Pilot Moldovan Cluj

AP i l ot
Refere nce
ntre for
Ce
H ered itary
dem a in
An gi oe
Rom a ni a

Dumitru MOLDOVAN *, Enikö MIHÁLY **

* University of Medicine and Pharmacy Tîrgu Mureş
Allergology Department, Mures County Hospital
** Romanian Hereditary Angioedema Network

What is Hereditary Angioedema?

recurrent, recurrent, non-pruritic, nonpainful
angioedema in the subcutis or submucosa

Throat
Glottal edema, laryngeal
edema starting with swallowing
difficulties, voice changes,
Abdomen hoarseness, dyspnea, in
Vomiting, colicky pain, extreme cases suffocation
diarrhea, malaise

Skin
Swelling and feeling of
tension and pressure

antige Functi Anti-
TYPE C1q n C1- onal C4
INH C1-INH C1-INH

HAE-I N ↓ ↓ ↓ -

HAE-II N N/↑ ↓ ↓ -

AAE-I ↓ ↓ ↓ ↓ -
AAE-II
(autoimune ↓ N/↓ ↓ ↓ +
)

2004

Upgrade course in Allergology and Clinical
Immunology organized yearly by the
Hungarian Society of Allergology and
Clinical Immunology (Budapest, December)

4th C1 INH Workshop
Budapest, Ibis Vaci út
Spring, 2005

14:00-15:45 HAE Centers
Chairpersons: H Farkas, Y Mykal

H Farkas, G Füst, L Varga:
Mission though not impossible
D Zabolotny, I Gogunska, L Zabrodska:
Start of the program to study hereditary angioedema (HAE) in Ukraine
R Stefanov, P Krastev, M Stefanova:
HAE patients healthcare and management in Bulgaria – history, present and
future
K Stavric, S Peova, K Mironska, L Kareva, S Nikolovska, Lj Pavlova, M
Spirovski:
Diagnosis and treatment of hereditary angioedema in Macedonia
D Moldovan, Cs Todea:
Hereditary angioedema: present and perspectives in Romania
S Cimbollek, T González-Queved, M Díaz Fernández:
Experience and goals in Familiar angioedema from the South of Spain
A Blanch, O Roche, T Caballero, N Sastre, D Callejo, M López-Trascasa:
Patient registry and approach to the prevalence of HAE in Spain
K Obtulowicz, G Porebski, B Bilo, M Kapusta, P Obtulowicz:
Diagnostic and therapeutic problems in management of patients with HAE in
Cracow/Poland
MM Gompels, RJ Lock, M Abinum, CA Bethune, G Davies, C Grattan, AC
Fay, HJ Longhurst, L Morrison, A Price, M Price, D Watters.:
C1 inhibitor deficiency: UK Consensus Document. Improving standards in the
UK

Romania is far behind other
nations in the diagnosis and
treatment of HAE patients

HAE is usually
No HAE centre, misdiagnosed
and no
register of
HAE patients

No drug for the treatment of
HAE is available in Romania

No genetic studies in HAE

Previous intentions to register
C1 INH purified extracts from
plasma were unsuccessful

Map of HAE in Romania

2005

c cases
Only sporadi ta base
i dual da
i n indiv

International support
would be of paramount importance

HAE exchange programme
Regional / International
support

for clinicians for complement
laboratory

Long term
difficulties

 unable to access appropriate care
− 40% rural
− poor income
 chronic lack of funds of the state
insurance company

Aim

• To build-up a national data base

Defining skils

WORKSHOPS

ARTICLES

PPT PRESENTATIONS

INTERNET SITE

LEAFLETS

Targets for the HAE
n e t w o r k (1)

Allergist

Internal
Medicine

Dermatologist

Peditrician

Targets for the HAE
n e t w o r k (2)

Gastroenterologist

Anesthesiologist

ENT
specialist

Surgeon

Targets for the HAE
n e t w o r k (3)

