Abstract
BackgroundHenoch-Schönlein purpura nephritis (HSPN), a small-vessel vasculitis, shares renal pathological features with immunoglobulin A nephropathy. Oxford classification of immunoglobulin A nephropathy pathology has been updated to the MEST-C score, but its application in HSPN remains unresolved.MethodsTwo hundred and thirteen patients with biopsy-proven HSPN were retrieved from the Seoul National University Hospital between 2000 and 2017. Renal outcome risks (i.e., end-stage renal disease or doubling of serum creatinine) were evaluated according to MEST-C scores after stratification by age: 113 children aged < 18 years (9.2 ± 3.6 years) and 100 adults aged ≥18 years (38.6 ± 18.3 years). We pooled our data with four previous cohort studies in which MEST or MEST-C scores were described in detail.ResultsTwenty-one child (19%) and 16 adult (16%) patients reached the renal outcome during the median follow-up periods of 12 years and 13 years, respectively (maximum 19 years). In children, M1 and T1/T2 scores revealed worse renal outcomes than did M0 and T0 scores, respectively, whereas the T score was the only factor related to worse outcomes in adult patients after adjusting for multiple clinical and laboratory variables. The pooled data showed that M1, S1, and T1/T2 in children and E1 and T1/T2 in adults were correlated with poorer renal outcomes than those of their counterpart scores.ConclusionsThe Oxford classification MEST-C scores can predict long-term renal outcomes in patients with HSPN.
Highlights
Henoch-Schönlein purpura nephritis (HSPN), a small-vessel vasculitis, shares renal pathological features with immunoglobulin A nephropathy
The histopathological classification methods for HSPN are inconsistent and depend on clinicians or societies, two main classifications exist for HSPN: the Meadow classification, which primarily focuses on the degree of mesangial hypercellularity [9] and the International Study Group of Kidney Disease in Children (ISKDC), which uses crescents as an important factor [10]
The updated Oxford classification of immunoglobulin A (IgA) nephropathy includes information on various pathological features, which are graded as the MEST-C score; applying this classification to HSPN cases requires further validation [14]
Summary
Henoch-Schönlein purpura nephritis (HSPN), a small-vessel vasculitis, shares renal pathological features with immunoglobulin A nephropathy. Oxford classification of immunoglobulin A nephropathy pathology has been updated to the MEST-C score, but its application in HSPN remains unresolved. Henoch-Schönlein purpura (HSP) is a small vessel vasculitis that features immunoglobulin A (IgA) deposition, mainly in the joints, skin, and kidneys. When the disease affects the kidneys, it is called HSP nephritis (HSPN). HSPN is rare but can potentially cause chronic kidney disease and end-stage renal disease (ESRD). The histopathological classification methods for HSPN are inconsistent and depend on clinicians or societies, two main classifications exist for HSPN: the Meadow classification, which primarily focuses on the degree of mesangial hypercellularity [9] and the International Study Group of Kidney Disease in Children (ISKDC), which uses crescents as an important factor [10]. Which classification is best for HSPN patients remains uncertain because of limited evidence [11], and the above classifications do not consider other
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
