There is a lack of systematic studies on single-organ cutaneous small vessel vasculitis (SOCV). To evaluate prognostic clinical and laboratory parameters, including systemic immune-inflammation biomarkers (SIIB) in SOCV inpatients. This study investigated the clinical and laboratory data of 178 inpatients. Blood tests were performed at baseline. SIIB were assessed based on neutrophil-to-lymphocyte ratio (NLR) and pan-immune-inflammation value (PIV). Univariable and multivariable statistics were performed. Both NLR and PIV were significantly higher in SOCV patients than in healthy controls. However, the SIIB values observed in SOCV patients were as high as those in psoriasis patients. On logistic regression analysis, disease manifestation on the upper extremities strongly predicted the absence of severe disease (OR: 0.31, 95% CI: 0.13 to 0.73; p = 0.0071). Moreover, older age (OR: 2.3, 95% CI: 1.11 to 4.77; p = 0.025) and severe disease (OR: 2.4, 95% CI: 1.16 to 4.94, p = 0.018) were significant independent predictors of longer hospital stay, whereas female sex was an independent protective factor for longer hospitalization (OR: 0.52, 95% CI: 0.28 to 0.96, p = 0.038). Lower serum C3 was a strong independent predictor of disease recurrence (OR: 13.9, 95% CI 3 to 63.4; p = 0.0007). The increase in SIIB observed in patients with SOCV reflects that systemic inflammatory alterations also play a role in SOCV patients. We identified several clinical and laboratory-based independent predictors of SOCV severity, length of hospitalization, and disease recurrence that may aid prognostication of SOCV patients.

Henoch-Schönlein purpura (HSP) also known as rheumatoid purpura is the most common vasculitis in children. This condition affects small blood vessels, predominantly targeting the skin, digestive system, joints, and kidneys. Short-term prognosis mainly depends on abdominal complications, while long-term prognosis is mainly determined by the severity of kidney involvement, which occurs in about 35% of cases. Although uncommon, other organs such as the lungs, heart, or nervous system may also be affected. Compartment syndrome of the hand and forearm is a very rare complication of HSP. To our knowledge, only two cases have been reported in the literature. We describe the case of a four-year-old child who presented with rheumatoid purpura complicated by compartment syndrome of the hand and forearm successfully managed through emergency fasciotomy.

UNASSIGNED: A variety of studies have shown a link between the gut microbiota and autoimmune diseases, but the causal relationship with Henoch-Schönlein purpura (HSP) and immune thrombocytopenic purpura (ITP) is unknown.
UNASSIGNED: This study investigated the bidirectional causality between gut microbiota and HSP and ITP using Mendelian randomization (MR). Large-scale genetic data of gut microbiota at phylum to species level from the MiBioGen consortium and the Dutch Microbiome Project were utilized. Genome-wide association studies (GWAS) summary statistics for HSP and ITP came from FinnGen R10. Various MR methods were applied to infer causal relationships, including inverse variance weighted (IVW), maximum likelihood (ML), cML-MA, MR-Egger, weighted median, weighted model, and MR-PRESSO. Multiple sensitivity analyses and Bonferroni correction were conducted to enhance robustness and reliability.
UNASSIGNED: Based on the IVW estimates, 23 bacterial taxa were identified to have suggestive associations with HSP and ITP. Remarkably, after Bonferroni correction, family Alcaligenaceae (OR = 2.86, 95% CI = 1.52-5.37; IVW, p = 1.10 × 10-3, ML, p = 1.40 × 10-3) was significantly associated with ITP as a risk factor, while family Bacteroidales S24 7group (OR = 0.46, 95% CI = 0.29-0.74; IVW, p = 1.40 × 10-3) was significantly associated with ITP as a protective factor. No significant associations between HSP and ITP and gut microbiota were found in reverse analyses.
UNASSIGNED: Our study provides evidence of causal effects of gut microbiota on HSP and ITP, highlighting the importance of further research to clarify the underlying mechanisms and develop targeted therapeutic interventions for these autoimmune diseases.

