scholarly journals EULAR guidelines on ANCA-associated vasculitis in the real life

2021 ◽  
Vol 2 (3) ◽  
pp. 74-78
Author(s):  
Chiara Marvisi ◽  
Elena Galli ◽  
Carlo Umberto Manzini ◽  
Gilda Sandri ◽  
Carlo Salvarani
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Inflammatory Diseases ◽  
Real Life ◽  
Clinical Manifestations ◽  
Remission Induction ◽  
Induction Treatment ◽  
Management Approach ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Remission Maintenance ◽  
The Future

Anti-neutrophil cytoplasmic antibodies-associated vasculitides (AAVs) are a heterogenous group of inflammatory diseases which primarily involve small vessels and include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). They present heterogeneous clinical manifestations, while their diagnosis and management still remain a challenge for clinicians. Nowadays, the treatment is based on two different regimens: the remission-induction treatment and the remission-maintenance treatment. The therapeutic armamentarium has grown over the years, with the aim to lessen adverse effects, improve quality of life of patients and maintain the disease under control. Biological treatments are the future: they act on different pathogenic pathways and may offer in the future a personalized management approach tailored to actual clinical manifestations. The latest guidelines were published in 2015 by the European League Against Rheumatism (EULAR) and still represent the vade mecum for the management of AAVs. In this review, we will focus on the principal strategies to treat AAVs. We discuss the remission-induction therapy and the remission- maintenance therapy; we have also distinguished the management of GPA and MPA from that of EGPA, because of their different clinical pictures.

scholarly journals ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS IN “REAL LIFE” – SERIES OF CLINICAL CASES IN A ROMANIAN REFERENCE CENTER

2017 ◽  
Vol 26 (4) ◽  
pp. 164-168
Author(s):  
Mihaela-Lavinia Popescu ◽  
◽  
Denisa Predeteanu ◽  
Andra Balanescu ◽  
F. Berghea ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Real Life ◽  
Maintenance Phase ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Cross Sectional Study ◽  
Remission Induction ◽  
Induction Treatment ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Positive Serology ◽  
Cross Sectional

Introduction. Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) represent a group of conditions evolving with necrotizing inflammation in small and medium-sized blood vessels. AAV are composed of GPA (granulomatosis with polyangiitis, former Wegener’s granulomatosis), MPA (microscopic polyangiitis) and EGPA (eosinophilic granulomatosis with polyangiitis, former Churg-Strauss syndrome). AAV receive immunosuppressive therapy associated with a high risk of complications. Objective. The aim of this study was to characterize a single center cohort of AAV patients regarding clinical, biological and therapeutic features. Method. We realized a cross-sectional study by consequently enrolling all the patients registered with AAV diagnosis between 2009 and 2017 in Department of Rheumatology of “Sfânta Maria” Hospital. Demographic, diseaserelated and therapeutic-related parameters were collected. The data was extracted from the clinical files. Results. The study sample included 26 cases, 15 females and 11 males: 20 patients GPA, 4 MPA and 2 cases EGPA. Mean age at the time of diagnosis was around 48 but 12 patients presented delays between age at the onset and age at the time of diagnosis (the mean delay was 2 years). The most frequent clinical manifestation identified where pulmonary, musculoskeletal and renal. 15 patients had a diagnostic biopsy performed. ANCA detection revealed 16 cases of c-ANCA and 7 cases of p-ANCA and 11 patients presented other positive serology. A combination of glucocorticoids and cyclophosphamide was used in most of the cases for remission-induction treatment and the same scheme was used for relapse cases. For maintenance phase a combination of glucocorticoids and azathioprine was preferred. 13 patients (50%) developed treatment related complications. Conclusion. Most of the patients were diagnosed with GPA (20) and the least were diagnosed with EGPA (2). Biopsy was performed in 15 cases and it was mostly nasal. For remission-induction prevailed the combination of glucocorticoids and cyclophosphamide. Most treatment related complications were due to glucocorticoids administration. Osteoporosis was predominant.

