Antibody-associated Vasculitis Research Articles

Diagnosis and Treatment of Renal ANCA Vasculitis: A Summary of the Consensus Document of the Catalan Group for the Study of Glomerular Diseases (GLOMCAT)

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.

Immune checkpoint molecules performance in ANCA vasculitis

ObjectiveThe PD-1 axis promotes protection against autoimmunity. Immune checkpoint (IC) molecules performance in anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) remains unknown. This study aims to assess the IC pathway’s role in...

Admission chest CT scan of intensive care patients with interstitial lung disease: Unveiling its limited predictive value through visual and automated analyses in a retrospective study (ILDICTO)

BackgroundClinical course prediction of patients with interstitial lung disease (ILD) admitted to the intensive care unit (ICU) for acute respiratory failure (ARF) can be challenging. This study aimed to characterize the prognostic value of admission chest CT-scan in this situation. MethodsWe retrospectively included ILD patients admitted to a French ICU for acute respiratory failure requiring oxygen. Patients with lymphangitis carcinomatosis and ANCA vasculitis were excluded. We analyzed every admission chest CT-scan using two different approaches: a visual analysis (grading the extent of traction bronchiectasis, ground glass and honeycomb) and an automated analysis (grading the extent of ground glass and consolidation with a dedicated software). The primary outcome was ICU mortality. ResultsBetween January 2014 and October 2020, 81 patients presented an acute respiratory failure with ILD on the admission chest CT-scan. In univariate analysis, only the main pulmonary artery diameter differed between patients who survived and those who died in ICU (30 vs 32 mm, p = 0.021). In multivariate analysis, none of the radiological funding was associated with ICU mortality. Visual and automated analyses did not yield different results, with a strong correlation between the two methods. However, the identification of an UIP pattern (and the presence of honeycomb) was associated with a poorer response to corticosteroid therapy. ConclusionOur study showed that the extent of radiological findings and the severity of fibrosis indices on admission chest CT scans of ILD patients admitted to the ICU for ARF were not associated with subsequent deterioration.

Mucosal dominant presentation of hydralazine-induced ANCA vasculitis.

The typical clinical spectrum of hydralazine induced AAV involves glomerulonephritis and pulmonary haemorrhage, with or without cutaneous features. Cases with isolated cutaneous features are exceptionally rare. We present an uncommon case of an 84-year-old woman with hydralazine-induced AAV presenting with violaceous plaques with ulceration and significant mucositis without other organ involvement.

7876 Complex Complications of Thionamide Therapy in One Patient: Methimazole-Induced Agranulocytosis and PTU-Induced ANCA-Vasculitis

Abstract Disclosure: E. Zweibach: None. A. Chang: None. Introduction: Thionamides, including methimazole and propylthiouracil (PTU), are essential for treating Graves' disease but can cause serious side effects such as agranulocytosis and ANCA-vasculitis. Although agranulocytosis itself is quite uncommon, the incidence of ANCA vasculitis is even more infrequent, making combination of conditions particularly exceptional. We present a unique case of a Graves' disease patient who suffered both complications: agranulocytosis due to methimazole and ANCA vasculitis from PTU, with successful resolution of both the clinical signs and laboratory findings following drug discontinuation. Case: 61-year-old female with a history of Graves' disease previously managed with methimazole but discontinued due to agranulocytosis, presented with lower extremity swelling, atrial fibrillation with RVR, and high output cardiac failure. Laboratory tests showed hyperthyroidism (TSH 0.008, Free T4 3.07), leading to a diagnosis of thyroid storm. Patient was started on PTU inpatient and discharged with this medication to home. Eight months later, the patient developed alarming signs including gross hematuria, oral ulcers, widespread muscle pain, found to have AKI with Cr of 2.79 and hospitalized for further eval. While hospitalized, the patient was found to have a range of complications including leukopenia with normal granulocyte count, anemia, AKI, proteinuria, and microscopic hematuria. Rheumatological evaluation showed +ANA and a high ANCA titer >1:1280, along with elevated levels of MPO and PR-3, and reduced complement levels. These symptoms and laboratory findings led to a diagnosis of ANCA vasculitis induced by PTU, resulting in the cessation of this medication. Following the discontinuation of PTU there was a notable improvement with normalization of serum Cr and resolution of proteinuria within 1.5 months. Concurrently, MPO and PR-3 levels began to decrease, and her hematological abnormalities resolved. During this period, her Free T4 levels gradually increased, leading to the administration of radioactive iodine for the treatment of her Graves' disease. Discussion: This case underscores the complexities in treating a patient with Graves' disease highlighting the unpredictable and severe side effects of antithyroid drugs like agranulocytosis and ANCA vasculitis. We report the first documented case of sequential thionamide-induced complications: agranulocytosis with methimazole, followed by ANCA vasculitis with propylthiouracil. Although both can arise from immune responses to these medications, they are distinct in their pathophysiology and are not associated with one another. The unpredictable nature of these side effects complicates prevention. Educating patients to promptly report any unusual symptoms and immediate medical intervention with drug discontinuation are key to managing these adverse reactions effectively. Presentation: 6/1/2024

