Table 5.
Onset of organ involvement among all PRESS participants during follow-up
| Visceral involvement or related outcome measure | Overall population n (%) |
Definite dcSSc n/N (%) |
At-risk for dcSSc n/N (%) |
P-value |
|---|---|---|---|---|
| Absolute increase in mRSS of ≥ 5 units or ≥ 25%, (n = 228)1 | 48 (21.1) | 36/202 (17.8) | 12/26 (45.2) | 0.0009¶ |
| Absolute decline of FVC % of ≥ 10%, (n = 176)1 | 41 (23.3) | 34/153 (22.2) | 7/23 (30.4) | 0.3850¶ |
| Pulmonary hypertension on RHC, (n = 296)2, 3,* | 7 (2.4) | 7/259 (2.7) | 0/37 (0.0) | 0.6021§ |
| LVEF of ≤ 45% on TTE, (n = 189)3 | 5 (2.6) | 5/166 (3.0) | 0/23 (0.0) | 1.0000§ |
| Scleroderma renal crisis, (n = 285)3 | 11 (3.9) | 9/248 (3.6) | 2/37 (5.4) | 0.6404§ |
| All-cause mortality, (n = 301) | 20 (6.6) | 19/263 (7.2) | 1/38 (2.6) | 0.4870§ |
mRSS, modified Rodnan Skin score; FVC, forced vital capacity; RHC, right heart catheterization; LVEF,left ventricular ejection fraction; TTE, transthoracic echocardiogram
1FVC and mRSS worsening: calculated change from baseline values
2mPAP threshold value for pulmonary hypertension was ≥25 mmHg on RHC. Participants who had PH before/at baseline were excluded from the denominator
3PH, LVEF, and scleroderma renal crisis: only counted events that occurred between the consent date and the cutoff date
*Based on the results of n = 33 RHC on 29 participants
¶Chi-squared test
§Fisher exact test