scholarly journals IgA vasculitis (Henoch-Schönlein purpura) nephritis and psoriasis in a child: is there a relationship?

2021 ◽  
Author(s):  
Ana Sofia Vaz ◽  
Raquel Penteado ◽  
Carolina Cordinhã ◽  
Carmen Carmo ◽  
Clara Gomes
Keyword(s):  
Iga Nephropathy ◽  
Systemic Vasculitis ◽  
Kidney Injury ◽  
Common Mechanism ◽  
Henoch Schonlein Purpura ◽  
Systemic Involvement ◽  
Kidney Involvement ◽  
Schonlein Purpura ◽  
Nephrotic Range Proteinuria ◽  
Henoch Schönlein Purpura

Abstract Background Psoriasis is a chronic immune-mediated disorder that primarily affects the skin in both adults and children but can also have systemic involvement, particularly with arthritis and kidney injury. IgA nephropathy is the most frequent kidney disorder associated with psoriasis. Approximately one third of all cases of psoriasis begin in childhood, but association between psoriasis and renal disorders has scarcely been reported in pediatric patients. Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by IgA deposits in the vessel walls of affected organs and in the mesangium of the kidney. HSP nephritis histopathology is identical to IgA nephropathy. Case report A 6-year-old boy with recent onset of psoriasis developed HSP with kidney involvement, clinically manifested by nephrotic-range proteinuria and hematuria. Kidney biopsy revealed fibrocellular glomerular crescents and mesangial IgA deposits compatible with IgA nephropathy. Treatment with systemic corticosteroids led to the control of hematuria, but as nephrotic-range proteinuria persisted, cyclophosphamide was added, leading to a gradual decrease in proteinuria. Conclusions We propose an underlying common mechanism in the pathogenesis of both HSP and psoriasis, involving a dysregulation of the IgA-mediated immune response, which could predispose to both entities as well as to kidney damage and IgA nephropathy in these patients.

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

2020 ◽  
Author(s):  
Eda Didem Kurt-Şükür ◽  
Thivya Sekar ◽  
Kjell Tullus
Keyword(s):  
Angiotensin Receptor Blockers ◽  
Laboratory Data ◽  
Treatment Modalities ◽  
Oxford Classification ◽  
Henoch Schonlein Purpura ◽  
Nephritic Syndrome ◽  
Kidney Involvement ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura ◽  
Purpura Nephritis

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.

Correlation between endocapillary proliferative and nephrotic-range proteinuria in children with Henoch-Schönlein purpura nephritis

2018 ◽  
Vol 34 (4) ◽  
pp. 663-670 ◽  
Author(s):  
Xiao-qing Yang ◽  
Yan-jie Huang ◽  
Wen-sheng Zhai ◽  
Xian-qing Ren ◽  
Qing-yin Guo ◽  
...  
Keyword(s):  
Henoch Schonlein Purpura ◽  
Schonlein Purpura ◽  
Nephrotic Range Proteinuria ◽  
Henoch Schönlein Purpura ◽  
Purpura Nephritis

scholarly journals Renal Outcomes of Childhood IgA Nephropathy and Henoch Schönlein Purpura Nephritis

2021 ◽  
Vol 73 (10) ◽  
pp. 687-694
Author(s):  
Thanaporn Chaiyapak ◽  
Anirut Pattaragarn ◽  
Suroj Supavekin ◽  
Nuntawan Piyaphanee ◽  
Kraisoon Lomjansook ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Complete Recovery ◽  
Clinicopathological Characteristics ◽  
Henoch Schonlein Purpura ◽  
Renal Outcomes ◽  
Nephritic Syndrome ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura ◽  
Purpura Nephritis

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.

Defective activation of the MAPK/ERK pathway, leading to PARP1 and DNMT1 dysregulation, is a common defect in IgA nephropathy and Henoch-Schönlein purpura

2018 ◽  
Vol 31 (5) ◽  
pp. 731-741 ◽  
Author(s):  
Annamaria Milillo ◽  
Clelia Molinario ◽  
Stefano Costanzi ◽  
Gisella Vischini ◽  
Francesca La Carpia ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Erk Pathway ◽  
Henoch Schonlein Purpura ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura ◽  
Common Defect

scholarly journals Neutrophil-to-lymphocyte Ratio: A Biomarker for Predicting Systemic Involvement in Henoch–Schonlein Purpura

