The Improved Kidney Risk Score in ANCA-Associated Vasculitis for Clinical Practice and Trials.

SIGNIFICANCE STATEMENT: Reliable prediction tools are needed to personalize treatment in ANCA-associated GN. More than 1500 patients were collated in an international longitudinal study to revise the ANCA kidney risk score. The score showed satisfactory performance, mimicking the original study (Harrell's C=0.779). In the development cohort of 959 patients, no additional parameters aiding the tool were detected, but replacing the GFR with creatinine identified an additional cutoff. The parameter interstitial fibrosis and tubular atrophy was modified to allow wider access, risk points were reweighted, and a fourth risk group was created, improving predictive ability (C=0.831). In the validation, the new model performed similarly well with excellent calibration and discrimination ( n =480, C=0.821). The revised score optimizes prognostication for clinical practice and trials. BACKGROUND: Reliable prediction tools are needed to personalize treatment in ANCA-associated GN. A retrospective international longitudinal cohort was collated to revise the ANCA renal risk score. METHODS: The primary end point was ESKD with patients censored at last follow-up. Cox proportional hazards were used to reweight risk factors. Kaplan-Meier curves, Harrell's C statistic, receiver operating characteristics, and calibration plots were used to assess model performance. RESULTS: Of 1591 patients, 1439 were included in the final analyses, 2:1 randomly allocated per center to development and validation cohorts (52% male, median age 64 years). In the development cohort ( n =959), the ANCA renal risk score was validated and calibrated, and parameters were reinvestigated modifying interstitial fibrosis and tubular atrophy allowing semiquantitative reporting. An additional cutoff for kidney function (K) was identified, and serum creatinine replaced GFR (K0: <250 µ mol/L=0, K1: 250-450 µ mol/L=4, K2: >450 µ mol/L=11 points). The risk points for the percentage of normal glomeruli (N) and interstitial fibrosis and tubular atrophy (T) were reweighted (N0: >25%=0, N1: 10%-25%=4, N2: <10%=7, T0: none/mild or <25%=0, T1: ≥ mild-moderate or ≥25%=3 points), and four risk groups created: low (0-4 points), moderate (5-11), high (12-18), and very high (21). Discrimination was C=0.831, and the 3-year kidney survival was 96%, 79%, 54%, and 19%, respectively. The revised score performed similarly well in the validation cohort with excellent calibration and discrimination ( n =480, C=0.821). CONCLUSIONS: The updated score optimizes clinicopathologic prognostication for clinical practice and trials.

A systematic review of patient-reported outcome measures in patients with anti-neutrophil cytoplasmic antibody associated vasculitis.

OBJECTIVES: ANCA-associated vasculitis (AAV) is associated with significant morbidity, fatigue, pain and poor health-related quality of life (HRQoL). This review aims to assess the comprehensiveness of existing patient reported outcome measures (PROMs) used in AAV and identify associations with poorer HRQoL outcomes. METHODS: A literature review of studies using PROMs, including those labelled HRQoL in people with AAV as a primary or secondary study outcome were screened and reviewed up to July 2023. Quality was assessed using the Critical Appraisal Skills Programme. RESULTS: A total of 30 articles were included which utilised 22 different PROM tools, 76.7% (n = 23) used the SF-36 or a variation as a generic measure of health status and or HRQoL. Two studies developed a disease-specific PROM. The AAV-PRO showed good psychometric properties but potential limitations in capturing all relevant aspects of the disease experience for AAV patients. Factors associated with poorer HRQoL included: neurological and sinonasal involvement, women and younger patients. A total of 86.6% of studies showed no meaningful relationships between the SF-36 and BVAS, VDI or disease duration. Depression and anxiety were common and socioeconomic factors such as unemployment were significantly associated with poorer mental health outcomes. Glucocorticoids were found to be independently associated with worse SF-36 scores. CONCLUSION: Generic PROMs are useful in measuring significant changes but lack sensitivity to specific symptoms and unique AAV-related issues, while existing disease-specific PROMs have some limitations and may not fully capture the AAV patient's perspective on disease and treatment burden.

