Persistent Purpura Research Articles

Incidence and risk factors of nephritis in childhood Henoch-Schonlein purpura

Background Henoch-Schönlein Purpura (HSP) is the most common systemic vasculitis disease in children. It is characterized by involvement of the skin, joints, gastrointestinal tract, and kidney. Kidney manifestations may progress to severe nephritis, even lead to end-stage kidney disease.
 Objective To identify the incidence and risk factors of nephritis in childhood HSP.
 Methods A retrospective cohort study was performed to evaluate clinical, demographic, laboratory, and therapeutic parameters of HSP patients aged 0-18 years between 2011-2019 at Dr. Cipto Mangunkusumo Hospital, Jakarta. Diagnoses of HSP were made according to the 2008 EULAR/PRES/PRINTO criteria. Wefollowed subjects’ medical records for at least 3 months after disease onset to observe incidence and risk factors of Henoch-Schönlein nephritis (HSN).Results There were 112 HSP patients (aged 2-17 years) included in this study. HSN was found in 40 out of 112 patients (35.7%). Nephritis developed within the first 4 weeks for a majority of cases. Multivariate analysis showed that persistent purpura (OR 3.306; 95%CI 1.315 to 8.315; P=0.011) and acute phase leukocytosis(OR 2.585; 95%CI 1.047 to 6.385; P=0.039) were significantly associated risk factors for HSN. We found that corticosteroid use did not reduce the risk of HSN. The accumulation of several risk factors was associated with the likelihood of developing HSN.
 Conclusion Persistent purpura and acute phase leukocytosis are independent risk factors for HSN. Therefore, blood tests are needed to estimate the risk of HSN. Early corticosteroid therapy do not reduce the risk of kidney impairment.

Prediction of renal damage in children with IgA vasculitis based on machine learning.

This article is objected to explore the value of machine learning algorithm in predicting the risk of renal damage in children with IgA vasculitis by constructing a predictive model and analyzing the related risk factors of IgA vasculitis Nephritis in children. Case data of 288 hospitalized children with IgA vasculitis from November 2018 to October 2021 were collected. The data included 42 indicators such as demographic characteristics, clinical symptoms and laboratory tests, etc. Univariate feature selection was used for feature extraction, and logistic regression, support vector machine (SVM), decision tree and random forest (RF) algorithms were used separately for classification prediction. Lastly, the performance of four algorithms is compared using accuracy rate, recall rate and AUC. The accuracy rate, recall rate and AUC of the established RF model were 0.83, 0.86 and 0.91 respectively, which were higher than 0.74, 0.80 and 0.89 of the logistic regression model; higher than 0.70, 0.80 and 0.89 of SVM model; higher than 0.74, 0.80 and 0.81 of the decision tree model. The top 10 important features provided by RF model are: Persistent purpura ≥4 weeks, Cr, Clinic time, ALB, WBC, TC, Relapse, TG, Recurrent purpura and EB-DNA. The model based on RF algorithm has better performance in the prediction of children with IgA vasculitis renal damage, indicated by better classification accuracy, better classification effect and better generalization performance.

Persistent Purpura on the Right Arm and Chest Due to Continuous Use of an Electric Vacuum Cleaner for Several Hours

Persistent Purpura on the Right Arm and Chest Due to Continuous Use of an Electric Vacuum Cleaner for Several Hours

Epidemiological and clinical aspects of immunoglobulin A vasculitis in childhood: a retrospective cohort study

