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 AB0521 COST OF ILLNESS OF ANCA-ASSOCIATED VASCULITIS IN ITALY: DATA LINKAGE ANALYSIS OF MULTIPLE CLINICAL AND ADMINISTRATIVE DATABASES IN THE PROVINCE OF UDINE, ITALY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1558.1-1558
Author(s):  
L. Quartuccio ◽  
E. Treppo ◽  
S. De Vita ◽  
F. Valent
Keyword(s):  
Health Care ◽  
Cost Of Illness ◽  
Systemic Vasculitis ◽  
Health Information System ◽  
Clinical Manifestations ◽  
Administrative Databases ◽  
Rank Test ◽  
Hospital Discharges ◽  
Severe Infections ◽  
Regional Population

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 cost of illness due to AAV are lacking.Objectives:to estimate the cost of illness in patients suffering from AAV in the province of Udine (about 500,000 inhabitants), Friuli Venezia Giulia (FVG), Italy, from year 2010 to 2018.Methods:integration of the information coming from many administrative databases were used to this end. The Regional Health Information System of FVG 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 health care beneficiaries (including demographic information and the residential history of all of the subjects living in FVG), the hospital discharge database, the database of exemptions from medical charges, the database of the laboratories. The population under study was selected based on the following inclusion criteria: patients were residents in the province of Udine and they had to carry the exemption code for AAV, including GPA, or EGPA, or MPA. This population was observed from 2010 to 2018.Results:57 patients (201 patient-years) with AAV were identified. They were ANCA-positive in 44/57 (77%). GPA, EGPA and MPA was diagnosed in 18 (31,6%), 15 (26,3%), 11 (19,3%) patients, respectively. The mean age at diagnosis was 54,5 (17,5) years. The disease itself was the main cause of hospitalization in almost half of the hospital discharges (60/126, 47,6%). Four patients died during the observation period due to vasculitis itself (1), pneumonia (2), or haematological malignancy (1). Time to the first event (death or hospitalization) was significantly higher in ANCA-negative AAV patients than in ANCA-positive AAV patients (p=0,03, Log-Rank test), ANCA-positive AAV patients having a three-times higher risk (HR 3,38 95%CI 1,13-10,08, p=0,03). Total estimated cost was € 1,215,078, corresponding to € 6,168 patient-year. Costs for ANCA-positive AAV patients were much higher than those for ANCA-negative AAV patients (€ 1,115,253 vs € 99,825, and € 7058 per person-year vs € 2,559 per person-year, respectively). GPA and MPA showed the highest costs if compared to EGPA [GPA: € 239,168 (€ 5199 per person-year) vs MPA: € 281,502 (€ 4771 per person-year) vs EGPA: € 214,287 (2329 per person-year), respectively]. Costs for hospitalization were the highest [€ 734,957 (€ 3731 per person-year) vs other costs € 480,121 (€ 2437 per person-year)].Conclusion:costs for AAV are very high, confirming the high health care burden of this illness. Management of ANCA-positive patients rather than ANCA-negative patients was burdened by the highest costs. GPA and MPA showed the highest direct costs for hospitalization, which very frequently occurred due to the vasculitis itself.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 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.

scholarly journals Effectiveness and Safety of Mepolizumab in Combination with Corticosteroids in Patients with Eosinophilic Granulomatosis with Polyangiitis

2020 ◽  
Author(s):  
Masanobu Ueno ◽  
Ippei Miyagawa ◽  
Kazuhisa Nakano ◽  
Shigeru Iwata ◽  
Kentaro Hanami ◽  
...  
Keyword(s):  
Real World ◽  
Eosinophil Count ◽  
Granulomatosis With Polyangiitis ◽  
Remission Rate ◽  
Retention Rate ◽  
Damage Index ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Interleukin 5 ◽  
Number Of Patients ◽  
Eosinophilic Granulomatosis

Abstract Objectives: Mepolizumab (MPZ), an anti-interleukin-5 antibody, is effective for treating eosinophilic granulomatosis with polyangiitis (EGPA). However, its effectiveness has not been adequately evaluated in real-world clinical practice. In this study, we assessed the effectiveness and safety of 300 mg MPZ for relapsing/refractory EGPA resistant to corticosteroids (CS) for 1 year in real-world settings. Methods: We administered MPZ (300 mg) to 16 patients with relapsing/refractory EGPA resistant to CS (with-MPZ). We also retrospectively collected data from the same patients for 12 months before administering MPZ (without-MPZ). The primary endpoint was the 12-month remission rate after MPZ administration, and the secondary endpoints were the Birmingham vasculitis activity score (BVAS), vasculitis damage index (VDI), eosinophil count, changes in concomitant CS doses/concomitant immunosuppressant use, MPZ retention rate, and incidence of adverse events. The clinical course was compared between Without-MPZ and With MPZ.Results: The 12-month remission rate after initiating MPZ was 75%. No change was observed in BVAS, eosinophil count, or concomitant CS dose in the without-MPZ, whereas all these parameters were significantly decreased in the with-MPZ. The number of patients on concomitant immunosuppressants also decreased in the with-MPZ. VDI did not increase in both groups. The MPZ retention rate was 100%, and only three patients (18.8%) had infections.Conclusion: This study demonstrated that MPZ is effective and safe for EGPA, furthermore, compared to Without-MPZ, MPZ improves disease activity and possesses a higher remission rate and CS sparing effect.

