Wegener's Granulomatosis Research Articles

Long-Term Clinical Landscapes of Spinal Hypertrophic Pachymeningitis With Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis.

Spinal hypertrophic pachymeningitis (HP) is an extremely rare disorder characterized by the thickening of the spinal dura mater, which harbors distinct repertoires of immune cells due to the unique partitioning of the arachnoid blood-CSF barrier. The objectives were to identify the pathogenesis and therapeutic strategies for spinal HP. This retrospective cohort study analyzed the clinical and pathologic profiles of patients with idiopathic/immune-mediated HP including spinal HP. Among 61 patients with idiopathic/immune-mediated HP, all 6 Japanese patients with spinal HP, with a median observation period of 88.8 months, were myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA)-seropositive. The MPO-ANCA+ spinal HP cohort had the following characteristics: (1) a predominance of older women; (2) all patients were classified as having microscopic polyangiitis based on the 2022 American College of Rheumatology/European League Against Rheumatism criteria; (3) 83% of patients developed subacute/chronic myelopathy due to extramedullary spinal cord compression; (4) 50% of patients had lesion extension to the epidural compartment and vertebral column; (5) 50% of patients presented with chronic sinusitis, otitis media, or mastoiditis; (6) 33% of patients had involvement of the lower airways or kidneys; (7) a higher disease activity of the nervous system was noted based on the Birmingham Vasculitis Activity Score (BVAS), in contrast to MPO-ANCA+ cranial HP; (8) granulomatous inflammation with myofibroblasts, immune cells including granulocytes, and B-cell follicle-like structures were observed in the thickened dura mater; (9) immunotherapies (with or without surgical decompression) were effective in reducing the modified Rankin Scale score and reduced BVAS during the first active insults; (10) combined immunotherapies with glucocorticoids and cyclophosphamide/rituximab helped in reducing relapses in the long term; and (11) surgical decompression, including laminectomy and duraplasty, was necessary for compressive myelopathy. These data suggest that MPO-ANCA+ spinal HP shares common features with MPO-ANCA+ cranial HP (1, 2, 6, 8, 9, and 10), but also has unique clinical features (3, 4, 5, 7, and 11). Our findings highlight the significant pathogenic role of ANCA in spinal HP. MPO-ANCA+ spinal HP, as an organ-threatening disease, should be positioned as having unique characteristics, whether limited to the CNS or as part of a generalized form in ANCA-associated vasculitis.

FEATURES OF LUNG DAMAGE IN ANCA-ASSOCIATED VASCULITIS

Systemic vasculitis is a large area of disease characterized by the induction of inflammation in the vessel wall due to various immune disorders. The formation of anti-neutrophil cytoplasmic antibodies which determine the development of the so-called anti-neutrophil cytoplasmic antibodies-associated vasculitis is of the variants of such disorders: microscopic polyangiitis, granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) and eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome). These features determine the exceptional importance of understanding their pathologic behaviour, clinical picture, and treatment, which will be discussed in this article Objectives: The main goal of the article is to investigate the frequency of lung damage in patients with ANCA-SV, to assess their impact on the course, and to adequately select immunosuppressive therapy that allows for achieving disease remission. To identify the incidence of lung damage in patients with ANCA-SV. To track the dynamics of pulmonary function indicators and outcomes of ANCA-SV under the treatment. Material and methods: To do this, we conducted a retrospective analysis of the medical records of 50 patients with a clinical diagnosis of ANCA-SV who were treated in the Department of Internal Medicine No. 2 of the Research Institute of Cardiology and Internal Diseases in Almaty, the Republic of Kazakhstan, through 2015 to 2024. The diagnosis was verified based on a life history, physical examination, immunological study (detection of ANCA), as well as by assessing kidney function indicators (creatinine, urea, electrolytes, the presence of proteins, diuresis volume), assessing pulmonary function indicators (spirography, peakflowmetry), instrumental survey such as CT scan of the chest, MRI of the brain, sinuses. Results and discussion. 29 women and 21 men aged 25 to 75 (average 50 years) were identified among those suffering from ANCA-SV. Our goal was to investigate the incidence of lung damage in patients with ANCA-SV. To do this, we have conducted some survey methods. In most cases, changes in the lungs were confirmed by clinical data, decreased saturation, laboratory parameters (general sputum analysis, sputum culture for MTB and GeneXpert), and results of an immunological blood test: enzyme multiplied immunoassay (Immunoglobulin E), CT scan of the chest, сhest X-ray, spirography, blood gases test. Among 50 patients with ANCA-SV, changes in the lungs were detected in 28 patients. Changes in the lungs with ANCA-SV are different. Some patients experience more than two changes in the lungs. Conclusions: Lung damage in the form of infiltrates, cavities of destruction, diffuse alveolar damage, interstitial fibrosis, pleurisy and bronchial asthma, destructive processes, ventilation disorders, changes in the lungs in the form of granulomas, pulmonary hemorrhages are symptoms of ANCA-SV. Timely hospitalization and modern diagnostic methods make it possible not only to determine the causes of pulmonary complications in the shortest possible time but also to begin therapy as quickly as possible and obtain a positive clinical response to treatment. ANCA-associated vasculitis is a difficult condition to diagnose, which is characterized by a high rate of progression, and malignancy and leads to the death of the patient in a short time. However, advances in medicine and pharmacology with the discovery of genetically engineered biological therapy (Rituximab) have improved the prognosis for patients.

