scholarly journals ANCA-Associated Vasculitis: An Update

2021 ◽  
Vol 10 (7) ◽  
pp. 1446
Author(s):  
Salem Almaani ◽  
Lynn A. Fussner ◽  
Sergey Brodsky ◽  
Alexa S. Meara ◽  
David Jayne
Keyword(s):  
Nervous System ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Treatment Options ◽  
Clinical Manifestations ◽  
Treatment Modalities ◽  
Proteinase 3 ◽  
Anca Associated Vasculitis ◽  
Organ Systems ◽  
Kidney Involvement

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) represents a group of small vessel vasculitides characterized by granulomatous and neutrophilic tissue inflammation, often associated with the production of antibodies that target neutrophil antigens. The two major antigens targeted by ANCAs are leukocyte proteinase 3 (PR3) and myeloperoxidase (MPO). AAV can be classified into 3 categories based on patterns of clinical involvement: namely, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA). Clinically, AAV involves many organ systems including the lungs, kidneys, skin, and nervous system. The prognosis of AAV has improved dramatically due to advances in the understanding of its pathogenesis and treatment modalities. This review will highlight some of the recent updates in our understanding of the pathogenesis, clinical manifestations, and treatment options in patients with AAV focusing on kidney involvement.

scholarly journals Immunopathogenesis of ANCA-Associated Vasculitis

2020 ◽  
Vol 21 (19) ◽  
pp. 7319
Author(s):  
Andreas Kronbichler ◽  
Keum Hwa Lee ◽  
Sara Denicolo ◽  
Daeun Choi ◽  
Hyojeong Lee ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Autoimmune Disorder ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Proteinase 3 ◽  
Disease Biomarkers ◽  
Disease Phenotypes ◽  
Anca Associated Vasculitis ◽  
Clinical Presentations ◽  
Eosinophilic Granulomatosis

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disorder which affects small- and, to a lesser degree, medium-sized vessels. ANCA-associated vasculitis encompasses three disease phenotypes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). This classification is largely based on clinical presentations and has several limitations. Recent research provided evidence that genetic background, risk of relapse, prognosis, and co-morbidities are more closely related to the ANCA serotype, proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA, compared to the disease phenotypes GPA or MPA. This finding has been extended to the investigation of biomarkers predicting disease activity, which again more closely relate to the ANCA serotype. Discoveries related to the immunopathogenesis translated into clinical practice as targeted therapies are on the rise. This review will summarize the current understanding of the immunopathogenesis of ANCA-associated vasculitis and the interplay between ANCA serotype and proposed disease biomarkers and illustrate how the extending knowledge of the immunopathogenesis will likely translate into development of a personalized medicine approach in the management of ANCA-associated vasculitis.

scholarly journals ANCA-ASSOCIATED VASCULITIS: HETEROGENEITY OF CLINICAL MANIFESTATIONS, PROGNOSIS, CURRENT OPPORTUNITIES OF PHARMACOTHERAPY

2017 ◽  
Vol 13 (1-2) ◽  
pp. 98-105
Author(s):  
O.B. Yaremenko ◽  
L.B.
Keyword(s):  
Nervous System ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Joint Involvement ◽  
Upper Respiratory Tract ◽  
Diagnosis And Prognosis ◽  
Anca Associated Vasculitis

The article highlights the new views on classification and nomenclature of systemic vasculitis, the meaning of detection of antineutrophil cytoplasmic antibodies (ANCA) in the diagnosis and prognosis of the disease. The literature data and own research regarding clinical manifestations of ANCA-associated vasculitis are presented.  Analyzing the first clinical manifestations in 41 patients with granulomatosis with polyangiitis, we identified four variants of the debut: with involvement of ENT organs (n ​=21), with lung lesions without involvement of the upper respiratory tract (n=8), with skin lesions, joint involvement and fever (n=7) and other variants (n=5). Fever (76%), involvement of ENT organs (51%), skin (41%), nervous system (39%) and arthritis/arthralgia (37%) were the most frequent first manifestations of granulomatosis with polyangiitis. In comparison with the presenting features there were more often lesions of the skin (66% vs. 41%), nervous system (51% vs. 39%), kidneys (41% vs. 10%), lungs (63% vs. 30%), eyes (32% vs. 10%) and myalgia (34% vs. 12%) throughout course of disease.  Among the lesions of the ENT organs, sinusitis (n=19), rhinitis (n=8) and otitis (n=6) were predominate, laryngotracheitis, sublottic stenosis, saddle-shaped deformation of the nose, destruction of the walls of the paranasal sinuses and mastoiditis were diagnosed less frequently. The latest clinical recommendations for treating patients with ANCA-associated vasculitis, including the use of immunobiological therapy are presented, as well as describing of the results of our own experience in the using of immunobiological therapy in patients with granulomatosis with polyangiitis.

