scholarly journals P0212IMMUNOSUPPRESSIVE TREATMENT OF PATIENTS WITH ANCA ASSOCIATED VASCULITIS (AAV) IN CHRONIC DIALYSIS PROGRAM

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Ibolya File ◽  
László Ujhelyi ◽  
Jozsef Balla ◽  
László Bidiga ◽  
Trinn Csilla ◽  
...  
Keyword(s):  
Maintenance Therapy ◽  
Relapse Rate ◽  
Granulomatosis With Polyangiitis ◽  
Combined Therapy ◽  
Immunosuppressive Treatment ◽  
Chronic Dialysis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Time To Death ◽  
Serious Infection ◽  
The Mean

Abstract Background and Aims There are numerous studies on AAV, however, data describing the use of immunosuppressive treatment in AAV patients (pts) on chronic dialysis are limited. Most studies found that the incidence of infection is much higher than that of relapses (0.05-0.1 pt per year), suggesting that immunosuppression should be stopped after 3 months in pts with end-stage renal failure. Material, Method Retrospective analysis of immunosuppressive treatment in pts on chronic dialysis due to AAV. From 1995 to 2019 139 pts were diagnosed with AAV. Among them 38 patients (26 female, 12 male, mean age 58 years) needed chronic dialysis (>90 days) due to severe renal AAV (29), relapsing renal disease (6) or progressive CKD without active vasculitis (3). Results Microscopic polyangiitis was diagnosed in 20, granulomatosis with polyangiitis in 14, eosinophilic granulomatosis with polyangiitis in 4 cases. MPO-ANCA was more frequent than PR3-ANCA (23 vs 12), in one case both ANCAs were positive, and in two cases both were negative. The mean dialysis duration was 55 months (4–180 months). 18 pts (47%) had at least one relapse during the observation period. The mean age, the clinical diagnosis, the type of ANCA did not cause any difference, the dialysis time was longer in the relapsed pts group (72+/-38 vs. 40+/-29 months) compared to pts without relapse. High relapse rate was seen in 6 cases. Three patient had continuously high level of PR3-ANCA without relapse or any sign of disease. Relapses were treated mostly with combined therapy of corticosteroids and oral/intravenous cyclophosphamide followed by azathioprin maintenance therapy. Cortocosteroid alone was used in 7 pts, rituximab in five pts as maintenance therapy. Serious infection (needing hospital care) rate during any immunosuppressive treatment was 0.12 per patient-years. There were 12 deaths on dialysis, the median time to death was 61 months (range 3–132). The deaths rate was higher (9/18 vs. 4/20) in relapsed pts, 4 occurring during immunosuppressive treatment. Causes of deaths were cardiovascular (8), sepsis (3), malignancy (2 pts). Seven pts received kidney grafts, 15 pts remained on dialysis, but in three case dialysis could be discontinued after 16, 35 and 36 months. Conclusion Our study points to higher relapse rate and lower serious infection rate in AAV pts on chronic dialysis compared to previous studies. It seems reasonable to continue immunosuppression above 3 months in pts remaining on chronic dialysis. This approach may decrease the relapse rate preventing damage of other organs, allowing transplantation and in some pts recovery of renal function could be achieved.

Efficacy of rituximab in refractory neuromyelitis optica

2015 ◽  
Vol 22 (7) ◽  
pp. 955-959 ◽  
Author(s):  
N Collongues ◽  
D Brassat ◽  
E Maillart ◽  
P Labauge ◽  
JC Ouallet ◽  
...  
Keyword(s):  
Body Mass Index ◽  
Maintenance Therapy ◽  
Immunosuppressive Therapy ◽  
Relapse Rate ◽  
Neuromyelitis Optica ◽  
Predictive Factor ◽  
Population Based ◽  
Annualized Relapse Rate ◽  
The Mean

