Search Results (66)

Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a heterogeneous group of small-vessel vasculitides, characterized by the presence of antibodies binding to myeloperoxidase (MPO) and proteinase-3 (PR3) found in neutrophil granules. Apart from being the target of ANCA, neutrophils actively contribute to the vicious cycle of inflammation and vascular damage in AAV. On the other hand, platelets have recently been recognized as essential for thrombosis and as inflammatory effectors that collaborate with neutrophils, reinforcing the generation of reactive oxygen species (ROS) and the formation of neutrophil extracellular traps (NETs) in those diseases. Neutrophils exhibit morphological and functional heterogeneity in AAV, reflecting the complexity of their contribution to disease pathogenesis. Since long-term immunosuppression may be related to serious infections and malignancies, there is an urgent need for reliable biomarkers of disease activity to optimize the management of AAV. This review summarizes the current understanding of the role of neutrophils and platelets in the pathogenesis of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), focusing on their crosstalk, and highlights the potential for identifying novel biomarkers relevant for predicting the disease course and its relapses. Full article

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), represent a spectrum of systemic disorders characterized by necrotizing inflammation of small- to medium-sized vessels. Nailfold videocapillaroscopy (NVC) is a validated, non-invasive technique routinely employed in the assessment of microvascular involvement in systemic sclerosis and in the differential diagnosis of Raynaud’s phenomenon; its application in the context of AAV, particularly EGPA, has not been investigated yet. The present study aims to assess the presence and the possible pattern of microcirculatory abnormalities detected by NVC in EGPA patients, and to explore potential correlations between capillaroscopic findings and disease activity status. Methods: A total of 29 patients with EGPA (19 women and 10 men), aged between 51 and 73 years, and 29 age- and sex-matched healthy controls were retrospectively enrolled between October 2023 and April 2025, after providing informed consent and meeting the inclusion and exclusion criteria. NVC was conducted in both groups to assess various morphological parameters, and mean capillary density was also calculated. Results: This study observed the presence of capillaroscopic alterations in the EGPA group, including decreased capillary density (38%), neoangiogenesis (72%), rolling (100%), pericapillary stippling (66%), and inverted capillary apex (52%). Overall, when comparing healthy controls with EGPA patients, microcirculatory abnormalities were significantly more prevalent in the latter. Specifically, scores for neoangiogenesis, capillary rolling, pericapillary stippling, and inverted capillary apex showed p-values < 0.001. Conclusions: Our study demonstrates a higher prevalence of four nailfold videocapillaroscopic abnormalities in patients with EGPA compared to healthy controls. However, the identification of these capillaroscopic alterations as specific to EGPA requires further confirmation. Ongoing studies aim to explore the potential role of NVC as a diagnostic marker and to investigate its correlation with the clinical manifestations of EGPA. Full article

Background/Objectives: Although endoscopic dacryocystorhinostomy (DCR) has been widely accepted as the procedure of choice for nasolacrimal duct obstruction (NLDO) management due to most etiologies, concerns regarding the reactivation of disease and involvement of surrounding structures add to hesitation in its utilization for granulomatosis with polyangiitis (GPA) patients. No study has directly compared outcomes of external vs. endoscopic DCR in GPA patients. This information can be helpful for patient counselling and choosing a personalized surgical approach for the best results. Methods: A scoping review of the literature was performed in January 2024. The following databases were searched using a combination of MeSH (Medical Subject Headings) and keywords: Ovid MEDLINE, Ovid EMBASE, Scopus, and Web of Science. This scoping review is not registered. Medical records of two GPA patients who underwent endoscopic DCR at our center were reviewed. Results: The search yielded 96 articles; 15 articles met the inclusion criteria for a full review. Six studies with 22 procedures reported 100% success with endoscopic DCR. Nine studies with 122 procedures reported success in 88.5% of cases with external DCRs. Additional perioperative immunosuppression was recommended in patients with severe mucosal inflammation. The case series presents the disease course, details of surgery, and perioperative management in two GPA patients with NLDO who underwent endoscopic DCR successfully. Conclusions: Endoscopic DCR was associated with equivalent or better success rates and lower complications compared to external DCR in GPA patients. Ensuring disease remission state and appropriate immunomodulatory therapy can help prevent the proposed risk of endonasal disease reactivation with endoscopic DCR. Full article

