Central Nervous System Involvement of Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss): Retrospective Analysis of 26 Cases and Review of the Literature

Raphael André¹, Chahéra Khouatra², Jean-Luc Saraux³, Francois Maurier⁴, Gilles Blaison⁵, Boris Bienvenu⁶, Pascal Cathebras⁷, Nathalie Costedoat-Chalumeau⁸, Robin Dhote⁹, Aurélie Foucher¹⁰, Helder Gil¹¹, Joelle Lapouarie¹², David Launay¹³, Edouard Pertuiset¹⁴, Valentine Loustau¹⁵, Thierry Zenone¹⁶, Claire Le Jeunne¹⁷, Luc Mouthon¹⁸, Loïc Guillevin¹⁹ and Benjamin Terrier²⁰, ¹Cochin Hospital, Paris, France, ²CHU Lyon, Lyon, France, ³CH Eaubonne, Eaubonne, France, ⁴HP Metz Belle Isle Hospital, Department of Internal Medicine, Metz, France, ⁵Internal medicine, CHR, Colmar, France, ⁶Internal Medicine, Hospital Caen, Caen, France, ⁷Internal Medicine, University Hospital St Etienne, St Etienne, France, ⁸Internal Medicine Department, Cochin Hospital, “René-Descartes Paris V” University, Paris, France, ⁹Internal Medicine, Hospital Avicenne, Bobigny, France, ¹⁰CHU, Saint Pierre de la Réunion, France, ¹¹CHU, Besancon, France, ¹²CHU, Pau, France, ¹³Service de Médecine Interne, Centre National de Référence des Maladies Systémiques Rares, Hôpital Claude Huriez, CHRU Lille, Lille, France, ¹⁴CH René Dubos, Pontoise, France, ¹⁵Hôpital Henri Mondor, Créteil, France, ¹⁶CH de Valence, Valence, France, ¹⁷Department of Internal Medicine, Hotel-Dieu Hospital, AP-HP, Paris, Paris, France, ¹⁸Department of Internal Medicine, Department of Internal Medicine, Cochin Hospital, Referent Center for Necrotizing Vasculitis and Systemic Sclerosis, Paris-Descartes University, AP-HP, Paris, France, ¹⁹Internal Medicine, Hopital Cochin, Paris, France, ²⁰Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Paris, France

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: central nervous system involvement, Churg-Strauss syndrome, Neurologic involvement and vasculitis

Session Information

Date: Sunday, November 8, 2015

Title: Vasculitis Poster I

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose:

Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by asthma, blood and tissue eosinophilia, vasculitis-related peripheral neuropathy, glomerulonephritis or skin symptoms. Although peripheral nervous system involvement occurs frequently, usually as multiple mononeuropathy, exceptional central nervous system (CNS) disease, severe and associated with poor prognosis according to the 1996 Five-Factor Score, has been described. This study aimed to describe CNS involvement in EGPA.

Methods: This retrospective, observational, multicenter study included patients with EGPA meeting ACR criteria and/or Chapel Hill definitions, and CNS involvement affecting cranial nerves, brain or spinal cord. We also undertook a systematic literature review.

Results:

We analyzed 26 patients (50% women, mean age 57±15 years) and 62 reported EGPA-CNS cases (52% women, mean age 47±16 years). At EGPA diagnosis, their main manifestations included: asthma in 97%, hypereosinophilia in 98% with median eosinophil count 7500/mm³, sinonasal abnormalities in 66%, peripheral neuropathy in 55%, pulmonary infiltrates in 52% and biopsy with eosinophilic infiltration in 46%; 41% had cardiac involvement, including myocarditis (27%), pericarditis (16%), endomyocardial fibrosis (8%) and cardiac arrhythmia (2%). 38/71 (55%) were ANCA-positive, with a perinuclear-labeling pattern and/or anti-MPO specificity.

The CNS was involved in 86% at EGPA diagnosis, preceded EGPA in 2%, and occurred during follow-up in 12% after a median of 24 months. The main neurological signs were: 46 (52%) ischemic cerebrovascular lesions, 21 (24%) intracerebral hemorrhage or subarachnoid hemorrhage, 28 (32%) visual acuity loss (15 with retrobulbar optic neuritis, 10 occluded central retinal artery or 4 cortical blindness), and 18 (20%) cranial nerves palsies; 25 patients had ≥1 of these clinical CNS signs.

Patients with visual acuity loss and cranial nerve palsies had similar clinical-biological parameters. In contrast, patients with intracerebral or subarachnoid hemorrhage compared to those with ischemic cerebrovascular lesions were younger (43 vs 52 years), had less frequent myocarditis (10% vs 35%), more frequent peripheral neuropathy (67% vs 41%), glomerulonephritis (29% vs 13%) and ANCA (79% vs 52%); and had higher median eosinophil counts (12850 vs 7500/mm³).

All patients were treated with corticosteroids, associated with cyclophosphamide in 63%, azathioprine in 4% or methotrexate in 1%. Among the 81 patients with assessable neurological responses, 43% had complete responses without sequelae, 43% had partial responses with long-term sequelae and 14% refractory disease. After a mean follow-up of 36 months, 11 patients died, half of intracerebral hemorrhages.

Conclusion: The main EGPA-CNS manifestations form 4 distinct neurological pictures: ischemic lesions, intracerebral hemorrhages, cranial nerve palsies and optic neuritis- or central retinal artery occlusion-related visual acuity loss. Such manifestation should prompt practitioners to consider an EGPA diagnosis in such conditions. Neurological sequelae were common.

Disclosure: R. André, None; C. Khouatra, None; J. L. Saraux, None; F. Maurier, None; G. Blaison, None; B. Bienvenu, None; P. Cathebras, None; N. Costedoat-Chalumeau, None; R. Dhote, None; A. Foucher, None; H. Gil, None; J. Lapouarie, None; D. Launay, None; E. Pertuiset, None; V. Loustau, None; T. Zenone, None; C. Le Jeunne, None; L. Mouthon, None; L. Guillevin, None; B. Terrier, None.

To cite this abstract in AMA style:

André R, Khouatra C, Saraux JL, Maurier F, Blaison G, Bienvenu B, Cathebras P, Costedoat-Chalumeau N, Dhote R, Foucher A, Gil H, Lapouarie J, Launay D, Pertuiset E, Loustau V, Zenone T, Le Jeunne C, Mouthon L, Guillevin L, Terrier B. Central Nervous System Involvement of Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss): Retrospective Analysis of 26 Cases and Review of the Literature [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/central-nervous-system-involvement-of-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-retrospective-analysis-of-26-cases-and-review-of-the-literature/. Accessed .

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