Rare lung diseases in Romania where are we and where we need to be?
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1 Rare lung diseases in Romania where are we and where we need to be? Ruxandra Ulmeanu Visiting Professor, MD, PhD,FCCP President of the Romanian Society of Pneumology National representative GOLD,Central- Eastern European Alpha-1 Antitrypsin Network Head of Pneumology Department Faculty of Medicine Oradea Institute of Pneumology"Marius Nasta Bucharest
2 RARE LUNG DISEASES one of the most challenging activity for chest physicians
3 Diagnostic challenges Alpha-1 antitripsin deficiency Idiopathic pulmonary fibrosis (IPF)
4 Alpha-1 antitripsin deficiency in Romania
5 AATD - a disease slowly investigated Time between first symptoms and diagnosis 7.2 years ± 8.3 years Before the diagnosis is established at least 3 doctors evaluation Peter J. Barnes, Chronic Obstructive Pulmonary Disease, N Engl J Med 2000; 343:
6 AATD in other countries represents an iceberg 5% of patients diagnosed 1. K. Stoller, A Review of a1-antitrypsin Deficiency, Am J Respir Crit Care Med Vol 185, Iss. 3, pp , Feb 1, Darren N. Saunders, A Novel SERPINA1 Mutation Causing Serum Alpha 1 -Antitrypsin Deficiency, PLoS One. 2012; 7(12): e Ruxandra Ulmeanu et al, Epidemiology of AADT in Central-Eastern Europe where are we now?,first CEE -AATD Network Conference,Warsaw,19 th April
7 before 2012 AATD in Romania frozen frozen diagnostic lake we just know that it exists Ruxandra Ulmeanu et al, Epidemiology of AADT in Central-Eastern Europe where are we now?,first CEE -AATD Network Conference,Warsaw,19 th April
8 Whom to test? in accordance with AATD Romanian Guideline indications 2013 A genetic cause of the premature cases of COPD emphysema bronchiectasis incomplete reversibility asthma
9 before 2012 AATD in Romania The disease was usually identified only by the plasmatic values Genetic testing - only in the private practice with samples worked abroad and costs fully covered by the patient. Ruxandra Ulmeanu et al, Epidemiology of AADT in Central-Eastern Europe where are we now?,first CEE -AATD Network Conference,Warsaw,19 th April 2013
10 Genetic screening for AATD in Romania
11 Genetic Screening for AATD in Romania We start to introduce the standards of the best medical practice for AATD patients in Romania since 2012
12 It was a complex and extremely helpful activity for Romanian team Trainings for doctors (6) biochemists (1) nurses (1) physiotherapists (1) in hospitals and laboratories from Warsaw, Hanover, Vilnius, Leiden
13 Genetic Screening for AATD in Romania goes on. although Leonardo project came to an end with the support of The National Institute of Lung Diseases - Warsaw Joanna Chorostowska-Wynimko Head of Central-Eastern European Alpha-1 Antitrypsin Network
14 AATD in Romania? Certainly AATD in Romania is an under-diagnosed pathology in patients with COPD Ruxandra Ulmeanu,Ana Nebunoiu et al, Alpha 1 antitrypsin deficiency in Romania: preliminary results from the genetic screening ,unpublished data
15 Preliminary results for Romania
16 Age distribution Minimum: The 5 average years age for testing fluctuated around 50 years, Maximum: 85 years in accordance with the decade Median: ±16.20 when years commonly Average: AATD is years symptomatic and diagnosed N= years Ruxandra Ulmeanu, Ana Zaharie et al, Alpha 1 antitrypsin deficiency in Romania: preliminary results from the genetic screening ,unpublished data
17 6.5 % of screened patients were identified to have genetic modification heterozygous, more rarely homozygous Normal genotype: 93.31% Modified genotype: 6.69% N=642 available results Classical mutations (83.72%) Rare mutations (16.28%) heterozygote c.1033g>t p.val321phe Ruxandra Ulmeanu, Ana Zaharie et al, Alpha 1 antitrypsin deficiency in Romania: preliminary results from the genetic screening ,unpublished data
18 Rare genotypes Genotype Number Percent (%) Median AAT level (mg/dl) SD (mg/dl) IM IZ P lowell Z undetectable plasma levels - - P lowell M M wurzburg M Average AAT level (mg/dl) heterozygote c.1033g>t p.val321phe Ruxandra Ulmeanu, Ana Zaharie et al, Alpha 1 antitrypsin deficiency in Romania: preliminary results from the genetic screening ,unpublished data
