Lack of efficacy of Cyclosporine A in a patient with episodic angioedema and eosinophilia (Gleich’s syndrome)

Immunoallergology Unit, Dept. of Biomedicine

University of Florence

June 9th, 2009

Fassio F, Matucci A, Vultaggio A, Rossi O, Almerigogna F, Agostini C, Liotta F, Annunziato F, Romagnani S, Maggi E

A 29yo man refers to our Unit for persistent eosinophilia, up to >18k/mm3

No target organ involvement

(skin, airways, cardiovascular, G-I, neurologic)

Fassio F, Matucci A, Vultaggio A, Rossi O, Almerigogna F, Agostini C, Liotta F, Annunziato F, Romagnani S, Maggi E

Clinical presentation: Eosinophilia

No genetic abnormalities

(FIP1L1, Bcr/Abl, JAK)

0

500

1000

1500

2000

2500

3000

1 2 3 4 5 6 7 8 9 10

cells/m

m3

Days

Eosinophils

1000-1500/mm3

Fassio F, Matucci A, Vultaggio A, Rossi O, Almerigogna F, Agostini C, Liotta F, Annunziato F, Romagnani S, Maggi E

Clinical presentation: Angioedema

Recurrent Angioedema, in the last 6 years

No evident trigger: food intake, drugs, insect stings…No C1-INH deficiency, either quantitative or functionalDiagnostic workup: negative

After a few days in hospital…

Fassio F, Matucci A, Vultaggio A, Rossi O, Almerigogna F, Agostini C, Liotta F, Annunziato F, Romagnani S, Maggi E

Clinical evolution: EAE

Episodic Angioedema with Eosinophilia: Gleich’s Syndrome

CCS

AE

5000/mm3

Fassio F, Matucci A, Vultaggio A, Rossi O, Almerigogna F, Agostini C, Liotta F, Annunziato F, Romagnani S, Maggi E

Diagnosis: Gleich’s Syndrome

Recurrent Angioedema + Eosinophilia

Described by Gleich in 1984

Unclear etiology

Very good prognosis (no target organ involvement)

Angioedema attacks can be treated with corticosteroids…

…but no prophylactic therapy!

Fassio F, Matucci A, Vultaggio A, Rossi O, Almerigogna F, Agostini C, Liotta F, Annunziato F, Romagnani S, Maggi E

Therapeutic Approach: Gleich’s Syndrome

Demonstration of an aberrant T Lymphocyte population

(CD3-CD4+CD5+)

Course of CyA as a steroid-sparing agent…

Failure!

Fassio F, Matucci A, Vultaggio A, Rossi O, Almerigogna F, Agostini C, Liotta F, Annunziato F, Romagnani S, Maggi E

Therapeutic Approach: Gleich’s Syndrome

Anti IL-5 MoAb

Procedure for compassionate use of Mepolizumab currently ongoing…

Increased production of Th2 cytokines, ↑↑↑ IL-5

Thank you!

fil.fassio@tin.itImmunoallergology Unit, Dept. of Biomedicine

University of Florence

June 9th, 2009