Abstract
We investigated the all-inclusive echocardiographic features in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) within 3 months before and after diagnosis. We reviewed the medical records of 89 AAV patients taking echocardiography and 35 age- and gender-matched controls. We collected clinical and laboratory data and echocardiographic results. We compared the variables between patients with AAV of each variant and controls and among those with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). The mean age and period from diagnosis to echocardiography were 54.5 years (30 men) and 1.4 months. The mean age of controls was 51.6 years. AAV patients exhibited lower mean left ventricle ejection fraction (LVEF) (64.0 vs. 69.1%, P = 0.002) and higher mean E/E′ ratio (11.7 vs. 8.8, P = 0.001) and right ventricle systolic pressure (RVSP) (30.2 vs. 23.2 mm Hg, P < 0.001) than the controls. Each variant of AAV presented a different pattern of echocardiographic features in comparison with controls. MPA patients exhibited systolic and diastolic dysfunctions and pulmonary arterial hypertension more often than controls. Meanwhile, GPA patients exhibited only diastolic dysfunction, and EGPA patients had systolic dysfunction and pulmonary arterial hypertension more frequently than controls. No meaningful differences in echocardiographic features appeared among AAV variants. AAV patients exhibited reduced systolic function and advanced diastolic dysfunctions and pulmonary arterial hypertension near the time of diagnosis compared with controls. Each variant of AAV presented a different pattern in echocardiographic features.
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Jennette JC, Falk RJ, Bacon PA et al (2013) 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 65(1):1–11
Mukhtyar C, Lee R, Brown D et al (2009) Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis 68(12):1827–1832
Stone JH, Hoffman GS, Merkel PA et al (2001) A disease-specific activity index for Wegener's granulomatosis: modification of the Birmingham Vasculitis Activity Score. International Network for the Study of the Systemic Vasculitides (INSSYS). Arthritis Rheum 44(4):912–920
Millet A, Pederzoli-Ribeil M, Guillevin L, Witko-Sarsat V, Mouthon L (2013) Antineutrophil cytoplasmic antibody-associated vasculitides: is it time to split up the group? Ann Rheum Dis 72(8):1273–1279
Koldingsnes W, Nossent JC (2003) Baseline features and initial treatment as predictors of remission and relapse in Wegener’s granulomatosis. J Rheumatol 30(1):80–88
Mukhtyar C, Hellmich B, Jayne D, Flossmann O, Luqmani R (2006) Remission in antineutrophil cytoplasmic antibody-associated systemic vasculitis. Clin Exp Rheumatol 24(6 Suppl 43):S-93–S-98
Gayraud M, Guillevin L, le Toumelin P, Cohen P, Lhote F, Casassus P, Jarrousse B (2001) Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum 44(3):666–675
Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P (2011) The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore) 90(1):19–27
Katsuyama T, Sada KE, Makino H (2014) Current concept and epidemiology of systemic vasculitides. Allergol Int 63(4):505–513
Lyons PA, Rayner TF, Trivedi S et al (2012) Genetically distinct subsets within ANCA-associated vasculitis. N Engl J Med 367(3):214–223
Schirmer JH, Wright MN, Herrmann K, Laudien M, Nolle B, Reinhold-Keller E, Bremer JP, Moosig F, Holle JU (2016) Myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA)-positive granulomatosis with polyangiitis (Wegener’s) is a clinically distinct subset of anca-associated vasculitis: a retrospective analysis of 315 patients from a German vasculitis referral center. Arthritis Rheumatol 68(12):2953–2963
Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, Mahr A, Segelmark M, Cohen-Tervaert JW, Scott D (2007) Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 66(2):222–227
Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, Calabrese LH, Fries JF, Lie JT, Lightfoot RW Jr et al (1990) The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 33(8):1101–1107
Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY et al (1990) The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 33(8):1094–1100
Cornec D, Cornec-Le Gall E, Fervenza FC, Specks U (2016) ANCA-associated vasculitis—clinical utility of using ANCA specificity to classify patients. Nat Rev Rheumatol 12(10):570–579
Yoo J, Kim HJ, Ahn SS, Jung SM, Song JJ, Park YB, Lee SW (2017) Clinical and prognostic features of Korean patients with MPO-ANCA, PR3-ANCA and ANCA-negative vasculitis. Clin Exp Rheumatol 35 Suppl 103(1):111–118
Quinones MA, Waggoner AD, Reduto LA, Nelson JG, Young JB, Winters WL Jr, Ribeiro LG, Miller RR (1981) A new, simplified and accurate method for determining ejection fraction with two-dimensional echocardiography. Circulation 64(4):744–753
Yock PG, Popp RL (1984) Noninvasive estimation of right ventricular systolic pressure by Doppler ultrasound in patients with tricuspid regurgitation. Circulation 70(4):657–662
Lee SW, Choi EY, Jung SY, Choi ST, Lee SK, Park YB (2010) E/E' ratio is more sensitive than E/A ratio for detection of left ventricular diastolic dysfunction in patients with systemic sclerosis. Clin Exp Rheumatol 28(2 Suppl 58):S12–S17
Hazebroek MR, Kemna MJ, Schalla S, Sanders-van Wijk S, Gerretsen SC, Dennert R, Merken J, Kuznetsova T, Staessen JA, Brunner-La Rocca HP, van Paassen P, Cohen Tervaert JW, Heymans S (2015) Prevalence and prognostic relevance of cardiac involvement in ANCA-associated vasculitis: eosinophilic granulomatosis with polyangiitis and granulomatosis with polyangiitis. Int J Cardiol 199:170–179
Neumann T, Manger B, Schmid M, Kroegel C, Hansch A, Kaiser WA, Reinhardt D, Wolf G, Hein G, Mall G, Schett G, Zwerina J (2009) Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis. Medicine (Baltimore) 88(4):236–243
Miszalski-Jamka T, Szczeklik W, Nycz K, Sokolowska B, Bury K, Zawadowski G, Noelting J, Mazur W, Musial J (2012) The mechanics of left ventricular dysfunction in patients with Churg-Strauss syndrome. Echocardiography 29(5):568–578
Yune S, Choi DC, Lee BJ, Lee JY, Jeon ES, Kim SM, Choe YH (2016) Detecting cardiac involvement with magnetic resonance in patients with active eosinophilic granulomatosis with polyangiitis. Int J Cardiovasc Imaging 32(Suppl 1):155–162
Li Y, Yi Q (2015) Pulmonary arterial hypertension associated with rare cause of ANCA-associated vasculitis misdiagnosed as idiopathic one. Int J Clin Exp Med 8(9):16850–16853
Launay D, Souza R, Guillevin L, Hachulla E, Pouchot J, Simonneau G, Humbert M (2006) Pulmonary arterial hypertension in ANCA-associated vasculitis. Sarcoidosis Vasc Diffuse Lung Dis 23(3):223–228
Lacoste C, Mansencal N, Ben M'rad M, Goulon-Goeau C, Cohen P, Guillevin L, Hanslik T (2011) Valvular involvement in ANCA-associated systemic vasculitis: a case report and literature review. BMC Musculoskelet Disord 12:50
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This study was supported by a faculty research grant from Yonsei University College of Medicine (6-2016-0159).
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This study was approved by the institutional review board of the Severance Hospital (4-2017-0673).
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Ahn, S.S., Park, E.S., Jung, S.M. et al. Echocardiographic features in patients with ANCA-associated vasculitis within 3 months before and after diagnosis. Clin Rheumatol 36, 2751–2759 (2017). https://doi.org/10.1007/s10067-017-3868-2
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DOI: https://doi.org/10.1007/s10067-017-3868-2