1. Komócsi A, Pintér T, Faludi R, Magyari B, Bozó J, Kumánovics G, Minier T, Radics J, Czirják L.: Overlap of coronary disease and pulmonary arterial hypertension in systemic sclerosis. Ann Rheum Dis. 2010 Jan;69(1):202-5. (IF 8.111, 2009) 2. Allanore Y, Meune C, Vonk MC, Airo P, Hachulla E, Caramaschi P, Riemekasten G,
Cozzi F, Beretta L, Derk CT, Komócsi A, Farge D, Balbir A, Riccieri V, Distler O, Chialà A, Del Papa N, Simic Pasalic K, Ghio M, Stamenkovic B, Rednic S, Host N,Pellerito R, Zegers E, Kahan A, Walker UA, Matucci-Cerinic M.: Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of systemic sclerosis patients. Ann Rheum Dis. 2010
Jan;69(1):218-21 (IF 8.111, 2009)
3. Faludi R, Komócsi A, Bozó J, Kumánovics G, Czirják L, Papp L, Simor T.: Isolated diastolic dysfunction of right ventricle: stress-induced pulmonary hypertension. Eur Respir J. 2008 Feb;31(2):475-6. (IF 5.545)
4. Pintér T, Faludi R, Magyari B, Vorobcsuk A, Kumánovics G, Minier T, Czirják L, Komócsi A: Mechanism of coronary flow reserve reduction in systemic sclerosis, insight from intracoronary pressure-wire studies.In Press Rheumatology
doi:10.1093/rheumatology/keq402 (IF 4.236, 2009)
5. Komócsi A, Vorobcsuk A, Faludi R, Pintér T, Lenkey Z, Magyari B, Költő G, Czirják L:
The impact of cardio-pulmonary manifestation on the outcome of systemic sclerosis:
a systematic review and meta-analysis. Submitted Semin Art Rheum
6. Czirják L, Komócsi A. Tóvári E.: Provokációs tényezök szerepe a szisztémás szklerozis patogenesisében. Allergológia és Klinikai Immunológia. 2000; 3: 1-6.
7. Kumánovics G., Zibotics H., Komócsi A., Czirják L.: Pulmonális fibrózis
pathomechanizmusa systemás autoimmun kórképekben. Allergológia és Klinikai Immunológia 2001;4 (3): 67-73.
8. Kumánovics G., Zibotics H., Komócsi A., Czirják L.: Pulmonális fibrózis, alveolitis systemás autoimmun kórképekben. Allergológia és Klinikai Immunológia 2001;4 (4):
98-106.
9. Komócsi A, Faludi R.: Pulmonális artériás hypertensio. Focus Medicinae 9.évf. 3 sz.
15-18. 2007
10. Faludi R, Komócsi A: Pulmonalis hypertonia:gyanú, diagnózis, kezelés.
Tüdőgyógyászat 2008 2.évf 8.sz 27-34.
25
7 Köszönetnyílvánítás
Szeretnék köszönetet mondani szüleimnek és családomnak a jó példákért és a támogatásért, valamint mindenki másnak aki munkámban segített, ahhoz hozzátett és végzése során támogatott.
A teljesség igénye nélkül szeretném köszönetemet kifejezni mestereimnek, tanáraimnak, munkatársaimnak, tanítványaimnak, segítőimnek:
Prof. Dr. Czirják László, Dr. Aradi Dániel, Dr. Faludi Réka, Dr. Kumánovics Gábor, Dr. Zibotics Hilda, Dr. Juhász Erzsébet, Dr. Dévényi Katalin, Prof. Dr. Pajor László, Dr. Peter Lamprecht, Dr. Antje Müller, Dr. Elena Csernok, Prof. Dr. Wolfgang Ludvig Gross, Dr. Armin Schnabel, Dr.
Frank Moosig, Dr Magyarlaki Tamás, Dr. Szekeres György, Dr. Pintér Tünde, Dr. Horváth Iván, Dr. Kónyi Attila, Dr. Papp Előd, Dr. Czopf László, Dr. Habon Tamás, Dr. Halmosi Róbert, Dr.
Horváth Beáta, Dr. Márton Zsolt, Dr. Tahin Tamás, Prof. Dr. Melegh Béla, Prof. Dr. Tóth Kálmán, Prof. Dr. Simor Tamás, Dr. Tóth Levente, Dr. Szabados Sándor, Dr. Mágel Ferenc, Prof. Dr. Papp Lajos, Prof. Dr. Kovács L. Gábor, Dr. Pálinkás László, Prof. Dr. Berki Tímea, Dr.