12238 A Case Of Drug-induced Vasculitis

Abstract Disclosure: C. Javier: None. M.H. Horani: None. Title: A Case of Drug-Induced VasculitisChristian Javier, DOMohamad Horani, MD Introduction: Graves’ disease is commonly managed with thionamides. These have rare but serious complications of drug-induced lupus-like syndrome and vasculitis. In patients who present with rheumatologic and inflammatory findings, it is important to consider the possibility of these complications . Case Presentation: We present a 30-year-old female with Graves disease, asthma, and hypertension with concerns for cough, body aches, fatigue, bilateral flank pain, erythema, itching and burning of the hands, in the setting of recent spontaneous miscarriage. She was diagnosed with Graves’ nearly 2 years ago and was started on methimazole, but discontinued due to adverse effects of the medication. She was then started on PTU. The patient had a seizure, and head CT revealed a left-sided 4 cm brain mass in the left frontal lobe. The patient was admitted to the ICU, intubated, and started on IV Decadron. Further imaging showed multiple enlarged lymph nodes in the axilla, mediastinum, and hilum. Her brain mass was biopsied showing acute inflammation with early acute necrosis and multifocal fibrinous vascular necrosis, which has been reported in ANCA vasculitis. She had undetectable TSH (0.35-5.0) and free T4 of 1.54 (4.87-11.72). She was also found to have proteinuria and AKI. At this point, PTU-induced vasculitis and drug-induced lupus were suspected and PTU was discontinued. She was found to have low complement levels (C3 40, C4 6.1), elevated p-ANCA (>1:1280), ANA (1:640), anti-histone IgG (2.3) dsDNA (1:160), SSB La antibodies (70), anti-Smith antibodies (319), anti-RNP antibodies (146). Renal biopsy is pending. After discontinuation of PTU and administration of IV steroids, her symptoms largely resolved and she was extubated and stable for discharge. Conclusion: In patients being treated for Graves’ disease with PTU, there are rare but life-threatening complications associated with this medication including lupus-like state or vasculitis with elevated ANCA antibodies. Common presenting symptoms of drug-induced vasculitis include fever, joint pains, myalgias, skin lesions, glomerulonephritis, cerebral angiitis, and pulmonary hemorrhage. The onset of vasculitis can occur at any time after starting medication, with one study showing an average onset of 36 months after initiation of therapy. Other case reports have demonstrated positive serologies for ANA and anti DS-DNA antibodies in those with PTU-induced lupus. Drug induced lupus can also occur in patients taking PTU, with kidney and CNS involvement being more uncommon. In those with p-ANCA associated vasculitis secondary to PTU, anti-GBM, antiphospholipid, and antinuclear antibodies can also be present. Presentation: 6/1/2024

The real-world use and effectiveness of avacopan in routine practice for the treatment of ANCA vasculitis. First experiences in Spain.

