A Case Report of Idiopathic Hyper Eosinophilic Syndrome (HES) in a Rheumatoid Arthritis Patient

Hala El-Hadary^1*, Dadour, N. M.², Ahmed, H.³ & Moenes, D.³

¹Consultant of Rheumatology & Immunology, Kasr El-Ainy, School of Medicine, Cairo University, Egypt
²Specialist of Rheumatology & Rehabilitation, Egyptian Fellowship, Cairo, Egypt
³Resident of Rheumatology & Rehabilitation, ELKatib Hospital, Cairo, Egypt

Hala El-Hadary, Consultant of Rheumatology & Immunology, Kasr El-Ainy, School of Medicine, Cairo University, Egypt.

Keywords: Hypereosinophilic syndrome; Eosinophilia; Rheumatoid Arthritis; Prednisolone

Abstract

Hypereosinophilic syndrome (HES) is a myeloproliferative disorder (MPD) characterized by persistent eosinophilia that is associated with damage to multiple organs.

Anderson first described the Peripheral eosinophilia with tissue damage in 1968. In 1975, Chusid defined the three features required for a diagnosis of hypereosinophilic syndrome: a sustained absolute eosinophil count (AEC) greater than >1500/µl, which persists for longer than 6 months, No identifiable etiology for eosinophilia and Signs and symptoms of organ involvement.

However, due to advances in the diagnostic techniques, secondary causes of eosinophilia can be identified in a proportion of cases that would have otherwise been classified as idiopathic hypereosinophilic syndrome.

Secondary eosinophilia is a cytokine-derived (interleukin-5 [IL-5]) reactive phenomenon. Parasitic diseases are the most common cause, whereas in developed countries world widely, but allergic diseases are the most common cause in developed countries. There are other causes such as: Malignancies, Metastatic cancer, Tcell lymphoma, colon cancer, pulmonary eosinophilia, Loffler syndrome, Churg-Strauss syndrome, allergic bronchopulmonary aspergillosis, Connective tissue disorders – Scleroderma, polyarteritis nodosa, Skin diseases, Dermatitis herpetiformis, Inflammatory bowel disease, Sarcoidosis and Addison disease.

Clonal eosinophilia is diagnosed by bone marrow histology, cytogenetics, and molecular genetics. Like Acute leukemia, PDGFRA (platelet-derived growth factor receptor, alpha polypeptide, PDGFRß -rearranged eosinophilia, KIT-mutated systemic mastocytosis Myelodysplastic syndrome (MDS) etc.

Idiopathic eosinophilia is a diagnosis of exclusion when secondary and clonal causes of eosinophilia are excluded.

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