Varga-Szemes Ákos, Dr. Bozó János, Dr. Tőkés-Füzesi Margit, Dr. Minier Tünde, Dr. Radics Judit, Prof. dr. Yannick Allanore, Dr. Vorobcsuk András, Dr. Ungi Imre, Prof. Dr. Yves Louvard, Dr. Lenkey Zsófia, Lóth Erika, Királyvári Erika, Ferk Imola, Dr. Melczer László, Dr Brolly Mária, Dr. Mühl Diana, Dr. Farkasfalvi Klára, Prof. Dr. Tekeres Miklós, Dr. Rideg Orsolya, Puskás Tünde, Dr. Tóvári Eszter, Pázmándy Ágnes, Ili Renáta, Dr. Hejjel László, Dr. Lénárd László, Illés Rita, Dr. Imre Jenő, Prof. Dr. Christian Spaulding, Dr. Habon László, Dr. Jáksó Krisztián, Dr.
Békési Bernadett, Dr. Csizmadia Péter, Dr. Herczeg Krisztina, Dr. Sayour Amer, Dr. Muraközy Henrietta, Dr. Nagy Zoltán, Dr. Nyárfás Géza, Dr. Kovács Erik, Dr. Magyari Balázs, Zentai Jánosné, Farkas Ibolya, Prof. Dr. Nagy Judit, Linda Carstens, dr. Tóth Tivadar, dr. Sárosi Veronika, dr. Vereb Éva
26
8 Irodalom
Reference List
1 Steen VD, Medsger TA, Jr. Changes in causes of death in systemic sclerosis. Ann Rheum Dis 2007; 47(Suppl. 5.):16-7.
2 Czirjak L, Kumanovics G, Varju C, Nagy Z, Pakozdi A, Szekanecz Z et al. Survival and causes of death in 366 Hungarian patients with systemic sclerosis. Ann Rheum Dis 2008; 67(1):59-63.
3 Ioannidis JP, Vlachoyiannopoulos PG, Haidich AB, Medsger TA, Jr., Lucas M, Michet CJ et al. Mortality in systemic sclerosis: an international meta-analysis of individual patient data. Am J Med 2005; 118(1):2-10.
4 Arias-Nunez MC, Llorca J, Vazquez-Rodriguez TR, Gomez-Acebo I,
Miranda-Filloy JA, Martin J et al. Systemic sclerosis in northwestern Spain: a 19-year epidemiologic study. Medicine (Baltimore) 2008;
87(5):272-80.
5 Clements PJ, Lachenbruch PA, Furst DE, Paulus HE, Sterz MG. Cardiac score. A semiquantitative measure of cardiac involvement that
improves prediction of prognosis in systemic sclerosis. Arthritis Rheum 1991; 34(11):1371-80.
6 Medsger TA, Jr., Masi AT, Rodnan GP, Benedek TG, Robinson H. Survival with systemic sclerosis (scleroderma). A life-table analysis of
clinical and demographic factors in 309 patients. Ann Intern Med 1971; 75(3):369-76.
7 Ferri C, Valentini G, Cozzi F, Sebastiani M, Michelassi C, La Montagna G et al. Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients. Medicine (Baltimore) 2002; 81(2):139-53.
8 Czirjak L, Pakozdi A, Kumanovics G, Varju C, Szekanecz Z, Nagy Z et al. Survival and causes of death in 366 Hungarian patients with systemic sclerosis. Ann Rheum Dis 2007.
9 Maione S, Cuomo G, Giunta A, Tanturri dH, La Montagna G, Manguso F et al. Echocardiographic alterations in systemic sclerosis: a
longitudinal study. Semin Arthritis Rheum 2005; 34(5):721-7.
10 Hegedus I, Czirjak L. Left ventricular wall motion abnormalities in 80 patients with systemic sclerosis. Clin Rheumatol 1995; 14(2):161-4.
11 Valentini G, Vitale DF, Giunta A, Maione S, Gerundo G, Arnese M et al. Diastolic abnormalities in systemic sclerosis: evidence for associated defective cardiac functional reserve. Ann Rheum Dis 1996;
55(7):455-60.
12 Ferri C, Giuggioli D, Sebastiani M, Colaci M, Emdin M. Heart involvement and systemic sclerosis. Lupus 2005; 14(9):702-7.
13 Tarek e, Yasser AE, Gheita T. Coronary angiographic findings in asymptomatic systemic sclerosis. Clin Rheumatol 2006; 25(4):487-90.