19 Reason for testing of modified genotype N= COPD is the main indication COPD Siblings Bronchiectasis Asthma COPD+bronchiectasis ACOS COPD+emphysema Emphysema+pneumothorax COPD+cirrhosis Others* *Others: Chronic bronchitis, interstitial fibrosis, neonatal hepatitis syndrome history Ruxandra Ulmeanu, Ana Zaharie et al, Alpha 1 antitrypsin deficiency in Romania: preliminary results from the genetic screening ,unpublished data
20 COPD remains the main indication for testing for patients with modified genotype screening of first-degree relatives in 2nd bronchiectasis ranks in 3rd place refractory asthma ACOS COPD+cirrhosis until now 6.50% of patients with COPD are carriers of a modified gene ( 15 from 228 COPD tested patients ) Ruxandra Ulmeanu, Ana Zaharie et al, Alpha 1 antitrypsin deficiency in Romania: preliminary results from the genetic screening ,unpublished data
21 N=43 Gender distribution in patients with modified genotype Sex ratio (M:F) 1.38:1 is higher among men 18 42% 25 male 58% female Ruxandra Ulmeanu, Ana Zaharie et al, Alpha 1 antitrypsin deficiency in Romania: preliminary results from the genetic screening ,unpublished data
22 What else have we done for AATD in Romania?
23 Alpha-1 antitrypsin deficiency Working Group of Romanian Pneumology Society Sibiu June 17th founding members Lavinia Davidescu Coordinator
24 We kept increasing the AATD awareness in Romanian medical community
25 We have the guideline for the diagnosis and management of AATD the version in Romanian language
26 Lavinia Davidescu Coordinator of AATD Working Group of the Romanian Society of Pneumology Ruxandra Ulmeanu National representative Central- Eastern European Alpha-1 Antitrypsin Network
27 Joanna Chorostowska-Wynimko Scientific Director National Institute of Tuberculosis & Lung Diseases, Warsaw, Poland Head of Central-Eastern European Alpha-1 Antitrypsin Network
28 Overview of the preliminary data for AATD screening for Romania in 2015
29 over 400 participants
30 Experts from 9 medical specialties
31 An exceptional involvement of young specialists, residents, students
32 Accession to the European DAAT organizations Founding members of the Network of Alpha 1 antitrypsin Deficiency in Central and Eastern Europe
33 Romania become member of AATD Network of Central Eastern Europe
34
35
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37 Authors in relevant publications of the European Respiratory Society The incidence of severe alpha-1-antitrypsin (AAT) deficiency alleles in COPD patients Preliminary results from Central Eastern European (CEE) AAT NETWORK Joanna Chorostowska-Wynimko, Anna Kubincova, Nikolay Yanev, Ruxandra Ulmeanu, Radoslaw Struniawski, Pavol Pobeha, Nikolay Kyuchukov, Oana Deleanu, Beata Poplawska, Ruzena Tkacova, Yavor Ivanov, Florin Dumitru Mihaltan, Arunas Valiulis, ERJ September 1, 2013 vol. 42 no. Suppl 57 P541 The incidence of severe alpha-1-antitrypsin (AAT) deficiency alleles in COPD patients Update from Central Eastern European (CEE) AAT Network Joanna Chorostowska-Wynimko, Anna Kubincova, Nikolay Yanev, Ruxandra Ulmeanu, Radoslaw Struniawski, Pavol Pobeha, Nikolay Kyuchukov, Oana Deleanu, Beata Poplawska-Wisniewska, Ruzena Tkacova, Yavor Ivanov, Florin Mihaltan, Arunas Valiulis, ERJ September 2014 vol. 44 no. Suppl ;
38 and starting with 2015 Romania was repesented at The Biennial Alpha-1 Global Patient Congress and International Research Conference 2015,2016,2017 Simona Olteanu Romanian patients representative Lavinia Davidescu Coordinator of Romanian AATD Working Group The Romanian stand Hillegonda Guttierez Alpha 1 Global Director
39 Alpha -1 Global Association website in Romanian language Bine ati venit la Pagina Romaniei Deficitul de Alfa 1 Antitripsina
40 First flyer with information about Alpha-1 antitrypsin deficiency for patients
41
42 The Unique National Register of Alpha-1 Antitrypsin Deficiency The Unique National Register of Alpha-1 Antitrypsin Deficiency was founded in 2016 with the support of the Romanian Society of Pneumology and is a database that includes cases of deficiency of alpha-1 antitrypsin and genetic modifications that are not accompanied by a significant decrease of the amount of alpha-1 antitrypsin in plasma.
