scholarly journals Recurrence or reactivation of SARS-CoV-2 infection after immunosuppressive therapy in patients with ANCA-associated vasculitis and COVID-19

2022 ◽  
Vol 10 (2196-5293) ◽  
pp. 6-10
Author(s):  
Mansour Mbengue ◽  
Bede Bigirimana ◽  
Lolly Romeo Irankunda ◽  
Mohamed Cherif Dial ◽  
Abdou Niang
Keyword(s):  
Immunosuppressive Therapy ◽  
Anca Associated Vasculitis

scholarly journals ANCA-associated vasculitis in a 14 years-old patient: a clinical case

2020 ◽  
Vol 27 (5) ◽  
pp. 184-194
Author(s):  
A. V. Burlutskaya ◽  
N. V. Savelyeva ◽  
N. S. Тaran
Keyword(s):  
Respiratory Failure ◽  
Immunosuppressive Therapy ◽  
Clinical Case ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Pulse Therapy ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Associated Vasculitis ◽  
Nasal Bleeding ◽  
Diagnostic Research

Background. ANCA-associated systemic vasculitis is a rare childhood disease. Antineutrophil cytoplasmic autoantibodies (ANCA)-related vasculitises include microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. Their rarity often leads to a late diagnosis, rapid disability and high mortality in patients due to aggressive respiratory, pulmonary lesion and renal failure.Clinical Case Description. The patient suffered from a recurrent bronchoobstructive syndrome with signs of respiratory failure, obscure origin fever and chronic rhinitis with nasal bleeding for 6 months. The patient was diagnosed with obstructive bronchitis (putative bronchial asthma debut), received antibacterial therapy and inhalation bronchodilators without stable improvement during the entire period. Skin haemorrhages and arthralgia stimulated diagnostic research to establish ANCA-associated systemic vasculitis (presence of proteinase 3-specifi c ANCAs in titre 1/80). CT lung scanning revealed frosted glass foci of reduced pulmonary pneumatisation and signs of bilateral bronchoobstruction. Immunosuppressive therapy with glucocorticosteroids (methylprednisolone pulse therapy No. 3, 1000 mg intravenously on alternate days, subsequent per os administration of 1 mg/kg/day) and cyclophosphamide (500 mg intravenously once per 28 days) was prescribed. This led to the positive dynamics with eliminated fever and skin haemorrhages, as well as essentially reduced signs of respiratory failure.Conclusion. Diagnosis of systemic vasculitis is often complicated and long-term due to commonly non-specifi c debut symptoms of autoimmune disorders. In the described case, the fi rst 6 months of illness displayed intoxication and bronchoobstruction with signs of respiratory failure. Haemorrhagic rashes, arthralgias and the presence of ANCAs are proxy to vasculitis. Standard immunosuppressive therapy for ANCA-associated vasculitis improved the patient’s condition.

scholarly journals Infective endocarditis mimicking ANCA-associated vasculitis: does it require immunosuppressive therapy?

Medicine ◽  
2020 ◽  
Vol 99 (29) ◽  
pp. e21358
Author(s):  
Xiao-dong Shi ◽  
Wan-yu Li ◽  
Xue Shao ◽  
Li-mei Qu ◽  
Zhen-yu Jiang
Keyword(s):  
Infective Endocarditis ◽  
Immunosuppressive Therapy ◽  
Anca Associated Vasculitis

scholarly journals Pituitary dysfunction in patients with ANCA associated vasculitis: prevalence, presentation, and outcomes

2020 ◽  
Vol 11 ◽  
pp. 204062232093063
Author(s):  
Chunjia Li ◽  
Yu Zou ◽  
Xin Lu ◽  
Guochun Wang ◽  
Xiaoming Shu
Keyword(s):  
Immunosuppressive Therapy ◽  
Clinical Symptoms ◽  
Clinical Manifestations ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Magnetic Resonance Images ◽  
Initial Symptom ◽  
Pituitary Fossa ◽  
Pituitary Dysfunction ◽  
Anca Associated Vasculitis ◽  
Positron Emission