Background: Despite a growing use of rituximab (RTX) in neuromyelitis optica (NMO), data are lacking in patients with refractory NMO (RNMO), defined as cases with at least one relapse during immunosuppressive therapy. Objective: The purpose of this study was to assess RTX as a maintenance therapy in RNMO. Methods: Out of a total of 305 NMO cases from a population-based cohort, 21 RNMO patients received RTX during a mean follow-up period of 31 months. Results: After RTX, 11 patients (52.3%) were relapse free, meaning that 47.7% were refractory to RTX. The mean annualized relapse rate decreased from 1.3 to 0.4 ( p<0.001) and median EDSS from 5 to 3 ( p=0.02). Body mass index (BMI) was predictive of EDSS worsening. Conclusions: RTX is an effective and well-tolerated treatment in RNMO. BMI could be a predictive factor for efficacy.

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

scholarly journals Cardiac Involvement in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Disease)

2021 ◽  
Author(s):  
Rashid Saif Al Umairi ◽  
Khalid Al Manei ◽  
Fatma Al Lawati ◽  
Yaqoob Al Mahrouqi ◽  
Farida Al Balushi
Keyword(s):  
Cardiac Involvement ◽  
Granulomatosis With Polyangiitis ◽  
Immunosuppressive Treatment ◽  
Vascular Diseases ◽  
Management Plan ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Heart Patients ◽  
Chest Clinic ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss disease, is a rare vasculitis that affects small- to medium-sized vessels and has a propensity to involve the heart. Patients with cardiac involvement have a poor prognosis and usually require immunosuppressive treatment along with corticosteroids. Cardiovascular magnetic resonance (CMR) is a noninvasive diagnostic tool that can detect cardiac involvement and guide the management plan. Herein, we present the case of a 39-year-old man with a known history of bronchial asthma who was referred to the chest clinic at the Royal Hospital for further assessment of persistent lung parenchymal changes on chest computed tomography. Given the clinical context of the patient and the radiological findings, EGPA was suspected. Lung biopsy confirmed the diagnosis of EGPA. CMR was performed for further assessment, which confirmed cardiac involvement. The patient was started on prednisolone and azathioprine and showed significant radiological and clinical improvement. Keywords: Eosinophilic Granulomatosis with Polyangiitis, Vasculitis, Eosinophils, Vascular Diseases, ANCA-associated Vasculiti

La granulomatosi eosinofilica con poliangioite: dalla pelle al cuore

2020 ◽  
Vol 39 (9) ◽  
pp. 569-574
Author(s):  
Matteo Pavan ◽  
Anna Agrusti ◽  
Andrea Trombetta ◽  
Serena Pastore ◽  
Alberto Tommasini ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Immunosuppressive Treatment ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Eosinophilic Inflammation ◽  
Peripheral Eosinophilia ◽  
Churg Strauss Syndrome ◽  
History Of ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

Key words: Churg-strauss syndrome, Eosinophilic granulomatosis with polyangiitis in childhood, Vasculitis, Asthma, Hypereosinophilia Background - Eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss syndrome, is an extremely rare systemic vasculitis in the paediatric population. The hallmarks of eosinophilic granulomatosis with polyangiitis are a long history of asthma and peripheral eosinophilia with eosinophilic inflammation that may involve several organs. Findings - The paper reports the clinical characteristics, courses, and outcomes of the four patients diagnosed with eosinophilic granulomatosis with polyangiitis at IRCCS Burlo Garofolo (Trieste, Italy) from 1996 to 2015. The mean age at diagnosis was 11.5 years. All the patients presented a history of asthma and peripheral eosinophilia at diagnosis. 3/4 of the children presented upper airway and pulmonary disease. Skin and heart involvement was present in half of the patients, whereas gastrointestinal and neurological symptoms were reported in 25% of the cases. When performed, tissue biopsy revealed eosinophilic inflammation in all the cases. Anti-neutrophil cytoplasmic antibodies were negative in 66% patients. One young child died shortly after presentation, one remitted after immunosuppressive treatment and two patients needed low-dose corticosteroid therapy to maintain the remission. Conclusion - Comparison with an updated review of the series and cases of childhood-onset eosinophilic granulomatosis with polyangiitis reported in the literature showed similar demographic characteristics, clinical features and outcomes. Cardiac disease represents the poorer prognostic factor, leading to the 60% of the deaths reported.