scholarly journals Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on ANCA type

2015 ◽  
Vol 75 (6) ◽  
pp. 1166-1169 ◽  
Author(s):  
Sebastian Unizony ◽  
Miguel Villarreal ◽  
Eli M Miloslavsky ◽  
Na Lu ◽  
Peter A Merkel ◽  
...  
Keyword(s):  
Disease Activity Score ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Proteinase 3 ◽  
Successful Completion ◽  
Anca Associated Vasculitis ◽  
Registration Number ◽  
Treatment Responses ◽  
New Onset

ObjectiveTo evaluate whether the classification of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) according to ANCA type (anti-proteinase 3 (PR3) or anti-myeloperoxidase (MPO) antibodies) predicts treatment response.MethodsTreatment responses were assessed among patients enrolled in the Rituximab in ANCA-associated Vasculitis trial according to both AAV diagnosis (granulomatosis with polyangiitis (GPA)/microscopic polyangiitis (MPA)) and ANCA type (PR3-AAV/MPO-AAV). Complete remission (CR) was defined as disease activity score of 0 and successful completion of the prednisone taper.ResultsPR3-AAV patients treated with rituximab (RTX) achieved CR at 6 months more frequently than did those randomised to cyclophosphamide (CYC)/azathioprine (AZA) (65% vs 48%; p=0.04). The OR for CR at 6 months among PR3-AAV patients treated with RTX as opposed to CYC/AZA was 2.11 (95% CI 1.04 to 4.30) in analyses adjusted for age, sex and new-onset versus relapsing disease at baseline. PR3-AAV patients with relapsing disease achieved CR more often following RTX treatment at 6 months (OR 3.57; 95% CI 1.43 to 8.93), 12 months (OR 4.32; 95% CI 1.53 to 12.15) and 18 months (OR 3.06; 95% CI 1.05 to 8.97). No association between treatment and CR was observed in the MPO-AAV patient subset or in groups divided according to AAV diagnosis.ConclusionsPatients with PR3-AAV respond better to RTX than to CYC/AZA. An ANCA type-based classification may guide immunosuppression in AAV.Trial registration numberNCT00104299; post-results.

scholarly journals AB0533 ANTI-NEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA) IN GENERAL POPULATION WITHOUT ANCA ASSOCIATED VASCULITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1563.3-1563
Author(s):  
H. Tamaki ◽  
S. Fukui ◽  
T. Nakai ◽  
G. Kidoguchi ◽  
S. Kawaai ◽  
...  
Keyword(s):  
Risk Factors ◽  
Autoimmune Disease ◽  
General Population ◽  
Granulomatosis With Polyangiitis ◽  
Dietary Habits ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Anca Associated Vasculitis ◽  
The Mean

Background:Currently it is hypothesized that many systemic autoimmune diseases occur due to environmental risk factors in addition to genetic risk factors. Anti-Neutrophil Cytoplasmic Antibody (ANCA) is mainly associated with three systemic autoimmune disease including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA). It is known that ANCA can be positive before clinical symptoms in patients with known diagnosis of GPA and ANCA titers rise before clinical manifestations appear. However, prevalence of ANCA among general population is not well known. It has not been described as well how many of people with positive ANCA eventually develop clinical manifestations of ANCA associated Vasculitis.Objectives:This study aims to estimate prevalence of ANCA in general population without ANCA associated Vasculitis. It also describes natural disease course of people with positive ANCA without ANCA associated Vasculitis. Risk factors for positive ANCA are also analyzed.Methods:This is a single center retrospective study at Center for Preventive Medicine of St. Luke’s International Hospital in Tokyo. ANCA was checked among the patients who wished to between 2018 and 2019. St. Luke’s Health Check-up Database (SLHCD) was utilized to collect the data. The patients whose serum was measured for ANCA were identified. The data for basic demographics, social habits, dietary habits and laboratory data were extracted. The charts of the patients with positive ANCA were reviewed.Results:Sera of total 1204 people were checked for ANCA. Of these 1204 people, 587 (48.8%) are male and the mean age was 55.8 years (32.6 to 79). There were total 11 patients with positive ANCA. Myeloperoxidase ANCA (MPO-ANCA) was positive for 3 patients and proteinase 3 ANCA (PR3-ANCA) was positive for 8 patients. Of these 11 patients, 5 were male (45.5%) and the mean age was 54.6 years. Two patients had history of autoimmune disease (primary biliary cirrhosis and ulcerative colitis). Five patients were evaluated by rheumatologists with the median follow-up period of 274 days. None of them developed clinical signs and symptoms of ANCA associated Vasculitis. Four out of five patients had ANCA checked later, two of which turned negative. The prevalence of ANCA in this cohort was 0.9% (95% confidence interval [95% CI]: 0.5% to 1.6%). Univariate analysis was performed to identify risk factors of positive ANCA. The variables analyzed include age, gender, body mass index (BMI), smoking habits, alcohol intake, dietary habits (fruits, fish, red meat), hypertension, dyslipidemia, and laboratory data. None of these variables demonstrated statistically significant differences except for positive rheumatoid factor (ANCA positive group: 33 % vs ANCA negative group: 9.1%, p value = 0.044).Conclusion:The prevalence of ANCA in this cohort was 0.9% (95% CI: 0.5% to 1.6%). None of them who had a follow-up developed ANCA associated Vasculitis during the follow-up period. Longer follow-up and more patients are necessary to determine natural course of people with positive ANCA.Disclosure of Interests:None declared