27
Rheumatology (Oxford) 2006; 45(11):1395-8.
15 Kahan A, Nitenberg A, Foult JM, Amor B, Menkes CJ, Devaux JY et al.
Decreased coronary reserve in primary scleroderma myocardial disease.
Arthritis Rheum 1985; 28(6):637-46.
16 D'Andrea A, Caso P, Cuomo S, Scotto dU, Scarafile R, Salerno G et al.
Myocardial and vascular dysfunction in systemic sclerosis: the potential role of noninvasive assessment in asymptomatic patients.
Int J Cardiol 2007; 121(3):298-301.
17 McGoon M, Gutterman D, Steen V, Barst R, McCrory DC, Fortin TA et al.
Screening, early detection, and diagnosis of pulmonary arterial
hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004; 126(1 Suppl):14S-34S.
18 Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med 1987; 107(2):216-23.
19 Lindqvist P, Caidahl K, Neuman-Andersen G, Ozolins C, Rantapaa-Dahlqvist S, Waldenstrom A et al. Disturbed right ventricular diastolic function in patients with systemic sclerosis: a Doppler tissue imaging study. Chest 2005; 128(2):755-63.
20 Huez S, Roufosse F, Vachiery JL, Pavelescu A, Derumeaux G, Wautrecht JC et al. Isolated right ventricular dysfunction in systemic
sclerosis: latent pulmonary hypertension? Eur Respir J 2007;
30(5):928-36.
21 Allanore Y, Meune C, Kahan A. Systemic sclerosis and cardiac dysfunction: evolving concepts and diagnostic methodologies. Curr Opin Rheumatol 2008; 20(6):697-702.
22 Montisci R, Vacca A, Garau P, Colonna P, Ruscazio M, Passiu G et al.
Detection of early impairment of coronary flow reserve in patients with systemic sclerosis. Ann Rheum Dis 2003; 62(9):890-3.
23 Montisci R, Ruscazio M, Lai S, Vacca A, Cauli A, Passiu G et al.
Effect of a single IV administration of L-propionylcarnitine on myocardial microcirculation assessed by coronary flow velocity reserve measurement in patients with systemic sclerosis: a pilot study. Clin Ther 2007; 29(1):163-71.
24 Kahan A, Devaux JY, Amor B, Menkes CJ, Weber S, Foult JM et al.
Pharmacodynamic effect of dipyridamole on thallium-201 myocardial perfusion in progressive systemic sclerosis with diffuse scleroderma.
Ann Rheum Dis 1986; 45(9):718-25.
25 Nakajima K, Taki J, Kawano M, Higuchi T, Sato S, Nishijima C et al.
Diastolic dysfunction in patients with systemic sclerosis detected by gated myocardial perfusion SPECT: an early sign of cardiac
involvement. J Nucl Med 2001; 42(2):183-8.
26 Meune C, Vignaux O, Kahan A, Allanore Y. Heart involvement in systemic sclerosis: evolving concept and diagnostic methodologies.
Arch Cardiovasc Dis 2010; 103(1):46-52.
28
27 Tzelepis GE, Kelekis NL, Plastiras SC, Mitseas P, Economopoulos N, Kampolis C et al. Pattern and distribution of myocardial fibrosis in systemic sclerosis: a delayed enhanced magnetic resonance imaging study. Arthritis Rheum 2007; 56(11):3827-36.
28 Hachulla AL, Launay D, Gaxotte V, de GP, Lamblin N, Devos P et al.
Cardiac magnetic resonance imaging in systemic sclerosis: a cross-sectional observational study of 52 patients. Ann Rheum Dis 2009;
68(12):1878-84.
29 Komocsi A, Pinter T, Faludi R, Magyari B, Bozo J, Kumanovics G et al.
Overlap of coronary disease and pulmonary arterial hypertension in systemic sclerosis. Ann Rheum Dis 2010; 69(1):202-5.
30 Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am 2003;
29(2):239-54.
31 Walker UA, Tyndall A, Czirjak L, Denton CP, Farge BD, Kowal-Bielecka O et al. Clinical risk assessment of organ manifestations in systemic sclerosis - a report from the EULAR Scleroderma Trials And Research (EUSTAR) group data base. Ann Rheum Dis 2007.
32 Stroup DF, Berlin JA, Morton SC, Olkin I, Williamson GD, Rennie D et al. Meta-analysis of observational studies in epidemiology: a
proposal for reporting. Meta-analysis Of Observational Studies in Epidemiology (MOOSE) group. JAMA 2000; 283(15):2008-12.