IL1B gene polymorphisms (rs1143627, rs16944) increase the risk of ANCA-associated vasculitis.

IL1B gene polymorphisms (rs1143627, rs16944) increase the risk of ANCA-associated vasculitis.

Myocardial infarction in ANCA-associated vasculitis: a population-based cohort study

ObjectivesTo determine the incidence rate (IR) and predictors of myocardial infarction (MI) in patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) as well as to estimate the IR ratio (IRR)...

Necrotizing granulomatous vasculitis of the gallbladder. A case report

Necrotizing granulomatous vasculitis of the gallbladder. A case report

Clinical Utility of Serum sCD200/sCD200R Ratios in Predicting Current Activity of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

Objectives: This study investigated whether serum soluble CD200 (sCD200) and soluble receptor for CD200 (sCD200R) concentrations and serum sCD200/sCD200R ratios at diagnosis could predict cross-sectional activity in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Methods: We included 70 patients with AAV in this pilot study, retrospectively reviewed their medical records, and collected clinical data at the time of AAV diagnosis. We also measured sCD200 and sCD200R in stored blood samples collected at diagnosis. In medical records, AAV activity at diagnosis had been assessed according to the Birmingham Vasculitis Activity Score (BVAS). The prediction potential of serum sCD200 and sCD200R concentrations and serum sCD200/sCD200R ratios for BVAS was evaluated using Pearson correlation analysis. Results: Among the 70 patients, the median age was 63.5 years, with 29 males and 41 females. Among the three CD200-related variables at diagnosis, serum sCD200/sCD200R ratios at diagnosis were significantly correlated with cross-sectional BVAS; however, serum sCD200 and sCD200R concentrations were not correlated with it. These results may indicate that serum sCD200/sCD200R ratios may better help predict cross-sectional AAV activity by increasing the range of opposing changes in the two variables. On the other hand, both serum sCD200 concentrations and serum sCD200/sCD200R ratios showed significant correlations with cross-sectional myeloperoxidase-ANCA titre, five-factor score, and serum creatinine levels at diagnosis. Conclusions: In this study, we demonstrated that serum sCD200/sCD200R ratios at diagnosis can be a useful and convenient biomarker to predict cross-sectional AAV activity calculated according to BVAS.