BackgroundA retrospective study was conducted in order to investigate and describe the characteristics of Immunoglobulin A vasculitis (IgAV), previously known as Henoch-Schӧnlein purpura, in the paediatric population of a community-based healthcare delivery system in the Italian region of Abruzzo.MethodsThis is a population-based retrospective chart review of the diagnosis of IgAV in children ages 0 to 18, admitted to the Department of Paediatrics of Chieti and Pescara between 1 January 2000 and 31 December 2016. All children enrolled presented with clinical symptoms and laboratory findings and met the EULAR/PRINTO/PRES 2008 criteria.ResultsTwo-hundred-eight children met the criteria for IgAV, with the highest incidence reported among children below 7-years of age. A correlation with recent infections was found in 64% of the cohort; the onset was more frequently during the winter and fall. Purpura had a diffuse distribution in the majority of patients; joint impairment was the second most frequent symptom (43%), whereas the gastrointestinal tract was involved in 28% of patients.ConclusionsHereby, we confirm the relative benignity of IgAV in a cohort of Italian children; with regards to renal involvement, we report a better outcome compared to other studies. However, despite the low rate of renal disease, we observed a wide use of corticosteroids, especially for the treatment of persistent purpura.

Persistence and Severity of Cutaneous Manifestations in IgA Vasculitis Is Associated with Development of IgA Vasculitis Nephritis in Children

Background/Objective: The purpose of this study was to evaluate the prevalence and severity of skin involvement in children with IgA vasculitis (IgAV) and its relationship with clinical and biochemical parameters and the risk of developing IgA vasculitis nephritis (IgAVN), the only cause of long-term morbidity and the main prognostic factor in IgAV patients. Methods: This national multicenter retrospective study included 611 patients under the age of 18 years with IgAV referred to five Croatian tertiary hospitals between 2009 and 2019. Patient data were collected from a database with systematic analysis of IgAV patients in the Croatian population. Results: Among the 611 children, 205 (33.55%) had purpura on the lower extremities, in 207 (33.88%) the rash extended on the trunk, in 149 (24.39%) it extended to the upper extremities, in 32 (5.24%) the rash was generalized, while 15 (2.47%) had the most severe skin symptoms: bullae, ulcerations, and necroses. IgAVN developed in 130 (21.28%) and persistent IgAVN (present for >3 months) in 48 (7.86%) children. Multivariate logistic regression found that presence of ulcerations and necroses (OR 3.20 [95% CI 1.03–9.91]), persistent purpura (OR 2.89 [95% CI 1.71–4.88]), and higher age (OR 1.16 [95% CI 1.09–1.23]) were significant predictors of IgAVN, whereas persistent purpura (OR 20.11 [95% CI 1.09–372.52]), male sex (OR 3.32 [95% CI 1.13–9.80]), and higher age (OR 1.15 [95% CI 1.00–1.30]) were predictors of persistent IgAVN. Among the laboratory parameters, higher serum urea (OR 1.43 [95% CI 1.03–2.00]) and reduction in activated partial thromboplastin time (OR 0.83 [95% CI 0.74–0.93]) were shown to have a significant impact on increasing the risk of persistent IgAVN. Conclusion: With increasing severity and duration of cutaneous manifestations in IgAV, the risk of developing IgAVN increases, making the prognosis worse, with a greater likelihood to need more aggressive treatment.

Henoch-Schönlein Purpura: Management and Complication

Henoch-Schönlein Purpura (HSP) is a disease that mainly affects children, while the incidence in adults is rarely reported. Low incidence in adults caused by undiagnosed or misdiagnosed. Course of the disease in adults is more complex, including high incidence of renal insufficiency. Renal manifestations need special attention because it can worsen the prognosis, so patients must be detected and treated as quickly as possible. Poor prognosis depends on the presence of renal clinical manifestations accompanied by an increase in the severity of renal histological grading, abdominal manifestations, and persistent purpura. We describe male patients with HSP, presenting with gastrointestinal, renal, and cutaneous manifestations. Gastroscopy showed superficial gastritis (reddish patches on almost all gastric mucosa. Skin biopsy showed lymphocytic vasculitis. Gastric biopsy shows infiltration of lymphocyte inflammation cells, histiocytes, plasma cells in the corpus and gastric antrum. Patients have received supportive therapy, steroid, and showed clinical improvement.

Obesity is associated with severe clinical course in children with Henoch-Schonlein purpura.

