Clinical characteristics and risk factors for kidney involvement in children with immunoglobulin A vasculitis.

BACKGROUND: Immumoglobulin A (IgA) vasculitis (IgAV), formerly known as Henoch-Schönlein purpura (HSP), is a self-limiting systemic vasculitis in children. Kidney involvement is associated with a long-term unfavorable outcome and can lead to significant morbidity. This study was conducted to describe the clinical and laboratory characteristics of childhood IgAV with kidney involvement and to identify risk factors associated with IgAV nephritis (IgAVN). METHODS: This was an ambidirectional descriptive study of 77 children with IgAV. All demographic data, clinical features, and laboratory tests were collected from electronic medical records from January 2010 to December 2022. Risk factors for kidney involvement in IgAV were assessed using multivariate logistic regression. Kaplan-Meier survival analysis was used to calculate the time to commencement of kidney involvement. RESULTS: Twenty-five children (32.4% of the IgAV patients) developed IgAVN. The common findings in IgAV with kidney involvement were microscopic hematuria (100%), nephrotic range proteinuria (44%), and non-nephrotic range proteinuria (40%). Multivariate logistic regression showed that age greater than 10 years (adjusted hazard ratio, AHR 4.66; 95% confidence interval, CI, 1.91-11.41; p = 0.001), obesity (body mass index, BMI, z-score ≥ +2 standard deviations, SDs) (AHR 3.59; 95% CI 1.41-9.17; p = 0.007), and hypertension at onset (AHR 4.78; 95% CI 1.76-12.95; p = 0.002) were associated significantly with kidney involvement. During follow up, most IgAV patients developed nephritis within the first 9 months. CONCLUSION: Age greater than 10 years, obesity, and hypertension at presentation were predictive factors for IgAVN. Our study emphasized that IgAV patients with risk factors should be closely monitored for at least 1 year after the onset of the disease.

Changes in the spectrum of biopsy-proven renal diseases over 11 years: a single-center study in China.

BACKGROUND: There have been some shifts in the frequency and distribution of biopsy-proven renal diseases in China over recent years. The aim of the study was to investigate the changing spectrum of renal diseases from the view of kidney biopsy data in a single center of China. METHODS AND RESULTS: A total of 10,996 cases of native renal biopsies from patients aged ≥15 years old in Huashan Hospital, Fudan University, between 2008 and 2018 were analyzed retrospectively. The results showed that primary glomerular nephropathy (PGN) remained the most common biopsy-proven renal disease (69.42% of total), with IgA nephropathy (IgAN) accounting for 44.40% of PGN, membranous nephropathy (MN) for 28.55%, minimal change disease (MCD) for 13.26% and focal segmental glomerulosclerosis (FSGS) for 8.00%. During the study period, the proportion of MN in PGN appeared an increasing tendency, while that of IgAN and MCD remained stable and that of FSGS showed a decline. Secondary glomerular nephropathy (SGN) constituted 21.54% of total cases, among which the leading two diseases were lupus nephritis (LN) and Henoch-Schonlein purpura nephritis (HSN) which accounted for 41.08% and 19.11% respectively. CONCLUSIONS: The 11-year retrospective study revealed that PGN was the predominant histologic diagnosis among patients undergoing renal biopsy and the most frequent type of PGN remained to be IgAN, followed by MN which increased dramatically.

Adult Henoch-Schönlein Purpura: Comprehensive Assessment of Demographic, Clinical, and Histopathological Features as Predictors for Systemic Involvement.

