Ascites in a patient with episodic angio-oedema and eosinophilia: thinking outside the box

Rigopoulou, Eirini I.; Ιωάννου, Μαρία; Papadamou, Georgia; Dalekos, Georgios N.

doi:10.1136/bcr-2017-219467

Cited by 2 publications

(2 citation statements)

References 12 publications

(7 reference statements)

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“…It is also important to note that seven patients had prior history of angioedema. 8 However, there was no relationship linking either of the aforementioned past medical conditions with the severity of AE or its remission following treatment.…”

Section: Resultsmentioning

confidence: 98%

Gleich syndrome: a systematic review

et al. 2020

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Characterized chiefly by hypereosinophilia and angioedema, Gleich syndrome is a rare disorder with unclear clinical and therapeutic findings. Other symptoms include increased IgM levels, weight gain, fever, and urticaria. Herein we review Gleich syndrome and assess clinical features, epidemiology, and treatment options. Thirty-two articles including case reports or case series of eosinophilic angioedema and Gleich syndrome were included. Data regarding patient age, gender, and history, clinical and biological manifestations, and treatment protocols were recorded. The most common clinical findings include recurrent or non-recurrent angioedema, fever, urticaria, weight gain, blood eosinophilia, and elevated immunoglobulin levels. Corticosteroid therapy is the mainstay of treatment. Gleich syndrome is a distinctive hypereosinophilic entity with a benign course and good response to systemic corticosteroids. More studies are needed to evaluate the pathophysiology of this syndrome and lead to better therapeutic options.

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Section: Resultsmentioning

confidence: 98%

Gleich syndrome: a systematic review

et al. 2020

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“…Moreover, the absence of concomitant allergic, infective, and connective tissue disorders, together with a significant clinical improvement (with a rapid decrease of the eosinophil count to normal levels) following corticosteroid therapy, was pivotal to assess the diagnosis. Our patient had normal immunoglobulin levels on admission, so we would like to stress that IgM levels are elevated in many, but not all EAE patients [ 4 , 5 , 11 , 16 , 17 , 18 ]. Laboratory findings are not pathognomonic in EAE, except for the typical elevation of the eosinophils count during attacks.…”

Section: Differential Diagnosismentioning

confidence: 93%

Episodic Angioedema with Hypereosinophilia (Gleich’s Syndrome): A Case Report and Extensive Review of the Literature

Mormile

Petraroli

Loffredo

et al. 2021

JCM

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Episodic angioedema with eosinophilia (EAE) (Gleich’s syndrome) is a rare disease characterized by hypereosinophilia (up to 95 × 109 cells/L), recurrent episodes of angioedema, urticaria, weight gain, and fever, that occur at periodical intervals (usually every 3–4 weeks). The exact etiology of EAE is still unclear, but both eosinophils and abnormalities of cytokines homeostasis seem to play a pivotal role in the pathogenesis of the disease. In particular, the cyclic elevation of serum interleukin-5 before the increase in eosinophil count has been reported. Herein, we performed a broad literature review and report the case of a thirty-two-year-old woman with a two-year history of cyclic angioedema attacks, urticaria, periodic weight gain, and severe hypereosinophilia, diagnosed with EAE and treated with oral corticosteroids. Describing the most relevant clinical features of EAE reported so far in the literature, we aim to provide physicians with some useful tools to help them deal with this disease. In addition, we aim to raise awareness about this rare condition in which approved diagnostic classification criteria are currently missing.

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Ascites in a patient with episodic angio-oedema and eosinophilia: thinking outside the box

Cited by 2 publications

References 12 publications

Gleich syndrome: a systematic review

Gleich syndrome: a systematic review

Episodic Angioedema with Hypereosinophilia (Gleich’s Syndrome): A Case Report and Extensive Review of the Literature

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