Risk Stratification to Predict Renal Survival in Anti-Glomerular Basement Membrane Disease.

SIGNIFICANCE STATEMENT: Most patients with anti-glomerular basement membrane (GBM) disease present with rapidly progressive glomerulonephritis, and more than half develop ESKD. Currently, no tools are available to aid in the prognostication or management of this rare disease. In one of the largest assembled cohorts of patients with anti-GBM disease (with 174 patients included in the final analysis), the authors demonstrated that the renal risk score for ANCA-associated vasculitis is transferable to anti-GBM disease and the renal histology is strongly predictive of renal survival and recovery. Stratifying patients according to the percentage of normal glomeruli in the kidney biopsy and the need for RRT at the time of diagnosis improves outcome prediction. Such stratification may assist in the management of anti-GBM disease. BACKGROUND: Prospective randomized trials investigating treatments and outcomes in anti-glomerular basement membrane (anti-GBM) disease are sparse, and validated tools to aid prognostication or management are lacking. METHODS: In a retrospective, multicenter, international cohort study, we investigated clinical and histologic parameters predicting kidney outcome and sought to identify patients who benefit from rescue immunosuppressive therapy. We also explored applying the concept of the renal risk score (RRS), currently used to predict renal outcomes in ANCA-associated vasculitis, to anti-GBM disease. RESULTS: The final analysis included 174 patients (out of a total of 191). Using Cox and Kaplan-Meier methods, we found that the RRS was a strong predictor for ESKD. The 36-month renal survival was 100%, 62.4%, and 20.7% in the low-risk, moderate-risk, and high-risk groups, respectively. The need for renal replacement therapy (RRT) at diagnosis and the percentage of normal glomeruli in the biopsy were independent predictors of ESKD. The best predictor for renal recovery was the percentage of normal glomeruli, with a cut point of 10% normal glomeruli providing good stratification. A model with the predictors RRT and normal glomeruli ( N ) achieved superior discrimination for significant differences in renal survival. Dividing patients into four risk groups led to a 36-month renal survival of 96.4% (no RRT, N ≥10%), 74.0% (no RRT, N <10%), 42.3% (RRT, N ≥10%), and 14.1% (RRT, N <10%), respectively. CONCLUSIONS: These findings demonstrate that the RRS concept is transferrable to anti-GBM disease. Stratifying patients according to the need for RRT at diagnosis and renal histology improves prediction, highlighting the importance of normal glomeruli. Such stratification may assist in the management of anti-GBM disease. PODCAST: This article contains a podcast at https://dts.podtrac.com/redirect.mp3/www.asn-online.org/media/podcast/JASN/2023_02_27_JASN0000000000000060.mp3.

Automated Computational Detection of Disease Activity in ANCA-Associated Glomerulonephritis Using Raman Spectroscopy: A Pilot Study.

Biospectroscopy offers the ability to simultaneously identify key biochemical changes in tissue associated with a given pathological state to facilitate biomarker extraction and automated detection of key lesions. Herein, we evaluated the application of machine learning in conjunction with Raman spectroscopy as an innovative low-cost technique for the automated computational detection of disease activity in anti-neutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis (AAGN). Consecutive patients with active AAGN and those in disease remission were recruited from a single UK centre. In those with active disease, renal biopsy samples were collected together with a paired urine sample. Urine samples were collected immediately prior to biopsy. Amongst those in remission at the time of recruitment, archived renal tissue samples representative of biopsies taken during an active disease period were obtained. In total, twenty-eight tissue samples were included in the analysis. Following supervised classification according to recorded histological data, spectral data from unstained tissue samples were able to discriminate disease activity with a high degree of accuracy on blind predictive modelling: F-score 95% for >25% interstitial fibrosis and tubular atrophy (sensitivity 100%, specificity 90%, area under ROC 0.98), 100% for necrotising glomerular lesions (sensitivity 100%, specificity 100%, area under ROC 1) and 100% for interstitial infiltrate (sensitivity 100%, specificity 100%, area under ROC 0.97). Corresponding spectrochemical changes in paired urine samples were limited. Future larger study is required, inclusive of assigned variables according to novel non-invasive biomarkers as well as the application of forward feature extraction algorithms to predict clinical outcomes based on spectral features.