scholarly journals AB0520 FIVE-YEAR RATE OF HOSPITALIZATIONS IN ANCA-ASSOCIATED VASCULITIDES IN THE ITALIAN REGION OF FRIULI VENEZIA GIULIA (2013-2017)

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1557.2-1558
Author(s):  
L. Quartuccio ◽  
E. Treppo ◽  
S. De Vita ◽  
F. Valent
Keyword(s):  
Health Care ◽  
Granulomatosis With Polyangiitis ◽  
Burden Of Illness ◽  
Clinical Manifestations ◽  
Administrative Databases ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Italian Region ◽  
Potential Health ◽  
Number Of Patients ◽  
Friuli Venezia Giulia

Background:ANCA-associated vasculitides (AAV) are a group of systemic vasculitis carrying a high risk of hospitalization because the multiorgan involvement, the acute nature of some clinical manifestations, the chronic but very disabling course of some other manifestations and finally the risk of severe infections due to chronic glucocorticoid and immunosuppressor administration. However, data on hospitalization due to ANCA-associated vasculitis are still scarce.Objectives:to estimate the rate of the first hospitalization or the death in patients suffering from AAV in the Italian region of Friuli Venezia Giulia (about 1,200,000 inhabitants) from year 2013 to 2017.Methods:integration of the information coming from many administrative databases were used to this end. The Regional Health Information System of Friuli Venezia Giulia was used as the source of information for this retrospective cohort study. The system covers the entire regional population and includes various electronic health administrative databases that can be linked with one another on an individual basis through a unique encrypted identifier. In particular, the following databases were matched: the database of the regional potential health care beneficiaries (including demographic information and the residential history of all of the subjects living in the region), the hospital discharge database, the database of exemptions from medical charges were used for this study, the database of the different regional laboratories. The population under study was selected based on the following inclusion criteria: patients were residents in Friuli Venezia Giulia and they had to carry the exemption code for AAV, including Granulomatosis with Polyangiitis (GPA), or Eosinophilic Granulomatosis with Polyangiitis (EGPA), or Microscopic Polyangiitis (MPA). This population was observed from 2013 to 2017. The coded event was the occurrence of the first hospitalization or the death. Also, all the hospitalization and their main discharge diagnoses were registered.Results:103 patient with AAV were identified. The number of patients with at least one hospitalization/death was 74/103 (71,8%). Seven patients died during the observation period (6,6%). The whole number of hospitalizations was 285 in 74 patients. 55/74 (74,3%) patients experienced more than one hospitalization. In the majority of the hospitalizations (119/285, 41,7%), the cause of hospitalization was directly attributable to the disease itself, while the second cause of hospitalization was the infections (26/285, 9,1%). In 10/103 patients (9,7%), an end stage renal disease was recorded as event. The presence of at least one positivity for ANCA antibodies was documented in 76/103 patients (73,8%), mainly in patients carrying GPA. Globally, the presence of ANCA antibody seems to be associated with greater likelihood of an event (p=0,07, log-rank test). The first event occurred in 50% of ANCA-positive patients within 180 days from diagnosis, while in 50% of ANCA negative patients in 859 days. 6 out of the 7 deaths occurred in ANCA positive patients.Conclusion:the rate of hospitalization in AAV is very high confirming the high health care burden of illness. The disease itself is often the cause of the hospitalization, as well as the infectious complication, highlighting the need for more effective treatments, and glucocorticoid sparing therapies. ANCA antibody may represent a biomarker of a more serious disease.Disclosure of Interests:Luca Quartuccio Consultant of: Abbvie, Bristol, Speakers bureau: Abbvie, Pfizer, Elena Treppo: None declared, Salvatore De Vita Consultant of: Roche, GSK, Speakers bureau: Roche, GSK, Novartis, Francesca Valent: None declared

scholarly journals B-Cell-Depleting Therapy Improves Myocarditis in Seronegative Eosinophilic Granulomatosis with Polyangiitis