We aimed to evaluate the role of obesity on the clinical course and response to treatment in patients with Henoch-Schonlein purpura (HSP). Data charts of children with HSP followed in a tertiary hospital between 2000 and 2018 were reviewed retrospectively. Persistent purpura was defined as skin involvement persisting for ≥ 30days. Mild nephropathy was defined as the presence of microscopical hematuria and/or non-nephrotic proteinuria, while severe nephropathy as nephrotic proteinuria, nephritic syndrome, and/or kidney insufficiency. Obese and non-obese patients were compared for demographic, clinical, and laboratory parameters. There were 199 patients (M/F, 104/95; median (IQR) presenting age 7.1 (5.0-9.2) years; follow-up period 17.5 (6-50) months). Obese patients (n = 35 (17.6%)) had significantly higher rate of persistent purpura (46% vs 21%), severe renal involvement (58% vs 31%), high-grade renal histopathological lesions (83% vs 39%), hypertension (29% vs 9%), and increased erythrocyte sedimentation rate (79% vs 56%). Obese patients also showed delayed improvement of cutaneous (25 vs 14days), articular (12.5 vs 10.0days), and kidney (280 vs 57days) symptoms. Obese children used steroids for significantly longer period of time (236 vs 40days). Furthermore, need for immunosuppressive medications were higher in obese patients (40% vs 9%). Obese children with HSP had higher erythrocyte sedimentation rate, hypertension, and severe renal involvement; showed delayed improvement of skin, joint, and kidney findings; and need more immunosuppressive medications and a longer period of steroid treatment. These findings may be associated with the effect of adipose tissue on inflammation.

Henoch-Schönlein purpura nephritis: initial risk factors and outcomes in a Latin American tertiary center.

The objective of this study was to evaluate prevalence, initial risk factors, and outcomes in Henoch-Schönlein purpura nephritis (HSPN) patients in Latin America. Two hundred ninety-six patients (validated EULAR/PRINTO/PRES HSP criteria) were assessed by demographic data, clinical/laboratorial involvements, and treatments in the first 3months after diagnosis. They were followed-up in a Latin American tertiary center and were divided in two groups: with and without nephritis. Persistent non-nephrotic proteinuria, nephrotic proteinuria, and acute/chronic kidney injury were also systematically evaluated at 1, 5, 10, and 15years after diagnosis. HSPN was evidenced in 139/296 (47%) in the first 3months. The median age at diagnosis was significantly higher in HSPN patients compared without renal involvement [6.6 (1.5-17.7) vs. 5.7 (0.9-13.5) years, p = 0.022]. The frequencies of persistent purpura (31 vs. 10%, p < 0.0001), recurrent abdominal pain (16 vs. 7%, p = 0.011), gastrointestinal bleeding (25 vs. 10%, p < 0.0001), and corticosteroid use (54 vs. 41%, p = 0.023) were significantly higher in the former group. Logistic regression demonstrated that the independent variables associated with HSNP were persistent purpura (OR = 3.601; 95% CI (1.605-8.079); p = 0.002) and gastrointestinal bleeding (OR = 2.991; 95% CI (1.245-7.183); p = 0.014). Further analysis of patients without HSPN in the first 3months revealed that 29/118 (25%) had persistent non-nephrotic proteinuria and/or hematuria in 1year, 19/61 (31%) in 5years, 6/17 (35%) in 10years and 4/6 (67%) in 15years after diagnosis. None of them had chronic kidney injury or were submitted to renal replacement therapy. The present study observed HSPN in almost one half of patients in the first months of disease, and HSPN was associated with persistent purpura and gastrointestinal bleeding. One fourth of patients had nephritis only evidenced during follow-up without severe renal manifestations.

Risk Factors Associated with Renal Involvement in Childhood Henoch-Schönlein Purpura: A Meta-Analysis.

