Modern diagnostic methods for early assessment of the abdominal involvement in Schönlein-Henoch disease.

INTRODUCTION: Schönlein-Henoch disease is a small vessel vasculitis resulting from IgA-mediated inflammation. It is the most common acute systemic vasculitis in childhood, mainly affecting the skin, gastrointestinal tract, joints, and kidneys. Although the prognosis of Schönlein-Henoch is generally good, gastrointestinal tract involvement is a potential complication, presenting as massive gastrointestinal bleeding, bowel infarction, perforation, as well as intussusception and peritonitis.

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.

An Assessment on the Effectiveness of the Immature Granulocyte Percentage in Predicting Internal Organ Involvement Among Children With Henoch-Schönlein Purpura.

Henoch-Schönlein purpura (HSP) is the most common childhood systemic vasculitis. The present study aims to investigate the effectiveness of the immature granulocyte (IG) percentage as a new marker for predicting internal organ involvement in HSP. This study included 75 patients below 18 years old who were diagnosed with HSP. The mean age was 7.48±2.77 years. The male/female ratio was 1.14. The findings showed that 35 (46.7%) of the patients had an internal organ involvement. The mean IG percentage was 0.88±0.68 among the patient group with HSP internal organ involvement, while it was 0.31±0.15 in the group without internal organ involvement, and a significant difference was determined between the 2 groups (P=0.000). The findings showed that the patients with renal involvement had the highest mean IG percentage (IG; 1.00±0.21). When the cutoff value for the IG percentage was specified as 0.45 to predict internal organ involvement, the sensitivity was 77.1%, and the specificity was 85%. In this study, the findings showed that IG percentage increased among patients with internal organ involvement in HSP and that its sensitivity, specificity, and predictive values were higher in predicting internal organ involvement compared with other markers.

National Trends in the Epidemiology and Resource Use for Henoch-Schönlein Purpura (IgA Vasculitis) Hospitalizations in the United States From 2006 to 2014.

OBJECTIVES: We examined the trends in the rate of Henoch-Schönlein purpura (HSP) hospitalizations and the associated resource use among children in the United States from 2006 through 2014. METHODS: Pediatric hospitalizations with HSP were identified by using International Classification of Diseases, Ninth Revision, code 287.0 from the National Inpatient Sample. HSP hospitalization rate was calculated by using the US population as the denominator. Resource use was determined by length of stay (LOS) and hospital cost. We used linear regression for trend analysis. RESULTS: A total of 16 865 HSP hospitalizations were identified, and the HSP hospitalization rate varied by age, sex, and race. The overall HSP hospitalization rate was 2.4 per 100 000 children, and there was no trend during the study period. LOS remained stable at 2.8 days, but inflation-adjusted hospital cost increased from $2802.20 in 2006 to $3254.70 in 2014 (P < .001). CONCLUSIONS: HSP hospitalization rate in the United States remained stable from 2006 to 2014. Despite no increase in LOS, inflation-adjusted hospital cost increased. Further studies are needed to identify the drivers of increased hospitalization cost and to develop cost-effective management strategies.

Analysis of neutrophil to lymphocyte ratio, platelet to lymphocyte ratio and mean platelet volume to platelet count ratio in children with acute stage of immunoglobulin A vasculitis and assessment of their suitability for predicting the course of the disease.

Immunoglobulin A vasculitis (IgAV) is the most common systemic vasculitis in developmental age. The disease is most often characterized by a self-limiting course and good prognosis, but sometimes serious complications, like gastrointestinal bleeding or glomerulonephritis, may develop. The neutrophil to lymphocyte (NLR) and the platelet to lymphocyte (PLR) ratios are indicators related to clinical outcome in various inflammatory diseases. The mean platelet volume to platelet count ratio (MPR) has not been evaluated in patients with IgAV. The aim of this study was to analyze the values of the NLR, PLR and MPR in patients with an acute stage of IgAV compared to healthy children and to assess their suitability for predicting the severity of the disease. All children with IgAV hospitalized in our institution between 2012 and 2017 were reviewed retrospectively. The selected laboratory data were recorded before starting the treatment; these results allowed for NLR, PLR, and MPR calculation. The study involved 71 IgAV children. 57.7% of patients revealed signs of systemic involvement (including GT bleeding and/or glomerulonephritis) and 42.3% were nonsystemic (presenting skin and joint symptoms). 83% of patients were classified as mild and 17% as severe course of the disease. The NLR and the PLR were significantly higher in all IgAV children and in the systemic involvement group in comparison with non-systemic. The MPR was significantly lower in all IgAV group with the exception of children without systemic involvement. The NLR is a more valuable indicator than the PLR to identify patients at higher risk of systemic involvement in the course of IgAV. Clinical usefulness of the MPR requires further research.

