Table 2

Diagnostic accuracy of clinical, laboratory and imaging findings for the identification of EGPA-related cardiomyopathy

FindingsEGPA-CM (n = 70)No CM (n = 106)Sensitivity (95% CI), %Specificity (95% CI), %PPV (95% CI), %NPV (95% CI), %Diagnostic OR (95% CI)P
Extra-cardiac features
 Peripheral neuropathy33/70 (47)69/106 (65)47.2 (35.9, 58.7)34.9 (26.5, 44.4)32.3 (24.1, 41.9)50.0 (38.9, 61.1)0.48 (0.26, 0.90)0.02
 Eosinophils >3000/mm349/63 (78)57/88 (65)77.8 (66.1, 86.3)35.2 (26.1, 45.6)46.2 (37.0, 55.7)68.9 (54.3, 80.5)1.90 (0.91, 3.85)0.10
 Eosinophils >5000/mm342/63 (67)37/88 (42)66.7 (54.4, 77.1)58.0 (47.5, 67.7)53.2 (42.2, 63.8)70.8 (59.5, 80.1)2.76 (1.39, 5.46)0.003
 Positive ANCA16/63 (25)45/92 (49)25.4 (16.3, 37.3)51.1 (41.0, 61.1)26.2 (16.8, 38.4)50.0 (40.0, 59.9)0.36 (0.18, 0.73)0.004
 Positive MPO-ANCA12/63 (19)35/92 (38)19.1 (11.3, 30.4)62.0 (51.8, 71.2)25.5 (15.3, 39.5)52.8 (43.4, 61.9)0.38 (0.18, 0.81)0.01
ECG6896
 Abnormal ECG49 (72)31 (32)72.1 (60.4, 81.3)67.7 (57.8, 76.2)61.3 (50.3, 71.2)77.4 (67.4, 85.0)5.41 (2.73, 10.24)<0.0001
 Repolarization abnormalities25 (37)12 (13)36.8 (26.3, 48.6)87.5 (79.4, 92.7)67.6 (51.5, 80.4)66.1 (57.6, 73.8)4.07 (1.93, 9.05)0.0005
 Rhythm abnormalities19 (28)10 (10)27.9 (18.7, 39.6)89.6 (81.9, 94.2)65.5 (47.4, 80.1)63.7 (55.3, 71.3)3.34 (1.40, 7.69)0.006
 Conduction abnormalities13 (19)12 (13)19.1 (11.5, 30.0)87.5 (79.4, 92.7)52.0 (33.5, 70.0)60.4 (52.1, 68.2)1.66 (0.68, 3.72)0.28
 Q wave abnormalities5 (7)4 (4)7.4 (3.2, 16.1)95.8 (90.0, 98.4)55.6 (26.7, 81.1)59.4 (51.5, 66.8)1.83 (0.52, 6.12)0.49
Laboratory findings
 Increased troponin44/51 (86)6/52 (12)86.3 (74.3, 93.2)88.5 (77.0, 94.6)88.0 (76.2, 94.4)86.8 (75.2, 93.5)48.2 (14.87, 133)<0.0001
 Increased NT-pro-BNP38/43 (88)10/46 (22)88.4 (75.5, 94.9)78.3 (64.4, 87.7)79.2 (65.7, 94.7)87.8 (74.5, 94.7)27.4 (8.46, 77.0)<0.0001
Transthoracic echography6889
 Abnormal TTE49 (72)34 (38)72.1 (60.4, 81.3)61.8 (51.4, 71.2)59.0 (48.3, 69.0)74.3 (63.4, 82.9)4.17 (2.10, 7.98)<0.0001
 Pericardial effusion28 (41)12 (13)41.2 (30.3, 53.0)86.5 (77.9, 92.1)70.0 (54.6, 81.9)65.8 (56.8, 73.8)4.49 (2.02, 9.94)<0.0001
 Left ventricular dysfunction21 (31)2 (2)30.9 (21.2, 42.6)97.8 (92.2, 99.6)91.3 (73.2, 98.5)64.9 (56.5, 72.5)19.44 (4.65, 85.5)<0.0001
 Hypokinetic areas22 (32)2 (2)32.4 (22.4, 44.2)97.8 (92.2, 99.6)91.7 (74.2, 98.5)65.4 (57.0, 73.0)20.80 (5.01, 91.3)<0.0001
