after the diagnosis of RA has already been established. [2] Thus, we present an atypical presentation of rheumatoid meningitis in a patient without a preexisting history of RA.…”

Section: Introductionmentioning

confidence: 99%

“…[2] However, rheumatoid meningitis is typically a late manifestation of disease, presenting after the diagnosis of RA has already been established. [2] Thus, we present an atypical presentation of rheumatoid meningitis in a patient without a preexisting history of RA.…”

Section: Introductionmentioning

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: 69%

Section: Introductionmentioning

confidence: 99%

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Rheumatoid arthritis presenting as rheumatoid meningitis: A case report

Jessee

Keenan

2017

CRIM

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“…ANCA, which is found in about 40% of EGPA patients, more frequently when glomerulonephritis is present [6], was negative.…”

Section: Discussionmentioning

confidence: 92%

“…EGPA is the less frequent of AAV, a group of chronic inflammatory pauci-imunne disorders that involve small vessels [6][7][8]. The 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides defines it as an eosinophil-rich and necrotizing granulomatous inflammation, often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium vessels, associated with asthma and eosinophilia [9].…”

Section: Discussionmentioning

confidence: 99%

Eosinophilic Granulomatosis with Polyangiitis Associated to Chromophobe Renal Cell Carcinoma: A Case Report

Oliveira¹,

Simaan²,

Neto³

et al. 2017

J Vasc

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Paraneoplastic syndromes are characterized by the set of signs and symptoms of an underlying cancer, frequently occult, due to a variety of remote tumor effects unrelated to the mechanical impact of the tumor mass or distant metastasis [1,2]. Among these syndromes, paraneoplastic vasculitis are very rare, especially necrotizing systemic vasculitis. It is estimated that 2-5% of all causes of vasculitis are paraneoplastic, with the most common presentation being leukocitoclastic cutaneous vasculitis related to hematological malignancies [1,3,4], and fewer cases related to solid organ tumors as an underlying cause.There are some reports of paraneoplastic ANCA-associated vasculitis (AAV), especially granulomatosis with polyangiitis associated with hematological malignancies and with solid organ tumors such as lung and renal carcinomas [5]. We report a case of eosinophilic granulomatosis with polyangiitis (EGPA) associated to chromophobe renal cell carcinoma (chCRR), an unusual form of malignant renal neoplasm, with complete remission of vasculitis after tumor resection.It is important to be aware of the rare association between vasculitis and cancer, since early recognition could provide proper treatment and better prognosis. A 62 year-old man presented with painful palpable purpura on lower limbs and palms, dry cough, dyspnea and fever four weeks prior to admission. He had a long medical history of hypertension and diabetes, and lifetime tobacco exposure of 35 packyears.There was also a severe persistent asthma starting three years before. Weight loss, abdominal pain and urinary symptoms were absent. On physical examination, there were skin lesions (Figure 1), wheezing on chest auscultation and a body mass index of 27.2 kg/m 2 . Laboratory tests showed marked eosinophilia (5,700 cells/mm 3 at admission, peaking 15,520 cells/mm 3 during hospital stay), elevated erythrocyte sedimentation rate (80 mm/h) and C-reactive protein (2,6 mg/dL, NR <0,5 mg/dL). Renal function and urine analysis were normal. Skin biopsy demonstrated eosinophilic leukocitoclastic vasculitis of small vessels with fibrinoid necrosis and eosinophilic perivascular infiltrates (Figure 2). Antinuclear antibody and antineutrophil cytoplasmic antibodies (ANCA) tested negative. Serological tests for hepatitis B and C viruses and acquired human immunodeficiency virus were negative. At admission, chest radiography revealed pulmonary infiltrates.A computerized tomography (CT) scan was performed, showing bilateral alveolar infiltrates and a small indeterminate solid nodule on left lower lobe. Daily oral prednisone was started (up to 1 mg/kg) with no improvement. The patient evolved with respiratory insufficiency, with severe hypoxemia, and then intravenous (IV) methylprednisolone 1 g/day was prescribed for three days. There was a striking improvement, with normalization of eosinophil count and complete resolution of respiratory symptoms. However, the cutaneous vasculitis not only persisted, but also progressed some days after the IV glucocorticoid therapy, ...

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Clinical Benefit of Mepolizumab in Eosinophilic Granulomatosis With Polyangiitis for Patients With and Without a Vasculitic Phenotype

Terrier

Jayne

Hellmich³

et al. 2023

ACR Open Rheumatology

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Objective To evaluate mepolizumab's efficacy in eosinophilic granulomatosis with polyangiitis (EGPA) with and without a vasculitic phenotype. Methods The MIRRA study (NCT02020889/GSK ID: 115921) included adults with relapsing/refractory EGPA and 4 or more weeks of stable oral glucocorticoids (OG). Patients received mepolizumab (300 mg subcutaneously every 4 weeks) or placebo, plus standard of care for 52 weeks. This post hoc analysis assessed EGPA vasculitic phenotype using antineutrophil cytoplasmic antibody (ANCA) history, baseline Birmingham Vasculitis Activity Score (BVAS), and Vasculitis Damage Index (VDI) score. Coprimary endpoints included accrued remission over 52 weeks and proportion in remission at Week 36 and Week 48. Remission was defined as a BVAS equal to 0 and an OG dose of 4 or more mg/day of a prednisone equivalent. Types of relapses (vasculitis, asthma, and sino‐nasal) and EGPA vasculitic characteristics (by study remission status) were also assessed. Results A total of 136 patients were included (n = 68, mepolizumab and placebo). Irrespective of history of ANCA positivity status, baseline BVAS, or baseline VDI, the accrued remission duration and the proportion of patients in remission at Weeks 36 and 48 were greater with mepolizumab compared with placebo. With mepolizumab, remission at both Week 36 and Week 48 was achieved by 54% of patients with and 27% of patients without a history of ANCA positivity compared with 0% and 4%, respectively (placebo); 45% of patients with a BVAS of 0 and 22% of patients with BVAS of greater than 0 compared with 5% and 2%, respectively (placebo); and 29% of patients with a VDI score of less than 5 and 37% of patients with a VDI score of 5 or more compared with 6% and 0%, respectively (placebo). Mepolizumab reduced all types of relapses as compared with placebo. Baseline vasculitic characteristics (neuropathy, glomerulonephritis, alveolar hemorrhage, palpable purpura, and ANCA positivity) were generally similar among patients with and without remission. Conclusion Mepolizumab is associated with clinical benefits for patients with and without a vasculitic EGPA phenotype.

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Churg–Strauss syndrome

Cited by 250 publications

References 77 publications

Rheumatoid arthritis presenting as rheumatoid meningitis: A case report

Rheumatoid arthritis presenting as rheumatoid meningitis: A case report

Eosinophilic Granulomatosis with Polyangiitis Associated to Chromophobe Renal Cell Carcinoma: A Case Report

Clinical Benefit of Mepolizumab in Eosinophilic Granulomatosis With Polyangiitis for Patients With and Without a Vasculitic Phenotype

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