ANCA-associated vasculitis (AAV) are chronic diseases with relapses that associate organic damage because of the disease and its treatment. Avacopan is a new treatment indicated for AAV. We present the first experiences with avacopan in Spain as part of an Early Access program. Patients with AAV who started avacopan between June 2022-September 2023 were included. For comparison, a historical cohort of patients diagnosed with AAV around the same time and treated without avacopan was also included. 29 patients treated with avacopan were analyzed. Twelve patients (41.4%) were male, median age was 56 years. Most of patients were ANCA MPO positive (21/29, 72.4%). The most frequently affected organ was the kidney (23/29, 79.31%), with a mean eGFR of 23.2 ml/minMedian follow-up was 456.8 ± 181.7 days with a remission rate of 86.2%. eGFR increased from 23.2 ± 11.2-38.38 ± 18.55 ml/min after 12 months of diagnosis.2 patients had Adverse Events related to avacopan as severe neutropenia and a gastrointestinal affectation , 13 infections were reported and 1 death.Patients treated with avacopan received a significantly lower cumulative dose of prednisone at 6 and 12 months (p-values of 0.02 and <0.01, respectively) compared with historical controls. The evolution of GFR at 1 year of follow-up and the incidence of relapse were similar in both groups. The combination of avacopan in clinical practice presents a good safety profile and provides added value by contributing to the control of AAV activity, increase GFR, and removal of steroids.

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.

Two Decades Rituximab Therapy in Anti-Neutrophil Cytoplasmic Antibody Associated Vasculitis.

Remission failure and relapse numerate as one of the main problems in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAVs). The need for new agents that provide effective and safe induction accompanied by sustained remission seems to be urgent in clinical care. The efficacy and safety of rituximab (RTX) for AAVs therapy has been reported in various studies. RTX therapy offers several advantages to treating AAVs patients compared to other therapeutic approaches including reduction of Glucocorticoids (GCs) and conventional Immunosuppressive therapy (IST) usage during both the induction of remission and maintenance phases. This reduction can lead to a lower rate of serious complications making RTX therapy a safer option. It seems that RTX may provide improved clinical outcomes in these patients mediated via B-lymphocyte depletion, Proteinase 3-antineutrophilic cytoplasmic antibody (PR3-ANCA), and myeloperoxidase-antineutrophilic cytoplasmic antibody (MPO-ANCA) titers reduction. In this regard, some uncertainties have been reported to validate the association between such depletion and clinical improvement, as suggested by other sources of autoreactive B cells that did not target with RTX. Due to the prolonged B cell depletion, fixed intervals and adjusted dosage of RTX may be required in patients with AAVs. In this narrative review, we aimed to insight better understand regarding the efficacy of RTX for effective induction and sustained remission in patients with AAVs. It seems that discovering new biomarkers predicting relapse in AAVs patients can lead to future targeted therapy.

Atypical Anti-GBM with ANCA Vasculitis- A Rarest of the Rare Entity: Index Case from Eastern India

Anti-glomerular basement membrane (GBM) antibody glomerulonephritis is an extremely rare glomerular disease. Around 90% of the patients are positive for serum anti-GBM antibodies while up to 10% can be negative. In such patients, only a kidney biopsy can reveal the anti-GBM disease it is then labeled as an atypical anti-GBM disease. Though anti-GBM disease can be associated with Anti Neutrophil Cytoplasmic Antibodies (ANCA) positivity, it is extremely rare to find atypical anti-GBM with ANCA positivity so much so that till now there are very few such cases reported from across the world. The case presented here is one such case where the patient presented with adult-onset nephrotic syndrome features with active urinary sediments and mildly deranged renal function. Myeloperoxidase (MPO) ANCA was positive and it was considered ANCA-associated crescentic glomerulonephritis (GN) but after the renal biopsy the picture was of anti-GBM disease. She was treated with pulse methylprednisolone but her creatinine increased in the meantime and considering anti-GBM she was put on Plasma Exchange (PLEX). She received 5 sessions of PLEX after which her renal function improved. She was also planned for Rituximab which could not be given due to local infection. As there are no protocols for treating such cases because of the extremely rare nature of the presentation, this case will increase the understanding of such presentations for the clinicians. This will help to plan for building the approach for such cases.

Infection prophylaxis among patients with antineutrophil cytoplasmic antibody (ANCA) vasculitis: a scoping review.

