Article Figures & Data
Figures
- Figure 1.
Flow chart of patient selection. aPL: antiphospholipid antibodies; PUMCH: Peking Union Medical College Hospital; TMA: thrombotic microangiopathy; HTN: hypertension.
- Figure 2.
Cumulative incidence of endpoint events in SLE patients with TMA. A. Cumulative incidence of partial remission. B. Cumulative incidence of complete remission. SLE: systemic lupus erythematosus; TMA: thrombotic microangiopathy.
Tables
- Table 1.
Baseline characteristics of patients with thrombotic microangiopathy associated with SLE.
Characteristics ADAMTS13 Inhibitor–negative, n = 13 ADAMTS13 Inhibitor–positive, n = 6 p Demographic data Age, yrs 21.8 ± 6.6 25.8 ± 11.6 0.351 Female 11 (84.6) 6 (100.0) 0.517 New-onset lupus 11 (84.6) 5 (83.3) 1.000 Interval from disease onset to TMA, mos 14.6 (1.4–93.3) 0.8 (0.3–110.4) 0.628 Interval from Dx of SLE to onset of TMA, mos 0.7 (0.6–2.4) 0.4 (0.2–31.8) 0.177 Overall disease activity SLEDAI 20.2 ± 4.4 20.2 ± 8.6 0.997 Complement C3 level, mg/dl 37.0 ± 17.3 74.9 ± 14.4 < 0.001 Complement C4 level, mg/dl 7.2 ± 5.4 10.8 ± 6.9 0.288 Positive anti-dsDNA antibody 7 (53.8) 2 (33.3) 0.628 Hematological abnormalities Severe ADAMTS13 deficiency 0 (0) 6 (100) < 0.001 Platelet count, ×109/l 25.0 ± 17.8 7.3 ± 5.1 0.005 Hemoglobin, g/l 51.3 ± 11.8 62.8 ± 8.0 0.046 Lactate dehydrogenase, U/l 860.5 ± 453.3 1301.7 ± 680.5 0.110 Renal involvement Renal impairment, n 13 (100.0) 0 (0) < 0.001 Serum creatinine, μmol/l 358.7 ± 193.4 64.3 ± 9.9 < 0.001 eGFR, ml/min/1.73 m2 21.6 ± 12.0 112.7 ± 18.0 < 0.001 Mean arterial pressure, mmHg 117.5 ± 13.1 95.3 ± 13.6 0.008 Proteinuria, g/d 8.1 (5.2–14.0) 0.6 (0.2–2.5) 0.011 CNS involvement, n 3 (23.1) 6 (100.0) 0.003 Values are expressed as the mean ± SD or median (interquartile range). Attribute values are expressed as number (percentage). Significant p values are in bold face. CNS: central nervous system; Dx: diagnosis; eGFR: estimated glomerular filtration rate; SLE: systemic lupus erythematosus; SLEDAI: SLE Disease Activity Index; TMA: thrombotic microangiopathy.
No. Sex Age Time of SLE Dx Relative to Initial Dx of TMA CNS Involvement Renal Insufficiency eGFR RRT Renal Biopsy, ISN/RPS Classification TPE Dose, Plasma Volume IVIG Pulse MP IS RTX Outcome Time to Outcome or End of Followup, d ADAMTS13 inhibitor (−) 1 M 13 Concurrent − + 20 − IV-G(A) + TMA 3.8 + − MMF − CR 431 2 F 24 Concurrent − + 10 + NA 6.4 + − CYC − Refractory 94 3 F 17 Concurrent − + 18 − NA 5.9 − + CYC − Refractory 30 4 F 30 Before + + 38 − NA 4.9 + + CYC − Death 15 5 F 33 Concurrent − + 5 + NA 4.3 − + CYC − Refractory 549 6 F 16 Concurrent − + 45 − NA 14.7 + + CYC − PR 55 7 F 24 Concurrent − + 7 + NA 4.7 + + CYC − Refractory 98 8 M 30 Concurrent − + 15 − NA 4.9 + + CYC − Refractory 192 9 F 20 Concurrent − + 22 − IV-G(A) + TMA 5.0 − + CYC − PR 33 10 F 20 Concurrent + + 21 − NA 6.7 + + CYC − Death 33 11 M 16 Before + + 31 − NA 9.3 + − MMF − CR 30 12 F 14 Concurrent − + 32 − NA 5.2 + + MMF − CR 40 13 F 27 Concurrent − + 17 + NA 14.6 + + CYC − PR 342 ADAMTS13 inhibitor (+) 1 F 29 Concurrent + − 115 − NA 0 − − MMF − CR 33 2 F 16 Concurrent + − 124 − NA 0 + + CYC − CR 18 3 F 46 Concurrent + − 91 − NA 6.6 + + CYC − CR 10 4 F 22 Concurrent + − 135 − NA 0 − + CYC − CR 14 5 F 28 Before + − 91 − NA 6.0 − + CYC − CR 18 6 F 14 Concurrent + − 120 − NA 10.5 + + − + CR 19 IS: immunosuppressants; CNS: central nervous system; CR: complete remission; CYC: cyclophosphamide; Dx: diagnosis; eGFR: estimated glomerular filtration rate (ml/min/1.73 m2); ISN/RPS: International Society of Nephrology/Renal Pathology Society; IVIG: intravenous immunoglobulin; MMF: mycophenolate mofetil; MP: methylprednisolone; PR: partial remission; RRT: renal replacement therapy; SLE: systemic lupus erythematosus; TMA: thrombotic micro-angiopathy; TPE: therapeutic plasma exchange; RTX: rituximab; NA: not available.
Variables ADAMTS13 Inhibitor − negative, n = 13 ADAMTS13 inhibitor−positive, n = 6 ADAMTS13 inhibitor (+)/(−), HR (95% CI) p Therapies TPA, n 13 (100.0) 3 (50.0) 0.021 No. TPA sessions, n 5.0 (3.5–8.5) 2.0 (0.0–8.2) 0.628 Steroid pulse therapy, n 10 (76.9) 5 (83.3) 1.000 Immunosuppressants, n 13 (100.0) 6 (100.0) 1.000 IVIG, n 10 (76.9) 3 (50.0) 0.320 Therapeutic responses Followup, mos 5.9 (1.5–10.9) 4.4 (2.5–7.4) 0.620 Complete remission 3 (23.1) 6 (100.0) 10.8 (1.8–65.5) < 0.001 Partial remission 6 (46.1) 6 (100.0) 6.9 (1.3–37.4) < 0.001 Social economy Hospitalization days 45.1 ± 13.4* 30.5 ± 3.4 0.005 Hospitalization cost, × CNY1000 151.0 ± 78.5 61.3 ± 44.2 0.019 Values are expressed as the mean ± SD or median (interquartile range) unless specified. Attribute values are expressed as number (percentage). Significant p values are in bold face.
↵* One patient died on Day 17 and one on Day 33 of hospitalization, and they were not included in the analysis. SLE: systemic lupus erythematosus; TMA: thrombotic microangiopathy; CNY: Chinese yuan; IVIG: intravenous immunoglobulin; TPA: therapeutic apheresis.