BACKGROUND: Henoch-Schönlein purpura (HSP) is a small-vessel IgA-predominant vasculitis. A major challenge in managing adult HSP is the difficulty assessing the risk of systemic involvement. There is currently a paucity of data in this area. OBJECTIVE: The objective of this study was to determine demographic, clinical, and histopathological features associated with systemic involvement in adult HSP. METHODS: In this retrospective study, we reviewed demographical features and clinical and pathology data of 112 adult HSP patients seen at Emek Medical Center between January 2008 and December 2020. RESULTS: Of these patients, 41 (36.6%) had renal involvement, 24 (21.4%) had gastrointestinal tract involvement, and 31 (27.7%) had joint involvement. Age >30 years (p = 0.006) at diagnosis was an independent predictor of renal involvement. Platelet count (<150 K/µL) (p = 0.020) and apoptosis of keratinocytes on skin biopsy (p = 0.031) were also associated with renal involvement. History of autoimmune disease (p = 0.001), positive c-antineutrophil cytoplasmic antibody (p = 0.018), positive rheumatoid factor (p = 0.029), and elevated erythrocyte sedimentation rate (p = 0.04) were associated with joint involvement. Female sex (p = 0.003), Arab race (p = 0.036), and positive pANCA (p = 0.011) were associated with gastrointestinal tract involvement. LIMITATIONS: This study is retrospective. CONCLUSION: These findings may serve as a guide to stratify risk in adult HSP patients so that those at higher risk can be monitored more closely.

Risk due to Elevated Uric Acid Levels in Children With Henoch-Schonlein Purpura.

PURPOSE: To compare uric acid levels in children with Henoch-Schonlein purpura (HSP)without nephritis and with renal damage, and at different pathological grades. METHODS: A total of 451 children were enrolled in this study, including 64 with HSP without nephritis and 387 HSP with kidney damage. Age, gender, uric acid, urea, creatinine and cystatin C levels were reviewed. Pathological findings of those with renal impairment were also reviewed. RESULTS: Among the HSP children with renal damage, 44 were grade I, 167 were grade II and 176 were grade III. There were significant differences in age, uric acid, urea, creatinine and cystatin C levels between the two groups (p<0.05, all). Correlation analysis showed that uric acid levels in children with HSP without nephritis were positively correlated with urea and creatinine levels (p<0.05). Uric acid levels in HSP children with renal damage was positively correlated with age, urea, creatinine and cystatin C levels (p<0.05, all). Regression analysis found that, without adding any correction factors, there were significant differences in uric acid levels between the two groups; however, after adjusting for pathological grade, there was no longer a significant difference. CONCLUSIONS: There were significant differences of uric acid levels in children with HSP without nephritis and with renal impairment. Uric acid levels in the renal impairment group were significantly higher than that in the HSP without nephritis group. Uric acid levels were related to only the presence or absence of renal damage, not to the pathological grade.

New insights into epidemiological data and impact of the COVID-19 pandemic on IgA vasculitis in children and adults: a French nationwide cohort.

IgA vasculitis (IgAV) is a small size vasculitis for which epidemiologic data are strikingly lacking, especially about the adult form. Additionally, the COVID-19 pandemic seems to have profoundly modified the incidence of this disease. Here, we aimed to establish some relevant epidemiological data in both pediatric and adult IgAV. We performed an observational study using a national database called "BNDMR" on IgAV, which gathers patients managed in the French network of experts on rare diseases. We primarily performed descriptive statistics over the 2010-2022 period. Then, we compared the North-South geographical areas, the seasonality, and the impact of COVID-19 with that of other patients reported in the same centers. We collected data from 1988 IgAV patients. The sex ratio was 1.57 for adults and 1.05 for children. The annual incidence in 2021 was 0.06 for 100,000 adults and 0.50 for 100,000 children. Compared with other diseases reported into the BNDMR, IgAV was more common in the South than in the North of France (OR 4.88 [4.17-5.74] in adults and OR 1.51 [1.35-1.68] in children). IgAV was also observed more frequently in winter and autumn. Strikingly, we observed a decrease in incidence during the COVID-19 pandemic period in children (OR 0.62 [0.47-0.81]). Our study provides both new insights and confirmations of IgAV epidemiological data: winter and autumn seasonality, more pronounced male predominance in adults, decreasing incidence of pediatric IgAV during the COVID-19 pandemic and increasing incidence in the South of France.

Online information-seeking behavior of Iranian web users on Google about Henoch-Schönlein purpura (HSP): an infodemiology study.

