Vasculitis Patients Research Articles

Systemic vasculitis with latent tuberculosis infection and associated factors: a cross-sectional multicenter study.

Systemic vasculitis patients are at a higher risk of developing latent tuberculosis infection (LTBI). However, there is currently no literature elucidating the positivity rate and risk factors for LTBI in systemic vasculitis patients. Our study is a multi-center, cross-sectional study that enrolled systemic vasculitis patients from 13 comprehensive hospitals in China. T-SPOT.TB as the screening method for LTBI, the study investigated the positivity rate of LTBI in systemic vasculitis patients and the factors associated with T-SPOT.TB results. A total of 191 systemic vasculitis patients were included and the positive rate of T-SPOT.TB was 31.4%. The highest T-SPOT.TB positivity rate was observed in Behçet's syndrome (BD) (72/191, 37.7%). There were statistically significant differences between the LTBI group and non-LTBI group in terms of systemic vasculitis type (P = 0.010), albumin levels (P = 0.034), erythrocyte sedimentation rate (P = 0.016), and corticosteroid dosage (P = 0.047). Multivariate regression analysis revealed that smoking history (aOR = 3.809, 95%CI: 1.341-10.817) and BD (aOR = 2.106, 95%CI: 1.042-4.254) were independent risk factors of T-SPOT.TB postive results, besides decreased lymphocyte count (aOR = 0.114, 95%CI: 0.013-0.973), and high-dose glucocorticoids use (aOR = 0.386, 95%CI: 0.149-1.003) were independent risk factors of T-SPOT.TB negative results. The prevalence of LTBI is high in systemic vasculitis patients, especially those with BD or smoking history. Patients with decreased lymphocyte counts and high-dose glucocorticoid use are more likely to have a negative T-SPOT.TB results. Therefore, LTBI screening should be performed based on the characteristics of the patient during the diagnosis and treatment of systemic vasculitis. Key Points • We explored the positivity rate and risk factors of LTBI in systemic vasculitis patients from 13 hospitals in China. • There were 191 systemic vasculitis patients in our study. The positive rate of T-SPOT.TB was 31.4%. The predominant type of systemic vasculitis was BD, with a T-SPOT.TB positive rate of 44.4%. The second type was TA, with a T-SPOT.TB positive rate of 25.0%. • The prevalence of LTBI is high in systemic vasculitis patients, especially those with Behçet's syndrome or smoking history. Decreased lymphocyte counts and high-dose glucocorticoid use are more likely to have a negative T-SPOT.TB results. • LTBI screening using T-SPOT.TB should be conducted during the diagnosis and treatment of systemic vasculitis.

Reclassification of the overlap syndrome of Behçet's disease and antineutrophil cytoplasmic antibody-associated vasculitis in patients with Behçet's disease.

This study applied the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to patients with Behçet's disease (BD) to investigate the proportion and clinical implications of the reclassification to the overlap syndrome of BD and AAV (OS-BD-AAV). We included 280 BD patients presenting with ANCA positivity but without medical conditions mimicking AAV at diagnosis. Demographic data, items from the 2014 revised International Criteria for BD and 2022 American College of Rheumatology and European Alliance of Associations for Rheumatology criteria for AAV, ANCA positivity, and laboratory results were recorded as clinical data at diagnosis. A total score ≥ 5 indicated microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA), whereas a total score ≥ 6 indicated a diagnosis of eosinophilic GPA (EGPA). The overall reclassification rate of OS-BD-AAV was 8.6%. Of the 280 patients, 16 (5.7%) and 8 (2.9%) were reclassified as having OS-BD-MPA and OS-BD-GPA, respectively; none were classified as having OS-BD-EGPA. ANCA, myeloperoxidase-ANCA (P-ANCA), proteinase 3-ANCA (C-ANCA) positivity, hearing loss, and interstitial lung disease (ILD) at diagnosis were more common in patients with OS-BD-AAV than in those without. ANCA positivity and ILD at BD diagnosis contributed to the reclassification of OS-BD-AAV. However, hearing loss was not considered a major contributor to BD due to its possibility of developing as a manifestation of BD. To our knowledge, this is the first study to demonstrate the reclassification rate (8.6%) of patients with BD and ANCA results at diagnosis as OS-BD-AAV.