Polyps, grommets and eosinophilic granulomatosis with polyangiitis

2018 ◽  
Vol 132 (3) ◽  
pp. 236-239 ◽  
Author(s):  
F G Kavanagh ◽  
W Hasan ◽  
D A Smyth ◽  
J E Fenton
Keyword(s):  
Observational Study ◽  
Diagnostic Criteria ◽  
Otitis Media ◽  
Nasal Polyps ◽  
Nasal Polyposis ◽  
Granulomatosis With Polyangiitis ◽  
Otitis Media With Effusion ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
The Mean ◽  
Eosinophilic Granulomatosis

AbstractObjective:To explore the link between nasal polyposis, refractory otitis media with effusion and eosinophilic granulomatosis with polyangiitis.Methods:A retrospective observational study was carried out of patients diagnosed with refractory otitis media with effusion necessitating grommet insertion and who had nasal polyps. Patients were evaluated to determine if they fulfilled the diagnostic criteria of eosinophilic granulomatosis with polyangiitis.Results:Sixteen patients (10 males and 6 females) were identified. The mean age of grommet insertion was 45.4 years. The mean number of grommets inserted per patient was 1.6. The mean number of nasal polypectomies was 1.7. All 16 patients had paranasal sinus abnormalities and otitis media with effusion, 14 had asthma, 9 had serological eosinophilia and 7 had extravascular eosinophilia. Nine patients met the diagnostic criteria for eosinophilic granulomatosis with polyangiitis.Conclusion:The co-presence of nasal polyps and resistant otitis media with effusion should raise the possibility of eosinophilic granulomatosis with polyangiitis.

Retrospective analysis of mortality within 30 days of systemic anticancer therapy and comparison with a previous audit at an Australian Regional Cancer Centre

2021 ◽  
pp. 107815522110160
Author(s):  
Bernadatte Zimbwa ◽  
Peter J Gilbar ◽  
Mark R Davis ◽  
Srinivas Kondalsamy-Chennakesavan
Keyword(s):  
Checkpoint Inhibitors ◽  
Anticancer Therapy ◽  
Mortality Rates ◽  
Feedback Process ◽  
Time To Death ◽  
Cancer Centre ◽  
Haematology Patients ◽  
The Mean ◽  
Regional Cancer Centre ◽  
Mean Time

Purpose To retrospectively determine the rate of death occurring within 14 and 30 days of systemic anticancer therapy (SACT), compare this against a previous audit and benchmark results against other cancer centres. Secondly, to determine if the introduction of immune checkpoint inhibitors (ICI), not available at the time of the initial audit, impacted mortality rates. Method All adult solid tumour and haematology patients receiving SACT at an Australian Regional Cancer Centre (RCC) between January 2016 and July 2020 were included. Results Over a 55-month period, 1709 patients received SACT. Patients dying within 14 and 30 days of SACT were 3.3% and 7.0% respectively and is slightly higher than our previous study which was 1.89% and 5.6%. Mean time to death was 15.5 days. Males accounted for 63.9% of patients and the mean age was 66.8 years. 46.2% of the 119 patients dying in the 30 days post SACT started a new line of treatment during that time. Of 98 patients receiving ICI, 22.5% died within 30 days of commencement. Disease progression was the most common cause of death (79%). The most common place of death was the RCC (38.7%). Conclusion The rate of death observed in our re-audit compares favourably with our previous audit and is still at the lower end of that seen in published studies in Australia and internationally. Cases of patients dying within 30 days of SACT should be regularly reviewed to maintain awareness of this benchmark of quality assurance and provide a feedback process for clinicians.

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