Mohsen Akhavan Sepahi
1*, Muhammed Mubarak
2 1 Department of Pediatric Nephrology, School of Medicine and Pediatric Clinical Research of Development Center, Qom University of Medical Sciences, Qom, Iran
2 Javed I. Kazi Department of Histopathology, Sindh Institute of Urology and Transplantation (SIUT), Karachi, Pakistan
3 Head International Research and Development, Mesencell Biotech International Ltd, 20-22 Wenlock Road, London, N1 7GU, United Kingdom
Abstract
Immunoglobulin A vasculitis nephritis (IgAVN) or Henoch-Schönlein purpura nephritis
(HSPN) is the most common type of secondary IgA nephropathy (IgAN), particularly in
children. Overall, its prognosis is good, but in a significant number of cases, it does lead to
chronic kidney disease (CKD) and end-stage renal disease (ESRD). Various classifications
exist to predict the long-term outcome of kidney disease in these diseases, but none is
universally accepted. We searched PubMed, Web of Science Embase, EBSCO, Scopus and
directory of open access journals (DOAJ) with keywords of Henoch-Schönlein purpura
nephritis, immunoglobulin A vasculitis nephritis, immunoglobulin A nephropathy, IgA
vasculitis, extracapillary proliferation, IgA nephropathy, leukocytoclastic vasculitis, endstage renal disease, Oxford classification, crescent and childhood IgA vasculitis. IgAVN in
children presents most often with crescents and endocapillary proliferation, with relapsing
and remitting course clinically. Due to morphological resemblance of IgAVN and IgAN,
the Oxford MEST-C scores could be applied for determining the long-term outcomes in the
former disease. A critical concern in applying Oxford classification for IgAVN is that limited
number of children with IgAVN exists in any one center and also a relatively short period of
follow-up. Hence, further work in this regard is necessary. Preliminary evidence suggests that
Oxford MEST-C classification is valid in predicting long-term kidney outcomes in children
with IgAVN and the classification can also be used in adults. However, further, large scale,
multicenter, international collaborative studies are needed to address the unmet issues.
Implication for health policy/practice/research/medical education:
It is possible to use Oxford MEST-C scores for predicting the long-term outcomes in IgAVN; however, further work is necessary
to demonstrate the full validation of morphologic variables of Oxford MEST-C classification in this disease.
Please cite this paper as: Akhavan Sepahi M, Mubarak M, Kellner JS. Predicting renal outcomes in immunoglobulin A vasculitis
nephritis; from ISKDC classification to Oxford MEST-C classification. J Renal Inj Prev. 2020; 9(1): e08. doi: 10.15171/jrip.2020.08.