33 Jadad AR, Moore RA, Carroll D, Jenkinson C, Reynolds DJ, Gavaghan DJ et al. Assessing the quality of reports of randomized clinical
trials: is blinding necessary? Control Clin Trials 1996; 17(1):1-12.
34 LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, Jr. et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988; 15(2):202-5.
35 Allanore Y, Meune C, Vonk MC, Airo P, Hachulla E, Caramaschi P et al.
Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of patients with systemic sclerosis. Ann Rheum Dis 2010; 69(1):218-21.
36 Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE et al.
Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 2010; 69(10):1809-15.
37 Czirjak L, Pakozdi A, Kumanovics G, Varju C, Szekanecz Z, Nagy Z et al. Survival and causes of death in 366 Hungarian patients with systemic sclerosis. Ann Rheum Dis 2008; 67(1):59-63.
38 Abbott KC, Trespalacios FC, Welch PG, Agodoa LY. Scleroderma at end stage renal disease in the United States: patient characteristics and survival. J Nephrol 2002; 15(3):236-40.
39 Al-Dhaher FF, Pope JE, Ouimet JM. Determinants of morbidity and mortality of systemic sclerosis in Canada. Semin Arthritis Rheum 2010; 39(4):269-77.
29
34(4):403-13.
41 Allanore Y, Avouac J, Kahan A. Systemic sclerosis: an update in 2008.
Joint Bone Spine 2008; 75(6):650-5.
42 DeMarco PJ, Weisman MH, Seibold JR, Furst DE, Wong WK, Hurwitz EL et al. Predictors and outcomes of scleroderma renal crisis: the high-dose versus low-high-dose D-penicillamine in early diffuse systemic sclerosis trial. Arthritis Rheum 2002; 46(11):2983-9.
43 Trad S, Amoura Z, Beigelman C, Haroche J, Costedoat N, Boutin lT et al. Pulmonary arterial hypertension is a major mortality factor in diffuse systemic sclerosis, independent of interstitial lung disease.
Arthritis Rheum 2006; 54(1):184-91.
44 Mathai SC, Hummers LK, Champion HC, Wigley FM, Zaiman A, Hassoun PM et al. Survival in pulmonary hypertension associated with the
scleroderma spectrum of diseases: impact of interstitial lung disease. Arthritis Rheum 2009; 60(2):569-77.
45 Simeon CP, Armadans L, Fonollosa V, Solans R, Selva A, Villar M et al. Mortality and prognostic factors in Spanish patients with systemic sclerosis. Rheumatology (Oxford) 2003; 42(1):71-5.
46 Hachulla E, Gressin V, Guillevin L, Carpentier P, Diot E, Sibilia J et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study.
Arthritis Rheum 2005; 52(12):3792-800.
47 Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP et al. Updated clinical classification of pulmonary hypertension.
J Am Coll Cardiol 2009; 54(1 Suppl):S43-S54.
48 Meluzin J, Spinarova L, Bakala J, Toman J, Krejci J, Hude P et al.
Pulsed Doppler tissue imaging of the velocity of tricuspid annular systolic motion; a new, rapid, and non-invasive method of evaluating right ventricular systolic function. Eur Heart J 2001; 22(4):340-8.
49 Ruan Q, Nagueh SF. Clinical application of tissue Doppler imaging in patients with idiopathic pulmonary hypertension. Chest 2007;
131(2):395-401.
50 Hsiao SH, Lee CY, Chang SM, Lin SK, Liu CP. Right heart function in scleroderma: insights from myocardial Doppler tissue imaging. J Am Soc Echocardiogr 2006; 19(5):507-14.
51 de GP, Gressin V, Hachulla E, Carpentier P, Guillevin L, Kahan A et al. Evaluation of cardiac abnormalities by Doppler echocardiography in a large nationwide multicentric cohort of patients with systemic sclerosis. Ann Rheum Dis 2008;
67(1):31-6.
52 Kahan A, Allanore Y. Primary myocardial involvement in systemic sclerosis. Rheumatology (Oxford) 2006; 45 Suppl 4:iv14-iv17.
53 Steen V, Medsger TA, Jr. Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum 2003; 48(2):516-22.
30
54 Hachulla E, Clerson P, Launay D, Lambert M, Morell-Dubois S, Queyrel V et al. Natural history of ischemic digital ulcers in systemic sclerosis: single-center retrospective longitudinal study. J Rheumatol 2007; 34(12):2423-30.