Using CPAP in COVID-19 patients outside of the intensive care setting: a comparison of survival and outcomes between dialysis and non-dialysis dependent patients.

BACKGROUND: SARS-CoV-2 (COVID-19) is a novel coronavirus associated with high mortality rates. The use of Continuous Positive Airway Pressure (CPAP) has been recognised as a management option for severe COVID-19 (NHS, Specialty guides for patient management during the coronavirus pandemic Guidance for the role and use of non-invasive respiratory support in adult patients with coronavirus (confirmed or suspected), https://www.nice.org.uk/guidance/ng159 ). We offered ward-based CPAP to COVID-19, dialysis patients not suitable for escalation to ICU. The aim of the study was to evaluate the use of CPAP for COVID-19 dialysis patients compared to non-dialysis COVID-19 patients outside of the intensive care setting. We further aimed to investigate factors associated with improved outcomes. METHODS: Data was collected from a single centre (Royal Preston Hospital, UK), from March to June 2020. Treatment outcomes were compared for dialysis and non-dialysis dependent patients who received CPAP with limitations on their escalation and resuscitation status. Kaplan-Meier survival curves and Cox regression models were used to compare outcomes. The primary study outcome was 30 day mortality. Confounders including length of admission, systemic anticoagulation and ultrafiltration volumes on dialysis were also analysed. RESULTS: Over the study period, 40 dialysis patients tested positive for COVID-19, with 30 requiring hospital admission. 93% (n = 28) required supplementary oxygen and 12% (n = 9) required CPAP on the ward. These patients were compared to a serial selection of 14 non-dialysis patients treated with CPAP during the same period. Results showed a significant difference in 30 day survival rates between the two groups: 88.9% in the dialysis group vs. 21.4% in the non-dialysis group. Statistical modelling showed that anticoagulation was also an important factor and correlated with better outcomes. CONCLUSION: This is to the best of our knowledge, the largest series of COVID-19 dialysis patients treated with CPAP in a ward-based setting. In general, dialysis dependent patients have multiple co-morbidities including cardiovascular disease and diabetes mellitus making them vulnerable to COVID-19 and not always suitable for treatment in ICU. We showed a significantly lower 30 day mortality rate with the use of CPAP in the dialysis group (11.1%) compared to the non-dialysis group (78.6%). Despite a small sample size, we believe this study provides impetus for further work clarifying the role of CPAP in treating COVID-19 dialysis dependent patients.

Teledermatology: How It Is Impacting the Underserved.

The rapid mobilization of telemedicine during the COVID-19 pandemic has brought about renewed concern about the digital divide. Understanding the benefits and limitations of remote medical care is an ongoing process. Access to telemedicine appointments, as well as the experience of the virtual appointment itself, varies across demographic groups. Telemedicine has promise in dermatology. However, if it continues to expand beyond the pandemic, federal changes may be necessary to improve access to remote care for all patients moving forward. This involves creating mandates for increased broadband access, deploying digital health tools and portals usable by underserved populations, and increasing access to interpreters. Information technology personnel may also become necessary members of the clinical care team. Parity between video and phone visit reimbursement for providers is also necessary.

Cutaneous Lupus Erythematosus: Progress and Challenges.