Severe infections associated with the use of strong immunosuppressive medication are a leading cause of morbidity and mortality in patients with ANCA vasculitis (AV). While guidelines conditionally recommend trimethoprim-sulfamethoxazole (TMP-SMX) prophylaxis for Pneumocystis jirovecii pneumonia in AV patients, robust evidence on prophylaxis strategies is lacking. This scoping review aimed to assess the existing evidence on infection prophylaxis in AV patients, identify knowledge gaps, and guide future study design. A comprehensive search of six databases and relevant references identified original studies in English from January 1, 2000, to July 31, 2020. Inclusion criteria encompassed studies evaluating the impact of any antimicrobial prophylaxis strategy on infection-related outcomes in AV patients receiving immunosuppressive treatment. Studies were screened by four researchers using a blinded approach. Data was extracted by two reviewers, with differences resolved via consensus in consultation with a third reviewer. Nineteen studies met inclusion criteria, including two randomized trials and 17 cohort studies, with TMP-SMX being the most commonly assessed prophylactic strategy. The studies varied in sample sizes, outcomes measured, prophylactic strategies employed, and proportion of patients who received the regimen. Most cohort studies included no or limited control of potential confounding factors. This scoping review suggests significant variation in AV patients' receipt of TMP-SMX and alternative infection prophylaxis approaches. Observational studies using large secondary healthcare databases with rigorous designs are needed to provide high-quality evidence of the real-world effectiveness of antimicrobial prophylactic regimens, to improve clinical decision-making and quality of care for AV patients receiving immunosuppressive treatment.

Granulomatosis with polyangiitis: clinical characteristics and updates in diagnosis.

Granulomatosis with polyangiitis (GPA) is a rare systemic disease characterized by granulomatous inflammation of the respiratory tract and necrotizing vasculitis of small and medium vessels often associated with the production of anti-neutrophil cytoplasmic antibodies (ANCA) directed mainly against leukocyte proteinase 3 (PR3). Usually, it involves upper airways, lungs, and kidneys, however any organ may be affected. The diagnosis is based on clinical, radiological, and serological findings. Biopsies, although strongly recommended, are not always feasible and often provides non-specific features. ANCA plays a crucial role in the diagnosis of GPA; nevertheless, ANCA detection is not a substitute for biopsy, which plays an important role in suspected cases, particularly when histological confirmation cannot be obtained. Significant advances have been made in classification criteria and phenotyping of the disease, particularly in determining the nuances between PR3-ANCA and myeloperoxidase (MPO)-ANCA vasculitis. This has led to better characterization of patients and the development of targeted treatment in the future. In addition, better identification of cytokine and immunological profiles may result in immuno-phenotyping becoming a new approach to identify patients with ANCA-associated vasculitis (AAV). Due to the chronic relapsing-remitting nature, strict follow-up of GPA is necessary to provide appropriate management. The search for the accurate marker of disease activity and to predict relapse is still ongoing and no predictor has been found to reliably guide therapeutic decision-making.

Unprecedented Encounter: A Rare Encounter of ANCA Vasculitis with Diffuse Alveolar Hemorrhage and Ileal Perforation

Unprecedented Encounter: A Rare Encounter of ANCA Vasculitis with Diffuse Alveolar Hemorrhage and Ileal Perforation

Patients with ANCA vasculitis treated with therapeutic plasma exchange in General Hospital Zadar, Croatia

Patients with ANCA vasculitis treated with therapeutic plasma exchange in General Hospital Zadar, Croatia

Atypical skin and mucosal lesions with renal involvement in ANCA vasculitis - a case report.

Atypical skin and mucosal lesions with renal involvement in ANCA vasculitis - a case report.

Kidney Biopsy Findings Among Patients With Diabetes in the Cleveland Clinic Kidney Biopsy Epidemiology Project