Obinutuzumab in Rituximab-Intolerant Antineutrophil Cytoplasmic Antibody–Associated Vasculitis Patients

Obinutuzumab in Rituximab-Intolerant Antineutrophil Cytoplasmic Antibody–Associated Vasculitis Patients

Muscle vasculitis in patients with polymyalgia rheumatica; three case series.

Polymyalgia rheumatica (PMR) is a common inflammatory disorder characterized by myalgia/stiffness in proximal hip and shoulder girdle, elevated C reactive protein (CRP) and erythrocyte sedimentation rate (ESR), but its pathogenesis is not fully elucidated. We report three cases of PMR who do not respond adequately to standard treatment. Those patients had typical symptoms of myalgia and muscle weakness, with elevated CRP in absence of creatine kinase elevation. Muscle specimen showed the findings of vasculitis in all cases, therefore muscular limited vasculitis (MLV) may be an underlying pathology in PMR in those refractory cases.

Management of hypertension and coronary artery disease in vasculitis patients using glucocorticoids

Systemic vasculitis is an autoimmune disorder characterized by inflammation and damage to blood vessels, often involving multiple organ systems. Glucocorticoids (GCs) are a cornerstone in managing vasculitis and other rheumatologic conditions due to their potent anti-inflammatory properties, but their use is associated with significant systemic side effects. This review evaluates the cardiovascular (CV) risks associated with glucocorticoid use, focusing on hypertension and atherosclerosis, and highlights strategies for minimizing these risks while managing systemic inflammation in vasculitis patients. Glucocorticoids, despite their efficacy in disease management, contribute to CV complications in a dose- and duration-dependent manner. Hypertension arises through mechanisms such as nitric oxide suppression, renin-angiotensin-aldosterone system activation, and vascular effects. Atherosclerosis is accelerated by endothelial dysfunction, oxidative stress, and inflammation, increasing risks for coronary artery disease and acute CV events. Daily doses exceeding 5 mg of prednisolone are linked to a marked rise in CV risks, even with short-term use. Moreover, systemic inflammation from vasculitis compounds these risks, making it challenging to disentangle GC side effects from the underlying disease pathology. Glucocorticoid therapy requires careful management, prioritizing the lowest effective dose and shortest duration possible. Routine CV risk assessments, blood pressure monitoring, and targeted interventions such as lifestyle modifications and pharmacologic therapies are crucial for mitigating adverse outcomes. Future studies should aim to define “safe” GC thresholds and refine treatment protocols to balance efficacy and safety in vasculitis management.

Can the affected intestinal segment visualized on abdominal ultrasound predict gastrointestinal tract bleeding in immunoglobulin A vasculitis?

To evaluate immunoglobulin A vasculitis (IgAV) patients with gastrointestinal (GI) tract involvement and to reveal the relationship between the location and extent of the affected intestinal segment detected on the initial abdominal ultrasound (US) and GI tract bleeding. This medical record review study was conducted on 117 IgAV patients with GI tract involvement between January 2016- June 2023. Patients were divided into two groups as those with (n=28) and without (n=89) GI tract bleeding. Predictors of GI tract bleeding were investigated by comparing demographic, clinical characteristics and laboratory findings. Gender, age at diagnosis, symptoms at admission, rash distribution, GI tract complaints, and the elapsed time until the development of GI tract symptoms were similar in both groups. There was no difference between small intestinal, large intestinal or small+large intestinal involvement (p=0.89). The ileum was the most commonly affected intestinal segment in patients with and without GI tract bleeding (p=0.37). Jejunal wall thickening (p=0.04) and the number of affected intestinal segments (p=0.008) were higher in patients with GI tract bleeding. In IgAV patients, jejunum involvement and affected multiple intestinal segments shown by abdominal US are associated with GI tract bleeding.

