1. 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

2. 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/mm3
Days
Eosinophils

3. 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 functional
Diagnostic workup: negative

4. 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

5. 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!

6. 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!

7. 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
Procedure for compassionate use of
Mepolizumab currently ongoing…
Increased production of Th2 cytokines, ↑↑↑ IL-5

8. Thank you!
fil.fassio@tin.itImmunoallergology Unit, Dept. of Biomedicine
University of Florence
June 9th, 2009