Cardiac MRI6760
 Abnormal CMRI66 (99)36 (60)98.5 (92.0, 99.9)40.0 (28.6, 52.6)64.7 (55.1, 73.3)96.0 (80.5, 99.8)44.0 (7.24, 461)<0.0001
 Pericardial effusion27 (40)25 (42)40.3 (29.4, 52.3)58.3 (45.7, 69.9)51.9 (38.7, 64.9)46.7 (35.8, 57.8)0.95 (0.46, 1.95)>0.99
 Pericardial GE18 (27)13 (22)26.9 (17.7, 38.5)78.3 (66.4, 86.9)58.1 (40.8, 73.6)49.0 (39.2, 58.8)1.33 (0.61, 3.07)0.54
 Left ventricular dysfunction32 (48)6 (10)47.8 (36.3, 59.5)90.0 (79.9, 95.3)84.2 (69.6, 92.6)60.7 (50.3, 70.2)8.23 (3.26, 20.97)<0.0001
 Dilated left ventricle7 (10)3 (5)10.5 (5.2, 20.0)95.0 (86.3, 98.6)70.0 (39.7, 89.2)48.7 (39.9, 57.7)2.22 (0.57, 8.14)0.33
 Myocardial oedema15 (22)4 (7)22.4 (14.1, 33.7)93.3 (84.1, 97.4)79.0 (56.7, 91.5)51.9 (42.5, 61.1)4.04 (1.28, 11.66)0.02
 EGE24 (36)8 (13)35.8 (25.4, 47.8)86.7 (75.8, 93.1)75.0 (57.9, 86.9)54.7 (44.7, 64.4)3.63 (1.43, 8.60)0.004
 LGE47 (70)13 (22)70.2 (58.3, 79.8)78.3 (66.4, 86.9)78.3 (66.4, 86.9)70.2 (58.3, 79.8)8.50 (3.88, 18.41)<0.0001
FindingsEGPA-CM (n = 70)No CM (n = 106)Sensitivity (95% CI), %Specificity (95% CI), %PPV (95% CI), %NPV (95% CI), %Diagnostic OR (95% CI)P
Extra-cardiac features
 Peripheral neuropathy33/70 (47)69/106 (65)47.2 (35.9, 58.7)34.9 (26.5, 44.4)32.3 (24.1, 41.9)50.0 (38.9, 61.1)0.48 (0.26, 0.90)0.02
 Eosinophils >3000/mm349/63 (78)57/88 (65)77.8 (66.1, 86.3)35.2 (26.1, 45.6)46.2 (37.0, 55.7)68.9 (54.3, 80.5)1.90 (0.91, 3.85)0.10
 Eosinophils >5000/mm342/63 (67)37/88 (42)66.7 (54.4, 77.1)58.0 (47.5, 67.7)53.2 (42.2, 63.8)70.8 (59.5, 80.1)2.76 (1.39, 5.46)0.003
 Positive ANCA16/63 (25)45/92 (49)25.4 (16.3, 37.3)51.1 (41.0, 61.1)26.2 (16.8, 38.4)50.0 (40.0, 59.9)0.36 (0.18, 0.73)0.004
 Positive MPO-ANCA12/63 (19)35/92 (38)19.1 (11.3, 30.4)62.0 (51.8, 71.2)25.5 (15.3, 39.5)52.8 (43.4, 61.9)0.38 (0.18, 0.81)0.01
ECG6896
 Abnormal ECG49 (72)31 (32)72.1 (60.4, 81.3)67.7 (57.8, 76.2)61.3 (50.3, 71.2)77.4 (67.4, 85.0)5.41 (2.73, 10.24)<0.0001
 Repolarization abnormalities25 (37)12 (13)36.8 (26.3, 48.6)87.5 (79.4, 92.7)67.6 (51.5, 80.4)66.1 (57.6, 73.8)4.07 (1.93, 9.05)0.0005
 Rhythm abnormalities19 (28)10 (10)27.9 (18.7, 39.6)89.6 (81.9, 94.2)65.5 (47.4, 80.1)63.7 (55.3, 71.3)3.34 (1.40, 7.69)0.006
 Conduction abnormalities13 (19)12 (13)19.1 (11.5, 30.0)87.5 (79.4, 92.7)52.0 (33.5, 70.0)60.4 (52.1, 68.2)1.66 (0.68, 3.72)0.28