Abstract 4119470: ST-Elevation Myocardial Infarction as a Complication of Granulomatosis with Polyangiitis

Description of Case: A 19-year-old female with history of granulomatosis with polyangiitis (GPA) presented to the emergency department after a syncopal episode at a concert. She reported a prodrome of feeling hot and dizzy prior to the event, followed by epigastric discomfort, nausea, and vomiting after regaining consciousness. She had been diagnosed with GPA one year prior to presentation based on serology and biopsy results. Because her disease was felt to be well-controlled on azathioprine, she had been tapered off of prednisone one month prior to presentation. Initial vitals included blood pressure of 90/60 mmHg, and heart rate of 94 bpm. A 12-lead EKG revealed ST segment elevation in leads I, II and aVF with T wave inversions in the lateral leads ( Figure 1 ). Initial bloodwork was notable for troponin I of 0.11ng/mL (upper limit of normal 0.034 ng/mL). A diagnosis of inferior ST-elevation myocardial infarction was made. She was treated with oral aspirin and prasugrel load as well as intravenous heparin and taken urgently to the catheterization laboratory. Diagnostic coronary angiography was significant for single vessel coronary artery disease (CAD) of the right coronary artery (RCA), with acute complete occlusion of the mid-RCA ( Figure 2A-B ). She was treated successfully with primary percutaneous coronary intervention and implant of two overlapping drug eluting stents in the mid-RCA ( Figure 2C ). Discussion: GPA is a systemic necrotizing vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA) that predominantly affects small to medium sized vessels in the respiratory tract and kidneys. Cardiovascular manifestations in GPA are uncommon, but include pericarditis, myocarditis, and coronary arteritis. GPA can affect the coronary arteries either primarily through inflammatory changes in the arterial wall or via systemic inflammation leading to accelerated atherosclerosis. No standardized criteria exist to establish the diagnosis of coronary arteritis and to differentiate it from accelerated atherosclerosis. Long-term treatment of vasculitis patients with coronary involvement is also poorly supported by evidence. Optimization of cardiovascular risk factors is advocated as per standard of care, and vasculitis control may slow disease progression. This case not only raises awareness of cardiac complications in GPA, but highlights the current lack of evidence surrounding secondary prevention in young patients with vasculitis and cardiac complications.

Clinical characteristics and risk factors analysis of abdominal symptoms in IgA vasculitis patients: a retrospective cohort study.

About 50% of children with IgA vasculitis (IgAV) have abdominal symptoms, usually colic mimic to acute abdomen. Since signs and symptoms of vasculitis may appear in any order, this may affect the diagnosis of children whose abdominal symptoms precede the appearance of purpura. It is necessary to identify the risk factors, pathogenesis, and specific biomarkers to improve the prevention and management of IgAV patients with abdominal symptoms. All the 278 patients were children who had been diagnosed with IgAV in Nanyang Central Hospital between January 2018 and December 2018. The patient's age, gender, clinical manifestations, laboratory examination, and medical history were retrospectively collected. All the patients were divided into two groups based on whether they had abdominal symptoms. Ridge regression and multivariate logistic regression model were used to find risk factors of IgAV patients with abdominal symptoms. Of the 278 patients, 54 patients had abdominal symptoms, and the remaining 224 patients had no abdominal symptoms. Patients with abdominal symptoms had a lower proportion of infections and higher IgM concentrations than patients with other symptoms. For patients over 12years of age, platelet counts were lower in patients with abdominal symptoms. In addition, basophil count was identified as a protective factor, while IgM was identified as a risk factor. Infections, platelet counts, basophil count, and IgM concentration may be associated with abdominal symptoms in IgAV patients. Basophils and IgM may be involved in the pathological mechanism of abdominal symptoms.

Carotid intima-media thickness correlated with age and pulse wave velocity in ANCA-associated vasculitis patients

Objective: Cardiovascular diseases are the main causes of mortality in the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients. Carotid intima-media thickness (CIMT) measurement and pulse wave velocity (PWV) were performed to determine atherosclerosis and arterial stiffness as cardiovascular risk markers. Patients and Methods: The data of 31 patients with AAV were compared with 21 healthy controls. Demographic and laboratory findings were recorded. Results: Seventeen patients (54.8%) were male. Mean age was 52.6±11.5 years. CIMT was higher in the patient group [0.74 (0.65 – 0.84) vs 0.63 (0.57-0.74) mm; p=0.048]. PWV [7.9 (6.7-9.3) vs 7.8 (6.8-8.5) m/s; p=0.295] and augmentation index (AI) [22.5 (11.0-30.0) vs. 23 (9.5-30.5) mm/Hg, p=0.801] were similar in both groups. CIMT was correlated with age (r: 0.538, p

Clinical associations with thyroid disease in ANCA-associated vasculitis.