MicroRNA expression profiles in sinonasal biopsies to support diagnosis of granulomatosis with polyangiitis

ObjectivesTo identify aberrantly expressed microRNAs (miRNAs) in sinonasal tissue biopsies of patients with granulomatosis with polyangiitis (GPA), associate their expression profiles to sinonasal histopathology, and assess their differential expression between subgroups of clinically proven GPA patients, healthy controls and patients exhibiting inflammation of other etiology.MethodsWe included formalin-fixed, paraffin-embedded biopsy tissue samples of sinonasal mucosa from 37 patients with clinically proven GPA, 15 patients with inflammation of other etiology and 14 control patients with normal histology. Of the included GPA patients, 20 patients had characteristic GPA-related histological features, while 17 patients displayed non-specific GPA histopathology in their sinonasal biopsy. Assessment of histological parameters was performed using histopathological techniques, and analysis of miRNA expression with miRCURY LNA miRNA miRNome Human PCR Panels and quantitative real-time PCR.ResultsWe determined expression of 306 miRNAs in sinonasal biopsy samples, which displayed different extent of dysregulation between individual patient groups. Based on their potential to discriminate between the controls, non-GPA and GPA patient subgroups, dysregulation of 11 miRNAs was further assessed, of which miR-1-3p/-21-3p/-93-5p/-155-5p/-1248/-31-3p/-182-5p/-183-5p and let-7b-5p held the potential to stratify patients based on their sinonasal tissue miRNA profile. Notably, several of these miRNAs were associated with the presence of granulomas, vasculitis and necrosis in sinonasal biopsies of GPA patients.ConclusionOur study identifies novel miRNAs putatively implicated in the pathogenesis of GPA, and highlights dysregulated miRNAs as supporting biomarkers in establishing GPA diagnosis, particularly in the early phases of the disease, or in patients with atypical GPA presentation.

Time-dependent effect of prophylactic trimethoprim-sulfamethoxazole on the incidence of serious infections in ANCA-associated vasculitis: A target trial emulation study.

To investigate the effect of prophylactic trimethoprim-sulfamethoxazole (TMP-SMX) on the incidence of serious infections in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). This multicenter cohort study designed to emulate a target trial studied 296 patients with AAV treated with rituximab or cyclophosphamide as induction therapy. Patients were grouped based on the administration of TMP-SMX within 14 days following induction therapy (n=240 and 56 patients in prophylaxis and control groups, respectively) (intention-to-treat) and also as a time-dependent exposure (time-varying). Inverse probability weighting was applied to minimize the baseline imbalance between the two groups. Primary outcome was 1-year incidence of serious infection. During the 252.1 person-years of observation, 77 cases of serious infections were recorded in 65 patients, with a fatality rate of 18.5%. Most serious infections (n=66, 85.7%) occurred within the first 180 days of observation. The prophylaxis group showed a significantly lower incidence of serious infections than the control group (HR 0.48 [0.32-0.72]). However, this beneficial effect of TMP-SMX was only significant during the first 180 days (HR 0.41 [0.22-0.76]) and not thereafter (HR 3.76 [0.46-29.43]) (interaction p=0.044). This result was also consistent with the time-varying analysis result. Based on one case of severe adverse drug reaction related to TMP-SMX, the number needed to harm was 127.4 whereas the number needed to treat to prevent one serious infection was 8.0. Prophylactic TMP-SMX significantly reduced the risk of serious infections in patients with AAV, particularly during the first 6 months of induction therapy with rituximab or cyclophosphamide.

Intravenous Methylprednisolone for Acute Right Middle Cerebral Artery Occlusion in Granulomatosis Polyangiitis: A Case Report.

A 17-year-old Japanese woman diagnosed with granulomatosis with polyangiitis (GPA) and was treated with immunotherapy suddenly developed an impaired consciousness, left hemiplegia, and conjugate eye deviation to the right. Magnetic resonance imaging showed an acute cerebral infarction in the right striatum and right middle cerebral artery (MCA) occlusion. As her GPA had worsened recently, intravenous methylprednisolone (IVMP) was started. Immediately after IVMP was started, recanalization of the MCA was detected on repeat cerebral angiography. Aspirin and argatroban dissolved the residual thrombi. A combination of IVMP and antithrombotic therapy may be effective for large-vessel occlusions associated with GPA.

Drug-induced liver injury due to avacopan improved by mycophenolate mofetil: A case report.

