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Abstract Background and Aims IgA Vasculitis (IgAV) frequently has a relapsing/refractory course despite glucocorticoids and immunosuppressive therapies and the management of severe disease remains controversial 1. Plasma exchange (PLEX) has been used as a rescue treatment in other vasculitides, particularly in cases with rapidly progressive glomerulonephritis, but little is known about its role in IgAV. Here we present outcomes of patients with refractory IgAV treated with PLEX at our centre. Method Clinical records of patients who met 1990 American College of Rheumatology classification criteria and 2012 Chapel Hill Consensus Conference definitions for IgAV were analysed and those receiving ≥1 course of PLEX (5 sessions) identified from our PLEX database. We assessed demographic and clinical features at diagnosis and at starting of PLEX. Response was defined as an improvement in vasculitis activity measured with Birmingham Vasculitis Activity Score (BVAS) 1 month after PLEX course completion and classified as “partial” (BVAS3 and prednisolone 10 mg/day) or “complete” (BVAS = 0). Both definitions included changes in eGFR and proteinuria. Relapse was defined as an increase in BVAS after initial response. Early adverse events occurring during PLEX course or within one week after completion of this were recorded. Results Among 174 patients with IgAV, 12 (7%) received ≥1 course of PLEX. This was started a median of 15 months after diagnosis (interquartile range, IQR 3-40). All patients received glucocorticoids and immunosuppressive therapy prior to PLEX (Table). At the time of starting PLEX, 8/12 patients had active skin involvement (7/8 had purpura and 2/8 ulcers) and 10/12 nephritis, with a median eGFR 34 mL/min (IQR 30-43) and a median urine albumin:creatinine ratio (UACR) of 298 mg/mmol (IQR 240-486). PLEX was combined with glucocorticoids and various immunosuppressive agents, most commonly cyclophosphamide (42%) or mycophenolate mofetil (33%). All but one patient had a response at 1 month, and this was “complete” in five (42%). The median eGFR of patients with active nephritis increased up to 44 mL/min/1.73 m2 (IQR 36-58) and the median UACR drop to 182 (IQR 159-408). Ten patients (91%) relapsed a median of 3 months (IQR 2-7) after completion of the PLEX course and 8/10 (80%) resumed PLEX and achieved response. Six patients (50%) continued a “chronic” regimen of PLEX (1-2 monthly sessions) for a median of 85 months (25-141), as this was the only therapy that could control skin (4/6) and/or kidney manifestations (3/6). Three patients experienced infection within a week of PLEX discontinuation and two reported reactions to FFP/albumin, but all recovered completely. Three patients (25%) developed kidney failure during follow-up and two died (one of whom had kidney failure), with death occurring 9 and 146 months after discontinuation of PLEX. Conclusion In this small cohort of adult patients with severe and refractory IgAV, PLEX was associated with improved disease control, stabilisation of renal parameters, and few early adverse events. The clinical response to PLEX appeared temporary, but some patients maintained remission through a “chronic” PLEX regimen. As highlighted by the high risk of death or kidney failure, more effective therapies for IgAV are needed but PLEX should be considered as a rescue treatment in severe/refractory cases.
Trivioli et al. (Wed,) studied this question.