Causes of death in 22 patients with mixed connective tissue disease (MCTD).
| Cause of Death | No. Patients (%) | Duration of MCTD at Time of Death, yrs |
|---|---|---|
| Disease manifestations | 12 (54.5) | |
| PAH | 9 (40.9) | 17, 9, 15, 14, 16, 8, 17, 16, 19 |
| TTP/HUS | 3 (13.6) | 3, 13, 1 |
| Disease complications | 10 (45.4) | |
| Infections | 3 (13.6) | 8, 12, 15 |
| Acute cardiovascular events | 7 (31.8) | 10, 4, 6, 15, 3, 12, 26 |
PAH: pulmonary arterial hypertension; TTP/HUS: thrombotic thrombocytopenic purpura or hemolytic uremic syndrome.