To evaluate the frequency and the clinical relevance of thyroid disease in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients. A total of 305 AAV patients admitted to the Second Affiliated Hospital of Chongqing Medical University between October 2010 and December 2023 were analyzed. Demographic, clinical, and laboratory data were compared between AAV patients with and without thyroid disease. Univariate and multivariate logistic regression analyses were conducted to identify factors associated with thyroid disease in AAV patients. Among the 305 AAV patients, 52 (17.0%) had concurrent thyroid disease. In univariate analysis, gender, coronary artery disease, renal involvement, anti-Ro/SSA antibodies, anti-Ro52 antibodies, anti-thyroglobulin antibodies (TgAb), and anti-thyroid peroxidase antibodies (TPOAb) exhibited significant differences between AAV patients with and without thyroid disease (P < 0.05). Multivariate analysis revealed that female gender (odds ratio (OR) = 2.423, 95% confidence interval (95% CI) 1.241, 4.729; P = 0.009), concurrent coronary artery disease (OR = 2.998, 95% CI 1.280, 7.019; P = 0.011), and positive anti-Ro/SSA antibodies (OR = 4.697, 95% CI 1.960, 11.257; P = 0.001) were associated with thyroid disease in AAV patients. AAV patients have a higher incidence of thyroid disease. Regular monitoring of thyroid function is advised for AAV patients, particularly for women, those with coronary artery disease, and those who are positive for anti-Ro/SSA antibodies. Key Points • AAV patients have a higher incidence of thyroid disease. • The potential clinical relevance of AAV patients with thyroid disease was explored. • Regular monitoring of thyroid function is advised for AAV patients.

Vasculitis associated with VEXAS syndrome.

To define the prevalence, distribution, and characteristics of patients with VEXAS who have confirmed vasculitis. Patients with VEXAS syndrome, verified by positive UBA1 mutation, were included. Chart review was performed to identify. characteristics and outcomes. Vasculitis diagnosis was based on either histopathology showing vascular inflammation or non-invasive angiography findings. Summary statistics were calculated. Eighty-nine patients met inclusion criteria. All were male with a median age of onset of 66.9 years (IQR 60.1, 72.7). Median (IQR) follow up was 3.8(2.2-5.5) years during which 21 patients (23.6%) had evidence of vasculitis. Vasculitis subtypes included small vessel vasculitis (19.1%), cutaneous medium vessel vasculitis (2.2%), and large vessel vasculitis (2.2%). No patient had more than one vessel size involved. Histopathology in small vessel vasculitis patients was consistent with cutaneous leukocytoclastic vasculitis in the majority, though one patient had leukocytoclastic peritubular capillaritis on renal biopsy. Cranial symptoms (headache, vision changes, or jaw pain) were noted in 18.0%. Two additional patients not experiencing cranial symptoms exhibited large vessel involvement with confirmed carotid thickening on non-invasive angiography; one of these had a positive temporal artery biopsy. VEXAS syndrome manifests as a variable vessel vasculitis in a quarter of patients, with cutaneous small and medium vessel involvement being particularly common. Some patients may have positive ANCA serologies or even renal vasculitis leading to misdiagnosis. Cranial symptoms are common and may mimic giant cell arteritis, though documented large vessel inflammation is rare.

Screening for Large Vessels Vasculitis in Patients with Acute Cerebrovascular ISCHEMIA

