AUTOINFLAMMATORY DISEASES IN HEMATOLOGY FROM DIAGNOSIS TO TREATMENT

AUTOINFLAMMATORY_DISEASES_IN_HEMATOLOGY_FROM_DIAGNOSIS_TO_TREATMENTkapak

HYPERIMMUNOGLOBULIN D SYNDROME (HIDS)

Sevde Yazıcı Şahin

Kocaeli University, Faculty of Medicine, Department of Hematology, Kocaeli, Türkiye

Yazıcı Şahin S. Hyperimmunoglobulin D Syndrome (HIDS). In: Kurt Yüksel M, editor. Autoinflammatory Diseases in Hematology from Diagnosis to Treatment. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.211-217.

ABSTRACT

Hyperimmunoglobulin D syndrome (HIDS) is a rare periodic fever syndrome with autosomal recessive inheritance. It is characterized by attacks of systemic inflammation triggered by physiological stress during the first year of life. The goals of treatment are attack prevention and symptom management through suppression of innate immunity. This chapter discusses the epidemiology, etiopathology, clinical manifestations, important laboratory findings, differential diagnosis, and treatment of HIDS.

Keywords: Hyperimmunoglobulin D syndrome; Periodic fever syndrome; Systemic inflammatory syndrome

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