Patients’ characteristics. The median age at baseline was 31 years (range: 14– 80 years). The median disease duration was 1 month (range: 0- 240 months). The median duration of follow-up was 33 months (range: 0– 115 months). The detailed clinical characteristics and laboratory parameters of the patients are reported in Table 1 and Table 2.
Table 1
Characteristics of the quantitative data.
Item | Valid N | Missing N | Mean | S. E | Median | P25 | P75 |
Age (years) | 162 | 0 | 34.296 | 14.1271 | 31.00 | 24.75 | 42.25 |
Disease duration (months) | 162 | 0 | 10.938 | 34.0045 | 1.00 | 0.00 | 6.00 |
PLT count | 162 | 0 | 11.488 | 8.7108 | 8.50 | 4.00 | 18.00 |
SLEDAI-2K score | 138 | 24 | 8.022 | 4.8698 | 7.00 | 5.00 | 11.00 |
Follow-up (months) | 162 | 0 | 38.33 | 29.606 | 33.00 | 14.75 | 61.25 |
Table 2
Characteristics of the qualitative data.
Item | Yes(n (%)) | No(n (%)) | Missing value(n(%)) |
Sex (male) | 17 (10.5) | 145 (89.5) | |
Mucocutaneous bleeding | 102 (63.0) | 60 (37.0) | |
Visceral hemorrhage | 9 (5.6) | 153 (94.4) | |
Mucocutaneous involvement | 40 (24.7) | 122 (75.3) | |
Arthritis | 40 (24.7) | 122 (75.3) | |
Raynaud's phenomenon | 13 (8.0) | 149 (92.0) | |
Fever | 45 (27.8) | 117 (72.2) | |
Proteinuria | 23 (14.2) | 139 (85.8) | |
NPSLE | 8 (4.9) | 154 (95.1) | |
PAH | 6 (3.7) | 129 (79.6) | 27 (16.7) |
Intestinal vasculitis | 5 (3.1) | 157 (96.9) | |
Myositis | 2 (1.2) | 160 (98.8) | |
Serositis | 40 (24.7) | 122 (75.3) | |
Leukopenia | 35 (21.6) | 127 (78.4) | |
Anemia | 86 (53.1) | 76 (46.9) | |
Hypocomplementemia (C3) | 100 (61.7) | 38 (23.5) | 24 (14.8) |
Hypocomplementemia (C4) | 57 (35.2) | 81 (50.0) | 24 (14.8) |
ANA | 162 (100) | 0 | |
Anti-dsDNA | 87 (53.7) | 75 (46.3) | |
Anti-Sm | 46 (28.4) | 115 (71.0) | 1 (0.6) |
Anti-nRNP | 76 (46.9) | 85 (52.5) | 1 (0.6) |
Anti-Nuc | 57 (35.2) | 104 (64.2) | 1 (0.6) |
Anti-Rib | 43 (26.5) | 118 (72.8) | 1 (0.6) |
Anti-His | 47 (29.0) | 114 (70.4) | 1 (0.6) |
Anti-SSA/Ro | 91 (56.2) | 70 (43.2) | 1 (0.6) |
Anti-SSB/La | 19 (11.7) | 142 (87.7) | 1 (0.6) |
GC pulses at week 1 | 25 (15.4) | 137 (84.6) | |
IgG pulses at week 1 | 18 (11.1) | 144 (88.9) | |
a. Mucocutaneous involvement: rash, alopecia or mucosal ulcers; |
b. NPSLE: seizure, psychosis, organic brain syndrome, visual disturbance or cranial nerve disorder; |
c. PAH: pulmonary arterial pressure measured by ultrasound > 36 mmHg; anemia: Hb < 100 g/L. |
Treatment response at week 1, week 4 and week 24. The treatment responses at three different times are displayed in Table 3, and the parallel program model is shown in Fig. 1. The response rates at week 1, week 2, and week 24 were 76.9%, 86.9%, and 91.2%, respectively, and the complete response rates were 35.9%, 75.9%, and 83.1%, respectively. We found that the rate of no response decreased as time went on, while the response and complete response rates increased.
Table 3
Treatment outcomes at three different points (N/%).
