scholarly journals Biopsy-proven Henoch-Schönlein purpura nephritis: a single center experience

Author(s):  
Eda Didem Kurt-Şükür ◽  
Thivya Sekar ◽  
Kjell Tullus

Abstract Background Knowledge on normal progress and treatment of Henoch-Schönlein purpura nephritis (HSPN) is limited. This study reviews outcome, clinical, pathological, and therapeutic factors affecting the prognosis of HSPN patients. Methods Forty-nine children with biopsy-confirmed HSPN diagnosed between September 2008 and 2018 were included. Demographics, clinical and laboratory data, treatment, and outcome were recorded at the time of biopsy, 3, 6, 12, and 24 months and at last visit. Clinical outcome was graded according to Meadow’s criteria. Results The median age at time of biopsy was 10.1 years (IQR:5.7) and female/male ratio 24/25. At presentation, 40.8% of patients had nonnephrotic proteinuria, 18.4% nephrotic syndrome (NS), 4.1% nephritic syndrome (NephrS), and 36.7% NephrS+NS. There were 11 patients with an estimated glomerular filtration rate below 90 ml/min/1.73 m2. Biopsy specimens were classified according to International Study of Kidney Diseases in Children (ISKDC) and Oxford Classification MEST-C scoring systems. Forty-one patients received angiotensin-converting enzyme inhibitors/angiotensin receptor blockers, 37 patients steroids, and 35 patients other immunosuppressive medications. At last visit, 24 patients had stage 1 chronic kidney disease (CKD), three stage 2 CKD, and two had stage 5 CKD. Neither clinical parameters nor ISKDC biopsy grade or treatment modalities effected the final outcome. The Oxford classification showed significantly increased segmental glomerulosclerosis in patients with unfavorable outcome. Favorable outcome was associated with shorter time from kidney involvement to biopsy and start of treatment. Conclusion A large proportion of patients continued to show signs of CKD at last follow-up while only a small proportion developed stage 5 CKD.

2021 ◽  
Vol 73 (10) ◽  
pp. 687-694
Author(s):  
Thanaporn Chaiyapak ◽  
Anirut Pattaragarn ◽  
Suroj Supavekin ◽  
Nuntawan Piyaphanee ◽  
Kraisoon Lomjansook ◽  
...  

Background: Henoch-Schönlein purpura nephritis (HSPN) is considered the systemic form of IgA nephropathy (IgAN). However, differing clinicopathological features and renal outcomes of children with IgAN and HSPN have been reported in some studies. Methods: This study retrospectively reviewed children with IgAN and HSPN younger than 18 years, between January 2004 and December 2015. The clinicopathological characteristics at diagnosis and the renal outcomes after at least 1 year of follow-up were compared between the two groups. Results: A total of 54 children, comprising 21 with IgAN and 33 with HSPN, were recruited. The children with HSPN were younger than the children with IgAN. Gross hematuria and nephritic syndrome at the initial presentation were more common in children with IgAN. Regarding the pathological findings, IgAN had greater chronicity than HSPN. After a median follow-up period from first presentation to renal outcomes measurement of 4.0 years (1.3-12.2) in children with IgAN and 4.2 years (1.1-11.4) in children with HSPN, the renal outcomes were better in the latter group. The incidence of chronic kidney disease (CKD) was 28.6% in children with IgAN and 6.1% in children with HSPN (p = 0.02). Complete recovery was observed more frequently in children with HSPN than in children with IgAN (57.1% in IgAN vs. 87.9% in HSPN, p = 0.01). Conclusions: Childhood IgAN has greater chronicity and worse renal outcomes than childhood HSPN, with a lower rate of complete recovery and a higher frequency of CKD. We recommend long-term follow-up for CKD in children with IgAN.


2020 ◽  
Author(s):  
Supatjaree Chanvitan ◽  
Kanchana Tangnararatchakit ◽  
Pawaree Saisawat ◽  
Songkiat Chantarogh ◽  
Suchin Worawichawong ◽  
...  

Abstract Introduction: There are limited data on the outcomes in children with Henoch-Schönlein purpura nephritis (HSPN) and IgA nephropathy (IgAN) in limited-resource countries. This study was aimed to evaluate the outcome of HSPN and IgAN and to evaluate the pathological findings associated with unfavorable outcomes.Materials and methods: This was a retrospective study conducted in children (≤18 years) diagnosed with HSPN or IgAN, had estimated glomerular filtration rate (eGFR) ≥ 60 mL/min/1.73 m2 and underwent renal biopsy between year 2002 - 2018 at Ramathibodi Hospital, Bangkok, Thailand. Clinical outcomes were graded according to the outcome classification by Counahan, as follows: favorable [A, normal; B, minor urinary abnormalities (proteinuria < 1 g/1.73 m2/day)]; unfavorable [C, active renal disease (proteinuria > 1 g/1.73 m2/day and/or hypertension); D, renal insufficiency (eGFR < 60 mL/min/1.73 m2 or died)]. Pathologies were graded according to Oxford classification.Results: A total of 47 patients (28 HSPN and 19 IgAN) were included with means of age at 9.4 ± 2.8 vs 11.3 ± 4.3 years, respectively. After a median follow-up time of 50 months, proportions of favorable outcomes in the patients with HSPN and IgAN were 82.1% (23/28) and 89.5% (17/19), respectively (p-value = 0.685). In multivariate analysis, only endocapillary proliferation (E) was associated with unfavorable outcomes in both diseases with the odds ratio (95% CI) of 12.46 (1.36 - 114.51, p-value = 0.026).Conclusion: The clinical outcomes of most patients with HSPN and IgAN were favorable and comparable. Endocapillary proliferation (E) was the only factor associated with poor outcome in both diseases.


2021 ◽  
Vol 9 ◽  
Author(s):  
Meiqiu Wang ◽  
Ren Wang ◽  
Xu He ◽  
Pei Zhang ◽  
Qianhuining Kuang ◽  
...  

Introduction: Henoch–Schönlein purpura nephritis (HSPN) and IgA nephropathy (IgAN) bear similarities in some aspects. The histological classification of HSPN was built on the International Study of Kidney Disease in Children (ISKDC) criteria, while IgAN was established on the 2016 Oxford classification (MEST-C scores). The purpose of this paper was to discuss the predictive value of the ISKDC classification and MEST-C scores in children with HSPN.Methods: We performed a retrospective study of 877 children with HSPN in a single center between 2001 and 2019. The primary outcome was defined as chronic kidney disease—estimated glomerular filtration rate (eGFR) &lt;90 ml/min/1.73 m2.Results: During the follow-up period of 23.3 (10.9–47.9) months, 51 (5.8%) patients reached the primary outcome. As revealed in a Kaplan–Meier plot, segmental glomerulosclerosis (S) (P &lt; 0.001) and tubular atrophy/interstitial fibrosis (T) (P &lt; 0.001) significantly predict poor renal outcome. Other Oxford lesions and the ISKDC classification, however, did not show a significant difference in a worse outcome. In a multivariate Cox model adjusted for pathological and clinical factors, eGFR [hazard ratio (HR) = 2.831, 95% confidence interval (95% CI) = 1.359–5.896], S lesion (HR = 3.936, 95% CI = 2.078–7.457), and T lesion (HR = 4.002, 95% CI = 1.733–9.242) were independent risk factors for the renal outcome.Conclusion: This series constitutes the largest series reported so far in the literature of such patients. According to our findings, S and T of the Oxford classification, which are ignored by the ISKDC classification, could be applied to predict the renal prognosis of children with HSPN.


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