Effects of clinical and serological characteristics on survival during the disease course of mixed connective tissue disease (MCTD).
| Characteristics | Living Patients, n = 258 | Deceased Patients, n = 22 | p | 95% CI | RR |
|---|---|---|---|---|---|
| Age at diagnosis of MCTD, yrs | 41.8 ± 10.7 | 35.5 ± 10.4 | 0.03 | ||
| Disease duration, yrs | 13.2 ± 7.6 (r: 1–29) | 12.3 ± 7.5 (r: 1–26) | 0.835 | ||
| Polyarthritis | 192 | 15 | 0.612 | 0.2878–1.886 | 0.736 |
| Raynaud phenomenon | 151 | 10 | 0.265 | 0.2461–1.417 | 0.5905 |
| Pulmonary arterial hypertension | 41 | 9 | 0.0071 | 1.47–9.133 | 3.664 |
| Myositis | 81 | 10 | 0.234 | 0.7556–4.389 | 1.821 |
| Interstitial lung disease | 124 | 8 | 0.3750 | 0.2504–1.523 | 0.6175 |
| Serositis | 69 | 14 | < 0.001 | 1.926–11.927 | 4.793 |
| Esophageal hypomotility | 133 | 6 | 0.04 | 1.076–7.483 | 2.837 |
| CNS involvement | 52 | 4 | 1.0 | 0.2856–2.713 | 0.8803 |
| Sclerodactyly | 102 | 15 | 0.8226 | 0.3544–2.040 | 0.8503 |
| Skin manifestations | 95 | 7 | 0.8181 | 0.3152–2.034 | 0.800 |
| Kidney involvement | 8 | 3* | 0.04 | 1.208–20.147 | 4.934 |
| Cardiovascular events | 84 | 14 | < 0.0001 | 1.463–8.979 | 3.625 |
| Cancer | 10 | 6 | < 0.001 | 2.999–28.837 | 9.3 |
| Antiphospholipid syndrome | 62 | 10 | 0.039 | 1.085–6.394 | 2.634 |
| AECA positivity | 78 | 16 | 0.002 | 2.30–16.321 | 6.154 |
| aCL IgG/M/A positivity | 85 | 13 | 0.0190 | 1.209–7.151 | 2.94 |
| Anti-ß2-GPI IgG/M/A positivity | 66 | 13 | 0.002 | 1.717–10.283 | 4.202 |
| Anti-SSA positivity | 88 | 4 | 0.1587 | 0.1409–1.308 | 0.4293 |
| Anti-dsDNA positivity | 9 | 0 | — | — | — |
| Anti-Sm positivity | 17 | 2 | 0.6516 | 0.1520–3.274 | 0.705 |
| ACPA positivity | 51 | 2 | 0.2710 | 0.0918–1.793 | 0.4059 |
| Treatment with high-dose steroid† | 201 | 18 | 0.783 | 0.2549–2.409 | 0.783 |
| Treatment with cytotoxic agent†† | 190 | 19 | 0.305 | 0.1265–1.538 | 0.4412 |
| Treatment with anti-TNF-α monoclonal antibodies | 42 | 0 | — | — | — |
↵* Kidney damage due to thrombotic thrombocytopenic purpura.
↵† Dosage ≥ 1 mg/kg/day methylprednisolone.
↵†† Methotrexate, cyclophosphamide, or azathioprine. CNS: central nervous system; AECA: antiendothelial cell antibody; aCL: anticardiolipin antibody; anti-ß2-GPI: ß2-glycoprotein I antibody; ACPA: anticitrullinated protein antibody; TNF-α tumor necrosis factor-α.