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitutes a group of rare diseases characterized by autoimmune-associated inflammation and vessel damage. Based on the clinical manifestations and involvement of immune components, three disease syndromes are distinguished: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). In this review, we present the current data on the epidemiology, the clinical manifestations of each syndrome, and the most up-to-date classification criteria. The role of the underlying genetic and epigenetic abnormalities, as well as their interplay, is described. The immunological diversification of AAV is also described, with a focus on the immune cell dysfunctions detected in patients. In conclusion, we emphasize the urgent need to unravel the sophisticated mechanisms of this disease, which would enable the development of new, effective therapeutic strategies. Full article

Background/Objectives: Granulomatosis with polyangiitis (GPA) is a necrotising vasculitis characterised by granulomatous inflammation involving small vessels. In addition to specific findings for the affected organ, constitutional symptoms and joint and muscle pain can be observed. The prodromal phase, where symptoms last for months before clinical diagnosis, may suggest infection or malignancy. This may cause a delay in GPA diagnosis. The period from the first symptoms to diagnosis may last from one month to three years. In this study, we aimed to demonstrate that, as the time between the onset of symptoms and diagnosis increases, the disease involvement may become more severe, and the possibility of recurrence may increase, indicating the importance of early diagnosis. Methods: For this cross-sectional retrospective study, data from 40 patients with GPA were evaluated. Demographic, clinical, treatment, and follow-up characteristics of the patients were obtained from their medical records. Results: The mean time between the presentation of the first complaints and GPA diagnosis was 7.40 ± 11.84 (0, 60, 3; mean ± standard deviation [minimum, maximum, and median]) months. The time between the first complaints and diagnosis was longer for patients with a history of recurrence (11.44 ± 16.73 [0, 60, 4.5] vs. 4.71 ± 6.04 [0, 24, 2.5], p value = 0.260). Conclusions: GPA is an inflammatory disease with various clinical presentations. In the management of patients with GPA, awareness of its presentation is important for rheumatologists as well as other clinicians during the initial evaluation, demonstrating the importance of interclinical collaboration. Full article

Background/Objectives: The evidence regarding the efficacy of probiotics in chronic rhinosinusitis (CRS) is very limited, prompting the EPOS2020 steering group to advise against their use in CRS treatment. Therefore, further research to evaluate the impact of probiotics on microbial communities is particularly important. This study aimed to assess the influence of probiotic nasal rinses on nasal microbiota profiles in patients with primary CRS, granulomatosis with polyangiitis (GPA), and nasal septal perforation (NSP) using 16S rRNA sequencing. Methods: Thirty-six patients with nasal mucosal diseases, including sixteen with primary CRS, eleven with GPA, and nine with NSP, were randomly assigned to either a study group receiving nasal rinses with probiotics containing Lactobacillus plantarum and Bifidobacterium animalis, or a control group using nasal rinses with saline. Metagenomic analysis targeting the V3–V4 hypervariable region of the 16S rRNA gene was performed to characterize bacterial and archaeal populations. Results: At the genus level, the most abundant co-colonizers included Staphylococcus, Streptococcus, and Haemophilus. After one month of probiotic rinsing, a decrease in abundance of the genera Finegoldia (p = 0.010), Haemophilus (p = 0.020), Streptococcus (p = 0.027), Staphylococcus (p = 0.033), Micrococcus (p = 0.035), Corynebacterium (p = 0.049), Gemella (p = 0.055), Rubrobacter (p = 0.055), and Pseudonocardia (p = 0.058) was observed. Conversely, the abundance of probiotic species Lactobacillus plantarum and Bifidobacterium animalis increased. Moreover, increases in the genera Dolosigranulum and Stenotrophomonas were observed, although they did not reach statistical significance. Conclusions: Probiotic nasal rinses may contribute to restoring microbial homeostasis by reducing genera associated with inflammatory dysbiosis in nasal inflammatory diseases, warranting further research on their clinical benefits. Full article