Abstract Background Systemic vasculitides are heterogeneous disabling diseases characterized by chronic inflammation of the blood vessels potentially leading to tissue destruction and organ failure, Giant cell arteritis (GCA) is a granulomatous subtype of vasculitis involving large and medium-size arteries, both cranial and extra-cranial, Imaging techniques, both morphological and metabolic, are required to confirm the diagnosis of large-vessel vasculitis, Arterial duplex is a moderately sensitive but highly specific test for exclusion of temporal arteritis. Purpose The purpose of this study is Screening of large vessels vasculitis in patients with cerebrovascular Ischemia by arterial transcranial and peripheral duplex. Patient and Methods 100 patients with cerebrovascular ischemia were further categorized into 3 groups: Group A: Seventy-four (74 %) pt of acute cerebrovascular stroke diagnosed according to National Institutes of Health Stroke Scale, Group B: Twenty-one (21%) pt with TIA diagnosed according to National Institutes of Health Stroke Scale, Group C: Five (5%) pt with GCA diagnosed according to 2022 American College of Rheumatology/EULAR classification criteria for Giant cell arteritis. Results Screening for large vessels vasculitis in patients with cerebrovascular Ischemia we found that five patients (5%) had GCA 2% with ischemic stroke 3% with TIA. Conclusion Our study indicates a significantly increased risk of CVA among patients with GCA

Diffuse Alveolar Hemorrhage Linked to Hydralazine

Hydralazine, a commonly prescribed vasodilator for hypertension, is associated with adverse effects such as vasculitis, glomerulonephritis, and drug-induced lupus. We present a rare case of hydralazine-induced diffuse alveolar hemorrhage (DAH) in a 74-year-old male with a history of hypertension. The patient was admitted with symptoms including hemoptysis, dyspnea, and dark urine. Initial findings included a biopsy-confirmed leukocytoclastic vasculitis, elevated MPO antibodies, and evidence of DAH on bronchoscopy. Despite the absence of glomerulonephritis, the patient exhibited signs of pulmonary-renal syndrome, including worsening renal function and anemia. The patient’s condition improved significantly after cessation of hydralazine and initiation of intravenous methylprednisolone. This case underscores the importance of considering hydralazine-induced ANCA vasculitis in patients presenting with unexplained pulmonary hemorrhage, particularly when other common causes are ruled out. Early recognition and management are crucial to prevent potentially life-threatening complications associated with this rare condition.

The impact of the COVID-19 pandemic on the incidence and clinical course of IgA vasculitis in paediatric patients

Introduction and objective: Immunoglobulin A vasculitis is an autoimmune disorder resulting from immune complex accumulation in small blood vessels, causing skin, joint, abdominal, and kidney manifestations. This study evaluated the impact of the COVID-19 pandemic on the incidence and clinical course of immunoglobulin A vasculitis in paediatric patients. Materials and methods: A retrospective analysis of medical records from a single university paediatric hospital was performed to compare 117 patients presenting with immunoglobulin A vasculitis before and 57 after the COVID-19 epidemic announcement in Poland on 20 March 2020. Laboratory results, hospitalisation duration, preceding infections, clinical presentation, history of allergies and COVID-19 vaccinations, and the proportion of immunoglobulin A vasculitis patients among all admissions were analysed. Results: The study of 174 patients showed that their average age during the pandemic (5.51 ± 3.10) was significantly lower than pre-pandemically (6.98 ± 3.67) (p &lt; 0.05). Before the pandemic, more hospitalised patients had immunoglobulin A vasculitis (1.14%) compared to during the pandemic (0.47%) (p &lt; 0.05). Food allergies were also more common during the pandemic (20.8%) than before (8.8%) (p &lt; 0.05). No significant differences were found in hospitalisation duration, and the incidence of immunoglobulin A vasculitis nephritis and abdominal symptoms (p = 0.194, p = 0.381, p = 0.968, respectively). Three patients had COVID-19 infection at admission. Conclusions: The pandemic led to fewer immunoglobulin A vasculitis hospitalisations but did not alter the clinical course of the disease or the incidence of immunoglobulin A vasculitis nephritis. In the context of the resurgence of COVID-19 infections, it is important to consider them as potential factors affecting immunoglobulin A vasculitis. Ongoing research is essential to understand these dynamics and guide effective clinical management of immunoglobulin A vasculitis amidst the evolving COVID-19 setting.