2021 ◽  
Vol 10 (19) ◽  
pp. 4577
Author(s):  
Chrong-Reen Wang ◽  
Yi-Shan Tsai ◽  
Hung-Wen Tsai ◽  
Cheng-Han Lee
Keyword(s):  
B Cell ◽  
Clinical Remission ◽  
Granulomatosis With Polyangiitis ◽  
Disease Onset ◽  
Cardiac Insufficiency ◽  
Induction Treatment ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Asthma Attack ◽  
Eosinophilic Granulomatosis ◽  
Eosinophil Counts

Cardiac involvement is a major mortality cause in eosinophilic granulomatosis with polyangiitis (EGPA), requiring novel therapeutics to spare the use of cyclophosphamide with known cardiotoxicity. Despite the observed efficacy of B-cell-depleting therapy in myocarditis of seropositive microscopic polyangiitis, it remains to be elucidated in seronegative EGPA. A retrospective study was performed in 21 hospitalized active patients aged 20 to 70 years with five-factor score 1 or 2, eosinophil counts 10,034 ± 6641/μL and vasculitis scores 27 ± 6. Overt myocarditis was identified in 10 cases, at disease onset in 6 and relapse in 4, with endomyocarditis in 4 and myopericarditis in 4. Five seronegative and one seropositive patient received rituximab with an induction regimen 375 mg/m2 weekly × 4 for refractory or relapse disease, and the same regimen for annual maintenance therapy. All cases had lower eosinophil counts, improved cardiac dysfunction and clinical remission with a relapse-free follow-up, 48 ± 15 months after the induction treatment. One seronegative endomyocarditis patient had eosinophilia and disease relapse with asthma attack and worsening cardiac insufficiency 24 months after induction, achieving clinical remission under anti-IL-5 therapy. Our findings suggest the suppression of IL-5-mediated eosinophilia as an action mechanism of B-cell-depleting therapy in seronegative EGPA myocarditis.

Treatment of ANCA-associated vasculitis

2013 ◽  
Author(s):  
David Jayne
Keyword(s):  
Tissue Damage ◽  
Drug Toxicity ◽  
Granulomatosis With Polyangiitis ◽  
Late Complication ◽  
Response To Therapy ◽  
Remission Induction ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Endstage Renal Disease ◽  
Steroid Dependent

The goals of treatment in anti-neutrophil cytoplasm antibody (ANCA) vasculitis are to stop vasculitic activity, to prevent vasculitis returning, and to address longer-term comorbidities caused by tissue damage, drug toxicity, and increased cardiovascular and malignancy risk. Cyclophosphamide and high-dose glucocorticoids remain the standard induction therapy with alternative immunosuppressives, such as methotrexate or azathioprine, to prevent relapse. Refractory disease resulting from a failure of induction or remission maintenance therapy requires alternative agents and rituximab has been particularly effective. Replacement of cyclophosphamide by rituximab for remission induction is supported by recent evidence. Additional therapy with intravenous methylprednisolone and plasma exchange is employed in severe presentations with failing vital organ function. Drug toxicity contributes to comorbidity and mortality and has led to newer regimens with reduced cyclophosphamide exposure. Glucocorticoid toxicity remains a major problem, with controversy over the rapidity with which glucocorticoids can be reduced or withdrawn. Disease relapse occurs in 50% and requires early detection at a stage when it will not adversely affect outcomes. Rates of cardiovascular disease and malignancy are higher than in control populations but strategies to reduce their risk, apart from cyclophosphamide-sparing regimens, have not been developed. Thromboembolic events occur in 10% and may be linked to the recently identified autoantibodies to plasminogen and tissue plasminogen activator. Outcomes of vasculitis depend heavily on the level of tissue damage at diagnosis, especially renal dysfunction, but are also influenced by patient age, ANCA subtype, disease extent, and response to therapy. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)is treated along similar principles to granulomatosis with polyangiitis (GPA) and microscopic polyangiitis but the persistence of steroid-dependent asthma in over one-third and differences in pathogenesis has suggested alternative treatment approaches. Chronic morbidity results from tissue damage and is especially common in the upper and lower respiratory tract and kidneys. Tracheobronchial disease is a severe late complication of GPA, while deafness, nasal obstruction, and chronic sinusitis are sequelae of nasal and ear vasculitis. Chronic infection of damaged epithelial surfaces acts as a drive for vasculitic activity and adequate infection control is necessary for stable remission. Chronic kidney disease can stabilize for many years but the risks of endstage renal disease (ESRD) are increased by acute kidney injury at presentation or renal relapse. Renal transplantation is successful, with similar outcomes to other causes of ESRD.