Time | NR | MR | MoR | CR | Death | Missing |
Week 1 | 35 (21.6) | 20 (12.3) | 44 (27.2) | 56 (34.6) | 1 (0.6) | 6 (3.7) |
Week 4 | 16 (9.9) | 3 (1.9) | 13 (8) | 110 (67.9) | 3 (1.9) | 17 (10.5) |
Week 26 | 5 (3.1) | 3 (1.9) | 8 (4.9) | 113 (69.8) | 7 (4.3) | 26 (16) |
Serious infections, death and LDG-CR. Serious infection was defined as infection that caused death or needed treatment in the hospital. There were 21 patients who had concomitant serious infections during follow-up. There were 12 deaths in total, of which 4 were due to pulmonary infection, 1 was due to a pelvic infection attributed to abortion surgery, 2 was due to alveolar hemorrhage, 1 was due to severe acute pancreatitis, 1 was due to hepatic failure, 1 was due to cardiac failure, 1 was due to multiple organ failure, and 1 was due to acute abdomen. There were 93 patients who achieved LDG-CR, accounting for 67.4%. The above parameters are shown in Table 4.
Table 4
Serious infections, death and LDG-CR
Outcome | Yes | No | Missing (%) |
Serious infection | 21 (13.0) | 121 (87.0) | |
Death | 12 | 150 | |
LDG-CR | 93 (57.4) | 45 (27.8) | 24(14.8) |
a. LDG-CR : Complete response with glucocorticoid that equals to no more than 7.5mg prednisone. |
Multivariate analysis of the risk factors. The significant results of multivariate analysis are shown in Table 5. Several factors, including baseline demographic features, clinical manifestations and autoantibodies, were associated with the outcome.
Table 5
Time | Outcome | Risk factor | B | S.E. | Wald | Sig. | OR | 95% CI |
Week 1 | R | male sex | -1.848 | 0.617 | 8.959 | 0.003 | 0.158 | 0.047–0.528 |
| | age | -0.049 | 0.015 | 10.364 | 0.001 | 0.952 | 0.924–0.981 |
| | *serositis | -0.884 | 0.453 | 3.799 | 0.051 | 0.413 | 0.170–1.005 |
| | anti-Sm | 1.125 | 0.563 | 4.003 | 0.045 | 3.082 | 1.023–9.282 |
Week 1 | CR | serositis | -1.123 | 0.495 | 5.155 | 0.023 | 0.325 | 0.123–0.858 |
Week 4 | R | *visceral hemorrhage | -1.593 | 0.820 | 3.775 | 0.052 | 0.203 | 0.041–1.014 |
| | *mucocutaneous involvement | 2.032 | 1.056 | 3.701 | 0.054 | 7.627 | 0.963–60.436 |
Week 4 | CR | age | -0.052 | 0.016 | 10.402 | 0.001 | 0.949 | 0.920–0.980 |
| | anti-nRNP | 1.418 | 0.455 | 9.708 | 0.002 | 4.128 | 1.692–10.071 |
| | anti-SSB/La | 2.219 | 1.074 | 4.268 | 0.039 | 9.194 | 1.120-75.445 |
Week 24 | R | age | -0.084 | 0.031 | 7.411 | 0.006 | 0.920 | 0.866–0.977 |
| | myositis | -3.493 | 1.609 | 4.715 | 0.030 | 0.030 | 0.001–0.712 |
| | *leukopenia | -1.423 | 0.738 | 3.721 | 0.054 | 0.241 | 0.057–1.023 |
| | anti-SSA/Ro | -2.559 | 1.040 | 6.055 | 0.014 | 0.077 | 0.010–0.594 |
Week 24 | CR | leukopenia | -1.382 | 0.523 | 6.998 | 0.008 | 0.251 | 0.090–0.699 |
| | anti-SSA/Ro | -1.083 | 0.536 | 4.076 | 0.044 | 0.339 | 0.118–0.969 |
Follow-up | Serious infection | pulmonary arterial hypertension | 1.955 | 0.859 | 5.178 | 0.023 | 7.062 | 1.311–38.037 |
| Death | myositis | 3.178 | 1.486 | 4.574 | 0.032 | 24.000 | 1.304-441.709 |
| | leukopenia | 1.603 | 0.640 | 6.271 | 0.012 | 4.966 | 1.417–17.404 |
| LDG-CR | age | -0.044 | 0.015 | 8.065 | 0.005 | 0.957 | 0.929–0.987 |
| | mucocutaneous hemorrhage | -0.920 | 0.438 | 4.418 | 0.036 | 0.399 | 0.169–0.940 |
| | anti-nRNP | 0.941 | 0.399 | 5.574 | 0.018 | 2.563 | 1.173–5.598 |
a. R: PLT > = 30*109/L; |
b. CR: PLT > = 100*109/L; |