General
practitioner

Targets for the HAE
network

Complement
laboratory

Local HAE
experts

National board
of HAE centres

Ministry of Health

Allergology and Clinical Immunology
meetings in Romania and HAE workshops

4th Balkan Congress of Allergy and Clinical Immunology
Bucharest September 2005

The Annual Conference of the Romanian Society of
Cluj-Napoca March 2006

3th HAE course
Budapest 8-10 March 2006

4th HAE course
Budapest 5-7 October 2006

First Congress of the Romanian Society of
Tirgu Mures, April 2007

Summer School
Cluj September 2007

Diagnostic and treatment opportunities and
limitations in care of patients with primary
imunodeficiencies in Romania and Moldavia
Iaşi 12-13 octombrie 2007

4th Balkan Congress of Allergy and
Clinical Immunology
Bucharest September 2005

HEREDITARY ANGIOEDEMA SYMPOSIUM

Chairpersons: Marco Ciccardi, Henriette Farkas

Henriette Farkas, Lilian Varga: Pathomechanisms, clinical
pictures and diagnosis of hereditary angioedema
Marco Cicardi:Hereditary angioedema: from pathophysiology to
treatment
Lilian Varga, Henriette Farkas: Regional HAE Network

Dumitru Moldovan, Csilla Todea: Steps in the improvement of
management of hereditary angioedema in Romania

The Annual Conference of the
Romanian Society of Allergology and
Clinical Immunology
Cluj-Napoca March 2006

WORKSHOP: HEREDITARY ANGIOEDEMA: PRESENT
AND FUTURE
Chairpersons: Henriette Farkas, Dumitru Moldovan
Florin Dan Popescu (Bucharest, Romania): Differential diagnosis
of angioedema without urticaria
Henriette Farkas (Budapest, Hungary): Clinical features of
herediatary angioedema (HAE)
Peter J. Späth (Switzerland): Pathomechanism and Diagnosis of
HAE
Henriette Farkas (Budapest): Therapy of HAE
Sylvia Herget (Germany): IMPACT. International, multicenter,
prospective, angioedema, C1-INH trials. The C1-INH program
with Berinert P for HAE a ZLB Behring
S Visscher , R Verdonk , R Schoemaker , G Choi , M Soeters, M
Levi, H Farkas, L Varga, J László, G Temesszentandrási, B Bilo,
G Porebski, K Obtulowicz, J Nuijens: Open-label Studies of
Recombinant Human C1 Inhibitor (rhC1INH) in Subjects with
Acute Attacks of HAE
Lilian Varga (Budapest): HAE NETWORK
Dumitru Moldovan (Tîrgu Mureş): HAE network in Romania
Dumitru Moldovan, Lorena Orosz, Linda Katona (Tîrgu Mureş):
Internet site of the Romanian HAE Network Association

3th HAE course
Budapest 8-10 March 2006

4th HAE course
Budapest 5-7 October 2006

Bowen T, Cicardi M, Bork K, Zuraw B, Frank M, Ritchie B, Farkas
H, Varga L, Zingale LC, Binkley K, Wagner E, Adomaitis P, Brosz
K, Burnham J, Warrington R, Kalicinsky C, Mace S, McCusker C,
Schellenberg R, Celeste L, Hebert J, Valentine K, Poon MC,
Serushago B, Neurath D, Yang W, Lacuesta G, Issekutz A, Hamed
A, Kamra P, Dean J, Kanani A, Stark D, Rivard GE, Leith E, Tsai E,
Waserman S, Keith PK, Page D, Marchesin S, Longhurst HJ, Kreuz
W, Rusicke E, Martinez-Saguer I, Aygoren-Pursun E, Harmat G,
Fust G, Li H, Bouillet L, Caballero T, Moldovan D, Spaeth PJ,
Smith-Foltz S, Nagy I, Nielsen EW, Bucher C, Nordenfelt P, Xiang
ZY. Hereditary angiodema: a current state-of-the- art review, VII:
Canadian Hungarian 2007 International Consensus Algorithm for the
Diagnosis, Therapy, and Management of Hereditary Angioedema.
Ann Allergy Asthma Immunol. 2008;100(Suppl 2):S30–S40.

