Background
Methods
Patient population
CMR protocol
CMR analysis
Evaluation of disease activity
Statistical analysis
Results
Patient characteristics
All patients n = 37 | EGPAa
n = 22 | GPAa
n = 15 | |
---|---|---|---|
Age (yrs) | 55 ± 16 | 54 ± 16 | 55 ± 16 |
Gender (male) | 13 (35%) | 5 (23%) | 8 (53%) |
Cardiovascular risk factors | |||
Diabetes | 7 (19%) | 5 (23%) | 2 (13%) |
Hypertension | 13 (35%) | 8 (36%) | 5 (33%) |
Smokingb
| 13 (35%) | 7 (32%) | 6 (40%) |
Hyperlipidemia | 6 (16%) | 5 (23%) | 1 (7%) |
Family history of CVD | 11 (30%) | 8 (36%) | 3 (20%) |
Obesity (BMI ≥ 30 kg/m2) | 9 (24%) | 5 (23%) | 4 (27%) |
Symptoms (multiple possible) | |||
Angina | 6 (16%) | 4 (18%) | 2 (13%) |
Dyspnea | 16 (43%) | 12 (54%) | 4 (27%) |
Palpitations | 4 (11%) | 4 (18%) | - |
Syncope | 1 (3%) | 1 (5%) | - |
ECG abnormality | 11 (30%) | 9 (41%) | 2 (13%) |
Years since diagnosis | |||
3 (0–11) | 4 (0–11) | 2 (0–11) | |
< 1 | 12 (32%) | 6 (27%) | 6 (40%) |
1–4 | 11 (30%) | 5 (23%) | 6 (40%) |
5–9 | 11 (30%) | 10 (46%) | 1 (7%) |
≥ 10 | 3 (8%) | 1 (5%) | 2 (13%) |
Disease Activity | |||
BVAS | 5.5 (1–14) | 5 (3–8) | 11.5 (0–24) |
Hematocrit | 0.39 (0.36–0.42) | 0.4 (0.38–0.42) | 0.38 (0.32–0.42) |
Medication | |||
Beta-blockers | 9 (24%) | 6 (27%) | 3 (20%) |
ARB | 14 (38%) | 7 (32%) | 7 (47%) |
ASA | 5 (14%) | 5 (23%) | - |
CCB | 8 (22%) | 4 (18%) | 4 (27%) |
Statins | 3 (8%) | 2 (9%) | 1 (7%) |
Diuretics | 8 (22%) | 7 (32%) | 1 (7%) |
Steroids | 37 (100%) | 22 (100%) | 15 (100%) |
NSAID | 1 (3%) | 1 (5%) | - |
Antibodies | 2 (5%) | 1 (5%) | 1 (7%) |
Cyclophosphamide | 17 (46%) | 4 (18%) | 13 (87%) |
Azathioprine | 4 (11%) | 2 (9%) | 2 (13%) |
Methotrexate | 5 (14%) | 4 (18%) | 1 (7%) |
LGE negative n = 21 | LGE positive n = 16 | p | |
---|---|---|---|
Cardiovascular risk factors | |||
Diabetes | 3 (14%) | 4 (25%) | 0.41 |
Hypertension | 8 (38%) | 5 (31%) | 0.67 |
Smokinga
| 8 (38%) | 5 (31%) | 0.98 |
Hyperlipidemia | 2 (10%) | 4 (25%) | 0.21 |
Family history of CVD | 6 (29%) | 5 (31%) | 0.86 |
Obesity (BMI ≥ 30 kg/m2) | 6 (29%) | 3 (19%) | 0.49 |
Symptoms (multiple possible) | |||
Angina | 2 (10%) | 4 (25%) | 0.21 |
Dyspnea | 8 (38%) | 8 (50%) | 0.55 |
Palpitations | 2 (10%) | 2 (13%) | 0.72 |
Syncope | - | 1 (6%) | 0.25 |
ECG abnormality | 5 (24%) | 6 (38%) | 0.37 |
Years since diagnosis | |||