 Q wave abnormalities5 (7)4 (4)7.4 (3.2, 16.1)95.8 (90.0, 98.4)55.6 (26.7, 81.1)59.4 (51.5, 66.8)1.83 (0.52, 6.12)0.49
Laboratory findings
 Increased troponin44/51 (86)6/52 (12)86.3 (74.3, 93.2)88.5 (77.0, 94.6)88.0 (76.2, 94.4)86.8 (75.2, 93.5)48.2 (14.87, 133)<0.0001
 Increased NT-pro-BNP38/43 (88)10/46 (22)88.4 (75.5, 94.9)78.3 (64.4, 87.7)79.2 (65.7, 94.7)87.8 (74.5, 94.7)27.4 (8.46, 77.0)<0.0001
Transthoracic echography6889
 Abnormal TTE49 (72)34 (38)72.1 (60.4, 81.3)61.8 (51.4, 71.2)59.0 (48.3, 69.0)74.3 (63.4, 82.9)4.17 (2.10, 7.98)<0.0001
 Pericardial effusion28 (41)12 (13)41.2 (30.3, 53.0)86.5 (77.9, 92.1)70.0 (54.6, 81.9)65.8 (56.8, 73.8)4.49 (2.02, 9.94)<0.0001
 Left ventricular dysfunction21 (31)2 (2)30.9 (21.2, 42.6)97.8 (92.2, 99.6)91.3 (73.2, 98.5)64.9 (56.5, 72.5)19.44 (4.65, 85.5)<0.0001
 Hypokinetic areas22 (32)2 (2)32.4 (22.4, 44.2)97.8 (92.2, 99.6)91.7 (74.2, 98.5)65.4 (57.0, 73.0)20.80 (5.01, 91.3)<0.0001
Cardiac MRI6760
 Abnormal CMRI66 (99)36 (60)98.5 (92.0, 99.9)40.0 (28.6, 52.6)64.7 (55.1, 73.3)96.0 (80.5, 99.8)44.0 (7.24, 461)<0.0001
 Pericardial effusion27 (40)25 (42)40.3 (29.4, 52.3)58.3 (45.7, 69.9)51.9 (38.7, 64.9)46.7 (35.8, 57.8)0.95 (0.46, 1.95)>0.99
 Pericardial GE18 (27)13 (22)26.9 (17.7, 38.5)78.3 (66.4, 86.9)58.1 (40.8, 73.6)49.0 (39.2, 58.8)1.33 (0.61, 3.07)0.54
 Left ventricular dysfunction32 (48)6 (10)47.8 (36.3, 59.5)90.0 (79.9, 95.3)84.2 (69.6, 92.6)60.7 (50.3, 70.2)8.23 (3.26, 20.97)<0.0001
 Dilated left ventricle7 (10)3 (5)10.5 (5.2, 20.0)95.0 (86.3, 98.6)70.0 (39.7, 89.2)48.7 (39.9, 57.7)2.22 (0.57, 8.14)0.33
 Myocardial oedema15 (22)4 (7)22.4 (14.1, 33.7)93.3 (84.1, 97.4)79.0 (56.7, 91.5)51.9 (42.5, 61.1)4.04 (1.28, 11.66)0.02
 EGE24 (36)8 (13)35.8 (25.4, 47.8)86.7 (75.8, 93.1)75.0 (57.9, 86.9)54.7 (44.7, 64.4)3.63 (1.43, 8.60)0.004
 LGE47 (70)13 (22)70.2 (58.3, 79.8)78.3 (66.4, 86.9)78.3 (66.4, 86.9)70.2 (58.3, 79.8)8.50 (3.88, 18.41)<0.0001

CM: cardiomyopathy; CMRI: cardiac MRI; EGE: early gadolinium enhancement; EGPA: eosinophilic granulomatosis with polyangiitis; LGE: late gadolinium enhancement; NPV: negative predictive value; NT-pro-BNP: N-terminal (NT)-pro hormone brain natriuretic peptide; OR: odds ratio; Pericardial GE: pericardial gadolinium enhancement; PPV: positive predictive value; TTE: transthoracic echocardiogram.