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (ANCA-vasculitis) is an autoimmune disease characterized by inflammation and necrosis of small or medium vessels. In the past, the role of the complement in the pathogenesis of ANCA-vasculitis has been underestimated, due to the paucity of the complement at sites of injured glomeruli. Following evidence from animal models of the major role of the complement in pathogenesis of ANCA-vasculitis, the complement has again attracted interest. Immunohistology analysis of pauci-immune glomerulonephritis—ANCA glomerulonephritis (ANCA-GN)—reveals the presence of complement products and membrane attack complex, suggesting their involvement in the disease process. Researchers emphasize the complement classical or lectin pathway as a contributor to the development of ANCA-vasculitis. The era of targeted therapies to suspend the complement activation as a therapy for ANCA-vasculitis has arrived, and thus, the comprehension of its role is very important. This review summarizes recent insights on the important role of complement activation in the development of ANCA-vasculitis as well as the emerging therapeutic possibilities that target complement components for the treatment of this condition. Full article

Background/Objectives: The overlap syndrome of primary Sjögren syndrome (pSS) with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) (OvSD/pSS/AAV) has been reported in other studies. This study applied the new criteria for AAV proposed by the American College of Rheumatology/European Alliance of Associations for Rheumatology in 2022 (the ACR/EULAR criteria) to patients with pSS presenting signs and symptoms suggestive of small- and medium-vessel vasculitis. It also investigated the overall frequency of OvSD/pSS/AAV and the major contributing factors to its reclassification. Methods: This study included 116 patients with pSS from March 2005 to December 2020, according to the inclusion criteria, and defined signs and symptoms suggestive of small- or medium-vessel vasculitides as lung parenchymal lesions supporting AAV, peripheral neuropathy, and suspected renal vasculitis. The classification could be made when the total scores for microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) are ≥5 points and the eosinophilic GPA (EGPA) score is ≥6 points. Results: The median age of the patients was 56.0 years, and 101 patients (87.1%) were women. In total, 95, 12, and 37 patients had lung parenchymal lesions supporting AAV, peripheral neuropathy, and suspected renal vasculitis, respectively. According to the ACR/EULAR criteria for AAV, 35 of 116 (30.2%) patients were reclassified as having OvSD/pSS/AAV. Among these 35 patients, 4 were reclassified as having both OvSD/pSS/MPA and OvSD/pSS/GPA and 1 as having both OvSD/pSS/MPA and OvSD/pSS/EGPA simultaneously. The major contributing factor to the reclassification of OvSD/pSS/AAV was ANCA positivity. Conclusions: The overall frequency of the reclassification of OvSD/pSS/AAV was 30.2% in pSS patients presenting signs and symptoms suggestive of small- and medium-vessel vasculitis. Its likelihood increased according to ANCA positivity. Full article

Background/Objectives: Granulomatosis with polyangiitis (GPA) is an autoimmune vasculitis, often presenting first with sinonasal symptoms diagnosed as vasculitis chronic rhinosinusitis (CRS). Patients with limited (L) GPA do not have renal involvement and often have more local sinonasal disease. Few studies have examined systemic progression in LGPA patients presenting with local sinonasal disease. Our objective was to compare GPA disease progression and activity in LGPA patients with and without CRS. Methods: Using the US Collaborative Network of the TriNetX platform, we conducted a retrospective study of adults with LGPA and CRS versus those without CRS. Outcomes were measured 1 month-5 years after patients met inclusion criteria. Primary outcomes were acute sinusitis, end-organ damage, and major GPA disease activity. Secondary outcomes were end-organ damage and major disease activity for each organ system and mortality. Results: There were n = 1097 in the LGPA with CRS cohort and n = 3331 in the LGPA without CRS cohort, with n = 1023 in each cohort after 1:1 propensity matching on age, gender, ethnicity, and race. We found a significantly greater risk of acute sinusitis (risk ratio: 4.80, 95% confidence interval: [2.89,7.99]), end-organ damage (2.99 [2.41, 3.70]), and major disease activity (2.41 [1.73, 3.35]) comparing patients with CRS to those without. LGPA patients with CRS had no significant difference in mortality compared to those without CRS (0.94, [0.64,1.38]). Conclusions: Patients with LGPA and CRS have greater risk of developing disease progression and increased organ system disease activity compared to LGPA without CRS. Full article