< 1 | 8 (38%) | 4 (25%) | 0.57 |
1–4 | 5 (24%) | 6 (38%) | 0.63 |
5–9 | 7 (33%) | 4 (25%) | 0.67 |
≥ 10 | 1 (5%) | 2 (13%) | 0.39 |
Disease Activity | |||
BVAS | 4 (1–19) | 6 (2.5–12.5) | 0.91 |
Hematocrit | 0.39 (0.32–0.41) | 0.41 (0.38–0.44) | 0.10 |
Medication | |||
Beta-blockers | 5 (24%) | 4 (25%) | 0.93 |
ARB | 10 (48%) | 4 (25%) | 0.16 |
ASA | - | 5 (31%) | |
CCB | 6 (29%) | 2 (13%) | 0.24 |
Statins | 1 (5%) | 2 (13%) | 0.37 |
Diuretics | 4 (19%) | 4 (25%) | 0.66 |
Steroids | 21 (100%) | 16 (100%) | 0.16 |
NSAID | 1 (5%) | - | |
Antibodies | 2 (10%) | - | |
Cyclophosphamide | 11 (52%) | 6 (38%) | 0.67 |
Azathioprine | 1 (5%) | 3 (19%) | 0.18 |
Methotrexate | 2 (10%) | 3 (19%) | 0.42 |
General CMR results
Controls (n = 20) | Patients (n = 37) | p | |
---|---|---|---|
LV-EF (%) | 65 ± 4 | 64 ± 10 | 0.84 |
LV-EDV (ml) | 109 ± 27 | 122 ± 41 | 0.38 |
LV-ESV (ml) | 38 ± 13 | 46 ± 31 | 0.49 |
LV-SV | 71 ± 15 | 76 ± 18 | 0.40 |
LV-EDD | 45 ± 5 | 48 ± 5 | 0.08 |
LA (cm2) | 20 ± 3 | 20 ± 5 | 0.84 |
IVS (mm) | 10 ± 2 | 10 ± 2 | 0.11 |
PA (mm) | 24 ± 4 | 25 ± 3 | 0.37 |
LV mass (g) | 85 ± 20 | 90 ± 25 | 0.67 |
LGE | - | 16 (43%) | |
epicardial | - | 5 (13%) | |
intramural | - | 7 (19%) | |
transmural | - | - | |
subendocardial | - | 4 (11%) | |
% LV mass | - | 6.8 ± 5 | |
Native T1 (ms) | 952 (923–960) | 988 (965–1017) |
<0.001
|
Post-contrast T1 (ms) | 524 (500–529) | 488 (468–528) |
0.03
|
ECV (%) | 24.5 (23–25) | 27.5 (26–29) |
<0.001
|
T2 (ms) | 49 (48–51) | 53 (51–55) |
<0.001
|
LGE negative (n = 21) | LGE positive (n = 16) | p | |
---|---|---|---|
LV-EF (%) | 69 ± 7 | 59 ± 12 |
0.01
|
LV-EDV (ml) | 117 ± 40 | 128 ± 44 | 0.37 |
LV-ESV (ml) | 38 ± 21 | 56 ± 38 | 0.06 |
LV-SV | 79 ± 22 | 72 ± 11 | 0.43 |
LV-EDD | 47 ± 6 | 49 ± 4 | 0.09 |
LA (cm2) | 21 ± 5 | 20 ± 4 | 0.54 |
IVS (mm) | 10 ± 2 | 11 ± 2 | 0.56 |
PA (mm) | 25 ± 4 | 25 ± 2 | 0.75 |
LV mass (g) | 90 ± 25 | 90 ± 24 | 0.93 |
LGE per patient | - | 16 (100%) | |
epicardial | - | 6 (37%) | |
intramural | - | 6 (37%) | |
transmural | - | - | |
subendocardial | - | 4 (25%) | |
% LV mass | - | 6.8 ± 5 | |
Native T1 (ms) | 1002 (970–1025) | 982 (957–998) | 0.10 |
Post-contrast T1 (ms) | 501 (478–542) | 477 (429–497) |
0.04
|
ECV (%) | 27 (25–28) | 28 (26–32) | 0.13 |
T2 (ms) | 51 (50–55) | 54 (52–56) | 0.14 |