Table 2

Diagnostic accuracy of clinical, laboratory and imaging findings for the identification of EGPA-related cardiomyopathy

FindingsEGPA-CM (n = 70)No CM (n = 106)Sensitivity (95% CI), %Specificity (95% CI), %PPV (95% CI), %NPV (95% CI), %Diagnostic OR (95% CI)P
Extra-cardiac features
 Peripheral neuropathy33/70 (47)69/106 (65)47.2 (35.9, 58.7)34.9 (26.5, 44.4)32.3 (24.1, 41.9)50.0 (38.9, 61.1)0.48 (0.26, 0.90)0.02
 Eosinophils >3000/mm349/63 (78)57/88 (65)77.8 (66.1, 86.3)35.2 (26.1, 45.6)46.2 (37.0, 55.7)68.9 (54.3, 80.5)1.90 (0.91, 3.85)0.10
 Eosinophils >5000/mm342/63 (67)37/88 (42)66.7 (54.4, 77.1)58.0 (47.5, 67.7)53.2 (42.2, 63.8)70.8 (59.5, 80.1)2.76 (1.39, 5.46)0.003
 Positive ANCA16/63 (25)45/92 (49)25.4 (16.3, 37.3)51.1 (41.0, 61.1)26.2 (16.8, 38.4)50.0 (40.0, 59.9)0.36 (0.18, 0.73)0.004
 Positive MPO-ANCA12/63 (19)35/92 (38)19.1 (11.3, 30.4)62.0 (51.8, 71.2)25.5 (15.3, 39.5)52.8 (43.4, 61.9)0.38 (0.18, 0.81)0.01
ECG6896
 Abnormal ECG49 (72)31 (32)72.1 (60.4, 81.3)67.7 (57.8, 76.2)61.3 (50.3, 71.2)77.4 (67.4, 85.0)5.41 (2.73, 10.24)<0.0001
 Repolarization abnormalities25 (37)12 (13)36.8 (26.3, 48.6)87.5 (79.4, 92.7)67.6 (51.5, 80.4)66.1 (57.6, 73.8)4.07 (1.93, 9.05)0.0005
 Rhythm abnormalities19 (28)10 (10)27.9 (18.7, 39.6)89.6 (81.9, 94.2)65.5 (47.4, 80.1)63.7 (55.3, 71.3)3.34 (1.40, 7.69)0.006
 Conduction abnormalities13 (19)12 (13)19.1 (11.5, 30.0)87.5 (79.4, 92.7)52.0 (33.5, 70.0)60.4 (52.1, 68.2)1.66 (0.68, 3.72)0.28
 Q wave abnormalities5 (7)4 (4)7.4 (3.2, 16.1)95.8 (90.0, 98.4)55.6 (26.7, 81.1)59.4 (51.5, 66.8)1.83 (0.52, 6.12)0.49
Laboratory findings
 Increased troponin44/51 (86)6/52 (12)86.3 (74.3, 93.2)88.5 (77.0, 94.6)88.0 (76.2, 94.4)86.8 (75.2, 93.5)48.2 (14.87, 133)<0.0001
 Increased NT-pro-BNP38/43 (88)10/46 (22)88.4 (75.5, 94.9)78.3 (64.4, 87.7)79.2 (65.7, 94.7)87.8 (74.5, 94.7)27.4 (8.46, 77.0)<0.0001
Transthoracic echography6889
 Abnormal TTE49 (72)34 (38)72.1 (60.4, 81.3)61.8 (51.4, 71.2)59.0 (48.3, 69.0)74.3 (63.4, 82.9)4.17 (2.10, 7.98)<0.0001
 Pericardial effusion28 (41)12 (13)41.2 (30.3, 53.0)86.5 (77.9, 92.1)70.0 (54.6, 81.9)65.8 (56.8, 73.8)4.49 (2.02, 9.94)<0.0001
 Left ventricular dysfunction21 (31)2 (2)30.9 (21.2, 42.6)97.8 (92.2, 99.6)91.3 (73.2, 98.5)64.9 (56.5, 72.5)19.44 (4.65, 85.5)<0.0001
 Hypokinetic areas22 (32)2 (2)32.4 (22.4, 44.2)97.8 (92.2, 99.6)91.7 (74.2, 98.5)65.4 (57.0, 73.0)20.80 (5.01, 91.3)<0.0001
