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1.
Curr Opin Pediatr ; 34(2): 197-202, 2022 04 01.
Article in English | MEDLINE | ID: covidwho-1853279

ABSTRACT

PURPOSE OF REVIEW: The purpose of this review is to highlight recent studies that have emerged on the topic of ANCA-associated vasculitis with some historical context. The review also discusses how the adult data is relevant to pediatric patients. RECENT FINDINGS: Pediatric studies on AAV are lacking. Therapies targeted to the inflammatory cascade specifically implicated in AAV, such as MPO inhibitors and complement mediators, are emerging. The PEXIVAS study recently called into question the routine use of plasma exchange (PLEX) in severe AAV, with no difference in ESKD or mortality found between patients who did or did not receive PLEX. Longer maintenance duration of nearly 48 months is preferred as compared with shorter duration in patients who are not on dialysis because of higher relapse rates in children with AAV. SUMMARY: Current treatment in AAV includes corticosteroids, rituximab, and cyclophosphamide for induction. Maintenance therapy commonly consists of azathioprine or rituximab. Plasma exchange (PLEX) is no longer recommended for induction therapy for AAV but some experts still consider this as an option for patients who are not responding to therapy or have severe disease at presentation. However, emerging novel therapies may be on the horizon.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , Adult , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/therapy , Azathioprine , Child , Humans , Immunosuppressive Agents/therapeutic use , Kidney , Plasma Exchange , Rituximab/therapeutic use
3.
Intern Med ; 61(4): 567-570, 2022 Feb 15.
Article in English | MEDLINE | ID: covidwho-1690562

ABSTRACT

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a systemic disease that causes vasculitis in various organs. Although the cause of the onset is unknown, infection has been reported to be a causative factor. The subsequent cytokine storm triggered by the immune response against SARS-CoV-2 infection has been reported to lead to symptoms being more severe. We herein report our experience with the onset of AAV following COVID-19 infection. We also report the course of anti-SARS-CoV-2 serum antibody titers following induction therapy, which suggests that vaccination and education concerning standard precautions are necessary in patients who require immunosuppressive therapy, even after COVID-19 infection.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , COVID-19 , Microscopic Polyangiitis , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis , Antibodies, Antineutrophil Cytoplasmic , Humans , Microscopic Polyangiitis/complications , Microscopic Polyangiitis/diagnosis , Peroxidase , SARS-CoV-2
4.
Front Immunol ; 13: 834981, 2022.
Article in English | MEDLINE | ID: covidwho-1686490

ABSTRACT

Humoral vaccine responses are known to be suboptimal in patients receiving B-cell targeted therapy, and little is known about vaccine induced T-cell immunity in these patients. In this study, we characterized humoral and cellular antigen-specific anti-SARS-CoV2 responses following COVID-19 vaccination in patients with ANCA-associated vasculitis (AAV) receiving anti-CD20 therapy, who were either B-cell depleted, or B-cell recovered at the time of vaccination and in normal control subjects. SARS-CoV-2 anti-spike (S) and anti-nucleocapsid (NC) antibodies were measured using electrochemiluminescence immunoassays, while SARS-CoV-2 specific T-cell responses to S glycoprotein subunits 1 (S1) and 2 (S2) and receptor binding domain peptide pools were measured using interferon-gamma enzyme-linked immunosorbent spot (ELISPOT) assays. In total, 26 recently vaccinated subjects were studied. Despite the lack of a measurable humoral immune response, B-cell depleted patients mounted a similar vaccine induced antigen-specific T-cell response compared to B-cell recovered patients and normal controls. Our data indicate that to assure a humoral response in patients receiving anti-CD20 therapy, SARS-CoV-2 vaccination should ideally be delayed until B-cell recovery (CD-20 positive B-cells > 10/µl). Nevertheless, SARS-CoV-2 vaccination elicits robust, potentially protective cellular immune responses in these subjects. Further research to characterize the durability and protective effect of vaccine-induced anti-SARS-CoV-2 specific T-cell immunity are needed.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy , COVID-19 Vaccines/immunology , COVID-19/immunology , Immunity, Cellular/immunology , Immunity, Humoral/immunology , Immunocompromised Host , Rituximab/therapeutic use , Adult , Aged , COVID-19/prevention & control , Female , Humans , Immunologic Factors/therapeutic use , Male , Middle Aged , SARS-CoV-2
5.
J Ren Care ; 48(2): 84-92, 2022 Jun.
Article in English | MEDLINE | ID: covidwho-1673225