55 Nitenberg A, Foult JM, Kahan A, Perennec J, Devaux JY, Menkes CJ et al. Reduced coronary flow and resistance reserve in primary
scleroderma myocardial disease. Am Heart J 1986; 112(2):309-15.
56 Sulli A, Ghio M, Bezante GP, Deferrari L, Craviotto C, Sebastiani V et al. Blunted coronary flow reserve in systemic sclerosis.
Rheumatology (Oxford) 2004; 43(4):505-9.
57 Stoker ME, Gerdes AM, May JF. Regional differences in capillary density and myocyte size in the normal human heart. Anat Rec 1982;
202(2):187-91.
58 Ng MK, Yeung AC, Fearon WF. Invasive assessment of the coronary microcirculation: superior reproducibility and less hemodynamic dependence of index of microcirculatory resistance compared with coronary flow reserve. Circulation 2006; 113(17):2054-61.
59 Tonino PA, Fearon WF, De BB, Oldroyd KG, Leesar MA, Ver Lee PN et al.
Angiographic versus functional severity of coronary artery stenoses in the FAME study fractional flow reserve versus angiography in multivessel evaluation. J Am Coll Cardiol 2010; 55(25):2816-21.
60 Fearon WF, Aarnoudse W, Pijls NH, De Bruyne B, Balsam LB, Cooke DT et al. Microvascular resistance is not influenced by epicardial coronary artery stenosis severity: experimental validation. Circulation 2004;
109(19):2269-72.
61 Aarnoudse W, Fearon WF, Manoharan G, Geven M, Van D, V, Rutten M et al. Epicardial stenosis severity does not affect minimal
microcirculatory resistance. Circulation 2004; 110(15):2137-42.
62 Caiati C, Zedda N, Cadeddu M, Chen L, Montaldo C, Iliceto S et al.
Detection, location, and severity assessment of left anterior descending coronary artery stenoses by means of contrast-enhanced transthoracic harmonic echo Doppler. Eur Heart J 2009; 30(14):1797-806.
63 Caiati C, Montaldo C, Zedda N, Montisci R, Ruscazio M, Lai G et al.
Validation of a new noninvasive method (contrast-enhanced
transthoracic second harmonic echo Doppler) for the evaluation of coronary flow reserve: comparison with intracoronary Doppler flow wire. J Am Coll Cardiol 1999; 34(4):1193-200.
64 Caiati C, Cioglia G, Montaldo C, Zedda N, Rubini G, Pirisi R et al.
Correlation of 99mTc-sestamibi SPECT myocardial perfusion imaging with absolute coronary flow reserve by a new noninvasive Doppler method in patients with stenoses of the left anterior descending coronary artery. A preliminary report. Cardiologia 1999; 44(9):809-16.
65 Caiati C, Montaldo C, Zedda N, Bina A, Iliceto S. New noninvasive method for coronary flow reserve assessment: contrast-enhanced transthoracic second harmonic echo Doppler. Circulation 1999;
99(6):771-8.
31
sclerosis: indications of the development of nitrate tolerance as a result of enhanced endothelial nitric oxide production. Arthritis Rheum 2002; 46(5):1324-32.
67 Soltesz P, Der H, Kerekes G, Szodoray P, Szucs G, Danko K et al. A comparative study of arterial stiffness, flow-mediated vasodilation of the brachial artery, and the thickness of the carotid artery intima-media in patients with systemic autoimmune diseases. Clin Rheumatol 2009; 28(6):655-62.
68 Szekanecz Z, Koch AE. Vascular involvement in rheumatic diseases:
'vascular rheumatology'. Arthritis Res Ther 2008; 10(5):224.
69 Szucs G, Timar O, Szekanecz Z, Der H, Kerekes G, Szamosi S et al.
Endothelial dysfunction precedes atherosclerosis in systemic sclerosis--relevance for prevention of vascular complications.
Rheumatology (Oxford) 2007; 46(5):759-62.
70 Allanore Y, Meune C, Vignaux O, Weber S, Legmann P, Kahan A. Bosentan increases myocardial perfusion and function in systemic sclerosis: a magnetic resonance imaging and Tissue-Doppler echography study. J Rheumatol 2006; 33(12):2464-9.
71 Mukerjee D, Yap LB, Ong V, Denton CP, Howells K, Black CM et al. The myth of pulmonary Raynaud's phenomenon: the contribution of pulmonary arterial vasospasm in patients with systemic sclerosis related
pulmonary arterial hypertension. Ann Rheum Dis 2004; 63(12):1627-31.