BACKGROUND: Intussusception, a common cause of abdominal pain in children, often lacks clear underlying causes and is mostly idiopathic. Recurrence, though rare, raises clinical concerns, with rates escalating after each episode. Factors like pathological lead points and Henoch-Schönlein purpura (HSP) are associated with recurrent cases. On the other hand, the prevalence of Helicobacter pylori (H. pylori), often asymptomatic, in children has been declining. Although its infection is reported to be linked with HSP, its role in recurrent intussusception remains unexplored. Further research is needed to understand the interplay among H. pylori (culprit pathogen), HSP (trigger), and intractable intussusception so as to develop effective management strategies.
METHODS: A two-year-old girl experienced four atypical episodes of intussusception at distinct locations, which later coincided with HSP. Despite treatment with steroids, recurrent intussusception persisted, suggesting that HSP itself was not a major cause for intractable presentations. Subsequent identification of H. pylori infection and treatment with triple therapy resulted in complete resolution of her recalcitrant intussusception.
CONCLUSIONS: This instructive case underscored a sequence wherein H. pylori infection triggered HSP, subsequently resulting in recurrent intussusception. While H. pylori infection is not common in young children, the coexistence of intractable intussusception and steroid-resistant recurrent HSP necessitates consideration of H. pylori infection as a potential underlying pathogen.

UNASSIGNED: Renal involvement is the most damaging long-term complication of Immunoglobulin-A (IgA) vasculitis. In the lack of a definite predictive biomarker for renal involvement, antiphospholipid antibodies (aPL) have been proposed in recent years.
UNASSIGNED: In this prospective cohort of 48 pediatric patients who were admitted with IgA vasculitis from September 2015 to June 2017, two serum samples were taken 12 weeks apart to detect Anti-Phospholipid antibodies. All patients were followed-up for renal involvement for six months.
UNASSIGNED: Renal involvement occurred in 14 out of 48 patients with IgA vasculitis (29.16%). APLs were positive in nine out of 14 patients with IgA vasculitis and renal involvement (64.28%), in contrast to only six out of 34 patients with IgA vasculitis without renal involvement (17.64%). The presence of aPL antibodies was statistically associated with renal involvement (P=0.002). Although, the relationship between both sex (P=0.025) and age (P=0.046) with aPL positivity was statistically significant, performing a modified logistic regression test, the odds ratio was significant between the groups with and without renal involvement only in term of age and aPL positivity).
UNASSIGNED: The presence of aPL antibodies was statistically associated with renal involvement. We found a significant relationship between the age and aPL positivity. Hence, we need multicenter, more extensive cohort studies to reach a better and more accurate conclusion on the relationship between serum aPLs and renal involvement in IgA vasculitis patients.

BACKGROUND: IgA vasculitis diagnosis relies primarily on clinical features and is confirmed by pathological findings. To date, there is no reliable noninvasive diagnostic biomarker.
OBJECTIVE: We aimed to explore the baseline serum metabolome of adult patients with IgA vasculitis to identify potential diagnostic biomarkers.
METHODS: We performed a study comparing the serum metabolome of patients with IgA vasculitis to that of patients with inflammatory condition, namely spondyloarthritis. Serum analyses were performed by high-performance liquid chromatography-mass spectrometry.
RESULTS: Fifty-five patients with IgA vasculitis and 77 controls with spondyloarthritis (age- and sex-matched) were included in this study. The median age of IgA vasculitis patients was 53 years. Two-thirds of patients were female (n = 32). At the time of vasculitis diagnosis, 100% of patients had skin involvement and 69% presented with glomerulonephritis (n = 38). Joint and digestive involvement were observed in 56% (n = 31) and 42% (n = 23) of patients. Four discriminative metabolites between the two groups were identified: 1-methyladenosine, L-glutamic acid, serotonin, and thymidine. The multivariate model built from the serum metabolomes of patients with IgA vasculitis and spondyloarthritis revealed an accuracy > 90%. As this model was significant according to the permutation test (p < 0.01), independent validation showed an excellent predictive value of the test set: sensitivity 98%; specificity 98%, positive predictive value 97% and negative predictive value 98%.
CONCLUSIONS: To our knowledge, this study is the first to use the metabolomic approach for diagnostic purposes in adult IgA vasculitis, highlighting a specific diagnostic metabolome signature.

