Table 2.

Disease evolution of the first flare, according to the treatment administered at onset

	Conservative Treatment (n = 6), HD = 2	FFP Infusion (n = 6), HD = 5	PE (n = 15), HD = 11	PE + IS (n = 3), HD = 1
No sequel	1	0	1	3
CRI	1	0	3	0
Late relapse	2	5	6	0
Treatment resistance	1	1	3	0
ESRD	1	0	1	0
Death	0	0	1	0

In total, 30 patients were documented. Follow-up occurred between 5 and 168 months with a mean of 48 months. The number of patients in each group is indicated in parentheses. HD indicates the number of patients needing hemodialysis at the first flare. Treatment resistance comprised cases without disease improvement under treatment and cases with relapse within the first month. Late relapses were defined by disease recurrence >1 month after onset and >15 days after disease remission.