Background and objectiveHenoch-Schönlein purpura (HSP) is an important cause of chronic kidney disease in children. This meta-analysis identified risk factors associated with renal involvement in childhood HSP.MethodsPubMed, Embase, and Web of Science were searched. The quality of all eligible studies was assessed using the Newcastle-Ottawa scale criteria. An analysis of possible risk factors was conducted to report the odds ratio (OR) and weighted mean difference (WMD).ResultsThirteen studies (2398 children) revealed 20 possible and 13 significant risk factors associated with renal involvement in HSP, with the following meta-analysis estimates of OR and WMD, with 95% confidence intervals: older age (0.90, 0.61–1.19); age > 10 y (3.13, 1.39–7.07); male gender (1.36, 1.07–1.74); abdominal pain (1.94,1.24–3.04); gastrointestinal bleeding (1.86, 1.30–2.65); severe bowel angina (3.38, 1.17–9.80); persistent purpura (4.02, 1.22–13.25); relapse (4.70, 2.42–9.14); WBC > 15 × 109/L (2.42, 1.39–4.22); platelets > 500 × 109/L (2.98, 1.22–7.25); elevated antistreptolysin O (ASO) (2.17, 1.29–3.64); and decreased complement component 3 (C3) (3.13, 1.62–6.05). Factors not significantly associated with renal involvement were: blood pressure; orchitis; elevated C-reactive protein; elevated erythrocyte sedimentation rate (ESR); and elevated serum IgA/IgE or IgG. Arthritis/arthralgia may be a risk factor according to the criteria of the American College of Rheumatology (1.41, 1.01–1.96).ConclusionThe following are associated with renal involvement in pediatric HSP: male gender; > 10 y old; severe gastrointestinal symptoms (abdominal pain, gastrointestinal bleeding, and severe bowel angina); arthritis/arthralgia; persistent purpura or relapse; WBC > 15 × 109/L; platelets > 500 × 109/L; elevated ASO; and low C3. Relevant clinical interventions for these risk factors may exert positive effects on the prevention of kidney disease during the early stages of HSP. However, the results should be interpreted cautiously due to the limitations of the studies.

Obesity increases the risk of renal involvement in children with Henoch-Schönlein purpura.

The main aim of this study was to evaluate the relationship between obesity and renal involvement in children with Henoch-Schönlein purpura (HSP). A retrospective study of 141 pediatric patients with HSP was conducted in our hospital. The clinical data of all patients were collected from the electronic medical record management system from January 2010 to June 2014. The possible risk factors of renal involvement, especially obesity, were analyzed using univariate and multivariate analyses. Renal involvement occurred in 45/141 of the patients. A univariate analysis showed that an age more than 7 years at onset, persistent purpura, obesity, time from symptoms onset to diagnosis more than 14 days, and decreased C3 all increased the risk of renal involvement in HSP. The forward stepwise logistic regression analysis indicated obesity (odds ratio (OR) 4.43, 95 % confidence interval (CI) 1.896 to 10.358), age more than 7 years at onset (OR 2.81, 95 % CI 1.142 to 6.907), and persistent purpura (OR 2.57, 95 % CI 1.119 to 5.909) were independent risk factors for renal involvement. Our results show that obesity can increase the hazard of renal involvement in children with HSP and reconfirm that older age at onset and persistent purpura are the independent risk factors for renal involvement. • There have been some reports that obesity was associated with the development of renal injury. • It is not clear whether obesity can increase the risk of renal involvement in children with HSP. What is New: • The main finding of this study is that obesity can increase the hazard of renal involvement in children with HSP.

Henoch Schonlein Purpura, with Special Reference to Renal Involvement: An Experience at SKIMS