Cardiac MRI6760
 Abnormal CMRI66 (99)36 (60)98.5 (92.0, 99.9)40.0 (28.6, 52.6)64.7 (55.1, 73.3)96.0 (80.5, 99.8)44.0 (7.24, 461)<0.0001
 Pericardial effusion27 (40)25 (42)40.3 (29.4, 52.3)58.3 (45.7, 69.9)51.9 (38.7, 64.9)46.7 (35.8, 57.8)0.95 (0.46, 1.95)>0.99
 Pericardial GE18 (27)13 (22)26.9 (17.7, 38.5)78.3 (66.4, 86.9)58.1 (40.8, 73.6)49.0 (39.2, 58.8)1.33 (0.61, 3.07)0.54
 Left ventricular dysfunction32 (48)6 (10)47.8 (36.3, 59.5)90.0 (79.9, 95.3)84.2 (69.6, 92.6)60.7 (50.3, 70.2)8.23 (3.26, 20.97)<0.0001
 Dilated left ventricle7 (10)3 (5)10.5 (5.2, 20.0)95.0 (86.3, 98.6)70.0 (39.7, 89.2)48.7 (39.9, 57.7)2.22 (0.57, 8.14)0.33
 Myocardial oedema15 (22)4 (7)22.4 (14.1, 33.7)93.3 (84.1, 97.4)79.0 (56.7, 91.5)51.9 (42.5, 61.1)4.04 (1.28, 11.66)0.02
 EGE24 (36)8 (13)35.8 (25.4, 47.8)86.7 (75.8, 93.1)75.0 (57.9, 86.9)54.7 (44.7, 64.4)3.63 (1.43, 8.60)0.004
 LGE47 (70)13 (22)70.2 (58.3, 79.8)78.3 (66.4, 86.9)78.3 (66.4, 86.9)70.2 (58.3, 79.8)8.50 (3.88, 18.41)<0.0001
FindingsEGPA-CM (n = 70)No CM (n = 106)Sensitivity (95% CI), %Specificity (95% CI), %PPV (95% CI), %NPV (95% CI), %Diagnostic OR (95% CI)P
Extra-cardiac features
 Peripheral neuropathy33/70 (47)69/106 (65)47.2 (35.9, 58.7)34.9 (26.5, 44.4)32.3 (24.1, 41.9)50.0 (38.9, 61.1)0.48 (0.26, 0.90)0.02
 Eosinophils >3000/mm349/63 (78)57/88 (65)77.8 (66.1, 86.3)35.2 (26.1, 45.6)46.2 (37.0, 55.7)68.9 (54.3, 80.5)1.90 (0.91, 3.85)0.10
 Eosinophils >5000/mm342/63 (67)37/88 (42)66.7 (54.4, 77.1)58.0 (47.5, 67.7)53.2 (42.2, 63.8)70.8 (59.5, 80.1)2.76 (1.39, 5.46)0.003
 Positive ANCA16/63 (25)45/92 (49)25.4 (16.3, 37.3)51.1 (41.0, 61.1)26.2 (16.8, 38.4)50.0 (40.0, 59.9)0.36 (0.18, 0.73)0.004
 Positive MPO-ANCA12/63 (19)35/92 (38)19.1 (11.3, 30.4)62.0 (51.8, 71.2)25.5 (15.3, 39.5)52.8 (43.4, 61.9)0.38 (0.18, 0.81)0.01
ECG6896
 Abnormal ECG49 (72)31 (32)72.1 (60.4, 81.3)67.7 (57.8, 76.2)61.3 (50.3, 71.2)77.4 (67.4, 85.0)5.41 (2.73, 10.24)<0.0001
 Repolarization abnormalities25 (37)12 (13)36.8 (26.3, 48.6)87.5 (79.4, 92.7)67.6 (51.5, 80.4)66.1 (57.6, 73.8)4.07 (1.93, 9.05)0.0005
 Rhythm abnormalities19 (28)10 (10)27.9 (18.7, 39.6)89.6 (81.9, 94.2)65.5 (47.4, 80.1)63.7 (55.3, 71.3)3.34 (1.40, 7.69)0.006
 Conduction abnormalities13 (19)12 (13)19.1 (11.5, 30.0)87.5 (79.4, 92.7)52.0 (33.5, 70.0)60.4 (52.1, 68.2)1.66 (0.68, 3.72)0.28