Rationale & ObjectiveDiabetic Kidney Disease (DKD) is a significant complication of diabetes mellitus, often leading to kidney failure. The absence of well-defined factors prevents distinguishing DKD from Non-Diabetic Kidney Disease (non-DKD; alternative primary diagnosis identified on kidney biopsy). Study DesignRetrospective cohort study. Setting& Participants: 1,242 patients with a history of diabetes from the Cleveland Clinic Kidney Biopsy Epidemiology Project between January 2015 and September 2021. ExposureProteinuria, retinopathy, A1c and eGFR. OutcomesNon-DKD, defined as an alternative primary diagnosis identified on kidney biopsy other than DKD. Analytical ApproachMultivariate logistic regression model with backward elimination method. ResultsAt the time of biopsy, the median (IQR) age was 63 (53-71) years, 58.8% were male, hemoglobin A1c was 6.7% (6.0-8.1), and serum creatinine was 2.5 (1.6-3.9) mg/dL. Among 1,242 patients, 462 (37.2%) had DKD alone, and 780 (62.8%) had non-DKD. Among those with non-DKD, the most common diagnoses were focal segmental glomerulosclerosis (24%), global glomerulosclerosis NOS (13%), acute tubular necrosis (9%), IgA nephropathy (8%), ANCA vasculitis (7%), and membranous nephropathy (5%). Factors associated with having non-DKD on biopsy were having no retinopathy (vs retinopathy) (adjusted odds ratio [aOR], 3.98; 95% CI, 2.69-5.90), lower A1c (<7% vs. ≥7%) (aOR, 3.08; 95%CI, 2.16-4.39), higher eGFR (≥60 vs. <60 ml/min/1.73m2) (aOR, 2.39; 95%CI 1.28-4.45), microalbuminuria (<300 vs. macroalbuminuria ≥300 [mg/g]) (aOR; 2.94; 95%CI, 1.84-4.72), and lower protein to creatinine ratio in random urine (<3 vs. ≥3mg/mg) (aOR; 1.80; 95%CI, 1.24-2.61). LimitationsSelection bias of clinically indicated biopsies, not protocol biopsies, which likely represent a ceiling (maximum) for non-DKD. ConclusionsAmong diabetic patients undergoing kidney biopsy, 63% have findings in addition to DKD on biopsy. We identified clinical parameters associated with non-DKD in the setting of diabetes. This provides valuable information for clinicians when kidney biopsy should be considered among diabetic patients to capture all etiologies of proteinuria and kidney dysfunction.

Epidemiology of ANCA vasculitis in Northern Spain.

The incidence of anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) shows disparate results due to variable classification criteria and heterogeneous-population series. We aimed to estimate the incidence of AAV in a well-defined population with standardized classification criteria. Population-based study of AAV patients diagnosed from January 2000 to December 2023 in Cantabria, Northern Spain. Patients were classified according to ACR/EULAR 2022 into granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), or unclassified vasculitis if the criteria were not met. Eosinophilic granulomatosis with polyangiitis (EGPA) patients were not included. The annual incidence rates were estimated by cases over 1,000 000 (106) (95% CI) including overall AVV, type of AAV, sex, and year of diagnosis. A literature review was also performed. We included 152 (80/72 men; mean age; 70.6 ± 13.18 years) patients. They were classified as MPA (67; 44%), GPA (64; 42.2%), and unclassified vasculitis (21; 13.8%). Annual incidence was 13.4 (10-16.8)/106 [male 14.5 (10.5-18.5); female 12.1 (8.7-15.6)]. The Annual incidence of MPA was 5.9 (4-7.8)/106 and GPA 5.6 (3.9-7.3)/106. The mean Annual incidence increased from 6.1 (4.5-7.7)/106-16.5 (5.6-27.4)/106 in the last three years, particularly, in GPA from 2.3 (0.3-4.9)/106-8.2 (2-14.5)/106. The prevalence of AAV was 184.7 (181-188)/106. During a 20-year period we found that the incidence of AAV (GPA and MPA) in Northern Spain is higher than Southern Spain, but lower than Northern European countries. An increase in the incidence was observed in the last years.

Brugada Syndrome and Sudden Cardiac Death: An Electrocardiographic History.

