MEST-C pathological score and long-term outcomes of child and adult patients with Henoch-Schönlein purpura nephritis

Abstract

Background: 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. Methods: Two 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.Results: Twenty-one child (19%) and 16 adult (16%) patients reached the renal outcome during the median followup 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. Conclusions: The Oxford classification MEST-C scores can predict long-term renal outcomes in patients with HSPN.

Using MEST-C Scores and the International Study of Kidney Disease in Children Classification to Predict Outcomes of Henoch–Schönlein Purpura Nephritis in Children

“…First, the onset age of T1/T2 was higher than in the T0 group in our group. In studies of adults with HSPN, the proportion of T1/T2 fluctuated between 11 and 54.1% (6,16,22), while that in children with HSPN was only 5.3-18.8% (6)(7)(8)24). Second, the Oxford classification is primarily derived from the data of adult IgAN patients, and thus, it may need to be modified when evaluating pediatric HSPN.…”

“…At present, we use the original four histopathologic features related to renal outcomes to five: mesangial hypercellularity (M), endocapillary proliferation (E), segmental sclerosis/adhesion (S), tubular atrophy/interstitial fibrosis (T), and cellular or fibrocellular crescents (C) (4,5). Currently, several studies have applied the updated Oxford classification to children with HSPN for predicting renal prognosis (Table 5) (6)(7)(8)(9)(10). In this paper, we use the Oxford classification and the ISKDC classification to predict the outcomes of 877 children with HSPN.…”

Global Glomerulosclerosis and Segmental Glomerulosclerosis Could Serve as Effective Markers for Prognosis and Treatment of IgA Vasculitis With Nephritis

“…Since IgAV-N was more common in children, few researches have investigated which pathological classification is suitable for adult-onset IgAV-N. Emerging studies have demonstrated that the indicators of the Oxford classification of IgAN, which shared the similar pathophysiological mechanism with IgAV-N, can indicate the renal outcomes of IgAV-N (14,(16)(17)(18)(19). A portion of scholars noted that they could be recommended for clinical practice.…”

“…However, whether segmental glomerulosclerosis can be used as a prognostic factor of IgAV-N does not reach a consensus. Some studies have proposed that segmental glomerulosclerosis is useful in predicting long-term renal outcome of IgAV-N (16,19). But K-M analysis in other studies have indicated that it fails to predict the prognosis (25,26).…”

“…We speculated that the causes of the inconsistent conclusions may result from the enrolled patients with different severity of segmental glomerulosclerosis. What is noteworthy is that the mainstream classification of segmental glomerulosclerosis scores was the presence or absence (14,16,19,(25)(26)(27)(28). Patients with higher proportion (>15%) and lower proportion (0-15%) of segmental glomerulosclerosis scores were assigned to the same group in their study.…”