Table 2.

History of patientswith CFI I416L variant at initial presentation

Patient	Known CKD (age)	Occurrence of TMA	Age at presentation	Concurrent trigger or feature	Associated organ damage	Biopsy results	Glomerulosclerosis	IFTA	Vascular lesions	IF	ESKD after the episodea	Relapseb	Duration of follow-up
P1	No	Yes	36	Malignant hypertension	AKI	TMA associated with nephrosclerosis	50%	50%	Severe	None	Yes	No	6 years
P2	CKD stage III (43)	Yes	45	Malignant hypertension	AKI	ND	-	-	-	-	Yes	No	7 years
P3	CKD stage V (37)	No	-	-	No	ND	-	-	-	-	-	-	6 years
P4	No	No	-	-	No	Fibrous endarteritis Intratubular hemoglobin casts	8%	5%	Moderate	Mesangial C3+	-	-	5 years
P5	No	Preeclampsia HELLP syndrome	19	Pregnancy	No	ND	-	-	-	-	No	No	2 years
P6	CKD stage IV (33)	Yes	41	CNI therapy with Tacrolimus	AKI	TMA lesions transplanted kidney	57%	70%	Severe	Arteriolar C3 +	Yes	No	15 years
P7	No	Yes	19	Malignant hypertension	AKI	Extensive fibrous lesions without deposits	84%	80%	Severe	Arteriolar C3 +	Yes	No	2 years
P8	CKD stage IV (35)	No	-	-	No	ND	-	-	-	-	-	-	9 years
P9	CKD stage V (28)	Preeclampsia	28	-	AKI Heart failure	ND	-	-	-	-	Yes	-	3 years
P10	No	Yes	40	Malignant hypertension	AKI	ND	-	-	-	-	Yes	No	11 years
P11	No	Preeclampsia	42	Pregnancy	No	ND	-	-	-	-	No	No	1 years
P12	Yes (38)	No	-	-	-	-	-	-	-	-	-	-	13 years

AKI, acute kidney injury; CKD, chronic kidney disease; CNI, calcineurin inhibitor therapy; ESKD, end- stage kidney disease; HELLP, hemolysis, elevated liver enzymes, and low platelet count; IFTA, interstitial fibrosis and tubular atrophy; ND, not determined; TMA, thrombotic microangiopathy.

Vascular lesions are classified as minimal, moderate, or severe; IF, Deposits in immunofluorescence.

^aNone of the patients received anti-C5 antibody therapy either after initial manifestation of TMA or as prevention of transplantation.

^bRelapses of TMA on native or transplanted kidney.