A 22-year-old male with a history of anti-neutrophil cytoplasmic antibody vasculitis, renal transplant, hypertension, and no known family history of sudden cardiac death suffered a witnessed cardiac arrest. An initial rhythm strip recorded by emergency medical services revealed ventricular fibrillation. Return of spontaneous circulation was achieved after three rounds of cardiopulmonary resuscitation, defibrillation, and intravenous epinephrine. The patient was brought to the emergency department and admitted to the intensive care unit. He was diagnosed with Brugada syndrome, and an automatic implantable cardioverter-defibrillator (AICD) was placed after discharge. Brugada syndrome is characterized electrocardiographically by ≥2 millimeters (mm) ST-segment elevation in leads V1-V2 with either "coved type" (type 1) or "saddleback" (type 2) ST-segment morphology, or ≤2mm ST-segment elevation in V1-V2 with either "coved" or "saddleback" morphology (type 3). The absence of these patterns on isolated electrocardiograms (ECG) does not exclude the diagnosis, as dynamic fluctuations in ECG patterns are well-documented and can be induced by various physiologic stressors. This case provides an uncommon, complete electrocardiographic history of Brugada syndrome, from out-of-hospital cardiac arrest to AICD placement and depicts dynamic fluctuations between Brugada patterns and normal ECGs. This highlights the importance of serial ECGs in diagnosis, as sudden cardiac death is often the first or only presentation of Brugada syndrome.

Clinical cases of vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA vasculitis) induced by COVID-19

COVID-19 is similar to immunoinflammatory rheumatic diseases in clinical manifestations, immune responses and pathogenetic mechanisms. Specific autoantibodies as markers of autoimmune diseases can be detected in patients with coronavirus infection. Most often, after COVID-19, antinuclear antibodies, antibodies to phospholipids, to cardiolipin, to β2-glycoprotein, to cytoplasmic antigens SS-A and SS/B, and cyclic citrulline-containing peptide are detected. In moderate and severe cases of COVID-19 infection with pulmonary-renal syndrome, cytokine storm, antineutrophil cytoplasmic antibodies, antibodies to myeloperoxidase and to proteinase 3 have become more frequent, which can trigger neutrophil NETosis with the formation of extracellular neutrophil traps — networks and induce the development of autoimmune processes and the appearance of de novo immunoinflammatory rheumatic diseases: arthritis , systemic lupus erythematosus, vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA vasculitis). Our study presents 4 clinical cases of patients with ANCA vasculitis with a history of recent coronavirus infection, chronologically verified. The average time after COVID-19 and the onset of ANCA vasculitis was about 3 months; damage to the upper respiratory tract, lower respiratory tract, skin and renal syndromes were observed. The severity of ANCA vasculitis did not depend on the severity of coronavirus infection: a mild course of COVID-19 infection did not exclude a severe course of ANCA vasculitis. It is recommended that all patients during and after coronavirus infection with post-COVID syndrome in the presence of damage to the ENT organs, kidneys, and skin are screened the presence of antineutrophil cytoplasmic antibodies to exclude the onset of ANCA vasculitis.

Examining the role of patient-reported external factors and risk of relapse in anti-neutrophilic cytoplasmic autoantibody vasculitis.

The role of stressors, insect bites, and infections on disease relapse of ANCA vasculitis has yet to be entirely explored, with limited retrospective studies focused on disease onset from small participant cohorts. Our study analyzes longitudinal survey data from 2011-2022 to evaluate this perspective from a large ANCA vasculitis cohort. We collected surveys every three to six months to obtain information on self-reported psychological stressors and significant life events, insect bites, and infections throughout clinical disease. We defined cohorts as those who relapsed (Relapse Cohort) and controls as those who did not relapse (Remission Cohort) during the study period. Survey responses were retrospectively reviewed during a 15-month timeframe prior to relapse or during 15 months of remission and categorized by type of stress event, insect bite, and infections at every available 3-month interval. There were no significant differences in stress and insect bites between the relapse and remission cohorts. Patients who relapsed reported more frequent upper respiratory infections and other infections, such as those affecting the skin and eyes, but there were no significant differences in the incidence of pulmonary or urinary infections compared to the remission cohort. There was a significant difference in reported upper respiratory infections 9 to 15 months prior to the relapse date, indicating a remote history of infections as a potentially significant physical stressor that may contribute to disease relapse. More frequent patient-reported infections, specifically upper respiratory infections, may contribute to patient vulnerability to relapse. Counseling and close monitoring of patients after infectious symptoms could aid in earlier detection of disease flares. Future studies are essential to further understand the importance of distal risk factors and how they impact relapse.