scholarly journals Addison’s disease presenting with perimyocarditis

2018 ◽  
Vol 31 (1) ◽  
pp. 101-105
Author(s):  
Elisa Baranski Lamback ◽  
Grazia Morandi ◽  
Eleni Rapti ◽  
Georgi Christov ◽  
Paul A. Brogan ◽  
...  
Keyword(s):  
Systemic Inflammation ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Manifestations ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Polyglandular Autoimmune Syndrome ◽  
Case Presentation ◽  
Prompt Treatment ◽  
Autoimmune Syndrome ◽  
Life Saving ◽  
Eosinophilic Granulomatosis

AbstractBackground:Polyglandular autoimmune syndrome (PGA) and eosinophilic granulomatosis with polyangiitis (EGPA) do not seem to represent a coincidental association.Case presentation:A case of a 15-year-old boy is reported who presented with severe systemic inflammation, perimyocarditis and cardiogenic shock, in whom EGPA was initially suspected and later diagnosed with autoimmune adrenalitis with PGA.Conclusions:The severity of the systemic inflammation and perimyocarditis suggests a more widespread autoimmune-mediated process. Autoimmune adrenal insufficiency should be considered in all cases of pericarditis and perimyocarditis, especially when the severity of clinical manifestations exceeds the expected for the severity of the cardiac findings, as timely identification and prompt treatment may be life-saving.

scholarly journals Differential diagnosis of granulomatous lung disease: clues and pitfalls

2017 ◽  
Vol 26 (145) ◽  
pp. 170012 ◽  
Author(s):  
Shinichiro Ohshimo ◽  
Josune Guzman ◽  
Ulrich Costabel ◽  
Francesco Bonella
Keyword(s):  
Differential Diagnosis ◽  
Lung Diseases ◽  
Granulomatosis With Polyangiitis ◽  
Wide Spectrum ◽  
Clinical Manifestations ◽  
Needle Aspiration ◽  
Endobronchial Ultrasound ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
High Resolution Computed Tomography ◽  
Transbronchial Needle Aspiration

Granulomatous lung diseases are a heterogeneous group of disorders that have a wide spectrum of pathologies with variable clinical manifestations and outcomes. Precise clinical evaluation, laboratory testing, pulmonary function testing, radiological imaging including high-resolution computed tomography and often histopathological assessment contribute to make a confident diagnosis of granulomatous lung diseases. Differential diagnosis is challenging, and includes both infectious (mycobacteria and fungi) and noninfectious lung diseases (sarcoidosis, necrotising sarcoid granulomatosis, hypersensitivity pneumonitis, hot tub lung, berylliosis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, rheumatoid nodules, talc granulomatosis, Langerhans cell histiocytosis and bronchocentric granulomatosis). Bronchoalveolar lavage, endobronchial ultrasound-guided transbronchial needle aspiration, transbronchial cryobiopsy, positron emission tomography and genetic evaluation are potential candidates to improve the diagnostic accuracy for granulomatous lung diseases. As granuloma alone is a nonspecific histopathological finding, the multidisciplinary approach is important for a confident diagnosis.