Henoch-Schönlein purpura nephritis in children: incidence, pathogenesis and management.

BACKGROUND: Henoch-Schönlein purpura (HSP) is one of the most common vasculitides in children. It is manifested by skin purpura, arthritis, abdominal pain, renal involvement, etc. Typically, HSP is considered to be self-limiting, although renal involvement (HSP purpura nephritis, HSPN) is the principal cause of morbidity from this disease. For this reason, it is important to clarify the mechanism of onset and clinical manifestations of HSPN and to ascertain the most appropriate treatment for HSPN. In this article, we review the updated pathophysiology and treatment strategies for HSPN. DATA SOURCES: We searched databases including PubMed, Elsevier and Wanfang for the following key words: Henoch-Schönlein purpura, nephritis, mechanism and treatment, and we selected those publications written in English that we judged to be relevant to the topic of this review. RESULTS: Based on the data present in the literature, we reviewed the following topics: 1) the possible pathogenesis of HSPN: several studies suggest that immunoglobulin A immune complexes deposit in the mesangium and induce renal injury; 2) multiple-drug treatment for HSPN: although there have been few evidence-based treatment strategies for HSPN, several studies have suggested that immunosuppressive drugs and multiple drug combination therapy were effective in ameliorating proteinuria and histological severity. CONCLUSIONS: HSPN is a severe disease of childhood. To better understand this disease, detailed investigations into the pathogenesis of HSPN and prospective randomized controlled treatment studies on children with severe HSPN are needed.

A systematic review of validated methods to capture several rare conditions using administrative or claims data.

PURPOSE: To identify and assess billing, procedural, or diagnosis code, or pharmacy claim-based algorithms used to identify the following health outcomes in administrative and claims databases: acute disseminated encephalomyelitis (ADEM), optic neuritis, tics, and Henoch Schönlein purpura (HSP). METHODS: We searched the MEDLINE database from 1991 to September 2012 using controlled vocabulary and key terms related to the conditions. We also searched the reference lists of included studies. Two investigators independently assessed the full text of studies against pre-determined inclusion criteria and extracted case validation data from those studies meeting inclusion criteria. RESULTS: Two eligible studies addressed ADEM, two addressed optic neuritis, and four studies addressed tics. Only one study addressed HSP. Among these, one study of ADEM reported a positive predictive value of 66%, however the identification algorithm contained a combination of International Classification of Diseases (ICD) codes and other identification methods and the performance of the ICD-9 codes alone was not reported. No other studies reported validation data. CONCLUSIONS: The lack of data on the validity of algorithms to identify these conditions may hamper our ability to determine incidence patterns with respect to infection and vaccination exposures. Further epidemiologic research to define validated methods of identifying cases could improve surveillance using large linked healthcare databases.

Performance of Birmingham Vasculitis Activity Score and disease extent index in childhood vasculitides.

OBJECTIVES: To evaluate the performance of the Birmingham Vasculitis Activity Score (BVAS) v3 and the Disease Extent Index (DEI) for the assessment of disease activity in 4 primary childhood (c-) systemic vasculitides. METHODS: Patients fulfilling the EULAR/PRINTO/PRES (Ankara) c-vasculitis classification criteria for Henoch-Schönlein purpura (HSP), childhood (c) polyarteritis nodosa (c-PAN), c-Wegener's granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) with disease duration at the time of diagnosis ≤3 months were extracted from the PRINTO database. The performance of the BVAS and DEI were examined by assessing convergent validity, the pattern of disease involvement, and responsiveness. We also evaluated alternative unweighted scoring methods for both tools. RESULTS: The analysis set included 796 patients with 669 HSP, 80 c-PAN, 25 c-WG and 22 c-TA. The median age at diagnosis was 6.9 years (6.6-12) and median delay in making the diagnosis from the onset of signs/symptoms was 0.01 (0.003-0.027) years. A strong correlation was found between the BVAS and DEI (rs=0.78) while correlation with the physician global assessment was moderate (rs=0.48) with BVAS and poor with DEI (rs=0.25). Both the BVAS and DEI sub-scores and total scores were able to descrive the disease involvement in the 4 childhood vasculitides. Responsiveness was large (>1.5) for both tools. The performance characteristics of the BVAS and DEI with the unweighted methods were comparable. CONCLUSIONS: This study demonstrates that both the BVAS and DEI are valid tools for the assessment of the level of disease activity in a large cohort of childhood acute and chronic vasculitides.

[Dapsone: an opportunity to spare corticoid use for Henoch-Schönlein treatment? A case report]. / La dapsone: une économie de corticoïdes dans le traitement du purpura rhumatoïde? A propos d'un cas observé.