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a systemic necrotizing vasculitis that predominantly affects small vessels. Glucocorticoids are the standard therapeutic agents for AAV; however, their long-term use can cause damage. Avacopan is a small-molecule complement component 5a receptor antagonist that reduces vasculitis and can potentially be used as an alternative to glucocorticoids. However, its therapeutic efficacy remains unknown, and drug-induced liver injury (DILI) is a concern associated with its use. A 74-year-old woman with a history of granulomatosis with polyangiitis was admitted to our hospital with a 1-week history of fatigue and anorexia. She had started avacopan 3 months before hospitalization. Blood tests showed severe liver injury, and since other diseases were ruled out, she was diagnosed with DILI secondary to avacopan. Avacopan was discontinued, and glucocorticoid doses were increased and ursodeoxycholic acid was administered; however, the liver injury did not resolve. Therefore, mycophenolate mofetil (MMF) was started. The liver injury was resolved after starting MMF. MMF is effective in treating DILI caused by avacopan.

Propylthiouracil-induced ANCA-associated vasculitis complicated by granulocytopenia and hemophagocytosis: a case report

ObjectiveTo analyze a rare case of ANCA-associated vasculitis (AAV) complicated by hemophagocytosis and granulocytopenia induced by long-term propylthiouracil (PTU) therapy, providing insights for clinical diagnosis and management.MethodsA retrospective analysis was conducted on the clinical data and treatment course of a patient who developed AAV with hemophagocytosis and granulocytopenia after prolonged PTU use.ResultsUpon admission, granulocytopenia secondary to PTU was suspected. Despite transient recovery of leukocyte counts with anti-infective therapy and granulocyte colony-stimulating factor (G-CSF), recurrent leukopenia and intermittent fever persisted. Bone marrow aspiration revealed hemophagocytic cells, while serologic testing showed positivity for both PR3-ANCA and MPO-ANCA. A definitive diagnosis of PTU-induced AAV was established. Glucocorticoid therapy normalized body temperature and restored leukocyte levels. Follow-up demonstrated resolution of thyrotoxicosis, stabilized leukocyte counts, and afebrile status.ConclusionLong-term PTU therapy may trigger AAV accompanied by hemophagocytosis. Clinicians should consider screening for hemophagocytic lymphohistiocytosis (HLH) in such cases to guide timely immunosuppressive intervention.

Successful treatment of MPO-ANCA positive crescentic IgA nephropathy/IgA vasculitis with nephritis potentially triggered by a COVID-19 vaccine in a young adult female using corticosteroids, rituximab, and avacopan.

An 18-year-old female presented with palpable purpura nine months before her hospital admission, which first appeared 1month after receiving a COVID-19 vaccine and recurred intermittently. One month prior to admission, she developed macrohematuria, abdominal pain, and a loss of appetite. Occult blood in urine had been noted during high school health check-ups. Upon admission, she continued to have macrohematuria, along with renal dysfunction and a nephritic urinalysis, serum myeloperoxidase-anti-neutrophil cytoplasmic antibodies (MPO-ANCA) positivity. A renal biopsy revealed crescentic glomerulonephritis with mesangial and endocapillary hypercellularity, and dominant IgA deposition and electron-dense deposits in the mesangial regions. The diagnosis was IgA nephropathy (IgAN) or IgA vasculitis with nephritis (IgAVN), with a possible overlap of MPO-ANCA-associated glomerulonephritis. Treatment began with methylprednisolone pulse therapy and prednisolone. After the diagnosis, rituximab (RTX) and avacopan were added to the regimen. Within two months, renal function, hematuria, and MPO-ANCA levels had normalized, and proteinuria was almost fully resolved by 13months. If IgAN/IgAVN and ANCA-associated vasculitis were indeed triggered by the COVID-19 vaccination in this case, it is plausible that both conditions share similar pathologic mechanisms. This case emphasizes the need for a reliable laboratory method to detect pathogenic ANCA to guide both induction and maintenance therapy. Further investigation into the effectiveness of the ANCA-associated glomerulonephritis treatment protocol including corticosteroids, RTX, and avacopan in managing crescentic IgAN/IgAVN could offer valuable insights into improving patient care.

