Table 2.

Summary of SLE patients with 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.