Syndrome de Churg et Strauss : caractéristiques et profils évolutifs d’une cohorte de 383 patients

Comarmond, Cloé; Pagnoux, Christian; Khellaf, M.; Cordier, Jean-François; Hamidou, M.; Viallard, Jean‐François; Séror, Raphaèle; Mahr, A.; Cohen, Patrice; Mouthon, Luc; Guillevin, Loı̈c

doi:10.1016/j.revmed.2011.10.386

Cited by 8 publications

(34 citation statements)

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“…Central nervous system involvement is uncommon (5.2% in our series 23 ), and reflects cerebral vasculitis. Clinical manifestations are nonspecific: seizures, hemiplegia, coma, brain hemorrhage.…”

Section: Neurological Manifestationsmentioning

confidence: 53%

“…Mean age at EGPA diagnosis is approximately 50 years, 23 which is lower than for GPA, MPA, or polyarteritis nodosa (PAN). Childhood-onset EGPA is very rare, as fewer than 50 cases have been reported [24][25][26] ; it has predominant pulmonary and cardiac involvements, and high mortality (18%).…”

Section: Incidence and Prevalencementioning

confidence: 97%

“…Notably, 38.9% of our EGPA patient series had fever and myalgias, 29.8% had arthralgias, and 50% had lost weight (mean loss: 7.8 AE 4.7 kg). 23…”

Section: General Symptomsmentioning

confidence: 99%

“…38 Ear, Nose, and Throat Manifestations Maxillary sinusitis is common and is one of the ACR classification criteria defining EGPA (►Table 1). Nasal polyposis and allergic rhinitis are found in 41 and 17% of EGPA patients 23 and must be distinguished from the destructive ear, nose, and throat (ENT) manifestations seen in GPA. Polyposis and rhinitis are usually present in EGPA patients before the vasculitis becomes overt.…”

Section: Pulmonary Manifestationsmentioning

confidence: 99%

“…Peripheral neuropathies, mainly mononeuritis multiplex, are frequent, affecting 46 to 75% of EGPA patients. 23 The most frequently involved nerves, in decreasing order of frequency, are the common peroneal, internal popliteal, then those of the arm (radial, cubital, and/or median), and, lastly, cranial nerves. 23 Paresthesia or sometimes painful hyperesthesia may occur before the onset of motor or sensory deficiencies.…”

Section: Neurological Manifestationsmentioning

confidence: 99%

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Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss)

Nguyen

Guillevin

2018

Semin Respir Crit Care Med

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Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg–Strauss syndrome, is a systemic necrotizing vasculitis of small- and medium-size vessels, characterized by asthma and blood eosinophilia. EGPA typically occurs in patients with preexisting asthma, and involves the skin, lungs, and peripheral nerves. Poor-prognosis factors (i.e., involvement(s) of the gastrointestinal tract, heart, and/or kidney) have been assessed with the Five-Factor Score (FFS). One-third of the patients have anti-myeloperoxidase antineutrophil cytoplasm antibodies, and their presence seems to differentiate between two phenotypes, with different clinical characteristics and prognoses. Overall survival has improved markedly since the use of glucocorticoids and immunosuppressants, but relapse rates remain high. All patients require glucocorticoids, and for those with severe/refractory disease and FFS-defined poor prognoses, immunosuppressants should be used (cyclophosphamide for induction and azathioprine for maintenance therapy). Recent advances in EGPA management, including several novel immunomodulatory drugs and targeted biotherapies, were or are being evaluated to improve EGPA patients' prognoses.

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Section: Neurological Manifestationsmentioning

confidence: 53%