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

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 Greatalys: Generating Real World Evidence across Italy in Sickle Cell Disease

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4707-4707
Author(s):  
Chiara Castiglioni ◽  
Claudia Condorelli ◽  
Paola Amore ◽  
Valentina Perrone ◽  
Davide Alessandrini ◽  
...  
Keyword(s):  
Health Care ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Real World ◽  
Diagnostic Tests ◽  
Burden Of Illness ◽  
Health Care Resource ◽  
Administrative Databases ◽  
Cell Disease ◽  
Real World Evidence

Background: Sickle Cell Disease (SCD) is a severe, potentially life threatening hereditary hemoglobin disorder requiring multidisciplinary care including screening, prevention, health education and management of acute and chronic complications. [ME Houwing et al, Blood Rev 2019] Although it affects millions of people throughout the world mainly individuals of African descent, Mediterranean (Caucasian) and Asiatic population, global migration from regions where the disease is endemic are changing its geographical distribution with a significant impact on patient survival, quality of life and costs for health systems requiring new treatment and preventive approaches. Data on epidemiological and clinical profiles of SCD have deeply changed also in Italy, a country with a high prevalence of hemoglobinopathies and where SCD is already present in the native population, due to an increasing number of immigrants from countries with high disease prevalence. Currently, approximately 2,000 SCD patients live in Italy. The creation of an Italian National Registry of Thalassemia and Hemoglobinopathies - was approved in 2017 thanks to a strong joint initiative of the Italian Society of Thalassemias and Hemoglobinopathies (SITE) and national patients associations. However the Registry is not yet completed, thus, the information currently available is very limited. [G Russo et al, Orphanet J Rare Dis. 2019] The Italian National Health Service is organized in Regions and Local Health Units (LHU). Each LHU reimburses health providers (eg, public and private hospital, general practitioners, outpatient services providers) for health assistance provided to citizens referring to the LHU, and uses specific databases to track the health assistance provided containing useful data to describe the type of assistance (eg, patient beneficiary, date of assistance) and to calculate the related charge. The main purpose of GREATalyS trial (CSEG101AIT01) is to collect data on SCD which may be representative of the national picture to better understand the burden of illness in terms of patient population, key disease features and resources consumption in Italy. Methods: Observational retrospective analyses will be carried out by integrating administrative databases for healthcare resources consumption (pharmaceuticals database, hospitalizations database, diagnostic tests and specialist visits database) from a sample of Italian Regions and LHUs, geographically distributed across the National territory (balanced between regions and number of covered residents). A representative sample of the whole national landscape through the involvement of approximately 11 LHUs and Regions will allow capture of approximately 23 million covered residents, representing about 38% of the entire Italian population. Patient Socio-Demographic characteristics (age, gender), all hospitalizations (SCD related and not related), previous SCD treatment type, co-morbidity profiles, patient treatment patterns, resources use and health services cost will be collected and analyzed. Anonymized patient data will be extracted into the Hospitalization database searching the relevant International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM codes). All analyses will be performed by STATA SE, version 12.0. The Local Ethics Committee of each participating Region/LHU has been notified about the study. Objectives: Composite objectives will collect both epidemiological and burden of illness data. Primary study objective is to estimate SCD prevalence and incidence rate from January 2010 to December 2018. Key secondary objectives include description of demographic and clinical characteristics, therapeutic pathways, health care resource use and direct costs (derived from health care resource utilization in term of drug treatments, diagnostic tests, specialist visits and hospitalizations) for patients affected by SCD. Conclusions: GREATalyS is to our knowledge the first SCD real world evidence study in Italy leveraging National Health System administrative databases to collect epidemiological and burden of illness data, and represents a useful health assessment model to better understand the evolving scenario in Italy, a country where SCD is becoming an emerging health problem. Figure Disclosures Castiglioni: Novartis Farma SpA: Employment. Condorelli:Novartis Farma SpA: Employment. Amore:Novartis Farma SpA: Employment. Perrone:CliCon srl: Employment. Alessandrini:CliCon srl: Employment. Veronesi:CliCon srl: Employment. Degli Esposti:CliCon srl: Employment. Premoli:Novartis Farma SpA: Employment. Fiocchi:Novartis Farma SpA: Employment.

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