Dapsone is a sulfone essentially used to treat leprae. We describe, here, a somewhat rare indication of dapsone: corticoid sparing in the treatment of particular forms of Henoch-Schönlein.

Practical aspects of treatment with drotrecogin alfa (activated).

In November 2001, drotrecogin alfa (activated) was approved by the US Food and Drug Administration; in August 2002 it was approved by the European Medicines Agency. Since the approval of drotrecogin alfa (activated), however, critical care physicians have been faced with several challenges, namely its costs, selection of patients who are more likely to benefit from it, and the decision regarding when to start drotrecogin alfa (activated) treatment. There are also operational issues such as how to manage the infusion to deliver an effective treatment while minimizing the risk for bleeding, particularly in patients with deranged clotting, at around the time of surgery or during renal replacement therapy. While addressing these issues, this review remains practical but evidence based as much as possible.

Stillbirths with placental hemorrhagic endovasculitis: a morphologic assessment with clinical implications.

CONTEXT: Hemorrhagic endovasculitis (HEV) is a vasodisruptive alteration affecting fetal-placental blood vessels of all calibers. Hemorrhagic endovasculitis is found in association with stillbirth and abnormalities of growth and development in livebirths. The role of HEV in the pathogenesis of these conditions is not known. OBJECTIVE: To further understand these events, we compare clinicopathologic features of HEV-affected placentas from stillbirths with those from livebirth pregnancies. Additionally, we assess the relationship of morphologic forms of HEV to clinical events and time of fetal death in utero and evaluate the significance of extensive versus localized HEV lesions in placentas of stillbirths. DESIGN: We reviewed the clinical records and slides from 119 stillbirths with placentas affected by HEV classified above a specified severity level (cases) and 119 matched stillbirths with placentas not affected by HEV (controls). A subset of 21 stillbirth placentas exhibiting focal HEV lesions was similarly evaluated. Slides were graded for HEV, villitis of unknown etiology, chorionic thrombi, villous fibrosis, erythroblastosis, and lesions indicative of maternal hypertension. Hemorrhagic endovasculitis was subcategorized into active, bland, and healed forms and clustered capillary lesions (hemorrhagic villitis). Focal, segmental, and diffuse patterns of villous fibrosis were delineated. Interlesional relationships were established by matching HEV severity indices with severity indices of co-existing lesions. Timing of fetal death was determined by published criteria. Data were analyzed for significance using chi2 and t tests. Results were compared with published analyses of livebirths with placental HEV. RESULTS: Lesions occurring with significant frequency in HEV-affected (case) placentas include villitis of unknown etiology, chorionic thrombi, villous fibrosis, erythroblastosis, and meconium staining. Interlesional relationships were evident between HEV and villous fibrosis, villitis of unknown etiology, and chorionic thrombi. Growth restriction was more common in case versus control infants (P = .02). A segmental pattern of villous fibrosis predominated in cases versus controls and within the case group (P < .001). Time to delivery after fetal death was longer in cases than controls. Active-vasodestructive forms of HEV correlate with shorter intervals of intrauterine retention, whereas bland forms correlate with longer intervals (P = .04). Placentas with focal HEV were associated with coexisting chorionic thrombi and villous fibrosis but not with fetal growth restriction. CONCLUSIONS: Patterns of interlesional interplay are similar in HEV-affected placentas of livebirths and stillbirths. This suggests that the pathogenesis of infant morbidity and mortality is similar in both groups. Active-vasodestructive forms of HEV may precede whereas bland forms may follow intrauterine demise. The segmental pattern of villous fibrosis and high incidences of growth restriction, erythroblastosis, and meconium in cases suggests a chronicity of adverse intrauterine events that may precede fetal loss. Stillbirths with focal HEV lesions are probably not at risk.

Placenta within the medicolegal imperative.

Bad pregnancy outcome includes abortion, stillbirth, neonatal death, morbidity, malformation, cerebral palsy, and mental retardation. These afflictions cause devastating personal impact. In the United States, almost 10% of all school-aged children are handicapped. Neurologic and communicative disorders affect 42 million people and cost $114 billion each year. The cause of cerebral palsy is known in less than 10% of these cases. Enormous litigations have threatened clinicians, paramedical personnel, hospitals, and insurance carriers. The placenta is an important potential means of establishing that fetal damage causes bad pregnancy outcome independently of clinical care. All pathologists serve an important role in the documentation of gross placental features and in the procurement of appropriate light microscopic slides. Pathologic placentas are common. Only an expert can determine whether an abnormal placenta represents the probable cause of bad pregnancy outcome.