Galactose-deficient IgA1 in skin and serum from patients with skin-limited and systemic IgA vasculitis. Neufeld Matthias,Molyneux Karen,Pappelbaum Karin I,Mayer-Hain Sarah,von Hodenberg Christina,Ehrchen Jan,Barratt Jonathan,Suzuki Yusuke,Sunderkötter Cord Journal of the American Academy of Dermatology BACKGROUND:IgA vasculitis (IgAV) encompasses a systemic form involving kidneys, gut, skin, or joints, and a skin-limited form. One characteristic feature of systemic IgAV is deposition of galactose-deficient IgA1 (GD-IgA1) in kidneys (as in IgA nephropathy). The relevance of GD-IgA1 for cutaneous vasculitis is unknown. OBJECTIVE:We investigated whether GD-IgA1 is deposited perivascularly in systemic and also skin-limited IgAV and whether its serum levels differ between both forms. METHODS:In a case-control study, deposition of GD-IgA1 was analyzed immunohistochemically by KM55 antibody in skin biopsy specimens from 12 patients with skin-limited IgAV and 4 with systemic IgAV. GD-IgA1 levels were compared by enzyme-linked immunosorbent assay in sera from 15 patients each with skin-limited and systemic IgAV and from 11 healthy individuals. RESULTS:All biopsy samples from systemic IgAV, and also from skin-limited IgAV, revealed perivascular GD-IgA1 deposition. The average GD-IgA1 concentration in serum was significantly higher in systemic IgAV than in skin-limited IgAV, despite overlap between the groups. LIMITATIONS:Although high GD-IgA1 levels may be predictive of systemic IgAV, patient numbers were too low to determine cutoff values for systemic versus skin-limited IgAV. CONCLUSION:Perivascular GD-IgA1 deposition is a prerequisite for systemic and skin-limited IgAV; however, high GD-IgA1 levels in some patients with systemic IgAV suggest a dose-dependent effect of GD-IgA1 in IgAV. 10.1016/j.jaad.2019.03.029

Do practical laboratory indices predict the outcomes of children with Henoch-Schönlein purpura? Ekinci Rabia Miray Kisla,Balci Sibel,Sari Gokay Sinem,Yilmaz Hayri Levent,Dogruel Dilek,Altintas Derya Ufuk,Yilmaz Mustafa Postgraduate medicine : Henoch Schönlein Purpura (HSP) is the most common systemic vasculitis of childhood and often has a self-limiting course. We aimed to study whether practical laboratory parameters at the diagnosis predict disease course including recurrence and nephritis in addition to severe gastrointestinal involvement in children with HSP. : This retrospective cohort study included 214 HSP patients, 43.5% (n = 93) female and 56.5% (n =121) male, who were diagnosed in our department. Laboratory parameters before treatment, including neutrophil, lymphocyte and platelet counts, mean platelet volume (MPV), neutrophil-to-lymphocyte (NLR), and platelet-to-lymphocyte ratios (PLR) were obtained retrospectively. Age at diagnosis, duration of follow-up, gender, preceding infections, medications, arthritis and arthralgia, abdominal pain, severe GI involvement, invagination, renal involvement and presence of nephritis, outcomes, and presence of recurrences were retrospectively recorded from medical files. Severe GI involvement was determined as severe colicky abdominal pain, bowel edema in ultrasonography or overt GI bleeding. A relapse was defined as a new flare of cutaneous lesions or other manifestations in a patient at least four asymptomatic weeks after the initial HSP episode. : Mean age at diagnosis was 7.6 ± 3.1 years. Biopsy-proven nephritis was found in 16 (7.5%) patients. Severe GI involvement was present in 77 (36%) patients, whereas only 12 (5.6%) patients were diagnosed with intussusception and in 29 (13.5%) patients, HSP recurred. Neutrophil count and NLR were found higher in HSP patients with severe gastrointestinal involvement and biopsy-proven nephritis. Additionally, only platelet count was lower and MPV was higher in patients with recurrent HSP. : Elevated neutrophil count and NLR may be relevant markers for severe GI involvement and nephritis, whereas platelet count and MPV were the only laboratory parameters associated with disease recurrence. 10.1080/00325481.2019.1609814