Advantages of metagenomic next-generation sequencing in the management of ANCA-associated vasculitis patients with suspected pulmonary infection as a rule-out tool

ObjectivePulmonary infection is one of the leading causes of death in patients with ANCA-associated vasculitis (AAV). It is sometimes difficult to differentiate pulmonary infection from pulmonary involvement of vasculitis in AAV patients. Fiberoptic bronchoscopy and bronchoalveolar lavage fluid (BALF) assays are useful diagnostic methods. In addition to conventional microbiological tests (CMTs), metagenomic next-generation sequencing (mNGS) facilitates rapid and sensitive detection of various pathogens. The current study aimed to evaluate the advantages of additional BALF mNGS in the management of pulmonary infection in AAV patients.Methods27 patients with active AAV and suspected pulmonary infection whose BALF samples were tested by mNGS and CMTs and 17 active AAV patients whose BALF were tested by CMTs alone were retrospectively recruited. The results of microbiological tests, and adjustments of treatment following BALF mNGS, were described. The durations of antimicrobial treatment and in-hospital mortality in patients were compared.ResultsAmong the 27 patients whose BALF samples were tested by mNGS, 25.9% of patients did not have evidence of pathogenic microorganism in their BALF samples, 55.6% had polymicrobial infections, including bacteria, fungi and viruses. Of these 27 patients, 40.7% did not have evidence of pathogenic microorganism in their BALF or serum samples according to CMTs. Patients in the BALF mNGS/CMT group received a significantly shorter duration of antibacterial and total antimicrobial treatment than patients in the CMT alone group (17.3 ± 14.7 vs. 27.9 ± 19.0 days, P = 0.044; 18.9 ± 15.0 vs. 29.5 ± 17.7 days, P = 0.040, respectively). Fewer patients in the BALF mNGS/CMT group died than in the CMT alone group (4/27 vs. 7/17, P = 0.049).ConclusionCompared with CMT alone, additional mNGS tests may shorten the duration of antimicrobial treatment and possibly decrease death from severe infection by providing precise and quick diagnosis of infection.

Prognostic Factors and Outcome Measures After Rituximab Therapy in Central Nervous System Vasculitis: A Systematic Review.

Given the growing popularity of Rituximab (Rmab) treatment as a potential substitute for cyclophosphamide, we conducted this review to determine aspects related to Rmab therapy in central nervous system vasculitis (CNSV) patients, aiming to establish both the beneficial and detrimental consequences of Rmab while providing clinical guidance for managing patients' conditions. This systematic review was prepared following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The PubMed, Web of Science, and Scopus databases were utilized to investigate Rmab treatment in CNSV from January 2015 to May 2024. The research question was structured using the Population, Intervention, Comparison, Outcome, and Study Design (PICOS) criteria. Case series with only three or more unique cases, prospective or retrospective non-randomized studies, and randomized controlled trials (RCTs) were addressed. The initial evaluations were performed in PubMed, Scopus, and Web of Science. After removing duplicate entries, 177 publications were obtained, and 41 were chosen for full-text assessment. The review then incorporated information gathered from 27 studies, including 4 case series, 15 non-randomized cohorts, and 8 RCTs. Rmab is generally regarded as effective for CNSV therapy. Given its success, induction Rmab therapy is now frequently employed as maintenance therapy for CNSV patients. Rmab is a viable option for the induction of remission and maintenance treatment, with a successful reduction in relapse rates.

A Familiar Face in the Treatment of Immunoglobulin A Vasculitis: Colchicine

Objective: The aim of the this study was to evaluate immunoglobulin A vasculitis (IgAV) patients on colchicine therapy and determine the causative factors leading to the initiation and maintenance of colchicine. Material and Methods: This retrospective study was conducted receiving IgAV patients on colchicine at our clinic between January 2013 and June 2023. Demographic, clinical, and laboratory data of IgAV patients were obtained from their electronic medical records. Results: A total of 33 IgAV patients receiving colchicine, 20 (60.6%) males and 13 (39.4%) females, were included in the study. The mean age at diagnosis of IgAV was 111.6±45.5 months. Colchicine was initiated in 13 (39.4%) patients with persistent rash, 8 (24.2%) with severe gastrointestinal tract involvement, seven (21.2%) with generalized/severe rash, and five (15.2%) with recurrent IgAV. The median elapsed time to colchicine initiation was 30 (10.5-60) days. The median duration of colchicine usage was ten (IQR, 6-54) months. Colchicine treatment was discontinued in 14 patients after a median 6.5 (IQR, 3-11.2) months. At the last visit, 13 (39.4%) patients were receiving colchicine for familial Mediterranean fever (FMF), four (12.1%) for recurrent IgAV, and two (6.1%) for persistent rash. Conclusion: Colchicine should be kept in mind especially in the presence of severe, persistent or recurrent rash. Early initiation of colchicine treatment should be considered in IgAV patients with severe GI tract disease, especially in countries where FMF is common.