BACKGROUNDS: Previous studies have indicated that users' health information-seeking behavior can serve as a reflection of current health issues within a community. This study aimed to investigate the online information-seeking behavior of Iranian web users on Google about Henoch-Schönlein purpura (HSP). METHODS: Google Trends (GTr) was utilized to collect big data from the internet searches conducted by Iranian web users. A focus group discussion was employed to identify users' selected keywords when searching for HSP. Additionally, keywords related to the disease's symptoms were selected based on recent clinical studies. All keywords were queried in GTr from January 1, 2012 to October 30, 2022. The outputs were saved in an Excel format and analyzed using SPSS. RESULTS: The highest and lowest search rates of HSP were recorded in winter and summer, respectively. There was a significant positive correlation between HSP search rates and the terms "joint pain" (P = 0.007), "vomiting" (P = 0.032), "hands and feet swelling" (P = 0.041) and "seizure" (P < 0.001). CONCLUSION: The findings were in accordance with clinical facts about HSP, such as its seasonal pattern and accompanying symptoms. It appears that the information-seeking behavior of Iranian users regarding HSP can provide valuable insights into the outbreak of this disease in Iran.

[Risk factors associated with different types of Henoch-Schönlein purpura in Tibetan patients at high altitude].

OBJECTIVE: To investigate the risk factors of different types of Henoch-Schönlein purpura (HSP) in Tibetan patients at high altitude, as to provide reference for correctly identifying high-risk patients. METHODS: A retrospective study was used to analyze the 304 HSP patients admitted to Tibet Autonomous Region People's Hospital from April 2014 to March 2022. The gender, age, allergic history, family history, clinical type, laboratory indexes (hemoglobin, platelet count, eosinophil, C-reactive protein (CRP), albumin, immunoglobulin G, immunoglobulin A, complement C3 and C4) were analyzed retrospectively. Univariate and multivariate Logistic regression analysis to screen for risk factors affecting different types of HSP. RESULTS: Renal HSP patients showed higher IgA [(9.2±1.7) g/L vs. (6.4±2.4) g/L, P=0.015], lower complement C3 [(203.3±21.6) mg/dL vs. (301.1±19.5) mg/dL, P=0.043], and complement C4 [(33.5±2.3) mg/dL vs. (53.0±7.2) mg/dL, P=0.032]. The patients with abdominal HSP showed lower levels of hemoglobin [(119.6±19.6) g/L vs. (146.6±47.3) g/L, P=0.038] and plasma albumin [24.8 (22.1, 33.9) g/L vs. 32.6 (24.6, 35.1) g/L, P=0.045]. The patients with articular HSP exhibited higher CRP [13.5 (0.2, 20.6) g/L vs. 7.5 (0.1, 15.2) g/L, P=0.036] and erythrocyte sedimentation rate (ESR) [24 (5, 40) mm/h vs. 15 (4, 30) mm/h, P=0.049]. Elevated IgA and decreased complement C4 were risk factors for renal HSP, anemia and decreased plasma albumin were risk factors for abdominal HSP, and elevated CRP was a risk factor for articular HSP. CONCLUSION: The clinical characteristics of different types of HSP in plateau areas were different. Patients with high IgA, low complement C4, anemia, hypoalbuminemia, and significantly elevated CRP should be highly vigilant. Early and effective intervention can improve the clinical efficacy, avoid severe development, and improve the prognosis.

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.

Epidemiology and Clinical Characteristics of Henoch-Schönlein Purpura Associated with Streptococcal Infection in 217 Children in Hubei Province, China.

Objectives The objectives of present study were to analyze the association of the streptococcal infection with childhood Henoch-Schönlein purpura (HSP) in China. Methods: We performed a case-control study over a period of five years to evaluate the epidemiology and clinical characteristics of group A ß-hemolytic streptococcal (GABHS) triggered HSP. Results: 1. The frequency of GABHS-triggered HSP was 15.1%, while that of GABHS infection developing HSP in children was 4.7%. 2.The epidemiological characteristics of HSP with streptococcal infection were similar to those of HSP alone. 3. The GABHS-triggered HSP cases had a significantly higher frequency of renal involvement than the noninfectious group. 4. IgA and IgG were significantly increased in the streptococcal infection group than in the noninfectious group, while the levels of C3 and C4 decreased significantly. Conclusions: GABHS infection is the most frequent agent in HSP children, and may aggravate the immune dysfunction and prolong the course of HSP.