Assessment of Coronary Microvascular Dysfunction in Patients with Systemic Vasculitis.

Coronary microvascular dysfunction (CMD) is characterized by impaired coronary blood flow in the absence of obstructive coronary artery disease. CMD primarily involves the microvasculature, leading to myocardial ischemia, angina, and increased cardiovascular risk. Systemic vasculitides (e.g., giant cell arteritis, antineutrophil cytoplasmic antibody-associated vasculitis, and Takayasu arteritis) are a group of autoimmune conditions known to affect the vasculature through inflammation of the blood vessels that have been associated with more prevalent and severe CMD. Although systemic inflammation likely plays a role in the increased risk of cardiovascular events, the underlying pathogenesis is not well understood. Invasive and non-invasive techniques for assessing coronary microvascular function have been developed to assess for blood flow and coronary flow reserve (CFR), defined as the ratio of the maximum achievable blood flow during stress to the resting blood flow. The purpose of this review is to further explore the relationship between vasculitis and CMD as well as the techniques available for assessing this association. Studies have shown that CMD is significantly more prevalent in patients with systemic vasculitis compared to the general population. Moreover, in the absence of significant atherosclerotic burden, patients with vasculitis have a lower CFR than controls, indicating more severely impaired coronary vasomotor function. This suggests that systemic inflammation itself is a factor in driving coronary vasomotor abnormalities and CMD development. CMD contributes to cardiovascular morbidity in patients with systemic vasculitis, underscoring the need for early recognition and management. Further studies are needed to determine whether therapies targeting the reduction of systemic inflammation can lead to improved coronary microvascular function and cardiovascular outcomes.

Relapse of indurative tuberculosis of the skin of the lower extremities against the background of undiagnosed spontaneously cured pulmonary tuberculosis

Introduction. In recent years, there has been a marked increase in the prevalence of cutaneous tuberculosis in Ukraine. Tuberculous lesions of the skin, as a rule, are secondary. Case description. Tuberculous lesions of the skin are typically secondary in nature. Patient N, aged 58 years, presented with a dermatological complaint on the lower extremities, characterised by pruritus, burning and functional impairment. He had been experiencing symptoms for approximately three years prior to seeking medical attention at his place of residence, where a diagnosis was not established. The dermal lesions present as dense, rounded nodules up to 1.0 cm in diameter on the lower limbs. The skin in the vicinity of the nodes displays a reddish hue with a bluish tinge. Irregularly shaped foci of cicatricial atrophy are present on the skin of the ankle-foot joints. An additional rounded formation is identified on the X-ray of the thoracic organs and lungs, taken in both direct and left lateral projections. The pathohistological examination revealed uneven infiltration in the surface layers of the dermis, comprising lymphocytes, plasma cells, and multinucleated Langhans cells, which formed non-caseous granulomas. Additionally, the dermis exhibited focal sclerosis, granulomatous vasculitis with damage to the walls of small and medium-sized vessels, and signs of lobular panniculitis. Discussion. In consideration of the patient's complaints, anamnesis data, data from objective and special research methods, a diagnosis was formulated. A relapse of indurative tuberculosis of the skin of the lower extremities was observed. The patient presented with residual changes in a previously treated case of pulmonary tuberculosis. Conclusions. In order to facilitate an early diagnosis of tuberculosis of the skin, it is necessary to perform a mandatory X-ray examination of the organs of the chest cavity and a histological examination of the affected area of the skin at the initial visit.

Retraction of: aPKC/Par3/Par6 polarity complexes regulate podocyte motility and crescent formation in the progression of ANCA-associated vasculitis

Retraction of: aPKC/Par3/Par6 polarity complexes regulate podocyte motility and crescent formation in the progression of ANCA-associated vasculitis

Granulomatosis with polyangiitis presenting with isolated external genital and urethral manifestations: a case-based review.

Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis primarily affecting the respiratory tract and kidneys, with external genital and urethral lesions (EGUL) being exceedingly rare. We present a case of a middle-aged man with relapsing proteinase 3-antineutrophil antibody-positive GPA who developed isolated granulomatous, ulcerative balanitis, and urethritis. His condition abruptly worsened after a prolonged indolent course, requiring treatment with glucocorticoids and rituximab, leading to successful remission. To better characterize EGUL in GPA, a systematic literature search was performed in PubMed, Scopus, and the NPO Japanese Society of Medical Abstracts databases using keywords related to GPA and EGUL. Cases meeting the American College of Rheumatology or Japanese Ministry of Health, Labor, and Welfare criteria for GPA and the 2012 Chapel Hill Consensus Conference definitions were included for analysis. Our review identified that EGUL often presents as an initial symptom and can be classified by the presence or absence of preceding urethritis. Cases with preceding urethritis had a higher risk of severe complications with extensive penile or urethral involvement. In contrast, penile lesions without preceding urethritis typically presented as characteristic mucosal lesions localized around the glans. In females, GPA-associated urethritis frequently led to periurethral mass formation, vaginal involvement, and significant sequelae. Given the potential for delayed diagnosis and serious complications, clinicians should be vigilant for EGUL in patients with suspected or diagnosed GPA. Future prospective studies with larger cohorts are needed to elucidate the prevalence, clinical spectrum, and optimal management of these rare but significant manifestations of GPA.

Evaluation of ANCA testing by antigen-specific assays in small vessel vasculitis patients

IntroductionAntineutrophil cytoplasmic antibodies (ANCA) testing is a screening method that may be applied in clinical practice. Testing for vasculitis linked to antineutrophil cytoplasmic antibodies (AAV) usually involves these measurements. ANCAs are not exclusive to vasculitis; immunofluorescence testing can also detect (atypical) ANCA patterns in other inflammatory diseases. As a result, using indirect immunofluorescence (IIF) in addition to an immunoassay was advised. These discoveries led to the establishment of a new worldwide consensus. The 2017 international agreement on ANCA testing states that when immunoassay is used as the primary screening method to detect AAV, IIF should not be necessary.AimAssess the efficacy of the suggested method for ANCA detection based on antigen-specific immunoassay screening. A number of patients with AAV and relevant disease controls, meaning patients for whom ANCA was ordered in relation to small-vessel vasculitis, will be evaluated. These patients may include those with infections, illnesses produced by drugs, connective tissue disease, etc.ResultsRegarding the respiratory features among the two studied groups (ANCA vasculitis group and non-ANCA vasculitis group), it was found that cough with fever (p = 0.024), pulmonary hemorrhage (p = 0.024), interstitial lung disease (ILD) (p = 0.024), pulmonary renal syndrome (0.001), and pleural thickening (p = 0.024) were the most common and the leading clinical manifestation that differentiate between ANCA and non-ANCA vasculitis. Also, in studying different extra pulmonary system features between the two clinical groups for differential diagnosis, it was found that rash (p = 0.001), dry mouth (p = 0.001), bloody diarrhea (p = 0.024), bilateral scleritis (p = 0.024), and vision loss (p = 0.001) were the most clinically leading symptoms Moreover, the laboratory parameters among the two clinically suspected vasculitis groups were in favor of vasculitis if proteinase-3 (pr3) ANCA (p < 0.001) and or the myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO) ANCA (p = 0.001) or the patient had proteinuria and hematuria. Regarding the diagnostic performance of different biomarkers to detect ANCA, it was found that at level > 1.5, the sensitivity of pr3 ANCA is 99.9% and the specificity is 92%, while at level > 3, the sensitivity of MPO ANCA is 90% and the specificity is 55%.ConclusionThe investigation’s findings support the global European consensus reached in 2017 that anti-PR3 and anti-MPO immunoassay should be used for first screening for ANCA-associated vasculitis instead of ANCA testing by IIF. Similar levels of specificity and sensitivity were shown by immunoassays employing direct, capture, and anchor. A positive anti-PR3 test would be very helpful when granulomatosis with polyangiitis (GPA) is suspected, and an anti-MPO might be used to diagnose MPA.