Craig TJ, Levy RJ, Wasserman RL, Bewtra AK, Hurewitz D,
Obtułowicz K, Reshef A, Ritchie B, Moldovan D, Shirov T,
Grivcheva-Panovska V, Kiessling PC,Bernstein JA. Efficacy of
Human C1-Esterase Inhibitor (C1-INH) concentrate compared to
placebo in patients with acute abdominal or facial HAE attacks. J
Allergy Clin Immunol (submitted 2009).

Kiessling PK, Craig T, Levy R, Wasserman RL, Bewtra AK,
Hurewitz D, Obtulowicz K, Reshef A, Ritchie B, Moldovan D,
Shirov T, Grivcheva-Panovska V, Kiessling PC, Bernstein J.
Treatment of hereditary angioedema with human C1 esterase
inhibitor: results of a global, multicenter, randomized, placebo-
controlled, phase II/III dose finding study (I.M.P.A.C.T.1) Clin
Experimental Immunol, 154 (Suppl. 1), 1-224, 2008.

Moldovan D. Angioedemul ereditar. De la un caz la o reţea
naţională. Medic.ro 4, 31, supl.85-88, 2007.

Moldovan D. Angioedemul ereditar: o afecţiune cu potenţial letal.
Al VI-lea Congres Naţional de Medicină Internă, 6-8 aprilie, 2006,
Călimăneşti-Căciulata. EMC 0903/27/02/2006.

Moldovan D. Hereditary angioedema: a life-threatening disorder. J
Romanian Soc Allergology Clin Immunol 2005; II, 3:18-24.

Moldovan D. Hereditary angioedema: a multifaced disease. EAACI
& Ga2len Allergy School, Cluj-Napoca, 5-10 Sept. 2007.

Moldovan D. Mission is possible. J Romanian Soc Allergol- ogy
Clin Immunol 2005; II, 3:47.

Moldovan D. Our first 3 years of experience in managing he-
reditary angioedema in Romania. Annual Conference of the Rom
Soc Allergol Clin Immunol, Baia Mare, 1-4 mai 2008.

Moldovan D. Reţeaua Română de Angioedem Ereditar. Conferinţa
„Bolile rare: de la evaluarea nevoilor la stabilirea priorităţilor”. UMF
„Victor Babeş” Timişoara, Zalău, 2-3 noiembrie 2007.

Moldovan D. Romanian Hereditary Angioedema Network. 5th C1
inh Deficiency Workshop, Budapest, 31 May-3 June 2007. Abstract
book, 53.

Moldovan D. Reţeaua de angioedem ereditar în România.
Conferinţa Naţională Anuală de Alergologie şi Imunologie Clinică,
24-26 Martie 2006, Cluj-Napoca.

Moldovan D, Dobreanu M, Mihaly E. Hereditary Angioedema:
diagnostic difficulties, the Romanian Network for HAE, and
IMPACT study. A 36-a Conferinţă Naţională de Imunologie, Tîrgu
Mureş, 21-23 septembrie 2006.

Moldovan D, Dobreanu M, Popescu FD, Vieru M, Cristea C, Tătaru
A. The Romanian Network for Hereditary Angioedema. Present and
perspectives. 1st Congress of the Romanian Society of Allergology
and Clinical Immunology, Tîrgu Mures, 26-28 April, 2007.

Moldovan D, Levy RJ, Visscher S, Relan A, Nuijens JH, Hack CE.
Interim results from ongoing open-label studies with recombinant C1
inhibitor (Rhucin; rC1INH) for treatment of patients with acute
attacks of hereditary angioedema. Oral presentation. 6th Workshop
on Hereditary Angioedema. Budapest, 21-24 May, 2009.

Moldovan D, Mihaly E. A MOGYE es a SOTE egyutmukodése a
herediter angioodemás betegek ellatásában. Orvostudomanyi ertesito,
82, suppl.1, 39 p, 2009.