Morbidity and mortality in adult-onset IgA vasculitis: a long-term population-based cohort study.

OBJECTIVES: With sparse data available, we investigated mortality and risk factors in adults with IgA vasculitis (IgAV). METHODS: This was an observational population-based cohort study using state-wide linked longitudinal health data for hospitalized adults with IgAV (n = 267) and matched comparators (n = 1080) between 1980 and 2015. Charlson comorbidity index (CCI) and serious infections (SIs) were recorded over an extensive lookback period prior to diagnosis. Date and causes of death were extracted from the Western Australia Death Registry. Mortality rate (deaths/1000 person-years) ratios (MRRs) and hazard ratio (HR) for survival were assessed. RESULTS: During 9.9 (9.8) years lookback patients with IgAV accrued higher CCI scores (2.60 vs 1.50, P < 0.001) and had higher risk of SI (OR = 8.4, P < 0.001), not fully explained by CCI scores. During 19 years' follow-up, the rate of death in patients with IgAV (n = 137) was higher than in comparators (n = 397) (MRR = 2.06, 95% CI: 1.70-2.50; P < 0.01) and the general population (standardized mortality rate ratio = 5.64, 95% CI: 4.25, 7.53; P < 0.001). Survival in IgAV was reduced at 5 (72.7 vs 89.7%) and 20 years (45.2% vs 65.6%) (both P < 0.05). CCI (HR = 1.88, 95% CI: 1.25, 2.73; P = 0.001), renal failure (HR = 1.48, 95% CI: 1.04, 2.22; P = 0.03) and prior SI (HR = 1.48, 95% CI: 1.01, 2.16; P = 0.04) were independent risk factors. Death from infections (5.8 vs 1.8%, P = 0.02) was significantly more frequent in patients with IgAV. CONCLUSION: Premorbid comorbidity accrual appears increased in hospitalized patients with IgAV and predicts premature death. As comorbidity does not fully explain the increased risk of premorbid infections or the increased mortality due to infections in IgAV, prospective studies are needed.

Clinical spectrum and outcome of immunoglobulin A vasculitis in children: A 10-year clinical study.

BACKGROUND: Immunoglobulin A (IgA) vasculitis is the most common vasculitides of childhood. The aim of this study was to characterise the disease and identify clinical and laboratory factors associated with renal involvement. METHODS: Medical records of children aged ≤18 years with the diagnosis of IgA vasculitis according to the EULAR/PRES criteria were retrospectively reviewed for demographic data, clinical manifestations and laboratory tests. Factors associated with renal involvement were determined using multivariable logistic regression analysis. RESULTS: A total of 113 patients were involved in the study. There were 57.5% of males (male: female ratio of 1.4:1). The mean (SD) age was 7.1 (3.0) years. Most of the patients presented during the fall (37.2%) and winter (29.2%). The most common presenting complaints were skin rash (83.2%) and arthralgia (9.7%). The most frequent presentation of the skin was palpable purpuric rash, except for two patients (1.8%) presenting with blistering eruptions in the form of hemorrhagic bullae and ecchymoses of both extremities. We have encountered one patient with pulmonary-renal syndrome. Renal involvement was found in 27.4% of the patients. In the multivariate analysis, the strongest factors associated with renal involvement were older age and elevated creatinine level. Factors that improved model fit were higher platelet and lower white blood cell counts, elevated anti-streptolysin O and a history of preceding infection. CONCLUSION: The clinical profile of the disease in our cohort was similar to other reports. However, we have encountered a few severe and rare atypical presentations. Over a quarter of children in our setting had renal involvement. Children presenting with these risk factors need to be closely monitored and long-term follow-up is recommended.

Effects of dexamethasone and gamma globulin combined with prednisone on the therapeutic effect and immune function of Henoch-Schonlein purpura nephritis in children.