Controls (n = 20) | Patients (n = 22) | p | |
---|---|---|---|
LV-EF (%) | 65 ± 4 | 62 ± 12 | 0.58 |
LV-EDV (ml) | 109 ± 27 | 124 ± 48 | 0.38 |
LV-ESV (ml) | 38 ± 13 | 51 ± 37 | 0.35 |
LV-SV | 71 ± 15 | 74 ± 17 | 0.76 |
LV-EDD | 45 ± 5 | 48 ± 6 | 0.06 |
LA (cm2) | 20 ± 3 | 21 ± 6 | 0.97 |
IVS (mm) | 10 ± 2 | 10 ± 2 | 0.23 |
PA (mm) | 24 ± 4 | 25 ± 3 | 0.27 |
LV mass (g) | 85 ± 20 | 89 ± 25 | 0.83 |
LGE per patient | - | 9 (41%) | |
epicardial | - | 3 (14%) | |
intramural | - | 2 (9%) | |
transmural | - | - | |
subendocardial | - | 4 (18%) | |
% LV mass | - | 7.1 ± 6 | |
Native T1 (ms) | 952 (923–960) | 982 (958–1007) |
0.003
|
Post-contrast T1 (ms) | 524 (500–529) | 481 (462–527) |
0.02
|
ECV (%) | 24.5 (23–25) | 28 (25–31) |
<0.001
|
T2 (ms) | 49 (48–51) | 53 (50–55) |
<0.001
|
Controls (n = 20) | Patients (n = 15) | p | |
---|---|---|---|
LV-EF (%) | 65 ± 4 | 68 ± 7 | 0.24 |
LV-EDV (ml) | 109 ± 27 | 118 ± 28 | 0.57 |
LV-ESV (ml) | 39 ± 13 | 39 ± 14 | 0.91 |
LV-SV | 71 ± 15 | 80 ± 20 | 0.19 |
LV-EDD | 45 ± 5 | 47 ± 4 | 0.36 |
LA (cm2) | 20 ± 3 | 20 ± 3 | 0.71 |
IVS (mm) | 10 ± 2 | 11 ± 2 | 0.11 |
PA (mm) | 24 ± 4 | 24 ± 4 | 0.78 |
LV mass (g) | 85 ± 20 | 92 ± 25 | 0.57 |
LGE per patient | - | 7 (47%) | |
epicardial | - | 3 (20%) | |
intramural | - | 4 (27%) | |
transmural | - | - | |
subendocardial | - | - | |
% LV mass | - | 6.3 ± 4 | |
Native T1 (ms) | 952 (923–960) | 1002 (972–1023) |
<0.001
|
Post-contrast T1 (ms) | 524 (500–529) | 490 (477–551) | 0.16 |
ECV (%) | 24.5 (23–25) | 27 (26–28) |
<0.001
|
T2 (ms) | 49 (48–51) | 53 (51–54) |
<0.001
|
T1 and ECV results
T2 results
Values above the 95% percentile of normal
AAV patients with ECG abnormality
ECG normal (n = 26) | ECG abnormal (n = 11) | p | |
---|---|---|---|
LV-EF (%) | 66 ± 9 | 61 ± 12 | 0.47 |
LV-EDV (ml) | 116 ± 28 | 136 ± 60 | 0.68 |
LV-ESV (ml) | 40 ± 17 | 59 ± 47 | 0.46 |
LV-SV | 76 ± 17 | 77 ± 21 | 0.79 |
LV-EDD | 47 ± 5 | 49 ± 6 | 0.31 |
LA (cm2) | 19 ± 3 | 23 ± 7 | 0.16 |
IVS (mm) | 10 ± 2 | 11 ± 2 | 0.06 |
PA (mm) | 25 ± 3 | 25 ± 4 | 0.80 |
LV mass (g) | 91 ± 23 | 89 ± 30 | 0.46 |
LGE positive (n) | 10 (39%) | 6 (55%) | 0.37 |
Native T1 (ms) | 983 (960–1010) | 1006 (983–1037) | 0.11 |
Post-contrast T1 (ms) | 487 (464–538) | 490 (476–512) | 0.62 |
ECV (%) | 26 (25–28) | 29 (28–32) |
0.01
|
T2 (ms) | 52 (50–54) | 55 (53–57) |
0.009
|