Immunoglobulin A vasculitis (IgAV), also known as Henoch-Schönlein purpura (HSP), is a disease that causes inflammation and bleeding in small blood vessels in the skin, joints, intestines, and kidneys. Although various infections and chemicals are known to be triggers, the underlying cause of IgAV remains unknown. Here, we describe a case of an 86-year-old male patient with IgAV that developed after anti-tuberculosis treatment for tuberculous pleurisy. There have been several case reports implicating Mycobacterium tuberculosis and other acid-fast bacterium in the development of IgAV, but only a few case reports implicating anti-tuberculous drugs. This case highlights the importance of recognizing that IgAV can be caused by anti-tuberculous drugs.

The coronavirus disease 2019 (COVID-19) vaccine was developed to provide immunity against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), which was first reported in 2019. The vaccine has proven to be effective in reducing severity and mortality and preventing infection. Henoch-Schönlein purpura is an autoimmune vasculitis (immunoglobulin A vasculitis). Historically, vaccines have been administered primarily to children, and Henoch-Schönlein purpura has often been reported in children following vaccination. However, since the start of COVID-19 vaccination, an increasing number of cases have been reported in adults. Here, we report a case of a patient who developed hematuria and proteinuria after receiving the messenger RNA COVID-19 vaccine. A 22-year-old man presented to the hospital with a lower extremity rash, bilateral ankle pain, and abdominal pain 18 days after receiving the COVID-19 vaccine. The man had no significant medical history and was not taking any medications. Laboratory tests showed normal platelet counts but elevated white blood cell counts and C-reactive protein and fibrinogen levels. He was treated with the non-steroidal anti-inflammatory drugs, pheniramine and prednisolone. At 40 days after starting treatment, C-reactive protein levels were within normal limits, and no hematuria was observed. Treatment was terminated when the purpura disappeared. This report is intended to highlight the need for further research to be proactive and carefully monitor for conditions associated with the COVID-19 vaccine.

IgA-associated vasculitis (IgAV) known as Henoch - Schönlein purpura (HSP) disease is an inflammatory disorder of small blood vessels. It\'s the most common type of systemic vasculitis in children which can be associated with the inflammatory process following infections. IgA vasculitis is a rare and poorly understood systemic vasculitis in adults. Coronavirus disease 2019 (COVID-19) has been associated with HSP in both adults and children. A 58-year-old woman was diagnosed with HSP, fulfilling the clinical criteria: palpable purpura, arthritis, hematuria. The disclosure of the HSP disease was preceded by a infection of the respiratory tract. COVID-19 infection was confirmed via the presence of IgM and IgG antibodies. This case indicates the possible role of SARS-CoV-2 in the development of HSP. The clinical course of IgAV in adults appears to be different from pediatric IgAV, especially due to higher risk of renal complications. Symptoms of the disease quickly resolved with low-dose of steroids.

Henoch-Schönlein purpura (HSP) is a common form of IgA1-mediated blood vessel inflammation affecting mainly children. Intercellular adhesion molecule-1 (ICAM-1) gene polymorphisms have been shown to be associated with HSP in different populations; in this study, we investigated its potential association and influence on the development of severe complications in Iranian HSP patients. Twenty-three patients diagnosed with IgAV/HSP according to the criteria of the American College of Rheumatology (ACR) with 53 age- and sex-matched control subjects were referred to us. Cases and controls were genotyped using Sanger sequencing. Based on our research data, we found an association between codon 469 K/E of the ICAM1 gene and risk of HSP. Our results revealed that KK genotype and allele K are more common in control than in the HSP group, therefore the subjects with KK genotype are protected against HSP. Our data also suggested that the genotype EE is associated with higher risk of HSP progression compared to KK genotype.