c. LDG-CR: Complete response with glucocorticoid that equals to no more than 7.5mg prednisone. |
d. The italicized variables marked with “*” were not statistically significantly associated with the outcome, but the P values were close to 0.05, and they could not be automatically eliminated by the software. |
Effect of baseline demographic features. Male patients were relatively less likely to have a response at week 1 (OR = 0.158; 95% CI: 0.047–0.528; p = 0.003). Older patients were relatively less likely to have a response at week 1 (OR = 0.952; 95% CI: 0.924–0.981; p = 0.001), less likely to have a complete response at week 4 (OR = 0.949; 95% CI: 0.920–0.980; p = 0.001), less likely to have a response at week 26 (OR = 0.920; 95% CI: 0.866–0.977; p = 0.006), and less likely to achieve LDG-CR (OR = 0.957; 95% CI: 0.929–0.987; p = 0.005).
Effect of clinical features. Patients with serositis were relatively less likely to have a complete response or response at week 1 (OR = 0.325; 95% CI: 0.123–0.858; p = 0.023; and OR = 0.413; 95% CI: 0.170–1.005; p = 0.051), even though the latter was not statistically significant. Patients with myositis were relatively less likely to have a response at week 24 (OR = 0.030; 95% CI: 0.001–0.712; p = 0.030). Patients with leukocytopenia were relatively less likely to have a complete response at week 24 (OR = 0.251; 95% CI: 0.090–0.699; p = 0.008). At week 4, patients with visceral hemorrhage were relatively less likely to have a response, and patients with mucocutaneous involvement were relatively more likely to have a response (OR = 0.203; 95% CI: 0.041–1.014; p = 0.052; and OR = 7.627; 95% CI: 0.963–60.436; p = 0.054), but the difference was not statistically significant. During follow-up, 21 patients had serious infections, and pulmonary arterial hypertension was the only risk factor for a serious infection (OR = 7.062; 95% CI: 1.311–38.037; p = 0.023). There were 12 deaths, and myositis and leukopenia were risk factors for mortality (OR = 24.000; 95% CI: 1.304–441.709; p = 0.032; and OR = 4.966; 95% CI: 1.417–17.404; p = 0.012). There were 93 patients who achieved LDG-CR, and patients with mucocutaneous hemorrhage were relatively less likely to achieve LDG-CR (OR = 0.399; 95% CI: 0.169–0.940; p = 0.036). The SLEDAI-2K score was not significantly associated with any outcome.
Effect of autoantibodies. Patients with anti-Sm antibodies were relatively more likely to have a response at week 1 (OR = 3.082; 95% CI: 1.023–9.282; p = 0.045). Patients with anti-nRNP antibodies or anti-SSB antibodies were relatively more likely to have a complete response at week 4 (OR = 4.128; 95% CI: 1.692–10.071; p = 0.002; and OR = 9.194; 95% CI: 1.120–75.445; p = 0.039). Patients with anti-Ro antibodies were relatively less likely to have a response or complete response at week 24 (OR = 0.077; 95% CI: 0.010–0.594; p = 0.014; and OR = 0.339; 95% CI: 0.118–0.969; p = 0.044). Patients with anti-nRNP antibodies were relatively more likely to achieve LDG-CR (OR = 2.563; 95% CI: 0.1173–5.598; p = 0.018).
Effect of glucocorticoid pulses and intravenous immunoglobulin pulses. There were 25 patients and 18 patients who were given glucocorticoid pulses and immunoglobulin pulses. However, neither glucocorticoid pulses nor immunoglobulin pulses were significantly associated with any outcome.