Moldovan D, Mihály E. Angioedemul ereditar: o imunodeficienţă
primară relativ bine conturată. Al 21-lea Simpozion “J Project”.
Posibilităţi şi limite în diagnosticul imunodeficienţelor primare în
România şi Moldova” 12-13 octombrie 2007, Iaşi.

Moldovan D, Mihaly E. Angioedemul ereditar: o imunodeficienţă
primară subevaluată. Conferinţa de Imunodeficienţe Primare, J
Project, Buziaş 19-20 martie 2009.

Moldovan D, Mihály E. Open label treatment with Rhucin. The
Experience from Romania. Investigator meeting. Amsterdam 2008.

Moldovan D, Mihaly E, Popescu FD, Dobreanu M. Hereditary C1-
inhibitor esterase deficiency: a rather well-defined entity. Rom Rev
Lab Med 2009 (in press).

Moldovan D, Mihály E, Szöllősi GA. A romániai herediter
angiooedema hálózat és nemzetközi együttműködése 36th Congress
of the Hungarian Society of Allergology and Clinical Immunology,
Siofok, Hungary, May 15-17 2008.

Moldovan D, Mihály E, Szöllösi, G. Model de asistenţămedicală
multicentrică în bolile rare: Reţeaua Română de Angioedem Ereditar.
Sesiunea Ştiinţifică Anuală a Cadrelor didactice, UMF Tîrgu Mureş,
13-14 Dec 2007.

Moldovan D, Orosz L, Katona L. Site-ul de Internet al Asociaţiei
Române pentru angioedemul ereditar. Conferinţa Naţională de
Alergologie şi Imunologie Clinică. Cluj-Napoca, 2006.

Moldovan D, Tătaru A. Angioedemul ereditar: de la erori de
diagnostic la o reţea naţională. Congresul National de Dermatologie,
Bucureşti, 1-4 noiembrie, 2006.

Moldovan D, Todea Cs. Angioedemul ereditar: o boală rară, dar
uneori fatală. Conferinţa Naţională a Societăţii de Pediatrie, Galaţi,
octombrie, 2005.

Moldovan D, Todea Cs. Hereditary angioedema: present and
perspectives in Romania. 4th C1 inhibitor Deficiency Work- shop.
Budapest, 29 April-1 May 2005, Abstract book pg.40.

Moldovan D, Todea Cs. Steps in the improvement of management
of hereditary angioedema in Romania. 4th Balkan Congress Allergy
Clin Immunol Bucureşti, 22-25 Sept 2005, Abstract book pg. 24.

Nuijens J, Verdonk R, Giannetti B, Visscher S, van Doorn M, Resink
T, Levi M, Hack C, Farkas H, Obtulowicy K, Caballero T, Perricone
R, Cancian M, Montinaro V, Cillari E, Gonzales-Quevedo T,
Moldovan D, Schelzinger M, Trig- giani M, Longhurst H, Neri S,
Cicardi M. Clinical studies of recombinant human C1 ihibitor
(rhC1INH) in Patients with Acute attacks of Hereditary Angioedema.
1st International Leadership Conf. Frankfurt/Main, 19-21 Oct 2007.

Popescu FD, Vieru M, Moldovan D. A study of drug-induced
angioedema without urticaria 5th C1-INH Deficiency Workshop,
Budapest, 31 May-3 June 2007. 32. Szöllösi G, Mihály E, Moldovan
D. Romanian Hereditary Angioedema Network, Allergy, 2007,62:
S83: 479. 33. Tătaru A, Moldovan D. Angioedemul ereditar: de la
fiziopa- tologie la tratamentul modern. Congresul National de Der-
matologie, Bucureşti, 1-4 noiembrie, 2006.

Moldovan D. Hereditary Angioedema, an example for the
understanding and management of rare diseases. Revista de
Medicină şi Farmacie - Orvosi és Gyógyszerészeti Szemle, 2009 55,
s3, 3-6.

Mihály E, Moldovan D. Some genetic issues in hereditary
angioedema. Revista de Medicină şi Farmacie - Orvosi és
Gyógyszerészeti Szemle, 2009 55, s3, 7-10.