Comparison of patient self-reported data to physician-reported data in eosinophilic granulomatosis with polyangiitis (Preprint)

2021 ◽  
Author(s):  
Irena Doubelt ◽  
Jason M. Springer ◽  
Tanaz A. Kermani ◽  
Antoine G. Sreih ◽  
Cristina Burroughs ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Clinical Manifestations ◽  
The United States ◽  
Research Network ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
American College Of Rheumatology ◽  
Organ Systems ◽  
Patient Reported ◽  
Reported Data ◽  
Eosinophilic Granulomatosis

BACKGROUND Patient-based registries can help advance research in rare diseases such as eosinophilic granulomatosis with polyangiitis (EGPA), a complex, multi-organ form of anti-cytoplasm neutrophil antibody (ANCA)-associated vasculitis. OBJECTIVE To compare patient-reported vs. physician-reported manifestations, treatments, and outcomes for patients with EGPA. METHODS Comparative analysis of patients ≥18 years with EGPA in Canada or the United States from two separate cohorts: i) The Vasculitis Patient-Powered Research Network (VPPRN), a self-enrolled, secure portal with patient-entered data updated quarterly (2013-2019), vs. ii) The Vasculitis Clinical Research Consortium (VCRC) observational studies, a physician-entered database (2003-2019) of patients who fulfilled the 1990 American College of Rheumatology classification criteria for EGPA. Studied parameters included demographics, clinical manifestations, ANCA status, treatments, and relapses. RESULTS Data from 195 patients with a validated diagnosis of EGPA in the VPPRN and 354 patients enrolled in the VCRC were analyzed. Compared to the VCRC cohort, the patients in the VPPRN cohort were more commonly female (69.2% vs. 59.0% in the VCRC cohort; P =.02), younger at diagnosis (47.3 vs. 50.0 years; P =.03), reported similar frequencies of asthma (96.2% vs 92.9% in VCRC; P =.13), cardiac manifestations (28.8% vs 21.2%; P =.06), but less frequent lung manifestations other than asthma, and more frequent disease manifestations in all other organ systems. ANCA positivity was 48.9% in the VPPRN patients vs. 38.9% (P=.05) in the VCRC cohort. Relapsing disease after study enrollment was reported in 32.3% patients in the VPPRN compared 35.7% of patients in the VCRC. Most therapies (glucocorticoids, cyclophosphamide, mepolizumab) were used at similar frequencies in both groups, except for rituximab with VPPRN patients reporting more use than VCRC cohort (24.1% vs. 10.5%; P =<.001). CONCLUSIONS Patients with EGPA generally report having more manifestations of disease than physicians report for patients with EGPA. These differences imply the need to reconsider how patient- and physician-reported data are collected for the study of EGPA, and reevaluate disease specific definitions. CLINICALTRIAL ClinicalTrials.gov: (1) VCRC Longitudinal Study (LS) NCT00315380 https://clinicaltrials.gov/ct2/show/NCT00315380 and (2) One-Time DNA (OT) study NCT01241305 https://clinicaltrials.gov/ct2/show/NCT01241305

Non-severe eosinophilic granulomatosis with polyangiitis: long-term outcomes after remission-induction trial

Rheumatology ◽  
2019 ◽  
Vol 58 (12) ◽  
pp. 2107-2116 ◽  
Author(s):  
Xavier Puéchal ◽  
Christian Pagnoux ◽  
Gabriel Baron ◽  
François Lifermann ◽  
Loïk Geffray ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Task Force ◽  
Controlled Trial ◽  
Remission Induction ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Long Term Outcomes ◽  
First Relapse ◽  
Eosinophilic Granulomatosis