ABSTRACT

BACKGROUND: Anti-neutrophil cytoplasmic antibody-associated vasculitis is a rare chronic autoimmune organ- and life-threatening disease primarily affecting kidneys and lungs. The clinical symptoms of the disease vary considerably, and patients may display varied symptoms. Healthcare professionals believe that the patients are well informed about their disease and symptoms of relapse. However, some patients contact the Department later than expected after the debut of symptoms of relapse. AIM: To investigate patients' experiences of informational needs living with anti-neutrophil cytoplasmic antibody-associated vasculitis. DESIGN: Individual semi-structured interviews by telephone due to the COVID-19 pandemic. Data were analysed through systematic text condensation. The Consolidated Criteria for Reporting Qualitative Research checklist was used. PARTICIPANTS: Ten patients were diagnosed with anti-neutrophil cytoplasmic antibodies-associated vasculitis. APPROACH: A qualitative study. FINDINGS: We identified five themes: 'Need oral and written information in a combination', 'Need information about living with the disease', 'Need information about symptoms and indications of relapse', 'Need psychological support to receive information about the disease' and 'Need a peer for sharing information'. CONCLUSION: To increase patients' self-management skills, healthcare professionals should focus on three areas of information: 'Provision of information', 'Content of the information' and 'Learning prerequisites'. This study indicates that patients have an increased need for more and clear information about the disease as well as psychological support to react accurately to symptoms that may lead to relapse. Most of the patients had limited knowledge, which indicates that patients need a better understanding of their disease, symptoms and relapse.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , COVID-19 , Humans , Pandemics , Qualitative Research , Recurrence
6.
Rheumatol Int ; 42(4): 749-758, 2022 04.
Article in English | MEDLINE | ID: covidwho-1669776

ABSTRACT

For the foreseeable future, vaccines are the cornerstone in the global campaign against the Coronavirus Disease-19 (COVID-19) pandemic. As the number and fatalities due to COVID-19 decline and the lockdown anywise rescinded, we recognize an increase in the incidence of autoimmune disease post-COVID-19 vaccination. However, the causality of the most vaccine-induced side effects is debatable and, at best, limited to a temporal correlation. We herein report a case of a 51-year-old gentleman who developed Anti-Neutrophil Cytoplasmic Antibody (ANCA)-associated vasculitis (AAV) 2 week post-COVID-19 vaccination. The patient responded favorably to oral steroids and rituximab. Additionally, we conducted a case-based review of vaccine-associated AAV describing their clinical manifestations and treatment response of this emerging entity.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , COVID-19 , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy , Antibodies, Antineutrophil Cytoplasmic , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Communicable Disease Control , Humans , Male , Middle Aged , Vaccination
9.
Intern Med ; 61(4): 567-570, 2022 Feb 15.
Article in English | MEDLINE | ID: covidwho-1542244

ABSTRACT

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a systemic disease that causes vasculitis in various organs. Although the cause of the onset is unknown, infection has been reported to be a causative factor. The subsequent cytokine storm triggered by the immune response against SARS-CoV-2 infection has been reported to lead to symptoms being more severe. We herein report our experience with the onset of AAV following COVID-19 infection. We also report the course of anti-SARS-CoV-2 serum antibody titers following induction therapy, which suggests that vaccination and education concerning standard precautions are necessary in patients who require immunosuppressive therapy, even after COVID-19 infection.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , COVID-19 , Microscopic Polyangiitis , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis , Antibodies, Antineutrophil Cytoplasmic , Humans , Microscopic Polyangiitis/complications , Microscopic Polyangiitis/diagnosis , Peroxidase , SARS-CoV-2
10.
Blood Purif ; 50(3): 290-297, 2021.
Article in English | MEDLINE | ID: covidwho-1533118

ABSTRACT

The principles and use of plasmapheresis are often little understood by intensivists. We propose to review the principles, the main indications, and the methods of using this technique.


Subject(s)
Critical Care/methods , Plasma Exchange/methods , Animals , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/therapy , COVID-19/therapy , Equipment Design , Guillain-Barre Syndrome/therapy , Humans , Liver Failure, Acute/therapy , Membranes, Artificial , Plasma Exchange/instrumentation , Purpura, Thrombotic Thrombocytopenic/therapy
12.
Curr Opin Rheumatol ; 34(1): 33-38, 2022 01 01.
Article in English | MEDLINE | ID: covidwho-1503782