Background: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are rare multisystem autoimmune diseases characterized by inflammatory cell infiltration causing necrosis of small blood vessels. Pituitary involvement in AAV is poorly described. This study aimed to describe the prevalence, clinical characteristics, and outcomes of pituitary involvement in patients with AAV. Methods: A total of 150 patients diagnosed with AAV and hospitalized in the China–Japan Friendship Hospital between 2009 and 2019 were enrolled in this retrospective study. Patients diagnosed with pituitary involvement in AAV were selected for inclusion. Results: Three patients (2%) were identified with pituitary involvement. Two patients had positive ANCA titers, one with proteinase 3 positive and one with myeloperoxidase positive antibodies. Pituitary dysfunction presented as an initial symptom in one patient and developed over the course of the diseases in the other two patients. All three patients had abnormal hormones. Among them, two patients had an enlarged pituitary, shown by magnetic resonance images (MRIs), and one patient had a normal sized pituitary, shown by MRI, but presented with increased linear radioactivity uptake in the pituitary fossa by positron emission tomography-computed tomography. All patients were treated with corticosteroid and immunosuppressive therapy. Both pituitary dysfunction and vasculitis were in remission. Conclusion: Pituitary involvement is uncommon in AAV and it can occur at any point during AAV. The main clinical manifestations are central diabetes insipidus and panhypopituitarism. Immunosuppressive therapy could significantly alleviate clinical symptoms as well as pituitary imaging.

scholarly journals Subarachnoid Hemorrhage and Prostatic Involvement in Granulomatosis with Polyangiitis – An Atypical Presentation

2019 ◽  
Vol 19 (01) ◽  
pp. 34-39
Author(s):  
Raymond Bak Hei Chu ◽  
Priscilla Ching Han Wong ◽  
Joey Ka Ming Wai ◽  
Florence Hiu Yi Yap
Keyword(s):  
Subarachnoid Hemorrhage ◽  
Vertebral Artery ◽  
Immunosuppressive Therapy ◽  
Acute Urinary Retention ◽  
Granulomatosis With Polyangiitis ◽  
Rare Complication ◽  
Artery Aneurysm ◽  
Atypical Presentation ◽  
Anca Associated Vasculitis ◽  
First Case

Granulomatosis with Polyangiitis (GPA) is a form of Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. We report a case of a 56-year-old man with GPA who first presented with acute urinary retention, and then developed pulmonarycavitating lesions and subarachnoid hemorrhage (SAH). The SAH was due to a ruptured right vertebral artery aneurysm, whichwas subsequently embolized. The patient initially had a good response to immunosuppressive therapy but later succumbed due to fulminant sepsis. SAH is a rare complication of GPA and usually not associated with a detectable aneurysm. Our patient is thesecond case in the literature where an aneurysm had been detected on angiography and the first case where examination of the culprit aneurysm revealed features of vasculitis.

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

STRONGYLOIDIASIS COMPLICATING IMMUNOSUPPRESSIVE THERAPY FOR ANCA-ASSOCIATED VASCULITIS

CHEST Journal ◽  
2007 ◽  
Vol 132 (4) ◽  
pp. 698A
Author(s):  
Chad I. Case ◽  
Donald H. Loebl ◽  
Joseph B. Barney ◽  
Keith M. Wille
Keyword(s):  
Immunosuppressive Therapy ◽  
Anca Associated Vasculitis

scholarly journals AB0472 INFECTIOUS PROFILE IN PATIENTS WITH ANCA-ASSOCIATED VASCULITIS: RETROSPECTIVE ANALYSIS IN A REFERRAL CENTRE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1534.3-1534
Author(s):  
M. Estévez Gil ◽  
B. Maure ◽  
A. Argibay ◽  
C. Vazquez-Triñanes ◽  
B. Gimena ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Immunosuppressive Therapy ◽  
Bacterial Infections ◽  
Granulomatosis With Polyangiitis ◽  
Response To Therapy ◽  
Upper Airways ◽  
Factors Associated ◽  
Icu Admission ◽  
Anca Associated Vasculitis ◽  
Increased Risk

