Gleich's syndrome

Overview

Gleich's syndrome is a rare and poorly understood disorder characterized by recurrent episodes of angioedema (swelling), elevated eosinophil counts in the blood (eosinophilia), and weight gain. It is also known as episodic angioedema with eosinophilia. The syndrome typically affects adults and is marked by cyclical or periodic symptoms that resolve on their own but recur over time. Unlike other eosinophilic disorders, Gleich’s syndrome is not usually associated with significant organ damage, which makes it clinically distinct and often more benign in nature.

Causes

The exact cause of Gleich's syndrome is unknown, but it is believed to involve an abnormal immune response. The condition is characterized by the overproduction of eosinophils—a type of white blood cell involved in allergic responses and parasitic infections. Research suggests that increased levels of certain immune system signaling molecules, such as interleukin-5 (IL-5), may play a role in stimulating eosinophil production and contributing to the recurring episodes of swelling and inflammation. There is no known genetic or infectious trigger, and most cases appear to be sporadic.

Symptoms

Gleich’s syndrome typically follows a cyclical pattern, with episodes recurring every few weeks or months. Common symptoms during episodes include:

• Angioedema: Sudden swelling of the face, limbs, or trunk

• Peripheral eosinophilia: Markedly elevated eosinophil counts during flares

• Weight gain: Often sudden and related to fluid retention

• Fever and fatigue

• Urticaria (hives): May occur in some cases

• Headache or malaise during active episodes

Between episodes, individuals are usually asymptomatic, and blood eosinophil levels return to normal or near-normal levels.

Diagnosis

Diagnosis of Gleich’s syndrome is primarily clinical, supported by laboratory findings and the exclusion of other conditions. Key diagnostic steps include:

• Complete blood count (CBC): Shows elevated eosinophil count during symptomatic periods

• Clinical history: Documentation of recurrent angioedema and weight gain episodes

• Serum IgM levels: Often elevated during episodes

• Interleukin-5 levels: May be increased, supporting immune dysregulation

• Exclusion of other causes: Rule out hypereosinophilic syndrome, parasitic infections, autoimmune diseases, and allergies

Treatment

There is no definitive cure for Gleich's syndrome, but treatment focuses on managing symptoms and preventing recurrences:

• Corticosteroids: Prednisone is often effective in rapidly reducing eosinophil counts and controlling swelling

• Immunomodulatory agents: In some cases, medications like interferon-alpha or anti-IL-5 therapies (e.g., mepolizumab) may be used

• Antihistamines: Used for symptomatic relief, especially in cases with urticaria

• Monitoring: Regular blood tests to track eosinophil levels and adjust treatment accordingly

Most patients respond well to treatment, and steroids can often be tapered between episodes.

Prognosis

The prognosis for individuals with Gleich’s syndrome is generally favorable. Unlike other eosinophilic syndromes, it typically does not cause end-organ damage. With appropriate management, most individuals can control symptoms and maintain a good quality of life. The condition may persist for many years, with intermittent episodes, but serious complications are rare. Long-term follow-up is recommended to monitor for changes in disease pattern or the development of complications.