BACKGROUND: Henoch-Schonlein purpura nephritis (HSPN) is a serious complication of Henoch-Schonlein purpura (HSP), which is usually treated with immunosuppressant and glucocorticoid. This study was designed to explore the effect of dexamethasone and gamma globulin combined with prednisone in the treatment of pediatric HSPN. METHODS: According to the treatment plan, 60 children treated with dexamethasone and gamma globulin were included in the control group, and the rest 55 children treated with dexamethasone and gamma globulin combined with prednisone were selected as the research group. The clinical manifestations, therapeutic effect, immune function, serum inflammatory factors, blood coagulation function, urine routine, renal function, and adverse reactions were compared between the two groups. RESULTS: The clinical manifestations of children in the research group were significantly better than those in the control group after treatment (P < .05). The total effective rate in the research group (94.55%) was markedly higher than that in the control group (76.67%) (P < .05). CD3+, CD4+, CD8+, IL-10, PT, and APTT increased while CD4+/CD8+, IgA, IL-8, TNF-α, FIB, urine protein, urine red blood cell, Scr, and BUN decreased in both groups after treatment, and the changes of all the above indexes in the research group were significant than those in the control group (P < .05). The incidence of adverse reactions in the research group was remarkably superior to that in the control group (P < .05). CONCLUSION: Dexamethasone and gamma globulin combined with prednisone can improve the immune function of children with HSPN and promote the recovery of renal function.

[Analysis of clinical characteristics of Henoch-Schonlein purpura patients from different altitudes in plateau areas].

OBJECTIVE: To investigate the clinical characteristics of Henoch-Schonlein purpura (HSP) patients from different altitudes in Tibet plateau areas of China. METHODS: A retrospective study was used to analyze the 190 HSP patients admitted to Tibet Autonomous Region People ' s Hospital form April 2014 to May 2021. The subjects were divided into 3 groups according to the altitude of long-term residence before onset and the clinical data at different altitudes were compared and analyzed. RESULTS: There were no significant differences in the age of onset and gender in HSP patients at different altitudes (P>0.05). The HSP patients in high altitude areas were more likely to have digestive symptoms (P < 0.01). The patients were more likely to have kidney or joint involvement at higher altitudes. The platelets [(512.1±55.0)×109 /L] and C reactive protein [11.2 (5.7, 19.4) g/L] in high altitude areas were significantly higher than at medium altitudes [(498.3±76.9)×109 /L and 9.5 (4.6, 13.5) g/L] and lower altitudes [(456.4±81.2)×109/L and 3.7 (0.2, 8.9) g/L] respectively. The effective rate of treatment was 98.9%, while there was no significant difference of outcome from different altitudes (P>0.05). The patients who were repeatedly hospitalized all had kidney involvement and no immunosuppressive agents were added in the initial treatment. CONCLUSION: HSP is common in high altitude areas. There was little difference in age of onset and gender at different altitudes. Abdominal pain was the most common clinical manifestation. Patients in high altitude areas were more likely to have severe abdominal problems. Kidney involvement may be poor prognostic factor. Early application of glucocorticoid combined with immunosuppressive agents can effectively control the disease and reduce the recurrence of HSP.

[TRENDS IN HOSPITALIZATION OF CHILDREN WITH HENOCH -SCHONLEIN PURPURA].

BACKGROUND: Traditionally, most children diagnosed with Henoch-Schonlein Purpura (HSP) were hospitalized. This policy gradually changed towards selective hospitalization, yet there are still no criteria for admitting pediatric patients with HSP. OBJECTIVES: To examine the clinical features and criteria for hospitalization of pediatric patients with HSP. METHODS: A retrospective analysis was conducted of medical records of pediatric patients with HSP examined in the emergency department (ED) of Schneider Children's Medical Center, during 2005-2015. We compared children who were admitted with those not admitted to the hospital. RESULTS: During the study period, 116 children diagnosed with HSP were examined in the ED of Schneider Children's Medical Center: 14(12%) were admitted at first referral, and 22 (19%) of the children were hospitalized subsequently. The average age of all the children with HSP was 7.4 years, 57% were boys, and approximately 10% of the children had additional diseases, most of them auto-inflammatory. The main indications for hospitalization were gastrointestinal involvement (abdominal pain or bleeding), renal involvement (hematuria or proteinuria), and inability to walk. Most of these symptoms were also present in the children who were not admitted. CONCLUSIONS: Approximately 30% of the children were admitted to the hospital, most of them not at first referral. No clear criteria for admission were found, except for gastrointestinal bleeding.