Moldovan D, Popescu FD. Pathophysiology of hereditary angioedema.
Revista de Medicină şi Farmacie - Orvosi és Gyógyszerészeti Szemle,
2009 55, s3, 11-15.

Moldovan D, Mihály E, Popescu FD. Clinical manifestations and
diagnosis of hereditary angioedema. Revista de Medicină şi
Farmacie - Orvosi és Gyógyszerészeti Szemle, 2009 55, s3, 16-21.

Mihály E, Moldovan D, Dobreanu M. Laboratory diagnosis in
evaluation of patients with hereditary angioedema. Revista de
s3, 22-28.

Moldovan D, Mihály E. The treatment of angioedema caused by C1-
Inhibitor deficiency. Revista de Medicină şi Farmacie - Orvosi és

Mihály E, Moldovan D. Other therapeutic applications of C1-
esterase inhibitor. Revista de Medicină şi Farmacie - Orvosi és

Căpâlnă B, Moldovan D. Hereditary angioedema in children.
Revista de Medicină şi Farmacie - Orvosi és Gyógyszerészeti
Szemle, 2009 55, s3, 39-45.

Nădăşan V, Moldovan D. Hereditary angioedema patient education
and professional Web resources. Revista de Medicină şi Farmacie -
Orvosi és Gyógyszerészeti Szemle, 2009 55, s3, 46-49.

Moldovan D, Mihály E, Dobreanu M, Brassai T, Popescu FD, Vieru
M, Deleanu D, Cristea V, Agache I, Ungureanu M, Leru P,
Iamandescu B, Tătaru A, NădăN
Hereditary Angioedema. Four-years experience. Revista de
s3, 50-56.

Romanian
Hereditary
Angioedema Network

Founded in 2006

5th C1 INH workshop
Budapest
Spring 2007

08:30-10:30Patients’ Associations and management
Chairpersons: T. Bowen, A. Menendez, S. Smith-Foltz
T. Bowen, B. Ritchie, J. Heber, E. Wagner, K. Brosz, J. Brosz, P.
Adomaitis, J. Burnham, B. Yang:
Canadian 2006 International Consensus Algorithm for the Diagnosis,
Therapy and Management of Hereditary Angioedema – Canadian
Review and Experience 2007
T. Caballero, S. Cimbollek, R. Cabañas, A. Campos, C. Gómez-
Traseira, MT. González-Quevedo, M. Guilarte, J. Jurado-Palomo, JI.
Larco, MC. López-Serrano, C. Marcos, M. Pedrosa, N. Prior, M.
Rubio:
Introduction to the Spanish Clinical Group for the Study of
Angioedema due to C1 inhibitor deficiency (SGACI)
T. Castaldo:
HAE International (HAEI)
G. Harmat:
In what ways should European action help support the health systems
of the Member States?
D. Moldovan:
Romanian Hereditary Angioedema Network
P. Nordenfelt, L. Mallbris, J. Björkander, P. Hellström, AK. Lefvert,
A. Lindfors, L. Lundblad, K. Löfdahl , L. Nordvall, L. Truedsson, S.
Werner, CF. Wahlgren:
Sweha a swedish project that will survey HAE in Sweden
N. Prior, E. Remor, C. Gómez Traseira, MC. López Serrano,V.
Cardona, S.Cimbollek, T. González Quevedo, M. Guilarte, D.
Hernández , C. Marcos, M. Rubio, T. Caballero:
Development of a disease specific health-related quality of life
(HRQOL) questionnaire in adults with hereditary angioedema due to
C1 inhibitor deficiency (HAE)

Annual Conference of Allergology and
Clinical Immunology
Baia Mare, May 2008

Workshop: C1 inibitor deficiency (Deficienta de C1-inhibitor
esteraza)