2021 ◽  
Vol 0 ◽  
pp. 1-4
Author(s):  
Wang Lei ◽  
Shan Yun-Yun ◽  
Xu Ai-E
Keyword(s):  
Inflammatory Marker ◽  
Renal Involvement ◽  
Neutrophil To Lymphocyte Ratio ◽  
Henoch Schonlein Purpura ◽  
Systemic Involvement ◽  
Lymphocyte Ratio ◽  
Number Of Patients ◽  
Pre Treatment ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Background: Henoch–Schonlein purpura (HSP) is one of the commonest entities included within the category of cutaneous vasculitis (CV). Our work is purposed to explore the predictive value of neutrophil-to-lymphocyte ratio (NLR) for systemic involvement in Henoch– Schonlein purpura patients. This ratio is known as an inflammatory marker, and is used to assess the systemic inflammation associated with various diseases. Our objective is to establish whether it can be applied for the prediction of renal and gastrointestinal (GI) or purely renal involvement in Henoch–Schonlein purpura. Aim: To determine the relationship between neutrophil-to-lymphocyte ratio and systemic involvement in Henoch–Schonlein purpura Methods: This is a retrospective review of the patients who were diagnosed with Henoch–Schonlein purpura in our hospital between 2012 and 2018. Results: A total of 57 patients met our inclusion criteria. Pre-treatment neutrophil-to-lymphocyte ratio was significantly associated with renal and/or GI manifestations of the disease (p<0.001). The optimal cut-off value of this ratio for predicting systemic involvement was 2.48, with a 95% specificity and a 94% sensitivity. In addition, pretreatment ratio was also found to be significantly correlated with the severity of relevant systemic manifestations of Henoch–Schonlein purpura (r=0.831; p<0.01). Limitations: The small number of patients recruited for our research, its retrospective design, and the inclusion of patients attending the same hospital. Conclusion: This study suggests that neutrophil-to-lymphocyte ratio is suitable as a potential indicator for predicting the systemic involvement in Henoch–Schonlein purpura.

Steroid Effects on the Course of Abdominal Pain in Children With Henoch-Schonlein Purpura

PEDIATRICS ◽  
1987 ◽  
Vol 79 (6) ◽  
pp. 1018-1021
Author(s):  
NORMAN D. ROSENBLUM ◽  
HARLAND S. WINTER
Keyword(s):  
Abdominal Pain ◽  
Gastrointestinal Symptom ◽  
Systemic Vasculitis ◽  
Skin Lesions ◽  
Intestinal Bleeding ◽  
Controlled Trials ◽  
Henoch Schonlein Purpura ◽  
Intestinal Lesions ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Henoch-Schonlein purpura is a systemic vasculitis of unknown cause that is characterized primarily by abdominal pain, arthritis, and purpuric skin lesions. Abdominal pain is the most common gastrointestinal symptom, but intestinal bleeding and intussusception may occur. Previous studies have supported the use of steroids in managing the abdominal pain of Henoch-Schonlein purpura.1,2 Because there are no controlled trials using steroids in this disease, their value in affecting the intestinal lesions of Henoch-Schonlein purpura remains unknown. The purpose of this retrospective study was to assess the effect of corticosteroids on the outcome of abdominal pain in children with Henoch-Schonlein purpura. PATIENTS AND METHODS

scholarly journals Decreased glycolysis induced dysfunction of NK cells in Henoch-Schonlein purpura patients

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Wenjia Chai ◽  
Xiaolin Wang ◽  
Wei Wang ◽  
Hui Wang ◽  
Wenjun Mou ◽  
...  
Keyword(s):  
Nk Cells ◽  
Systemic Vasculitis ◽  
Metabolic Reprogramming ◽  
Healthy Children ◽  
Henoch Schonlein Purpura ◽  
Reduced Frequency ◽  
Activating Receptors ◽  
Innate Lymphocytes ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Abstract Background Henoch-Schonlein purpura (HSP) is the most common systemic vasculitis of the childhood. However, its mechanisms and pathogenesis still need more exploration. Natural killer (NK) cells are innate lymphocytes, and there is a growing appreciation that cellular metabolism is important in determining the immune responsiveness of lymphocytes. Thus, we aimed to analyze the NK cells phenotype and explore the association between glucose metabolism and NK cells function in HSP patients. Results A total number of 64 HSP patients and 34 healthy children were included. The HSP patients were divided into two groups according to whether accompanied with nephritis or not. NK cells in HSP patients without nephritis showed a reduced frequency in peripheral blood, a down-regulated expression of activating receptors both NKp30 and NKp46, and an attenuated cytotoxic function against tumor cells. In addition, the function impairment of NK cells was shown to exacerbate in HSPN. Our data further revealed an aberrant metabolic reprogramming of NK cells in HSP patients. Upon stimulation with cytokines (IL-15, IL-12 and IL-2), NK cells from healthy controls switched to an elevated glycolysis rate to support their effector function. By contrast, the glycolysis rate of activated NK cells in HSP group was not significantly up-regulated from the resting level possibly owing to the inhibition of mTORC1. Conclusions Our study found that HSP patients were accompanied with dysfunction of NK cells. We concluded that the dysfunction of NK cells in HSP patients was induced with a decreased glycolysis rate and suggested that metabolic reprogramming of NK cells might be a player in the pathogenesis of HSP.

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