BACKGROUND: Henoch Schonlein Purpura (HSP) or anaphylactoid purpuracharacterized by purpura, arthritis, abdominal pain, glomerulonephritis and gastrointestinal bleed, is one of the most common types of vasculitis of childhood. We see patients of HSP from all parts of Kashmir in our department at Sher-i-Kashmir Institute of Medical Sciences (SKIMS). Many of these patients develop complications and invariably have critical hospital course.&#x0D; OBJECTIVES: To study the epidemiology, clinical spectrum and outcome of HSP patients seen at SKIMS.&#x0D; METHODS: Patients of HSP who visited our hospital over last 6 years, between November 2005 and October 2011, were studied for the epidemiological aspects, clinical presentation, complications, duration of purpura and response to treatment in the our Pediatric department.&#x0D; RESULTS: HSP patients constituted approximately 0.32% of total admissions. Purpura was present in all patients, 83.95% had arthritis, 80.24% had abdominal symptoms, 35.80% patients had persistent purpura and 34.56% patients had nephritis&#x0D; CONCLUSION: Majority of HSP patients are in the age group of 6-12 years. Renal involvement is more common in older age group; steroids seem have a role in preventing the severity of nephritis in HSP. JMS 2012;15(2):128-31

Kampo medicines improved blood test and QOL in two vasculitis cases of Churg&amp;ndash;Strauss syndrome and Henoch&amp;ndash;Sh&amp;ouml;nlein purpura after inadequate treatment with conventional therapies

Based on the tenets of traditional Chinese medicine (TCM) theory, Kampo medicines were selected and applied to two cases of Churg-Strauss syndrome and Henoch-Shönlein purpura. Two vasculitis syndrome patients exhibited persistent symptoms and abnormal blood tests after treatment with conventional therapies. As the two cases had "blood stagnation" and "damps and heat" and one had a "yang deficiency" in terms of TCM theory, we applied certain selected Kampo medicines. In case 1, the patient presented with hypereosinophilia, venous thrombosis, pulmonary infarction, decreased platelet count, ulner nerve palsy and Raynaud's phenomena, which led to a diminished quality of life. After starting the Kampo medicines, the patient improved quickly and recovered within 11 months. In case 2, persistent purpura, abdominal pain, and bloody feces quickly improved and disappeared after Kampo treatment. After starting the Kampo medicines, prednisolone was stopped at 21 days without any sign of relapse to date. Kampo medicines helped clear the persistent abnormal symptoms and laboratory findings of vasculitis syndromes, Churg-Strauss syndrome and Henoch-Shönlein purpura, which had responded inadequately to the conventional therapies administered.

Predictive factors for nephritis, relapse, and significant proteinuria in childhood Henoch–Schönlein purpura

Objective: To identify predictive factors for nephritis, relapse, and significant proteinuria in childhood Henoch–Schönlein purpura (HSP).Methods: Two hundred and six consecutive patients with HSP (93 female, 113 male), followed up at a single centre between 1996 and 2001, were analysed retrospectively. They were regularly monitored for clinical and laboratory parameters for renal sequelae and relapse.Results: Nephritis was seen in 78 patients (38%), relapse in 52 (25%), and significant proteinuria in 39 (19%). In univariate analysis, an older age at onset (>10 years), persistent purpura, severe bowel angina, and relapse were identified as factors associated with nephritis and significant proteinuria. Relapse‐related factors were an older age, persistent purpura, severe bowel angina, and leucocytosis. Logistic regression analysis showed that nephritis was significantly associated with an older age, persistent purpura, and relapse, and significant proteinuria was closely related to severe bowel angina and relapse.Conclusion: We identified some predictors for nephritis, relapse, and significant proteinuria in childhood HSP, and close attention should be paid to those patients with the risk factors, such as an older age at onset, persistent purpura, severe bowel angina, and relapse.

Risk factors of renal involvement and significant proteinuria in Henoch-Schönlein purpura