Abstract Objective In a previous controlled trial, 1-year adjunction of AZA to glucocorticoids (GC) for patients with non-severe, newly diagnosed eosinophilic granulomatosis with polyangiitis (EGPA) failed to lower remission failure, vasculitis relapse and isolated asthma/rhinosinus exacerbation rates, or cumulative GC use at month (M) 24. The aim of this study was to analyse longer-term outcomes to determine whether subsequent vasculitis relapse or isolated asthma/rhinosinus exacerbation (IARE) rates differed. Methods After M24, patients were followed prospectively, being treated based on physicians’ best judgment. Flares and reasons for increased GC dose or immunosuppressant use were recorded, and reviewed according to randomization group to distinguish vasculitis relapses from IAREs according to EGPA Task Force recommendations. Results Fifty EGPA trial participants were followed for a median (interquartile range) of 6.3 (5.4–7.6) years; two (4%) died 11 months post-inclusion. By M24, vasculitis had relapsed in 21/49 (43%) patients and 14/50 (28%) had IAREs. Another patient died 4.8 years post-inclusion (infection). Among nine patients with subsequent vasculitis relapses, three had a major relapse and three had their first relapse after M24; among 25 patients with later IAREs, 17 occurred after M24. At 5 years, respective vasculitis relapse and IARE rates were 48% (95% CI 34.0, 62.6) and 56% (95% CI 41.7, 70.8), with no between-arm differences (P = 0.32 and 0.13). No entry clinical or biological parameter was associated with these outcomes during follow-up. Conclusion These results confirmed that 1-year AZA and GC induction obtained good overall survival but no long-term benefit for non-severe EGPA patients. Vasculitis relapses, occurring mostly during the first 2 years, and IAREs, occurring throughout follow-up, require other preventive treatments. Trial registration ClinicalTrials.gov, https://clinicaltrials.gov, NCT00647166.

scholarly journals FRI0219 MEPOLIZUMAB FOR EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS IN REAL WORLD DATA

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 693.2-693
Author(s):  
T. Shoda ◽  
T. Takeuchi ◽  
K. Nagai ◽  
J. Konma ◽  
S. Arawaka
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Age Of Onset ◽  
Remission Induction ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Clinical Settings ◽  
Osaka Medical College ◽  
Median Dose ◽  
Real World Data ◽  
Medical College ◽  
Eosinophilic Granulomatosis

Objectives:To investigate the treatments of eosinophilic granulomatosis with polyangiitis (EGPA) and evaluate the usage of mepolizumab in clinical settings.Methods:The subjects were consecutive EGPA patients who were hospitalized and treated at our department and the Rheumatology, Department of Internal Medicine IV, Osaka Medical College between 2002 and 2018. Their clinical data, treatments, and courses were examined, and the usage of mepolizumab was evaluated.Results:Of 49 EGPA patients, 41 could be analyzed (14 males and 27 females, mean age of onset: 56.4 years). The percentage of positive ANCA was 31.7%, and affected sites were peripheral nerve (92%), central nervous system (17%), skin (51%), ENT (39%), lungs (29%), heart (22%), digestive organs (12%), and kidneys (15%). Remission induction therapy was performed with PSL (41 cases, 100%), PSL pulse (16 cases, 39%), IVCY (17 cases, 41%), RTX (4 cases, 10%), IVIG (22 cases, 54%), AZA (22 cases, 54%), MTX (4 cases, 10%), MMF (2 cases, 5%), MIZ (1 case, 2%), and MEPO (1 case, 2%). Maintenance therapy was performed with PSL (41 cases, 100%), AZA (21 cases, 51%), MTX (6 cases, 15%), MMF (2 cases, 5%), MIZ (3 cases, 7%), and MEPO (10 cases, 24%). In 10 patients who received mepolizumab, the percentage of positive ANCA was 40%, and the median dose of PSL was reduced from 9.5 mg to 5.5 mg after administration. Neither relapses nor adverse events occurred in patients who had received mepolizumab.Conclusion:Mepolizumab reduced the dose of steroids and improved tolerability in EGPA patients with or without ANCA.Disclosure of Interests:None declared

scholarly journals POS1230 OUTCOME FOLLOWING COVID-19 INFECTION IN ANTI-NEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA)-ASSOCIATED VASCULITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 898.2-898
Author(s):  
A. Antovic ◽  
B. Lövström ◽  
A. Hugelius ◽  
O. Borjesson ◽  
A. Bruchfeld ◽  
...  
Keyword(s):  
Maintenance Therapy ◽  
Immunosuppressive Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Data Retrieval ◽  
Induction Treatment ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Upper Airways ◽  
Anca Associated Vasculitis