ABSTRACT

PURPOSE OF REVIEW: This review paper evaluates the use of patient reported outcome (PROs) in systemic vasculitis and the increasing incorporation of these measures in the evaluation of clinical outcomes and healthcare provision. RECENT FINDINGS: Generic PROs such as the SF-12, SF-36, EQ-5D have been used to evaluate health-related quality of life (HRQOL) across the spectrum of vasculitis; including giant cell arteritis, antineutrophil cytoplasmic antibody (ANCA)-related vasculitis and immunoglobulin A vasculitis (IgA) vasculitis. More recently disease-specific PROs have been developed including the associated vasculitis (AAV)-PRO and GCA-PRO, whilst further work is ongoing including a Steroid-PRO. SUMMARY: Generic and disease-specific PROs are complimentary in nature, but the advent of disease-specific PROs allows evaluation of the impact of specific symptoms and intervention on patient HRQOL. Following the COVID-19 pandemic, the advent of increasing virtual work has brought the potential for electronic-PRO measures to the forefront and is a current area of interest.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , COVID-19 , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/therapy , Antibodies, Antineutrophil Cytoplasmic , Humans , Pandemics , Patient Reported Outcome Measures , Quality of Life , SARS-CoV-2
13.
Autoimmun Rev ; 20(12): 102986, 2021 Dec.
Article in English | MEDLINE | ID: covidwho-1491723

ABSTRACT

The Coronavirus Disease 2019 (COVID-19) pandemic influenced the management of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. A paucity of data exists on outcome of patients with vasculitis following COVID-19, but mortality is higher than in the general population and comparable to patients undergoing haemodialysis or kidney transplant recipients (reported mortality rates of 20-25%). Delays in diagnosis have been reported, which are associated with sequelae such as dialysis-dependency. Management of ANCA-associated vasculitis has not changed with the aim to suppress disease activity and reduce burden of disease. The use of rituximab, an important and widely used agent, is associated with a more severe hospital course of COVID-19 and absence of antibodies following severe acute respiratory syndrome (SARS)-CoV-2 infections, which prone patients to re-infection. Reports on vaccine antibody response are scarce at the moment, but preliminary findings point towards an impaired immune response, especially when patients receive rituximab as part of their treatment. Seropositivity was reported in less than 20% of patients when rituximab was administered within the prior six months, and the antibody response correlated with CD19+ B-cell repopulation. A delay in maintenance doses, if disease activity allows, has been suggested using a CD19+ B-cell guided strategy. Other immunosuppressive measures, which are used in ANCA-associated vasculitis, also impair humoral and cellular vaccine responses. Regular measurements of vaccine response or a healthcare-policy time-based strategy are indicated to provide additional doses ("booster") of COVID-19 vaccines. This review summarizes a recent educational forum and a recent virtual meeting of the European Vasculitis Society (EUVAS) focusing on COVID-19.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , COVID-19 , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/epidemiology , Antibodies, Antineutrophil Cytoplasmic , COVID-19 Vaccines , Humans , Pandemics , Rituximab , SARS-CoV-2
14.
Curr Opin Rheumatol ; 34(1): 25-32, 2022 01 01.
Article in English | MEDLINE | ID: covidwho-1483554

ABSTRACT

PURPOSE OF REVIEW: The main purpose of this review is to present newly reported cutaneous manifestations of systemic vasculitis, updates in investigations to verify systemic involvement in cases with cutaneous vasculitis and new therapeutic guidelines. The spectrum of COVID-19-related vasculitis is also covered. RECENT FINDINGS: Only a few reports highlighted new cutaneous presentations or associations with some systemic vasculitic entities. For example, the association of inflammatory disorders with Takayasu arteritis, the importance of considering Kawasaki disease in febrile children with erythema nodosum, the development of necrotic ulcers on fingers and toes in Behçet's disease and the possible presence of polyarteritis nodosa-like pathological features in vulvar ulcers of Behçet's disease. New attempts to classify cutaneous manifestations of giant cell arteritis (GCA) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV) and the diagnostic investigations for cutaneous vasculitis cases to verify systemic involvement are discussed. Treatment of systemic vasculitis with cutaneous vasculitis should be tailored according to disease status. A plethora of reports in the past 2 years focused on the broad spectrum of COVID-19 vasculitic manifestations. SUMMARY: Although newly reported cutaneous manifestations of systemic vasculitis are relatively uncommon, the plethora of reports in the past 2 years on COVID-19 vasculitis necessitates the expansion of the classification of vasculitis associated with probable cause to include severe acute respiratory syndrome coronavirus-2 (SARS-Cov-2) vasculitis.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , COVID-19 , Skin Diseases, Vascular , Takayasu Arteritis , Humans , SARS-CoV-2 , Takayasu Arteritis/complications , Takayasu Arteritis/diagnosis
15.
Front Immunol ; 12: 762006, 2021.
Article in English | MEDLINE | ID: covidwho-1477832