Wegener’s granulomatosis (WG), or granulomatosis with polyangiitis (GPA), is a rare autoimmune disease that can cause inflammation in various organs, including the kidneys. Renal involvement in GPA is a major cause of morbidity and mortality in both adults and children, and early detection and effective treatment are essential for preventing renal failure. This review aims to summarize the current evidence on the incidence, clinical features, treatment, and outcomes of renal involvement in children with Wegener’s granulomatosis. The incidence of renal involvement in children with GPA ranged from 26% to 56%. Renal involvement is a common and serious complication of GPA in children, and early detection and effective treatment are crucial for preventing renal failure. The most common clinical features were proteinuria, hematuria, and reduced glomerular filtration rate. The majority of children with renal involvement in GPA required treatment with corticosteroids and immunosuppressive agents. The treatment outcomes varied among the studies, with some children achieving remission of renal involvement while others developed end-stage renal disease. Although most features are the same in children and adult patients, this review summed up some important differences between these two different populations. Further studies are needed to identify the most effective treatment strategies for renal involvement in children with GPA. Full article

Background/Objectives: This study compares the clinical features and treatment outcomes of granulomatosis with polyangiitis (GPA) based on age at onset. Methods: A retrospective longitudinal cohort of patients with GPA diagnosed between January 1978 and December 2015 was analyzed, stratified by age at diagnosis: ≤30 years (young group), 31–59 years (middle-aged group), and ≥60 years (older group). The comparative analysis included demographic data, organ involvement, laboratory results, anti-neutrophil cytoplasmic antibody (ANCA) status, comorbidities, treatments, and outcomes. Results: The analysis included 264 patients newly diagnosed with GPA. Older patients exhibited significantly higher rates of peripheral neuropathy and liver involvement. They had more severe lung diseases and required lung biopsies more frequently. Patients in the middle-aged group exhibited the highest likelihood of severe anemia. Peripheral neuropathy was more common in this group than in younger patients, and their lung disease was less severe than in older patients but more severe than in younger patients. Young patients exhibited mild disease with the least severe lung involvement, mild anemia, and highest albumin levels. Baseline comorbidities and post-treatment adverse events increased significantly with age at diagnosis. Treatment strategies and efficacy were similar across groups, although older patients tended to receive lower initial doses of cyclophosphamide and corticosteroids. Conclusions: Age at diagnosis influenced GPA clinical characteristics. While the treatment did not vary significantly by age at onset, tailoring therapy to a patient’s age is crucial to optimize outcomes and minimize complications. Full article

Background: Tracheobronchial stenosis is a significant complication in granulomatosis with polyangiitis (GPA), a systemic vasculitis that primarily affects the upper respiratory tract, kidneys, and lungs. The involvement of the tracheobronchial tree in GPA leads to airway narrowing, which can result in severe respiratory symptoms and increased morbidity, often requiring prompt diagnosis and management to prevent life-threatening airway obstruction. Method: We present the case of a 28-year-old male with mild exertional dyspnea, stridor, and retropharyngeal sputum. Clinical investigations revealed subglottic and bronchial concentric stenosis with granulomatous inflammation. A diagnosis of granulomatosis with polyangiitis (GPA) with isolated tracheobronchial stenosis (TBS) was confirmed. Results: Given the severity of airway obstruction, multidisciplinary management was initiated, combining rigid bronchoscopy with systemic immunosuppressive therapy. Post-intervention follow-up demonstrated significant airway improvement and maintained remission after two years. Conclusions: This case highlights TBS as a potentially debilitating GPA manifestation requiring a combination of systemic and endoscopic therapies. Further studies are needed to optimize therapeutic approaches and improve outcomes in GPA-associated TBS. Full article