Comorbidities in familial Mediterranean fever: analysis of 2000 genetically confirmed patients.

OBJECTIVES: FMF is the most common periodic fever syndrome, characterized by recurrent episodes of fever and serosal inflammation accompanied with high acute phase reactants. The analysis of possible comorbidities is important to understand the impact of these conditions on clinical care and whether they share a common aetiological pathway. In this study, we aimed to evaluate the comorbidities associated with FMF patients in a large genetically diagnosed cohort. METHODS: We retrospectively evaluated the medical and genetic records of FMF patients who were followed up by rheumatologists in Hacettepe University for 15 years. The FMF patients who had homozygous or compound heterozygous mutations were included in the study. Comorbidities associated with FMF were divided into three groups: (i) comorbidities directly related to FMF, (ii) comorbidities due to increased innate inflammation, and (iii) comorbidities that were regarded as being incidental. RESULTS: A total of 2000 patients with a diagnosis of FMF were enrolled in the study. Among them 636 were children (31.8%) and M694V was the most common mutation in patients with associated inflammatory conditions. The frequency of AS, Iga Vasculitis (Henoch-Schönlein purpura), juvenile idiopathic arthritis, polyarteritis nodosa, multiple sclerosis and Behçet's disease were increased in patients with FMF when compared with those in the literature. CONCLUSION: This study represents the largest genetically confirmed cohort and compares the frequencies with existing national and international figures for each disease. The increased innate immune system inflammation seen in FMF may be considered as a susceptibility factor since it predisposes to certain inflammatory conditions.

Familial Mediterranean fever is associated with a wide spectrum of inflammatory disorders: results from a large cohort study.

Familial Mediterranean fever (FMF) is characterized by recurrent short-lived/self-limiting inflammatory attacks. Besides these, a substantial number of patients with FMF present with a variety of other inflammatory diseases; however, this issue has not been systematically studied previously. Hence, we aimed to investigate the frequency of inflammatory comorbid diseases in a large FMF cohort. All patients were recruited from "FMF in Central Anatolia (FiCA) Cohort", comprising 971 (mean age 35.3 ± 12 years, 61.5% female) adult subjects. All patients fulfilled Tel Hashomer criteria. Demographic data, FMF disease characteristics, MEFV gene mutations, and comorbid inflammatory diseases were meticulously questioned, and laboratory features and genotype data were retrieved from hospital records. There were comorbid inflammatory diseases in 205 (21.1%) patients. The most common inflammatory disease was spondyloarthritis (12.9%). Other remarkable inflammatory disorders were psoriasis, immunoglobulin A vasculitis/Henoch-Schönlein purpura, Behçet's disease and inflammatory bowel diseases. Cryptogenic organizing pneumonia is a newly defined entity in our cohort which is seemed to be associated with FMF (0.3%). Number of patients with persistent inflammation was higher in those with comorbid diseases (p < 0.001). Our results suggest that FMF is commonly associated with other inflammatory diseases. Therefore, clinicians should be cautious about comorbid inflammatory diseases in FMF patients, particularly in those with persistent inflammation. Identification of pathogenic pathways linking FMF to these diseases warrants further investigations.

Evaluation of co-existing diseases in children with familial Mediterranean fever.

