Management Of Associated Vasculitis Research Articles

Plasmapheresis in ANCA-Associated Vasculitis with Active Kidney Involvement in the United States (2016-2020): A Cross-Sectional Study.

Plasmapheresis is currently recommended when antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) presents with severe kidney and/or lung involvement. This cross-sectional study aimed at describing characteristics of hospitalized patients diagnosed with AAV with severe kidney involvement undergoing plasmapheresis in the US. We defined the study population as adults hospitalized for active kidney involvement with a new diagnosis of AAV (by subtype or unspecified). We established the cohort from the 2016-2020 National Inpatient Sample by ICD-10-CM codes. In this cross-sectional study, we described demographic and clinical characteristics, associated inpatient procedures, lengths of stay, hospital costs, and disposition at discharge comparing patients treated and not treated with plasmapheresis. We identified a total of 975 cases of hospitalized AAV with acute kidney involvement in the US treated by plasmapheresis over the 5-year period. Demographic characteristics of patients who received plasmapheresis were similar to those in patients who did not (n=5670). There were no regional differences in the proportion of patients who received plasmapheresis; however, plasmapheresis was deployed more frequently among patients admitted to urban teaching hospitals relative to rural and non-teaching hospitals. Cases treated with plasmapheresis were more likely to have had acute kidney injury (AKI) (96% vs. 90%, p=0.0007), AKI requiring dialysis (52% vs 16%, p<0.001), hypoxia (40% vs. 16%, p<0.0001), and respiratory failure requiring mechanical ventilation (13% vs. 3%, p=0.0003). During 2016-2020, plasmapheresis was deployed in approximately 20% of patients being admitted for AAV and acute kidney involvement in the US. As standards of care and practice evolve, the role of plasmapheresis in the management of AAV with acute kidney involvement will require further study.

Management of anti-neutrophil cytoplasmic antibody associated vasculitis with glomerulonephritis as proposed by the ACR 2021, EULAR 2022 and KDIGO 2021 Guidelines/Recommendations.

Updated guidelines on the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) were released in 2021 by the American College of Rheumatology jointly with the Vasculitis Foundation and, subsequently, in 2022 by the European Alliance of Associations for Rheumatology. In addition, in 2021, the Kidney Disease: Improving Global Outcomes had released updated recommendations on the treatment of AAV with glomerulonephritis (AAV-GN). Kidney involvement is particularly relevant in microscopic polyangiitis and granulomatosis with polyangiitis, but is less frequent in eosinophilic granulomatosis with polyangiitis. The management of AAV-GN has been a focus for drug development and change over the past ten years. Avoidance of progression to end-stage kidney disease (ESKD) or kidney failure is one of the main unmet needs in the management of AAV, with ESKD having a major impact on morbidity, health costs and mortality risk. Relevant changes in AAV-GN management are related to remission-induction treatment of patients with severe kidney disease, the use of glucocorticoids, avacopan and remission-maintenance treatment. All the documents provide guidance in accordance with the evidence-based standard of care available at the time of their release. With our work we aim to (i) show the progress made and identify the differences between guidelines and recommendations, (ii) discuss the supporting rationale for those, and (iii) identify gaps in knowledge that could benefit from additional research and should be revised in subsequent updates.

Recurrent ‘Occult’ 18F-FDG Uptake in an Atypical Case of Anti-Myeloperoxidase Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

A 72-year-old woman, with anti-myeloperoxidase antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), presented with two episodes of spinal pachymeningitis (at two different levels 9 years apart, cervical in 2011 and dorso-lumbar in 2020) associated with aortitis and only demonstrated by F-18 fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT). This association between aortitis and pachymeningitis in AAV appears exceptional. Moreover, the relapse of aortitis and pachymeningitis in 2020 was not accompanied by an increase in ANCA.This case demonstrates the value of 18F-FDG PET/CT in the management of AAV, providing evidence of the recurrence and distribution of lesions in various organs, including those with unexpected involvement.LEARNING POINTSInvolvement of large vessels such as the aorta is rarely associated with anti-myeloperoxidase antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), but has been described in a few cases. Possible aortic involvement should always be kept in mind while managing a patient with AAV.Pachymeningitis is rarely associated with AAV, but in case of unexplained and unspecific neurological symptoms in patients with AAV, such involvement should be considered.18F-FDG PET/CT is a promising tool for the management of patients with AAV, allowing unexpected sites, undetected by usual examinations, to be highlighted. In contrast to giant-cell arteritis, this exam has not, until now, been included in the recommended/systematic work-up of AAV.