Background and Clinical Significance: Granulomatosis with polyangiitis (GPA) represents a rare autoimmune disease with granulomatous inflammation, tissue necrosis, and systemic vasculitis of the small and medium blood vessels. Although the clinical elements vary, aortic involvement is exceptional and it represents a challenge that requires a rapid intervention with the potential of displaying a fulminant evolution. Case Presentation: We report a 64-year-old male with an 18-year history of GPA who presented atypical low back pain. Following ultrasound and computed tomography exams, the initial suspicion was an intramural descending aorta hematoma, surrounded by a peri-aortic sleeve suggesting a chronic inflammation. Serial non-invasive assessments revealed a progressive lesion within the next 10 to 12 days to an aortic wall rupture, despite the absence of previous aneurysmal changes. The peri-aortic fibrous inflammatory sleeve was life-saving, and emergency minimally invasive surgery was successful, including the massive improvement in back pain. Conclusions: To our knowledge, this is a very rare scenario in GPA; we found only 18 other cases (the oldest report being from 1994). An interventional approach was mentioned in a few cases as seen in this instance. Glucocorticoid medication for GPA might act as a potential contributor to symptomatic osteoporotic fractures which require a prompt differential diagnosis. Unusual aortic manifestations (such as intramural aortic hematoma or aortic wall rupture) are difficult to recognize since the index of clinical suspicion is rather low. A prompt intervention may be life-saving and a multidisciplinary team is mandatory. Minimally invasive surgical correction of the aortic event represents an optimum management in the modern era. Such cases add to the limited data we have so far with respect to unusual outcomes in long-standing GPAs. Full article

Background: Calcified amorphous tumor (CAT) is a rare, mostly incidental tumor-like cardiac lesion of unknown histogenesis. Current imaging modalities do not differentiate between CAT and other masses. As it can be a source for embolization, surgical excision of CAT is mandatory. CAT in patients with Granulomatosis with polyangiitis (GPA) is exceedingly rare. Methods: This systematic literature review was prompted by the case of a CAT in a patient with GPA. The search of all types of studies in two databases (PubMed and Scopus) was conducted through November 2024 to identify the relevant studies. Results: Nine studies were included describing cases of patients being diagnosed with GPA and a cardiac mass. All included patients had a histopathological examination of the either biopsied or surgically resected mass. Only one case reported a CAT. In our case, the patient was diagnosed with GPA through a kidney biopsy, whereas a cardiac mass in the right atrium was diagnosed by echocardiography during evaluation for possible kidney transplantation. One year later a progression was observed, and the mass was resected. The histopathological examination revealed a CAT. The patient could be successfully discharged in a good clinical condition. Conclusions: This systematic literature search and case report highlight the importance of regular echocardiographic examination in patients with GPA. Moreover, surgical excision is crucial for the diagnosis and for further therapy planning, regardless of whether the mass is neoplastic or not. Full article

The document provides a comprehensive overview of the diagnosis, monitoring, and treatment of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) with renal involvement, focusing on granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). It outlines the definitions, clinical presentation, histopathological classification, monitoring strategies, induction and maintenance treatments, as well as special considerations for relapsing, refractory, and frail patients with renal AAV. The document was prepared by the Catalan Group for the Study of Glomerular Diseases (GLOMCAT), which comprises nephrologists with extensive experience in the diagnosis and treatment of AAV patients. Several virtual and face-to-face meetings were held for coordination, section assignments, and content discussion. An exhaustive and systematic search of the literature was carried out, which included, among others, the following databases: PubMed, EMBASE, Cochrane Library, Google Scholar, and ClinicalTrials.gov, as well as the abstract books of national and international congresses. Overall, the document provides a comprehensive guide for clinicians managing patients with renal AAV, offering evidence-based recommendations for diagnosis, monitoring, and treatment across various clinical scenarios. Full article