Pulmonary haemorrhage in a patient with IgA nefropathy

Vega, Lara Belmar; Fernández-Díaz, C.; Fontana, Natalia Palmou; Calabia, Emilio Rodrigo; Penagos, Luis Martín; Rodríguez, M.; Fresnedo, Gema Fernández

doi:10.1016/j.nefroe.2017.05.013

Cited by 1 publication

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“…In contrast, nine of the ten patients who achieved at least partial remission and had stable kidney function over a follow-up period of 24–72 months had initiated plasmapheresis treatment within 1 month of symptom onset. This relationship was redemonstrated in the Shenoy et al case series where all patients that initiated PLEX within 2 weeks of symptom onset achieved remission, whereas the single patient who delayed treatment until 2 months after symptom onset ultimately developed ESKD and required a kidney transplant [ 41 ]. The findings of this review support the KDIGO 2021 clinical practice guidelines that suggest treating RPGN due to IgA vasculitis similarly to ANCA-associated vasculitis where PLEX is sometimes utilized [ 2 ].…”

Section: Discussionmentioning

confidence: 99%

Efficacy and Safety of Plasma Exchange as an Adjunctive Therapy for Rapidly Progressive IgA Nephropathy and Henoch-Schönlein Purpura Nephritis: A Systematic Review

Nguyen

Acharya

Tangpanithandee

et al. 2023

IJMS

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Patients with IgA nephropathy (IgAN), including Henoch-Schönlein purpura nephritis (HSP), who present with rapidly progressive glomerulonephritis (RPGN) have a poor prognosis despite aggressive immunosuppressive therapy. The utility of plasmapheresis/plasma exchange (PLEX) for IgAN/HSP is not well established. This systematic review aims to assess the efficacy of PLEX for IgAN and HSP patients with RPGN. A literature search was conducted using MEDLINE, EMBASE, and through Cochrane Database from inception through September 2022. Studies that reported outcomes of PLEX in IgAN or HSP patients with RPGN were enrolled. The protocol for this systematic review is registered with PROSPERO (no. CRD42022356411). The researchers systematically reviewed 38 articles (29 case reports and 9 case series articles) with a total of 102 RPGN patients (64 (62.8%) had IgAN and 38 (37.2%) had HSP). The mean age was 25 years and 69% were males. There was no specific PLEX regimen utilized in these studies, but most patients received at least 3 PLEX sessions that were titrated based on the patient’s response/kidney recovery. The number of PLEX sessions ranged from 3 to 18, and patients additionally received steroids and immunosuppressive treatment (61.6% of patients received cyclophosphamide). Follow-up time ranged from 1 to 120 months, with the majority being followed for at least 2 months after PLEX. Among IgAN patients treated with PLEX, 42.1% (n = 27/64) achieved remission; 20.3% (n = 13/64) achieved complete remission (CR) and 18.7% (n = 12/64) partial remission (PR). 60.9% (n = 39/64) progressed to end-stage kidney disease (ESKD). Among HSP patients treated with PLEX, 76.3% (n = 29/38) achieved remission; of these, 68.4% (n = 26/38) achieved CR and 7.8% achieved (n = 3/38) PR. 23.6% (n = 9/38) progressed to ESKD. Among kidney transplant patients, 20% (n = 1/5) achieved remission and 80% (n = 4/5) progressed to ESKD. Adjunctive plasmapheresis/plasma exchange with immunosuppressive therapy showed benefits in some HSP patients with RPGN and possible benefits in IgAN patients with RPGN. Future prospective, multi-center, randomized clinical studies are needed to corroborate this systematic review’s findings.

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Section: Discussionmentioning

confidence: 99%