ABSTRACT

As the coronavirus disease 2019 (COVID-19) pandemic is ongoing and new variants of severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) are emerging, there is an urgent need for COVID-19 vaccines to control disease outbreaks by herd immunity. Surveillance of rare safety issues related to these vaccines is progressing, since more granular data emerge with regard to adverse events of COVID-19 vaccines during post-marketing surveillance. Interestingly, four cases of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) presenting with pauci-immune crescentic glomerulonephritis (GN) after COVID-19 mRNA vaccination have already been reported. We here expand our current knowledge of this rare but important association and report a case of AAV presenting with massive rhabdomyolysis and pauci-immune crescentic GN after Pfizer-BioNTech COVID-19 mRNA vaccination. As huge vaccination programs are ongoing worldwide, post-marketing surveillance systems must continue to assess vaccine safety important for the detection of any events associated with COVID-19 vaccination. This is especially relevant in complex diseases where diagnosis is often challenging, as in our patient with AAV presenting with massive rhabdomyolysis and pauci-immune crescentic GN.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/pathology , COVID-19 Vaccines/adverse effects , Glomerulonephritis/pathology , Rhabdomyolysis/pathology , Aged , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/immunology , Antibodies, Antineutrophil Cytoplasmic/blood , Antibodies, Antineutrophil Cytoplasmic/immunology , COVID-19/prevention & control , COVID-19 Vaccines/immunology , Female , Glomerulonephritis/diagnosis , Glomerulonephritis/immunology , Humans , RNA, Messenger/immunology , Rhabdomyolysis/diagnosis , Rhabdomyolysis/immunology
16.
Front Immunol ; 12: 712572, 2021.
Article in English | MEDLINE | ID: covidwho-1472386

ABSTRACT

The complement system is central to first-line defense against invading pathogens. However, excessive complement activation and/or the loss of complement regulation contributes to the development of autoimmune diseases, systemic inflammation, and thrombosis. One of the three pathways of the complement system, the alternative complement pathway, plays a vital role in amplifying complement activation and pathway signaling. Complement factor D, a serine protease of this pathway that is required for the formation of C3 convertase, is the rate-limiting enzyme. In this review, we discuss the function of factor D within the alternative pathway and its implication in both healthy physiology and disease. Because the alternative pathway has a role in many diseases that are characterized by excessive or poorly mediated complement activation, this pathway is an enticing target for effective therapeutic intervention. Nonetheless, although the underlying disease mechanisms of many of these complement-driven diseases are quite well understood, some of the diseases have limited treatment options or no approved treatments at all. Therefore, in this review we explore factor D as a strategic target for advancing therapeutic control of pathological complement activation.


Subject(s)
Complement Factor D/antagonists & inhibitors , Complement Pathway, Alternative/drug effects , Molecular Targeted Therapy , Adipose Tissue/metabolism , Aging/immunology , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/immunology , Antibodies, Monoclonal, Humanized/therapeutic use , Autoimmune Diseases/immunology , Autoimmune Diseases/therapy , Complement Factor D/biosynthesis , Complement Factor D/deficiency , Complement Factor D/physiology , Energy Metabolism , Geographic Atrophy/genetics , Geographic Atrophy/immunology , Hemoglobinuria, Paroxysmal/drug therapy , Hemoglobinuria, Paroxysmal/genetics , Hemoglobinuria, Paroxysmal/immunology , Hepatocytes , Humans , Kidney Diseases/immunology , Liver/injuries , Oligonucleotides, Antisense/therapeutic use , Peptides, Cyclic/therapeutic use , Phagocytosis
19.
Cells ; 10(7)2021 07 04.
Article in English | MEDLINE | ID: covidwho-1394870

ABSTRACT

Neutrophil extracellular traps (NETs) are web-like structures of decondensed extracellular chromatin fibers and neutrophil granule proteins released by neutrophils. NETs participate in host immune defense by entrapping pathogens. They are pro-inflammatory in function, and they act as an initiator of vascular coagulopathies by providing a platform for the attachment of various coagulatory proteins. NETs are diverse in their ability to alter physiological and pathological processes including infection and inflammation. In this review, we will summarize recent findings on the role of NETs in bacterial/viral infections associated with vascular inflammation, thrombosis, atherosclerosis and autoimmune disorders. Understanding the complex role of NETs in bridging infection and chronic inflammation as well as discussing important questions related to their contribution to pathologies outlined above may pave the way for future research on therapeutic targeting of NETs applicable to specific infections and inflammatory disorders.


Subject(s)
Cardiovascular System/pathology , Extracellular Traps/metabolism , Infections/pathology , Inflammation/pathology , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/pathology , Humans , Infections/virology , Models, Biological
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