Background:Patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) require immunosuppressive therapy for disease control and reduction of disease relapse and may be at risk for complications during Sars-CoV-2 (COVID-19) infection.Objectives:To analyze the consequences of COVID-19 in a large cohort of AAV patients regarding occurrence, need of hospitalization, treatment at the intensive care units (ICU), or death.Methods:Data were retrieved from March 2020 to mid-January 2021 from medical records from the AAV cohort (n=233). Patients diagnosed with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) or eosinophilic granulomatosis with polyangiitis (EGPA) were included. Data included age, gender, diagnosis, ongoing immunosuppressive medication at onset of COVID-19 or at last follow-up in non-COVID individuals. Renal involvement (ever) and estimated glomerular filtration rate (eGFR) were included. COVID-19 was confirmed either by a positive PCR test in the upper airways or by serology. Severe COVID-19 was defined as need of non-invasive ventilation, ICU care, and/or death.Results:The cohort comprised of 172 patients with GPA, 50 with MPA and 11 with EGPA. There were 121 females (52%). During the study period, 20 patients (8.6%) were diagnosed with COVID-19. The median age at data retrieval in all patients was 68 years (21-93), in the COVID-19 group 63 (29-93) and 68.5 (21-90) years in the non-COVID patients.Fourty-three patients in all (18%) were hospitalized during the study period of which 11 (4.7%) due to COVID-19 infection. In all, 8 deaths occurred of which 3 were related to COVID-19.At data retrieval, 110 (47%) patients were on prednisolone treatment, 10/20 (50%) in the COVID-19 group and 100 (47%) in the non-COVID-19 group (p=0.5), with significantly higher doses in COVID-19 patients (p<0.001). In patients hospitalized with COVID-19, 6/11 (54.5%) were on prednisolone, median dose 5 mg/day (0-50). In the total group 112 (48%) were on disease modifying anti-rheumatic drugs (DMARD) and 64 (27.5%) on rituximab as maintenance therapy. Eight patients were on induction treatment with either cyclophosphamide or rituximab.Of the 20 COVID-19 cases, 8 had severe COVID-19. Of these, 2 were inactive without immunosuppressive treatment, 4 had stable disease with prednisolone (5-7.5 mg/day) in combination with DMARDs, and 2 were active treated with high dose prednisolone (25-50 mg/day) in combination with cyclophosphamide and rituximab (n=1) or rituximab (n=1).A higher proportion of patients had active AAV (p=0.03) in the severe COVID-19 then in the non-COVID group (10/213 patients).In the group with the severe COVID-19, 1/8 (12%) patient had rituximab as maintenance therapy, compared to 61/213 (28.6%) in the group of non-COVID-19 patients (p=0.5).Renal involvement (ever) was present in 144 patients (62%), in 6 patients (30%) with COVID- 19, from which 5 (62%) were in the group of severe COVID-19 patients. Median eGFR did not differ between severe COVID-19 and remaining patients with renal involvement independently of COVID-19 infection.Conclusion:We found a high rate of severe COVID-19 infection in our cohort of AAV patients which indicates risk for serious complications, especially in patients with active disease and intense immunosuppressive therapy. Maintenance therapy with rituximab did not seem to increase the risk for severe COVID-19. The findings stress the need for continued shielding and early vaccination in AAV patients.Disclosure of Interests:None declared

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