Konrad Bork (Guthenberg University, Maiz, Germany): Clinical
manifestations of hereditary angioedema
Lilian Varga (Semmelweis University Budapest, Hungary):
Diagnosis of hereditary angioedema
Henriette Farkas (Semmelweis University Budapest, Hungary):
New therapies in hereditary angioedema
J Nuijens, R Verdonk, B Giannetti, S Visscher, M van Doorn, T
Resinka, M Levic, C Hack, H Farkas, K Obtulowicz, T Caballero,
R Perricon*, M Cancia, V Montinaro, E Cillari, T Gonzales-
Quevedo, D Moldovan, M Schlezinger, M Triggiani, H
Longhurst, S Neri, M Cicardi: Clinical Studies of Recombinant
Human C1 Inhibitor (rhC1INH) in Patients with Acute Attacks of
Hereditary Angioedema
Ioana Agache, Liliana Duca (Transylvania University, Medicine
Faculty, Department of Immunology-Allergology, Brasov): Our
experience on acquired C1 inhibitor deficiency in autoimmune
disorders
Dumitru Moldovan, Enikö Mihály, Géza-Attila Szöllösi
(University of Medicine and Pharmacy, Tirgu Mures): Three
years experience in managing hereditary angioedema in Romania

14.00 – 14.45 HANO SZIMPÓZIUM
Üléselnök: Dr. Farkas Henriette, Dr. Kadocsa Edit

14.00–14.20 Icatibant - a novel treatment for hereditary angioedema Werner Aberer
/JERINI szimpózium University of Graz, Department of Dermatology, Graz, Austria

14.25–14.35 Új eredmények a Herediter angioneuroticus oedema kutatásában és
kezelésében Farkas H., L. Varga, G. Széplaki, Gy. Temesszentandrási, L. Jakab, B.
Fekete, G. Füst, I. Karádi

14.35–14.45 A romániai herediter angiooedema hálózat és nemzetközi
együttműködése Moldovan D., Mihály E., Szöllősi GA.

The National Alliance for Rare
Diseases in Romania signed a
cooperation protocol with Ministry of
Health, and in the coming year are
expected a development of centres of
excelence.

Good news
The Romanian HAE Network
have started to work

The awareness of HAE is
improving

40 of our patients have C1 INH
antigen and function measured

Some improvement in
complement laboratory

Two clinical trials, one still
ongoing


2008

∗
∗ ∗

∗ ∗ ∗
∗
∗ ∗
∗ ∗
∗ ∗
∗
∗
∗
∗
∗
∗
∗
∗

6th C1 INH workshop
Budapest
May 2009


2009

53 confirmed cases

 10 were diagnostized in Romania

 21 diagnosed in Budapest, and 22 in
Karlsruhe and Groningen

 belong to 19 families

 7 are type II and 44type I

 we didn’t find yet any type III HAE patients

DEZIDERATA

HAE REFERENCE CENTRE

COMPLEMENT
LABORATORIES

HAE REGISTRY

PATIENTS’ ORGANIZATION

REGISTRATION OF DRUGS FOR
EMERGENCY AND PREVENTIVE TREATMENT

TASKS OF THE CENTER

 Diagnostic procedure
 Differential diagnostic procedure
 Treatment
 Follow-up
 Register
 Spread of knowledgeed
 Educating of colleagues
(postgraduate and graduate courses)
 Research
 Conference activity
 Publications
 Joining to the international projects
(research and trials)

i o na l
te rna t
G ood i n
coope ra ti o n

What can we offer?

 HAE training
 Diagnostic and therapeutic protocols
 Measurements of complement
parameters
 Telephone and e-mail hot-line service
for physicians and patients
 Storage of samples (-800 C)
 Referals to the most outstanding
HAEcentres

Acknowledgments

Patients

Acknowledgments

Industry

Acknowledgments

Network for
Complement
Related
Diseases

Colleagues and friends

• Prof. dr. Henriette Farkas
• Dr. Lilian Varga
• Prof.dr. Gyorgy Fust
• Prof. dr. Marco Cicardi
• Prof. dr. Konrad Bork
• Peter Spaeth
• Tom Bowen
• Avner Reshef
• Timothy Craig

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