Serum ferritin is a superior biomarker for evaluating disease activity and kidney injury compared with C-reactive protein in anti-neutrophil cytoplasmic antibody-associated vasculitis.

Elevated C-reactive protein (CRP) is a characteristic of active anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). However, its relationship with organ involvement severity is ambiguous. Serum ferritin (SF) is a proinflammatory marker elevated in many autoimmune diseases, yet its role in AAV is poorly studied. A total of 399 AAV patients were retrospectively selected and divided into four groups based on SF and CRP levels. Clinical, laboratory, and pathological data were compared. Survival analyses were conducted to estimate end-stage renal disease (ESRD) and death. In this cohort, 256 patients (64.16%) had elevated SF, and 289 (72.43%) had elevated CRP. Patients with elevated SF and normal CRP had the highest BVAS, lowest initial estimated glomerular filtration rate, highest 24-h proteinuria, and worst renal prognosis. SF showed a weak positive correlation with BVAS (R = 0.201, p < 0.001), while CRP showed a weak negative correlation with proteinuria (R = - 0.158, p = 0.002). Univariate Cox regression analysis revealed high SF level was a risk factor for ESRD and death, whereas CRP was not. Pathological tests showed that patients with elevated SF had a higher proportion of glomeruli with cellular crescents compared to those with normal SF. CRP positively correlated serum complement 3 (C3) with SF as a control variable (R = 0.434, p < 0.001), while SF negatively correlated with C3 with CRP as a control variable (R = - 0.201, p < 0.001). In active AAV, high SF is associated with severe injury and poor kidney prognosis, whereas CRP is not. The underlying mechanism may be related to the different impacts on the complement system. Key Points • Patients with elevated SF and normal CRP had the highest BVAS, lowest initial estimated glomerular filtration rate, highest 24-hour proteinuria, and worst renal prognosis. . • CRP positively correlated serum C3 with SF as a control variable, while SF negatively correlated with C3 with CRP as a control variable. • High SF reflects a high proportion of glomeruli with cellular crescents in renal histopathology.

ANCA-associated vasculitis in patients with rheumatoid arthritis: A single-center cohort study.

ANCA-associated vasculitis in patients with rheumatoid arthritis: A single-center cohort study.

Cardiac Magnetic Resonance Imaging Findings in Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitides: A Systematic Review.

Our objective was to review the available literature on cardiac magnetic resonance imaging (cMRI) findings in patients with antineutrophil cytoplasmic antibody-associated vasculitides (AAV), evaluate its diagnostic utility, and assess its potential as a screening tool. We systematically searched PubMed, Embase, Scopus, and Web of Science from inception to March 29, 2023, following Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) 2020 guidelines. English-language studies involving adult patients diagnosed with AAV-eosinophilic granulomatosis with polyangiitis (EGPA), granulomatosis with polyangiitis (GPA), or microscopic polyangiitis (MPA)-using recognized classification criteria were included. Studies had to report specific cMRI parameters in at least three patients. Three independent reviewers conducted study selection, data extraction, and quality assessment. Of 2,251 studies, 30 met the inclusion criteria, encompassing 1,149 patients with AAV (87% with EGPA, 13% with GPA, and 0.3% with MPA). The mean patient age was 52 ± 5 years, with 50.4% being female. The mean left ventricular ejection fraction (LVEF) was 55.6% ± 11.3%, and 29% of patients had an LVEF less than 50%. Myocardial fibrosis, indicated by late gadolinium enhancement (LGE), was present in 49% of patients, with predominantly subendocardial or endocardial (23%), intramyocardial (14%), and subepicardial (10%) patterns. Patients in remission (26%), when compared to those not in remission (74%), exhibited higher proportions of LGE (55% vs 47%) and glucocorticoid use (77% vs 68%), despite similar rates of abnormal electrocardiograms (44% vs 42%). This systematic review reveals a high prevalence of myocardial fibrosis detected by cMRI in patients with AAV, even during remission. Significant subclinical cardiac involvement may be missed by conventional diagnostic methods, underscoring the utility of cMRI during routine evaluation.