Familial Mediterranean fever (FMF) is A common periodic fever syndrome. The causative gene of the FMF is named Mediterranean Fever gene (MEFV). Increased inflammation in FMF may play a role as a trigger for the development of some diseases. The objective of the study is to evaluate the frequency of comorbid disorders in children followed up with diagnosis of FMF. Additionally, we aimed to assess the association between FMF and other inflammatory conditions in a large pediatric FMF cohort. A total of 686 FMF patients were included in the cross-sectional study. A questionnaire including questions about characteristics of fever episodes, presence of arthralgia, arthritis, abdominal pain, chest pain during and co-existence of any other disease diagnosed by a physician was filled out by face-to-face interviews with patients or their parents. Female-male ratio was 0.85. Median age at the time of study, age at disease onset and at the time of diagnosis were 12.9 (1.7-22.3), 3 (0.08-17), and 6 (0.75-17) years, respectively. In 130 (18.9%) FMF patients we detected co-existing inflammatory condition. The most common co-existing diseases were: juvenile idiopathic arthritis 42 (6.1%), asthma/reactive airway disease 29 (4.2%), Henoch-Schönlein purpura 20 (2.9%), uveitis 12 (1.7%) and inflammatory bowel disease 10 (1.4%). Except for asthma/reactive airway disease and inflammatory bowel disease, there was no significant difference regarding the type of MEFV gene mutation. We have reported increased frequencies of various inflammatory conditions and decreased frequency of asthma in patients with FMF.

Predictive role of laboratory markers and clinical features for recurrent Henoch-Schönlein Purpura in childhood: A study from Turkey.

Objectives: Henoch Schönlein Purpura is the most common systemic vasculitis of the childhood. The objective of this study was to evaluate whether there is a clinical significance of laboratory parameters and clinical features on the prediction of the recurrent Henoch Schönlein Purpura in children.Methods: For recurrent Henoch Schönlein Purpura, several laboratory results and clinical features were examined to identify the predictor factors via the logistic regression model in 99 children with Henoch Schönlein Purpura. A p-value of <.05 was considered significant. The patients were divided into two groups as the first attack and the recurrent.Results: The demographic features and laboratory results were not found statistically significant between the two groups (p > .05). The multiple logistic regression model showed that the risk of recurrent Henoch Schönlein Purpura increased 17.2-fold in children with Henoch Schönlein Purpura affected by the four systems including skin, joint, kidney and gastrointestinal system [Odds Ratio (OR) 17.2, 95% CI 1.4-12; p < .001].Conclusion: In our inpatient population, the laboratory characteristics were detected not to be a factor for the prediction of recurrence. However, the four systems including skin, joint, kidney and gastrointestinal involvement may be used as a predictive factor for recurrent HSP.

A retrospective analysis of children with Henoch-Schonlein purpura and re-evaluation of renal pathologies using Oxford classification.

BACKGROUND: Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood. The long-term prognosis is variable and depends on renal involvement. The aims of this study were to investigate the clinical and laboratory characteristics of our HSP patients, to identify the risk factors for the development of Henoch-Schönlein purpura nephritis (HSPN) and to assess the efficacy of the Oxford Classification system for predicting renal outcomes. METHODS: We performed a retrospective review of HSP patients who admitted to our center between 2001 and 2016, and were < 18 years on admission. RESULTS: A total of 1120 children with HSP were analyzed. Their mean age was 7.4 ± 3.4 years. At onset, purpura was present in all cases, arthritis/arthralgia in 42.4%, abdominal involvement in 39% and renal involvement in 37%. Risk factors for the development of nephritis were age ≥ 8 years, atypical distribution of purpura, ESR > 20 mm/h and abdominal pain. Renal biopsy was performed on 75 patients before immunosuppressive treatment. The mesangial score was strongly associated with proteinuria. Segmental glomerulosclerosis, tubular atrophy/interstitial fibrosis, and crescent formation of ≥ 50% were associated with reduced eGFR at the time of biopsy. A Kaplan-Meier plot showed that segmental glomerulosclerosis and tubular atrophy/interstitial fibrosis significantly predict poor renal outcome. CONCLUSION: The long-term morbidity of HSP is predominantly attributed to renal involvement. Patients with HSP, who have a high risk to develop nephritis, could be followed for longer periods of time. The Oxford classification is useful in predicting long-term outcomes of HSPN.