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Different histological classifications for Henoch-Schönlein purpura nephritis: which one should be used?

Marija Jelusic¹, Mario Sestan², Rolando Cimaz³

¹Department of Paediatrics, University Hospital Centre Zagreb, University of Zagreb School of Medicine, Zagreb, Croatia. marija.jelusic@mef.hr.

Pediatric Rheumatology Online Journal

|March 2, 2019

Insights

IgA Vasculitis nephritis (HSPN) lacks standardized histological classifications, impacting prognosis and treatment. Collaborative studies are needed to establish guidelines for better patient outcomes.

Area of Science:

Pediatric Rheumatology
Nephrology
Pathology

Background:

IgA Vasculitis nephritis (HSPN) is a significant complication of IgA Vasculitis (IgAV/HSP), affecting childhood vasculitis prognosis.
Current treatment and prognosis rely heavily on interpreting biopsy materials, but histological classifications are unevenly applied.

Purpose of the Study:

To address the challenges posed by the varied application of histological classifications in IgA Vasculitis nephritis (HSPN).
To highlight the lack of consensus on biopsy interpretation for treatment decisions in HSPN.

Main Methods:

Review of existing histological classification systems for HSPN, including ISKDC, Oxford, Haas, and Koskela classifications.
Discussion of the limited validation of these systems, particularly the Oxford classification, which is primarily validated for IgA nephropathy.

Main Results:

Multiple histological classification systems are used in clinical practice for HSPN, but their correlation with disease severity and renal outcome is unclear.
Treatment strategies for HSPN are not standardized and are not consistently based on histological classifications.

Conclusions:

Implementation of multicenter, multinational prospective studies is crucial for correlating clinical features and outcomes with classification systems.
Collaboration among pediatric rheumatologists, nephrologists, and nephropathologists is essential to develop standardized guidelines for HSPN treatment based on histological classification.

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Abstract

Background:

Nephritis is the most important chronic complication of IgA Vasculitis (IgAV)/Henoch-Schönlein purpura (IGAV/HSP) and thus the main prognostic factor of this most common childhood vasculitis. Since the prognosis and treatment selection depends on the mode of interpretation of biopsy material, in this manuscript we have presented several issues related to the uneven application of different histological classifications in IgAV/Henoch-Schönlein purpura nephritis (HSPN). The nephritis of IgAV/IGAV/HSP will be abbreviated as HSPN for this paper.

Main Body:

In clinical practice we use different histological classifications for HSPN. It is not known which of these classifications best correlates with severity of renal disease and renal outcome in IgAV/IGAV/HSP. One of the major problem with existing histological classifications is that there is no consensus on the implementation of biopsy in the treatment of HSPN. There is a histologic classification system conventionally used in HSPN, of the International Study of Kidney Disease in Children (ISKDC). On the other hand there is the new classification system suggested for IgA nephropathy, the Oxford classification. The latter has been validated only in IgA nephropathy. There are also two further histologic classifications of Haas and Koskela that have been developed. Current treatment strategies in HSPN are not standardised nor predominantly based on histological classification.

Conclusion:

One of the possible solutions to problems related to the application of different histological classification in HSPN is the implementation of multicenter multinational prospective studies with joint collaboration between pediatric rheumatologists, nephrologists and nephropathologists to correlate the clinical features and outcome with the classification systems as well among the classifications. This classification should be the basis for the construction of guidelines for the treatment of patients with HSPN.

Keywords:

Biopsy Children Glomerulonephritis Henoch-Schönlein purpura Histology IgA Vasculitis

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