Update of Management of ANCA-Associated Vasculitis

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is associated with significant morbidity and mortality. Management of AAV is divided into induction of remission and maintenance of remission. Management of AAV has evolved with the aim of improving treatment outcomes and minimizing treatment toxicities. In this article, we will review the latest evidence on the treatment of AAV.

ANCA-associated Vasculitis Management in the United States: Data From the Rheumatology Informatics System for Effectiveness (RISE) Registry.

The management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has evolved substantially over the last 2 decades. We sought to characterize AAV treatment patterns in the United States. We identified patients with AAV in the Rheumatology Informatics System for Effectiveness (RISE) registry who had at least 2 rheumatology clinician visits between January 1, 2015, and December 31, 2017. Demographics, medications, laboratory test results, and billing codes were extracted from the medical record. Demographic and prescription trends were assessed overall and across US regions. We identified 1462 patients with AAV, 259 (18%) with new or relapsing AAV. The majority were classified as having granulomatosis with polyangiitis (75%). The mean age was 59.8 years and 59% were female. The majority of patients were in the South (45%) followed by the Mid-West (32%), West (12%), and Northeast (8%). Patients had a median of 3 visits and follow-up of 579 days. The most commonly prescribed medications during the study period were glucocorticoids (86%) followed by rituximab (45%), methotrexate (33%), azathioprine (32%), and mycophenolate mofetil (18%); cyclophosphamide (CYC) was rarely used (7%). At the most recent visits in RISE, 47% of patients were on glucocorticoids. Prescription trends were similar across regions. To our knowledge, this is the first study to evaluate the demographics and management of AAV by rheumatologists outside of major referral centers. Management strategies vary widely, but CYC is rarely used. These observations can be used to inform future research priorities. Additional studies are needed to characterize AAV severity in RISE as well as patient and provider treatment preferences.

SILENT CORONARY VASCULITIS PRECEDING FULMINANT ANTI-NEUTROPHILIC CYTOPLASMIC ANTIBODY-ASSOCIATED PULMONARY-RENAL SYNDROME: A RARE DIAGNOSTIC AND THERAPEUTIC DILEMMA

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Various forms of vasculitides ranging from benign and self-limiting to potentially life-threatening exist. Here, we report a case of a patient who harbored an asymptomatic coronary vasculitis followed by a life threatening pulmonary-renal syndrome (PRS) secondary to anti-neutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV). CASE PRESENTATION: 75-year-old male presented to us with hemoptysis. Four months prior to current admission, he reported dyspnea upon exertion and was diagnosed with atrial fibrillation with rapid ventricular response for the first time. A chest x-ray was unremarkable whereas an echocardiogram revealed worsening of his aortic stenosis compared to the prior year. Elevated troponin and ST-T wave changes in EKG prompted a left heart catheterization that revealed multivessel coronary artery disease necessitating consideration of coronary artery bypass graft (CABG), surgical aortic valve replacement, and a maze procedure. During the procedures, his left atrial appendage was excised and sent for histopathological examination (HPE). Post CABG, on a routine outpatient follow up, he was found to have hyperkalemia with a potassium of 6.0 mmol/L and acute renal failure with a creatinine of 6 mg/dL (baseline creatinine of 1.0 mg/dl), prompting a second hospitalization. Bilateral renal ultrasound were unremarkable. Serum myeloperoxidase anti-neutrophilic cytoplasmic antibodies (MPO-ANCA) were elevated. Renal biopsy revealed a severe vasculitis of small vessels without any granulomas. No immune deposits were noted. He was diagnosed with AAV and was treated medically. Later in the course, he was also started on hemodialysis. Four months after the onset of his initial dyspnea patient presented with frank hemoptysis leading to a third hospitalization. CT of the chest with contrast revealed bilateral new extensive mixed ground-glass and consolidative opacities. He had developed severe PRS. Patient failed to improve with medical management; decided to undergo comfort care measures and passed away the same day. Retrospective chart review of the patient's left atrial appendage biopsy revealed vasculitis involving medium-sized myocardial and pericardial blood vessels. DISCUSSION: In this case, coronary vasculitis was a clinically silent disease with a histopathologic diagnosis whereas PRS secondary to AAV was a fulminant vasculitis with a fatal course despite therapy with pulse steroids, cyclophosphamide, plasmapheresis and hemodialysis. It is unclear whether coronary vasculitis in the patient was a prodrome to an impending serious illness or merely an incidental finding with no association to subsequent AAV. CONCLUSIONS: Vasculitis of the coronary arteries is uncommon, yet when involved in a patient portends a poor prognosis. While immunosuppression and plasma exchange therapy remain the cornerstone in management of AAV, therapeutic dilemma persists with regard to the clinically silent coronary vasculitis. DISCLOSURES: No relevant relationships by Ismael ABU LIMON, source=Web Response Speaker/Speaker's Bureau relationship with Otsuka America Pharmaceutical, Inc Please note: $1001 - $5000 Added 05/24/2020 by Arvind Garg, source=Web Response, value=Honoraria Consultant relationship with Fresenius Kidney Care Please note: $1001 - $5000 Added 05/24/2020 by Arvind Garg, source=Web Response, value=Honoraria no disclosure on file for Odalys Estefania Lara-Garcia; No relevant relationships by Ruby Maini, source=Web Response No relevant relationships by Priyanka Parajuli, source=Web Response No relevant relationships by Manjari Regmi, source=Web Response

