Medium and Small-Sized Vessel Involvement in Hypereosinophilic Syndrome

Julien Rohmer¹, Matthieu Groh², Maxime Samson³, Jonathan London⁴, Marie JACHIET⁵, Diane Rouzaud⁶, Antoinette Perlat⁷, Romain Paule⁸, Felipe SUAREZ⁹, Jean-Emmanuel Kahn¹⁰, Loïc Guillevin¹¹ and Benjamin Terrier¹², ¹National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Internal medicine, France, Paris, France, ²Internal Medicine, Foch, Suresnes, France, ³Dijon University Hospital, Dijon, France, ⁴Service de Médecine Interne, Hôpital Cochin, Centre de référence national pour les maladies systémiques autoimmunes rares d’Ile de France, DHU Authors, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, Paris, France, ⁵Dermatology department, St Louis Hospital, Paris, France, ⁶Université Paris-Diderot, Paris, France, ⁷Internal medicine, CHU de Rennes, Rennes, France, ⁸Department of Internal Medicine, Department of Internal Medicine, Cochin University Hospital, Paris, France, ⁹Hematology Department, Necker Hospital, Paris, France, ¹⁰Service de Médecine Interne, Centre de Référence des Syndromes Hyperéosinophiliques-CEREO, Hôpital Foch, Université Versailles–Saint-Quentin-en-Yvelines, Suresnes, France, ¹¹Medecine Interne, Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, ¹²National Referral Center for Rare Systemic Autoimmune Diseases, Paris Cochin, France, Paris, France

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: Vasculitis

Session Information

Date: Sunday, October 21, 2018

Title: Vasculitis Poster I: Non-ANCA-Associated and Related Disorders

Session Type: ACR Poster Session A

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

Background/Purpose:

Primary systemic vasculitis, especially eosinophilic granulomatosis with polyangiitis and polyarteritis nodosa, can be associated with blood and tissue eosinophilia. Conversely, eosinophilia in the context of hypereosinophilic syndrome (HES) may involve various organs, including blood vessels. While HES-related superficial and/or deep venous thromboses have been extensively reported, little is known about its medium- and small-sized–vessel involvement.

Methods:

This multicenter retrospective study concerned patients with medium/small-sized–vessel vasculopathy and eosinophilia >1,000/mm³. Patients with cardiac embolism or preexisting thrombophilia and those with primary systemic vasculitis (as defined by the 2012 revised International Chapel Hill Consensus Conference) were excluded.

Results:

Among 13 patients with eosinophilia and medium- and/or small-sized–vessel involvement, 1 had mononeuritis multiplex with biopsy-proven eosinophilic vasculitis and 1 had retinal vasculitis and coronary spasm. For the remaining 11 patients (median age: 43 (21–62) yr), clinical characteristics included distal ischemia (digital ischemia and/or necrosis for 9 and pulse abolition with paresthesia for 2), with splinter hemorrhages for 5, purpura for 2 and Raynaud’s syndrome for 2. Eosinophilia had been detected before vasculopathy onset for 6 patients and concomitantly for 5. Their etiological work-ups for eosinophilia, including the search for the FIP1L1–PDGFRA fusion transcript and T-cell lymphoproliferative disorders, were unremarkable. At vasculopathy diagnosis, median eosinophil count was 5,500/mm³ (2,500–9,000/mm³). Doppler ultrasonograms of 9 patients revealed arterial thrombosis in 5 and/or stenosis in 4. Magnetic resonance imaging, CT angiography or arteriography, obtained for 7 patients, showed thrombosis and/or vascular involvement. Two patients had histological evidence of small-vessel vasculitis. First-line therapies included glucocorticoids (GCs) for 9, antiplatelet drugs for 9, anticoagulants for 7, iloprost for 3, immunosuppressants for 2 and anti-eosinophil drugs for 2. Eight of the 11 patients entered remission but 3 of them relapsed and 1 required high-dose GCs. Seven (63%) patients received further-line treatments, including immunosuppressants for 4 and/or anti-eosinophil drugs (interferon-a, imatinib, hydroxycarbamide or cyclosporine) for 5. Despite initially severe disease, 10 patients had complete remissions when their eosinophil counts normalized and 1 required transmetatarsal amputation prior to remission. During follow-up, clinical status always corresponded to blood eosinophilia.

Conclusion:

Medium- and small-sized–vessel vasculopathy associated with eosinophilia is a rare entity, frequently manifesting with distal ischemia that may not fulfill primary systemic vasculitis criteria but is consistent with HES. Despite initially severe disease, favorable outcomes were obtained for most cases with immunosuppressants and/or anti-eosinophil drugs.

Disclosure: J. Rohmer, None; M. Groh, None; M. Samson, None; J. London, None; M. JACHIET, None; D. Rouzaud, None; A. Perlat, None; R. Paule, None; F. SUAREZ, None; J. E. Kahn, None; L. Guillevin, None; B. Terrier, None.

To cite this abstract in AMA style:

Rohmer J, Groh M, Samson M, London J, JACHIET M, Rouzaud D, Perlat A, Paule R, SUAREZ F, Kahn JE, Guillevin L, Terrier B. Medium and Small-Sized Vessel Involvement in Hypereosinophilic Syndrome [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/medium-and-small-sized-vessel-involvement-in-hypereosinophilic-syndrome/. Accessed .

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