43 Novelties Interactive Session with Romanian Patients Associations
44 Frank Willersinn Alpha-1 Global & Alpha-1 Foundation Bruxelles, Belgium
45 Saturday October 8th, 2016
46
47 Summarizing
48 Almost 1000 patients screened Mostly heterozygotes Rare genotypes 3 PIZZ, 1 PISZ, 1 PIP Lowell Z Mostly in COPD patients
49 AATD in Romania continuing screening among risk persons establishing a national reference laboratory Genetic Screening for AATD in Romania creating a national database for patients goes on! identified with AATD
50 Idiopathic pulmonary fibrosis (IPF)
51 Idiopathic pulmonary fibrosis (IPF) Is a devastating condition barely 1/2 of patients surviving the 3 years Carries a prognosis worse than that of many cancers. 1. King TE, Pardo A, Selman M: Idiopathic pulmonary fibrosis. Lancet 2011, 378: Ganesh Raghu et al :An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med Vol 183. pp , Spagnolo et al : Idiopathic pulmonary fibrosis: diagnostic pitfalls and therapeutic challenges. Multidisciplinary Respiratory Medicine 2012, 7:42 4. Athol U. Wells, Ulrich Costabel et al: Challenges in IPF diagnosis, current management and future perspectives. Sarcoidosis,vasculitis and diffuse lung diseases 2015; 32; Suppl. 1: Ganesh Raghu et al :An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis An Update of the 2011 Clinical Practice Guideline, Am J Respir Crit Care Med Vol 192, Iss 2, pp e3 e19, Jul 15, Fidler, Lee et al: Diagnostic disparity of previous and revised American Thoracic Society guidelines for idiopathic pulmonary fibrosis/la disparité diagnostique entre les lignes directrices passées et révisées de l'american Thoracic Society à l'égard de la fibrose pulmonaire idiopathique.canadian Respiratory Journal :
52 Suspicion of IPF often months/years after the initial manifestations The most common presenting symptoms dry cough exertional dyspnea wrongly attributed to smoking habits or aging. 1. Flaherty KR et al: Idiopathic interstitial pneumonia: do community and academic physicians agree on diagnosis? Am J Respir Crit Care Med 2007, 175: Thomeer M et al: Idiopathic Pulmonary Fibrosis International Group Exploring N-Acetylcysteine I Annual (IFIGENIA) study group: Multidisciplinary interobserver agreement in the diagnosis of idiopathic pulmonary fibrosis. Eur Respir J 2008, 31: Spagnolo et al : Idiopathic pulmonary fibrosis: diagnostic pitfalls and therapeutic challenges. Multidisciplinary Respiratory Medicine 2012, 7:42 4. Wells A. The revised ATS/ERS/JRS/ALAT diagnostic criteria for idiopathic pulmonary fibrosis (IPF) practical implications. Respiratory Research 2013; 14 (Suppl 1): S2. 5. Athol U. Wells, Ulrich Costabel et al: Challenges in IPF diagnosis, current management and future perspectives. Sarcoidosis,vasculitis and diffuse lung diseases 2015; 32; Suppl. 1: 28-35
53 IPF - definition A fibrosing interstitial pneumonia unknown cause older adults - primarily occurring limited to the lungs Associated with the histopathologic and/or radiologic pattern of UIP- usual interstitial pneumonia 1. King TE, Pardo A, Selman M: Idiopathic pulmonary fibrosis. Lancet 2011, 378: Ganesh Raghu et al :An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med Vol 183. pp , Ganesh Raghu et al :An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis An Update of the 2011 Clinical Practice Guideline, Am J Respir Crit Care Med Vol 192, Iss 2, pp e3 e19, Jul 15,
54 IPF -Diagnostic challenges Delay in diagnosis Alternative diagnosis IPF and other forms of ILD? Special conditions Acute exacerbation of IPF Unclassifiable ILD Obtaining a surgical lung biopsy
55 Irina Strambu Voicu Tudorache Claudia Toma
56 r o l oli B a iu Z a int C r e f on re a R
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59 The Network of Alpha 1 antitrypsin Deficiency in Central and Eastern Europe
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