Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
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.
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.
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 September 30, 2020.
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ACR Meeting Abstracts - 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/