PURPOSE OF REVIEW: The review provides an update on the diagnosis, pathogenesis, and treatment of cutaneous lupus erythematosus (CLE). RECENT FINDINGS: Diagnostic challenges exist in better defining CLE as an independent disease distinct from systemic lupus erythematosus with cutaneous features and further classifying CLE based on clinical, histological, and laboratory features. Recent mechanistic studies revealed more genetic variations, environmental triggers, and immunologic dysfunctions that are associated with CLE. Drug induction specifically has emerged as one of the most important triggers for CLE. Treatment options include topical agents and systemic therapies, including newer biologics such as belimumab, rituximab, ustekinumab, anifrolumab, and BIIB059 that have shown good clinical efficacy in trials. CLE is a group of complex and heterogenous diseases. Future studies are warranted to better define CLE within the spectrum of lupus erythematosus. Better insight into the pathogenesis of CLE could facilitate the design of more targeted therapies.

Developing a disease-specific patient reported outcome measure to enhance understanding of the lived experiences of ANCA associated vasculitis: A protocol paper.

Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a chronic, relapsing-remitting condition associated with increased morbidity. Previous research has shown patients with AAV report high levels of fatigue, pain, depression and anxiety. Over recent years successful work has been carried out to improve clinical outcomes, resulting in reduced mortality and end stage kidney disease (ESKD). Despite this, little work has been done to better understand the role of the patient within this condition. The prevalence of AAV is increasing and to date, there is a shortage of specific tools that assess and measure key features relating to patient reported outcomes (PROs). This protocol details how we can better understand the lived experiences of those with AAV through the development of a disease specific, patient reported outcome measure (PROM), to be used in clinic practice. This will allow us to recognise and validate PROs and the impact the disease and its treatment has on patients' health related quality of life (HRQoL). In addition, we aim to identify potential differences in PRO's between demographics, organ involvement and treatment subgroups in AAV as well as outcomes relating to the patient experience. Patients from a single centre in the UK will be recruited to take part in the exploratory qualitative study which will include focus groups and semi-structured interviews. The inclusion criteria comprise anyone with a diagnosis of AAV and willing to participate, including those who have active or relapsing disease, those are economically active, unemployed, retired and patients receiving renal replacement therapy. The aim of the project is to identify key issues patients experience in relation to their disease and its management and how these can be better assessed in a new PROM developed for use in the clinic setting. This will enable better delivery of individualised care and inform shared decision making, while also serving as a platform for future research looking at PROs in other glomerulonephritides.

Rituximab in the treatment of primary FSGS: time for its use in routine clinical practice?

Focal segmental glomerulosclerosis (FSGS) is a common cause of nephrotic syndrome and whilst advances have been made in the pathophysiology, diagnostics and management of other podocytopathies, primary FSGS remains the most elusive. It has been assumed for a long time that a circulatory permeability factor exists that mediates podocyte injury, and the potential for autoantibody-mediated disease therefore raises the question as to whether patients may benefit from targeted B-cell therapy with rituximab. The prospective case series of seven patients by Roccatello et al. adds to the limited but growing evidence suggesting that B-cell depletion therapy can be safe and effective in the treatment of primary FSGS. In this editorial we explore the available evidence that suggests how and in whom rituximab may play a role in the management of primary FSGS, as well as the limitations and other potential future treatments. Further research and randomized controlled trials are needed to include larger numbers of patients, feature genetic screening and incorporate data on B-cell kinetics as a potential guide for dosing and frequency of rituximab.

COVID-19 Infection and Vaccination and Its Relation to Amyloidosis: What Do We Know Currently?