Background:The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are rare multisystem autoimmune diseases of unknown cause, characterised by inflammatory cell infiltration causing necrosis of blood vessels. The treatment of AAV requires prolonged immunosuppressive therapy. Infections remain a major cause of morbidity and mortality.Objectives:The aim of our study was to investigate the prevalence and characteristics of infection, and analyse the factors associated with infection in patients with AAV from Northern of Spain.Methods:Retrospective, descriptive study of patients with AAV followed in a specific Systemic Autoimmune Diseases and Thrombosis Unit from January 2000 to December 2019. Demographic, laboratory, microbiology, treatment and clinical data were collected from the medical records. AAV was diagnosed according to the definitions of the Chapel Hill nomenclature and designated as granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA) or pauci-immune necrotizing and/or crescentic glomerulonephritis without systemic vasculitis (renal-limited vasculitis, RLV). Disease activity of AAV was evaluated by Birmingham Vasculitis Activity score (BVAS). The infection episode was considered on the basis of clinical, laboratory, microbiology, radiology information, and response to therapy. Different episodes of infection in one patient were independently reflected. Data were analysed using SPSS 25.0Results:Thirty-six patients of which 20 (55.6%) were males. Median follow-up was 42 months. The mean age at the diagnosis was 61.14 ± 17.49 years and mean BVAS was 18.81 ± 5.96. 15 patients were diagnosed of GPA, 13 of MPA, 5 of EGPA and 3 of RLV. 72.2% MPO, 11.1% PR3. Lung involvement occurred in 75% of patients, upper airways was detected in 41.7%, skin involvement in 16.7%, Nervous system affectation occurred in 33.3%. 30 patients (83.3%) had renal affectation with a mean of 1.93± 1.66 gr/dl of Proteinuria and 2.9±2.17mg/dl of creatinine. We detected hypocomplementemia in 27.8% of patients (C3 in 19.4% and C4 in 16.7%). Regarding induction treatments, all patients received corticoids at high doses, 21 (58.3%) Cyclophosphamide, 3 (20%) Rituximab and 2 (13.3%) patients, Azathioprine. When we analyse infections, we detected 15 patients (41.66%) who presented any infection after the diagnosis of AAV, with a total of 71 episodes of infection. The most frequent were bacterial infections (29 episodes), specifically gram negative pathogens. The most frequent location was the respiratory (56.3%) followed by urinary (22.5%) and Skin (8.5%). Also opportunistic infections were described: 3 patients with Aspergillus fumigatus and one patient with Cryptococcus neoformans. 41 of these episodes needed hospitalisation with a median stay of 11 days. 6 episodes warranted intensive care unit (ICU) admission. Infection related mortality was 2.82%. We made latent tuberculosis screening and Pneumocystis prophylaxis in all our patients. No cases of Tuberculosis or Pneumocystis were recorded. Factors associated with increased risk of hospitalisation with statistical signification in univariated study were MPA, Hypocomplementemia and increased BVAS. But in the logistical regression study, only the value of the BVAS maintained statistical significance. The only factor associated with elevated risk of ICU admission was IgG deficit in the multivariate analysis. Neither immunosuppressive therapy nor age was associated with increased risk of infection in our study.Conclusion:More than 50% of the episodes of infection needed hospitalisation in patients with AAV. Risk factors for hospitalisation and ICU admission were BVAS and IgG deficit respectively. Bacterial infections were the most frequent but fungal infections were the most severe.Disclosure of Interests:None declared

scholarly journals Evaluation of the Safety and Efficacy of Avacopan, a C5a Receptor Inhibitor, in Patients With Antineutrophil Cytoplasmic Antibody–Associated Vasculitis Treated Concomitantly With Rituximab or Cyclophosphamide/Azathioprine: Protocol for a Randomized, Double-Blind, Active-Controlled, Phase 3 Trial

10.2196/16664 ◽  
2020 ◽  
Vol 9 (4) ◽  
pp. e16664 ◽  
Author(s):  
Peter A Merkel ◽  
David R Jayne ◽  
Chao Wang ◽  
Jan Hillson ◽  
Pirow Bekker
Keyword(s):  
Immunosuppressive Therapy ◽  
Membrane Attack Complex ◽  
Ethics Committees ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Sustained Remission ◽  
Double Blind ◽  
C5a Receptor ◽  
Safety And Efficacy ◽  
Anca Associated Vasculitis ◽  
Orally Bioavailable