 Q wave abnormalities5 (7)4 (4)7.4 (3.2, 16.1)95.8 (90.0, 98.4)55.6 (26.7, 81.1)59.4 (51.5, 66.8)1.83 (0.52, 6.12)0.49
Laboratory findings
 Increased troponin44/51 (86)6/52 (12)86.3 (74.3, 93.2)88.5 (77.0, 94.6)88.0 (76.2, 94.4)86.8 (75.2, 93.5)48.2 (14.87, 133)<0.0001
 Increased NT-pro-BNP38/43 (88)10/46 (22)88.4 (75.5, 94.9)78.3 (64.4, 87.7)79.2 (65.7, 94.7)87.8 (74.5, 94.7)27.4 (8.46, 77.0)<0.0001
Transthoracic echography6889
 Abnormal TTE49 (72)34 (38)72.1 (60.4, 81.3)61.8 (51.4, 71.2)59.0 (48.3, 69.0)74.3 (63.4, 82.9)4.17 (2.10, 7.98)<0.0001
 Pericardial effusion28 (41)12 (13)41.2 (30.3, 53.0)86.5 (77.9, 92.1)70.0 (54.6, 81.9)65.8 (56.8, 73.8)4.49 (2.02, 9.94)<0.0001
 Left ventricular dysfunction21 (31)2 (2)30.9 (21.2, 42.6)97.8 (92.2, 99.6)91.3 (73.2, 98.5)64.9 (56.5, 72.5)19.44 (4.65, 85.5)<0.0001
 Hypokinetic areas22 (32)2 (2)32.4 (22.4, 44.2)97.8 (92.2, 99.6)91.7 (74.2, 98.5)65.4 (57.0, 73.0)20.80 (5.01, 91.3)<0.0001
Cardiac MRI6760
 Abnormal CMRI66 (99)36 (60)98.5 (92.0, 99.9)40.0 (28.6, 52.6)64.7 (55.1, 73.3)96.0 (80.5, 99.8)44.0 (7.24, 461)<0.0001
 Pericardial effusion27 (40)25 (42)40.3 (29.4, 52.3)58.3 (45.7, 69.9)51.9 (38.7, 64.9)46.7 (35.8, 57.8)0.95 (0.46, 1.95)>0.99
 Pericardial GE18 (27)13 (22)26.9 (17.7, 38.5)78.3 (66.4, 86.9)58.1 (40.8, 73.6)49.0 (39.2, 58.8)1.33 (0.61, 3.07)0.54
 Left ventricular dysfunction32 (48)6 (10)47.8 (36.3, 59.5)90.0 (79.9, 95.3)84.2 (69.6, 92.6)60.7 (50.3, 70.2)8.23 (3.26, 20.97)<0.0001
 Dilated left ventricle7 (10)3 (5)10.5 (5.2, 20.0)95.0 (86.3, 98.6)70.0 (39.7, 89.2)48.7 (39.9, 57.7)2.22 (0.57, 8.14)0.33
 Myocardial oedema15 (22)4 (7)22.4 (14.1, 33.7)93.3 (84.1, 97.4)79.0 (56.7, 91.5)51.9 (42.5, 61.1)4.04 (1.28, 11.66)0.02
 EGE24 (36)8 (13)35.8 (25.4, 47.8)86.7 (75.8, 93.1)75.0 (57.9, 86.9)54.7 (44.7, 64.4)3.63 (1.43, 8.60)0.004
 LGE47 (70)13 (22)70.2 (58.3, 79.8)78.3 (66.4, 86.9)78.3 (66.4, 86.9)70.2 (58.3, 79.8)8.50 (3.88, 18.41)<0.0001

CM: cardiomyopathy; CMRI: cardiac MRI; EGE: early gadolinium enhancement; EGPA: eosinophilic granulomatosis with polyangiitis; LGE: late gadolinium enhancement; NPV: negative predictive value; NT-pro-BNP: N-terminal (NT)-pro hormone brain natriuretic peptide; OR: odds ratio; Pericardial GE: pericardial gadolinium enhancement; PPV: positive predictive value; TTE: transthoracic echocardiogram.

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