Amyloidosis is a complex disorder characterized by deposited insoluble fibrillar proteins which misfold into ß-pleated sheets. The pathogenesis of amyloidosis can vary but can be the result of immune dysregulation that occurs from sustained high inflammatory states, often known as AA amyloidosis. Multi-organ involvement including hepatic, gastrointestinal, renal, cardiac and immunological pathological manifestations has been observed amongst individuals presenting with amyloidosis. The recent global pandemic of severe acute respiratory syndrome coronavirus 2, also referred to as coronavirus 2019 (COVID-19), has been shown to be associated with multiple health complications, many of which are similar to those seen in amyloidosis. Though COVID-19 is recognized primarily as a respiratory disease, it has since been found to have a range of extra-pulmonary manifestations, many of which are observed in patients with amyloidosis. These include features of oxidative stress, chronic inflammation and thrombotic risks. It is well known that viral illnesses have been associated with the triggering of autoimmune conditions of which amyloidosis is no different. Over the recent months, reports of new-onset and relapsed disease following COVID-19 infection and vaccination have been published. Despite this, the exact pathophysiological associations of COVID-19 and amyloidosis remain unclear. We present a scoping review based on our systematic search of available evidence relating to amyloidosis, COVID-19 infection and COVID-19 vaccination, evaluating current perspectives and providing insight into knowledge gaps that still needs to be addressed going forward.

Recrudescence of Severe Carbamazepine- Induced DRESS Syndrome after Initiation of Levetiracetam.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a potentially fatal cutaneous hypersensitivity reaction commonly precipitated by antiepileptic drugs (AEDs). Cross-reactivity among aromatic AEDs is well-documented, but between aromatic and nonaromatic AEDs. We report a patient with severe DRESS syndrome precipitated by aromatic AED carbamazepine with recrudescence approximately 2 weeks after substitution with nonaromatic AED levetiracetam. The patient was treated with high-dose corticosteroids and switched to the benzodiazepine AED clobazam. At follow-up appointment several weeks later, the patient's rash, liver injury, and eosinophilia had resolved.

A Meta-Analysis and Cohort Study of Histopathologic and Clinical Outcomes in ANCA-Negative versus -Positive Vasculitis.

BACKGROUND: ANCA-negative pauci-immune glomerulonephritis (PIGN) represents a rare and often under-studied subgroup of the vasculitides. This study aims to investigate differences in the clinical phenotype, renal histological features, and clinical outcomes of patients with PIGN, with and without serum ANCA positivity. METHODS: A cohort of biopsy-proven PIGN with and without detectable circulating ANCA was constructed from a single center between 2006 and 2016. Primary outcomes compared clinical presentation and histopathological features according to ANCA status, with multivariate Cox regression to compare mortality and ESKD. A systematic review and meta-analysis of the published literature was undertaken. RESULTS: In our cohort of 146 patients, 22% (n=32) had ANCA-negative disease, with a comparatively younger mean age at diagnosis; 51.4 versus 65.6 years (P<0.001). In total, 14 studies, inclusive of our cohort, were eligible for meta-analysis, totaling 301 patients who were ANCA negative. Those with ANCA-negative disease tended to have fewer extrarenal symptoms and a higher frequency of renal-limited disease, but both failed to reach statistical significance (P=0.92 and P=0.07). The risk of ESKD was significantly higher in seronegative disease (RR, 2.28; 95% confidence interval, 1.42 to 3.65; P<0.001), reflecting our experience, with a fivefold increased risk of ESKD in ANCA-negative disease (P<0.001). No significant difference in the chronicity of histopathological findings was seen and the meta-analysis showed no difference in morality (RR, 1.22; 95% confidence interval, 0.63 to 2.38; P=0.55). CONCLUSION: Our findings demonstrate that ANCA-negative PIGN presents in younger patients, with fewer extrarenal manifestations and higher ESKD risk, despite a lack of difference in histopathology. This study provides the impetus for further research into the pathogenesis, treatment response, and duration of immunotherapy in ANCA-negative disease. We suggest that the absence of positive ANCA serology should not discourage treatment and for clinical trials to include patients who are ANCA negative.

Cardiovascular disease and ANCA-associated vasculitis: are we missing a beat?