Background Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis is a serious, often life-threatening disease. In new-onset disease or a relapse, the standard treatment is immunosuppressive therapy with glucocorticoids; these therapies are associated with substantial short- and long-term toxicity. Complement component 5a (C5a) binding to C5a receptor (C5aR) may play a central role in the pathogenesis of ANCA-associated vasculitis. Avacopan is a novel, orally bioavailable, and highly selective antagonist of human C5aR. Avacopan does not interfere with the production of C5b or the membrane attack complex (ie, terminal complement complex) and does not block C5a binding to a second receptor, C5L2 (also called C5aR2), shown to be protective in antimyeloperoxidase glomerulonephritis. This trial will evaluate if avacopan replaces the need for chronic glucocorticoids in the treatment of ANCA-associated vasculitis. Objective The aim of this study is to determine the proportions of patients in remission at week 26 and with sustained remission at week 52, defined as Birmingham Vasculitis Activity Score=0, and not taking glucocorticoids within the 4 weeks before week 26 and week 52, respectively. Methods The Avacopan Development in Vasculitis to Obtain Corticosteroid elimination and Therapeutic Efficacy study is a randomized, double-blind, active-comparator (prednisone), 2-arm study evaluating the safety and efficacy of avacopan versus prednisone, administered in combination with other immunosuppressive therapy. Eligible subjects will have active disease requiring induction of remission. Subjects are stratified based on the type of immunosuppressive therapy, ANCA subtype, and new or relapsing disease. Target sample size is 300 patients, enrolled at over 200 sites globally. All authors and local ethics committees approved the study design. All patients will provide informed consent. Results Enrollment of patients was completed in Q4 2018. Topline results are anticipated to be published by Q3 2020. Conclusions Results will be released irrespective of whether the findings are positive or negative. Trial Registration ClinicalTrials.gov NCT02994927; https://clinicaltrials.gov/ct2/show/NCT02994927 International Registered Report Identifier (IRRID) DERR1-10.2196/16664

scholarly journals Cutaneous manifestations of ANCA-associated vasculitis and immunosuppressive therapy: cause-effect relationships (a case report)

2020 ◽  
Vol 12 (3) ◽  
pp. 79-84
Author(s):  
Madina A. Kitova ◽  
Maksim V. Maksimov ◽  
Valeriy N. Marchenko ◽  
Elena A. Bruchkus ◽  
Denis A. Davydov
Keyword(s):  
Immunosuppressive Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Secondary Infection ◽  
Skin Lesions ◽  
Cutaneous Manifestations ◽  
Multiple Organs ◽  
Anca Associated Vasculitis ◽  
Clinical Triad ◽  
The One

Granulomatosis with polyangiitis, formerly known as Wegeners granulomatosis, is an autoimmune ANCA-associated systemic vasculitis characterized by extensive damage to multiple organs and systems. Besides a typical clinical triad of ENT, lungs, and kidneys injury, various types of skin lesions can be found in 1050% of cases. A severe course of the disease and low survival of patients often requires using aggressive treatment in a form of combined immunosuppressive therapy. On the one hand, it generally improves the prognosis, and on the other is itself associated with numerous complications. One of them is a secondary infection. Skin is the second most common localization of infection after the respiratory system. Preceding skin lesions caused by vasculitis may increase the risk of infection. Thus, patients with ANCA-associated vasculitis should be carefully observed for cutaneous manifestation, both before and during the immunosuppressive therapy.

Recurrences and Infections During Continuous Immunosuppressive Therapy After Beginning Dialysis in ANCA-Associated Vasculitis

2007 ◽  
Vol 50 (1) ◽  
pp. 36-46 ◽  
Author(s):  
Frauke Weidanz ◽  
Clara J. Day ◽  
Peter Hewins ◽  
Caroline O. Savage ◽  
Lorraine Harper
Keyword(s):  
Immunosuppressive Therapy ◽  
Anca Associated Vasculitis
Sign in / Sign up

Export Citation Format

Share Document