Neutrophil-to-lymphocyte ratio to predict gastrointestinal bleeding in Henoch: Schönlein purpura. Hong Seung Hee,Kim Chan Jong,Yang Eun Mi Pediatrics international : official journal of the Japan Pediatric Society BACKGROUND:Henoch-Schönlein purpura (HSP) is a common form of vasculitis in children. It typically involves small vessels of the skin, the gastrointestinal (GI) tract, joints, and kidneys. GI involvement is the most severe short-term complication and GI bleeding is a major complication of HSP, but there is no established predictive marker of GI bleeding. Blood neutrophil-to-lymphocyte ratio (NLR) has been proposed as a potentially useful marker of clinical outcome in diseases with an inflammatory component. The aim of this study was to clarify the association of NLR with HSP and investigate the usefulness of NLR as a marker to predict GI bleeding in children with HSP. METHODS:All patients with newly diagnosed HSP were reviewed retrospectively. White blood cell count, hemoglobin, platelet counts, mean platelet volume, neutrophil and lymphocyte count were evaluated. NLR and platelet-to-lymphocyte ratio (PLR) were calculated using complete blood count data. RESULTS:This study involved 141 HSP patients. GI involvement was found in 65 patients (46.1%), and, of these, 15 (10.6%) had GI bleeding. At the time of diagnosis, NLR was significantly higher (P = 0.001) and PLR significantly lower (P = 0.032) in patients with GI bleeding than in those without GI bleeding. On logistic regression analysis, NLR was the only independent predictor of GI bleeding (P = 0.004). The optimal cut-off of NLR for predicting GI bleeding was 2.86 (sensitivity, 73%; specificity, 68%). CONCLUSIONS:NLR, a simple and easily obtainable parameter, is a potential predictive marker of GI bleeding in children with HSP. 10.1111/ped.13652

The relevance of practical laboratory markers in predicting gastrointestinal and renal involvement in children with Henoch-Schönlein Purpura. Karadağ Şerife Gül,Çakmak Figen,Çil Burcu,Tanatar Ayşe,Sönmez Hafize Emine,Kıyak Aysel,Yavuz Sevgi,Çakan Mustafa,Aktay Ayaz Nuray Postgraduate medicine OBJECTIVES:Henoch-Schönlein Purpura (HSP) is the most common self-limiting vasculitis of childhood. Both serious gastrointestinal and renal complications may be observed during the disease course. The aim of this study was to evaluate the role of hematological markers in predicting the likely complications of the disease. METHODS:The demographic findings, clinical features, organ involvements and laboratory findings including white blood cell count (WBC), neutrophil, lymphocyte and platelet counts, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), neutrophil/lymphocyte ratio (NLR), platelet/lymphocyte ratio (PLR), mean platelet volumes (MPV), MPV/platelet count ratio (MPR) were evaluated retrospectively from the charts of the patients with HSP and all these parameters were compared with the same parameters of healthy children. RESULTS:A total of 376 patients with HSP and age- and sex-matched 233 healthy children were evaluated. Mean age at the diagnosis was 7.5 ± 3.5. All patients had palpable purpura, 46% had arthritis, 56.1% GIS involvement and 21.3% had renal involvement. While platelet counts, neutrophil counts, NLR, and PLR were higher, lymphocyte counts, MPV, and MPR were lower in patients with GIS involvement. NLR was the sole biomarker that was higher in patients with renal involvement. CONCLUSIONS:This study had shown that platelet counts, neutrophil counts, NLR, and PLR were increasing and lymphocyte counts, MPV, and MPR were decreasing when the patients had GIS involvement. However, these parameters were not relevant in distinguishing severe and mild GIS involvement. When patients had renal involvement NLR was the unique elevated parameter. 10.1080/00325481.2020.1807161

Neutrophil to Lymphocyte Ratio and Gastrointestinal Involvement Among Henoch Schonlein Purpura Patients: A Systematic Review and Meta-Analysis. Journal of pediatric gastroenterology and nutrition ABSTRACT:Neutrophil to lymphocyte ratio has been examined among several studies regarding Henoch Schonlein purpura patients with gastrointestinal involvement. The current meta-analysis aims to evaluate neutrophil to lymphocyte ratio differences between Henoch Schonlein purpura patients with and without gastrointestinal manifestations.An extensive search of the available literature was done using MEDLINE (1966-2020), Clinicaltrials.gov (2008-2020), Cochrane Central Register of Controlled Trials (CENTRAL) (1999-2020) and Google Scholar (2004-2020) databases and statistical analysis was mainly conducted using RevMan 5.3 software.The current meta-analysis is based on data from six studies. Neutrophil to lymphocyte ratio was higher in Henoch Schonlein patients with gastrointestinal involvement than those without with a mean difference of 0.88 (95% confidence interval [CI]: 0.55, 1.22 [P < 10-5, I2 = 8%]). Low heterogeneity (I2) was observed between the included studies. Subgroup analysis, leave one out meta-analysis and meta-regression analysis were performed which led to the lowering of I2 to 0.The findings of our meta-analysis suggest that the neutrophil to lymphocyte ratio could serve as a marker of gastrointestinal involvement in patients with Henoch Schonlein purpura; however, most of the included studies are case-control, therefore future cohort studies should clarify the value of this marker. 10.1097/MPG.0000000000003185