The association between cardiovascular (CV) disease and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is well documented. The recent work by Massicotte-Azarniouch et al. confirms the risk and adds to the existing evidence by describing the highest risk in the first 3 months after diagnosis. In this review, we aim to put their findings into perspective and formulate implications for the care of AAV patients. We discuss mechanisms for increased CV disease in AAV, including the impact of traditional risk factors and disease-related risks such as renal impairment and anti-myeloperoxidase (MPO) ANCA serotype. We also provide a brief primer on the impact of inflammatory-driven endothelial dysfunction and platelet activation on accelerated atherosclerosis in AAV patients. These features alongside the impact of disease activity and systemic inflammation provide potential explanations to why the incidence of CV events is highest in the first 3 months from diagnosis. We suggest future avenues of research, provide some suggestions to address and treat CV risk based on current evidence, and highlight the importance of addressing this topic early on. Addressing modifiable risk factors, dialogue with patients, patient information and a structured approach overall will be key to improve CV outcomes in AAV.

Monoclonal gammopathy of undetermined significance causing large vessel vasculitis.

A man in his late 50s presented with unilateral pain and discolouration of his fourth and fifth toes suggestive of digital ischaemia. He had a medical history of two unprovoked venous thromboembolisms in the preceding 18 months and a history of monoclonal gammopathy of undetermined significance (MGUS). A CT scan showed evidence of large vessels vasculitis in the absence of circulating antineutrophil cytoplasmic antibodies. Biopsy of the toes showed evidence of light chain and immunoglobulin deposition on immunofluorescence suggesting vasculitis secondary to his haematological diagnosis of MGUS. The patient was treated with high dose glucocorticoids and immunosuppressive treatment with a significant improvement in his symptoms and features of digital ischaemia.

Glucocorticoid Therapy in ANCA Vasculitis: Using the Glucocorticoid Toxicity Index as an Outcome Measure.

Background: ANCA-associated vasculitis (AAV) is an autoimmune disease. Induction remission and maintenance treatment typically includes high-dose, tapering glucocorticoids (GC), in addition to other immunosuppressive medication. The use of theGlucocorticoid Toxicity Index (GTI) provides a global, quantifiable assessment tool in which clinicians can assess GC-associated morbidity. Recent trials in AAV have exposed the need for systemic assessment of GC burden. In this small cohort study, we look to address these issues and the justification of newer GC sparing agents, such as C5a inhibitors. Methods: A retrospective cohort study of 43 patients with biopsy AAV was constructed from a single center between 2012-2016, and followed up for 48 months. The GTI table made up of adverse features was used to quantify patients' GC toxicity. Electronic patient records were reviewed and scores calculated according to published methods. GTI scores were compared with cumulative steroid doses at separate intervals and incidences of adverse features in relation to the treatment timeline. Results: The mean age was 65.9 (±11.06) years and treatment regimens consisted of glucocorticoids alongside cyclophosphamide or rituximab. Our results showed statistical significance in the association of cumulative GC doses and GTI scores (P=0.008; 95% CI, 1.31 to 8.05). Adverse features relating to mood disturbance and GC-induced psychosis occurred early, in contrast to adrenal insufficiency, which typically presented later in the follow-up. Infection-related adverse events were consistent throughout. Conclusions: We demonstrated that higher cumulative doses of steroids in AAV lead to worse glucocorticoid-related toxicity. Using the GTI creates the potential to individualize and quantify the adverse effects patients experience as a result of GC treatment and permits more patient-centered management. Although glucocorticoids remain the main adjunctive immunosuppression of AAV treatment, the narrow therapeutic window supports the need for GC-sparing treatments.

Thrombotic microangiopathy secondary to recurrent prostate cancer.