Cutaneous Manifestations of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

Cutaneous manifestations of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), are poorly characterized. This report describes the dermatologic features of AAV and their association with systemic manifestations of vasculitis. A cross-sectional study identifying and comparing the cutaneous manifestations of AAV was performed using data from a large, international, collaborative effort in order to collect comprehensive clinical data on patients with vasculitis. Data from 1,184 patients with AAV from 130 centers worldwide were available. Cutaneous manifestations were common in all AAV subtypes: GPA (223 of 656, or 34%), MPA (85 of 302, or 28%), and EGPA (106 of 226, or 47%). The most frequent cutaneous manifestation in AAV (all types) was petechiae/purpura, which was observed in 181 patients (15%). Allergic and nonspecific manifestations, such as pruritus, urticaria, and maculopapular rash, were more common in EGPA than in other disease subtypes (all P < 0.01). Skin biopsy, while underutilized (performed in 22-44% of patients), was frequently found to be an effective test suitable for diagnosis of AAV (diagnostic in 68-94% of patients). Compared to patients without cutaneous manifestations, those with skin lesions more frequently had severe systemic manifestations of vasculitis (such as alveolar hemorrhage and glomerulonephritis), specifically patients with GPA or EGPA and cytoplasmic/anti-proteinase 3 (anti-PR3) ANCA-positive or ANCA-negative patients (hazard ratio >1.9 for all), but not those with MPA or perinuclear/antimyeloperoxidase ANCAs. Cutaneous manifestations are common and varied in AAV and are associated with disease severity in patients with GPA, EGPA, cytoplasmic/anti-PR3 ANCA, or ANCA-negative disease. These findings underscore the potential diagnostic and prognostic importance of the cutaneous examination in the evaluation and management of AAV.

Treatment of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Is There Still a Role for Cyclophosphamide?

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of systemic necrotizing vasculitides that includes granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis. Treatment of these conditions has improved during the past 2 decades with better understanding of these conditions and availability of newer agents. Cyclophosphamide (CYC) was the first drug demonstrated to afford successful treatment and improvement in AAV. With the emergence of newer agents with more favorable safety profiles, CYC is no longer the cornerstone of management of AAV. This article reviews existing data for treatment and the current role of CYC in the management of AAV.

Comparing outcomes of biopsy-proven anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis patients treated with cyclophosphamide in the 20th and 21st centuries: a 23-year study.

BackgroundAnti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is a multisystem autoimmune disorder associated with significant morbidity and mortality. Approximately 80–90% of patients have circulating ANCAs. Long-term outcomes appear to be improving. This retrospective study analyses the incidence and patient outcomes over a period of 23 years at a single tertiary centre.MethodsOutcomes of patients diagnosed with AAV between 1 January 1988 and 31 December 2010 were collected retrospectively. Data including patient demographics, age of diagnosis, dates of starting renal replacement therapy, death and biochemistry results were collected. Patients were divided into two cohorts (1988–99 and 2000–10) and analysed using Stata software (StataCorp, College Station, TX, USA).ResultsA complete dataset was obtained for 273 patients. Of these patients, 101 were diagnosed between 1988 and 1999 while 172 were diagnosed between 2000 and 2010. The number of patients diagnosed with AAV increased from 2.2/million in 1988 to 10.3/million in 2010. A higher proportion of patients (56.4%) in the earlier cohort presented with creatinine >500 μmol/L compared with the later cohort (30.2%; P < 0.001). Overall patient survival improved significantly between the two cohorts. Cohort 1 had a median survival of 59 months compared with 125 months for Cohort 2 (P = 0.003).ConclusionsThis study shows that AAV is being diagnosed at an earlier stage, resulting in improved outcomes. This may be because of improvements in the management of AAV and chronic kidney disease.