MTOR signalling controls the formation of smooth muscle cell-derived luminal myofibroblasts during vasculitis

The accumulation of myofibroblasts within the intimal layer of inflamed blood vessels is a potentially catastrophic complication of vasculitis, which can lead to arterial stenosis and ischaemia. In this study, we have investigated how these luminal myofibroblasts develop during Kawasaki disease (KD), a paediatric vasculitis typically involving the coronary arteries. By performing lineage tracing studies in a murine model of KD, we reveal that luminal myofibroblasts develop independently of adventitial fibroblasts and endothelial cells, and instead derive from smooth muscle cells (SMCs). Notably, the emergence of SMC-derived luminal myofibroblasts—in both mice and patients with KD, Takayasu’s arteritis and Giant Cell arteritis—coincided with activation of the mechanistic target of rapamycin (mTOR) signalling pathway. Moreover, SMC-specific deletion of mTOR signalling, or pharmacological inhibition, abrogated the emergence of luminal myofibroblasts. Thus, mTOR is an intrinsic and essential regulator of luminal myofibroblast formation that is activated in vasculitis patients and therapeutically tractable. These findings provide molecular insight into the pathogenesis of coronary artery stenosis and identify mTOR as a therapeutic target in vasculitis.

Vasculitis associated with psoriatic arthritis: a case report

BackgroundPsoriatic arthritis is a chronic inflammatory condition associated with psoriasis, which affects joints. The coexistence of vasculitis, an inflammation of blood vessels, with psoriatic arthritis is rare, highlighting a complex interplay between autoimmune diseases that affect both joints and vascular structures.Case presentationWe report on a 56-year-old male with a long-standing history of psoriasis and psoriatic arthritis, who presented with new-onset skin lesions and neurological symptoms. Clinical evaluations confirmed the presence of leukocytoclastic vasculitis and asymmetric sensory motor axonal neuropathy. The absence of positive results from various standard tests led to a challenging diagnostic process, ultimately pointing towards vasculitis associated with psoriatic arthritis. The patient was treated with corticosteroids and immunosuppressive therapy, leading to partial symptom improvement.ConclusionsThis case underlines the complex and often overlapping nature of autoimmune conditions, particularly the occurrence of vasculitis in patients with psoriatic arthritis, which is rare. It stresses the importance of considering vasculitis in patients with psoriatic arthritis who present with unexplained skin lesions and neurological symptoms. This case adds to the spectrum of psoriatic disease manifestations and suggests a need for further research to explore the underlying mechanisms and improve management strategies.

Hydroxychloroquine as an add-on therapy for the induction therapy of MPO-AAV: a retrospective observational cohort study.

The remission rate of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients who received standard induction therapy is far from satisfactory. Improving the remission rate of MPO-AAV patients is essential. Hydroxychloroquine (HCQ), one of the classic antimalarial drugs, has been widely used in various autoimmune rheumatic diseases. This retrospective observational cohort study is aimed to evaluate the efficacy and safety of HCQ during induction treatment for MPO-AAV. The medical records of patients diagnosed with MPO-AAV at Xiangya Hospital, Central South University from January 2021 to September 2023 were collected. They were assigned to the HCQ group or control group according to whether they used HCQ. The patients included were screened by propensity score matching. To evaluate whether MPO-AAV patients benefited from HCQ, we compared the prognosis of the two groups. The adverse effects of HCQ during follow-up were recorded. The composition ratio of complete remission, response and treatment resistance between HCQ group and control group were different statistically (P=.021). There was no significant difference between the two groups in 1-year renal survival (P=.789). The HCQ group had better 1-year patient survival than the control group (P=.049). No serious adverse effects were documented in the HCQ group. HCQ together with standard induction treatment may improve the remission rate of MPO-AAV patients, and HCQ had good safety in our study.