An 86-year-old man returned to the UK from Spain with symptoms suggestive of gastrointestinal bleeding. He was found to have an acute kidney injury and thrombocytopenia. Further investigations identified the presence of a microangiopathic hemolytic anemia, supporting the diagnosis of a thrombotic microangiopathy. Differentials included atypical hemolytic uremic syndrome and secondary thrombotic microangiopathy. Thrombotic thrombocytopenic purpura (TTP) and STEC (Shiga toxin-producing E. coli) hemolytic uremic syndrome were excluded by a normal ADAMTS-13 and negative E. coli serology and stool PCR. The patient was treated with blood and platelet transfusions. He received eculizumab and hemodialysis whilst a screen for secondary causes was undertaken. Thrombotic microangiopathy was shown to be secondary to recurrence of prostate cancer, which had been treated 16 years previously. He later recovered his renal function and receives ongoing hormonal treatment for his prostate cancer.

Opportunities in the cloud or pie in the sky? Current status and future perspectives of telemedicine in nephrology.

The use of telehealth to support, enhance or substitute traditional methods of delivering healthcare is becoming increasingly common in many specialties, such as stroke care, radiology and oncology. There is reason to believe that this approach remains underutilized within nephrology, which is somewhat surprising given the fact that nephrologists have always driven technological change in developing dialysis technology. Despite the obvious benefits that telehealth may provide, robust evidence remains lacking and many of the studies are anecdotal, limited to small numbers or without conclusive proof of benefit. More worryingly, quite a few studies report unexpected obstacles, pitfalls or patient dissatisfaction. However, with increasing global threats such as climate change and infectious disease, a change in approach to delivery of healthcare is needed. The current pandemic with coronavirus disease 2019 (COVID-19) has prompted the renal community to embrace telehealth to an unprecedented extent and at speed. In that sense the pandemic has already served as a disruptor, changed clinical practice and shown immense transformative potential. Here, we provide an update on current evidence and use of telehealth within various areas of nephrology globally, including the fields of dialysis, inpatient care, virtual consultation and patient empowerment. We also provide a brief primer on the use of artificial intelligence in this context and speculate about future implications. We also highlight legal aspects and pitfalls and discuss the 'digital divide' as a key concept that healthcare providers need to be mindful of when providing telemedicine-based approaches. Finally, we briefly discuss the immediate use of telenephrology at the onset of the COVID-19 pandemic. We hope to provide clinical nephrologists with an overview of what is currently available, as well as a glimpse into what may be expected in the future.

The impact of COVID-19 pandemic on patients with ANCA associated vasculitis.

INTRODUCTION: The coronavirus 2019 (COVID-19) pandemic has brought on challenges not only to acute care, but also chronic care of patients. Individuals maintained on immunosuppression appear to be especially susceptible to COVID-19 infection. Patients with ANCA-associated vasculitis (AAV) frequently require immunosuppression and may be at increased risk for developing COVID-19. The incidence and impact of COVID-19 on patients with AAV is currently not known. We aimed to investigate this impact via a telephone questionnaire-based patient survey and chart review. METHODS: A cross-sectional study of AAV patients followed at two centers was conducted. Data regarding demographics, disease characteristics and therapy were confirmed by chart review. A telephone survey was conducted to ascertain symptoms and contact exposure related to COVID-19, as well as changes in health care delivery during the pandemic period between January and July, 2020. RESULTS: Of the 206 patients surveyed, the median age was 64 years, 51% were female and mean (SD) disease duration was 7 (5) years. The majority had kidney (n = 160) and lung (n = 108) involvement. Seventy-five percent (n = 155) were receiving immunosuppression, with 77 patients (50%) receiving rituximab during the pandemic period. Of the 10 patients tested for severe acute respiratory syndrome coronavirus 2 (SARS CoV-2) by PCR, three were positive. Patients had a significant disruption in care; none had an in-person visit and 69% had a telemedicine consultation. Rituximab maintenance was postponed in 21 patients. Twelve patients experienced disease relapse. CONCLUSION: The incidence of COVID-19 in patients with AAV appears to be similar to that of the general population. For a patient population that requires active clinical surveillance, there is significant disruption in care as a result of the pandemic. Reduction of immunosuppression may not be indicated, and the risk of relapse likely far outweighs the risk of COVID-19.