Risk factors of renal involvement and significant proteinuria in patients with Henoch-Schönlein purpura (HSP) were retrospectively evaluated by univariate and multivariate analyses. The analysis was performed in 134 patients with HSP. Renal involvement was found in 65 patients (49%) and 97% of the renal involvement was found within 3 months of disease onset. Moderate or severe proteinuria was recognised in 25 patients. A univariate analysis revealed that an age of more than 4 years at the onset, severe abdominal pain with gastrointestinal bleeding, persistent purpura over a month, coagulation factor XIII activity < 80%, and treatment with factor XIII concentrate were associated with developing renal involvement. A multivariate analysis showed that severe abdominal symptoms, an age of more than 4 years, and persistent purpura increased the risk of renal involvement. Risk factors of moderate or severe proteinuria were also examined. The risk factors in a univariate analysis were severe abdominal symptoms, persistent purpura, decreased factor XIII activity, treatment with steroids, and treatment with factor XIII concentrate. Of those, persistent purpura, treatment with factor XIII concentrate, and factor XIII activity < 80% were associated with significant proteinuria in a multivariate analysis. Among the patients with severe abdominal symptoms, factor XIII activity was significantly decreased in patients with significant proteinuria compared to other patients without significant proteinuria. Long-term prognosis of Henoch-Schönlein purpura is dependent on the severity of renal involvement. In those patients who have the risk factors of renal involvement, especially significant proteinuria, close attention should be paid to a urinalysis for at least 3 months from the onset of the disease.

Renal involvement in Henoch-Schönlein purpura: A multivariate analysis of prognostic factors

This study is the first report in which the relationship between the progression of renal involvement in Henoch-Schönlein purpura (HSP) and various factors was evaluated using a multivariate analysis. Sixty-five (33.5%) of 194 patients with HSP developed renal involvement from three days to 17 months after the onset of the disease. The plasma coagulation factor XIII (F XIII) activity of 97 patients was examined, and 51 (54.3%) of them showed a decreased activity. A univariate analysis showed that an age at the onset of more than seven years, persistent purpura and a decreased F XIII activity all increased the risk of developing renal involvement. A Cox regression model analysis indicated severe abdominal symptoms, persistent purpura and decreased F XIII activity to be significant risk factors and their hazard ratios were 3.26, 11.53 and 2.27, respectively. Corticosteroid treatment had a hazard ratio of 0.36 and was considered to decrease the risk of developing renal involvement. Based on these findings, patients who have the risk factors for renal involvement should be treated with corticosteroids at the onset of the disease to prevent developing renal involvement.

Immunotactoid Glomerulopathy and Cutaneous Vasculitis

A 22-year-old woman presented glomerulonephritis with Schönlein-Henoch-like syndrome and monoclonal abnormality. One month later, she developed a rapidly progressive glomerulonephritis with hypertension and persistent purpura. In the two renal biopsies performed during the first and the second attack, mesangial expansion and thickening of the glomerular capillary walls (associated with 50% of crescents in the second biopsy) were observed on light microscopy. By immunofluorescence faint deposits of immunoglobulins (light and heavy chains) and complement components were found present in the mesangium. Electron microscopy showed tubular microfibrils measuring 19-24 nm in the mesangium, subendothelial and subepithelial areas. A skin biopsy performed during the first attack demonstrated leukocytoclastic skin vasculitis. By immunofluorescence, no deposits were observed. Congo red staining for amyloid and cryoglobulinemia were negative. This case is similar to an entity recently described and named immunotactoid glomerulopathy.

Remission of Macroglobulinemia Coincident With Hepatitis

Monoclonal gammopathies may be either associated with manifestations of multiple myeloma or macroglobulinemia (Waldenstrom) or unassociated with clinically apparent illness.1-4Diminution or disappearance of M-proteins has been observed following chemotherapy.4-8Rare instances of spontaneous remission of monoclonal gammopathies have been reported.9-20In the patient to be presented, a complete remission of macroglobulinemia (Waldenstrom) with hyperviscosity syndrome occurred coincident with an episode of hepatitis. The association of hepatitis and remission of monoclonal gammopathy21-23may not be fortuitous and may suggest a cause-effect relationship. Patient Summary A 59-year-old Latin American man was first hospitalized on Jan 16, 1965, with purpura of the legs precipitated by exposure to cold weather. No etiology was established. The patient was readmitted on Feb 18, 1965, because of pedal edema and persistent purpura. Examination revealed only the presence of purpuric, ulcerated lesions on the lower legs and feet and bilateral pedal edema. Serum