scholarly journals POS0830 FACTORS INFLUENCING PATIENT-REPORTED OUTCOMES IN ANCA-ASSOCIATED VASCULITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 668.2-669
Author(s):  
S. Monti ◽  
P. Delvino ◽  
C. Klersy ◽  
G. Coppa ◽  
A. Milanesi ◽  
...  
Keyword(s):  
Disease Activity ◽  
Global Assessment ◽  
Patient Reported Outcomes ◽  
Granulomatosis With Polyangiitis ◽  
Disease Duration ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Associated Vasculitis ◽  
Organ Systems ◽  
Damage Accrual ◽  
Patient Reported

Background:Patient-reported outcomes (PROs) are currently poorly integrated in the clinical evaluation of disease activity in patients with ANCA-associated vasculitis (AAV).Objectives:To assess the distribution of the Patient Global Assessment (PtGA) in patients with AAV in stable remission, and to identify correlates of PtGA; to assess the discordance between PtGA score and PhGA.Methods:Patients with a diagnosis of AAV [eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, microscopic polyangiitis] in stable, complete remission (defined by a BVAS=0) and with a Physician Global Assessment (PhGA)=0 were included. A questionnaire including several aspects of disease captured by PROs was collected. PtGA on a 0-100 mm visual analogue scale (VAS) was assessed, with higher scores representing higher/worse levels of disease activity. Similarly, VAS for pain, chronic damage according to the patient’s opinion, general health (GH), fatigue, and sleep quality were collected. The worst symptom in the patient’s opinion affecting the overall assessment of disease activity was recorded. The Cragg Hurdle model was used to assess the predictors of PtGA.Results:65 patients were included, female 57%, mean age 61±12 years. Mean disease duration at enrollment was 8±6 years. Mean vasculitis damage index (VDI) was 4.4 ±2.3, with 45% of patients having a VDI ≥ 5. Despite having been classified as being in remission, PtGA was elevated in 37% of patients. We explored several correlates of PtGA. Higher degree of damage accrual (VDI) did not influence the patient’s evaluation of current disease activity. Similarly, we did not identify a correlation between older age, educational level, number of organ-systems involved, number of comorbidities, the number of previous major or minor relapses, higher disease duration, nor the type of AAV diagnosis (figure 1, panel A). Only sex significantly correlated with PtGA scores: 19 (51%) of female patients reported an elevated PtGA compared to only 5 (18%) of male (p=0.009). PtGA resulted to be significantly correlated with other (mostly modifiable) PROs including VAS pain, perception of the level of chronic damage accrual, GH, and fatigue (figure 1, panel B). The agreement between patients’ and physicians’ assessments of disease activity was 63%. Patients reported pain, followed by chronic respiratory symptoms to be the worst-experienced ongoing manifestations affecting their evaluation of disease activity.Conclusion:A significant proportion of patients with AAV considered to be in remission by the physician still declares to have persistent aspects of uncontrolled disease. PtGA is significantly influenced by persistent pain and fatigue, which warrant better assessment in the future.Disclosure of Interests:None declared

Irritable Bowel Syndrome with Diarrhea

2018 ◽  
Author(s):  
Judy Nee ◽  
Jacqueline L. Wolf
Keyword(s):  
Irritable Bowel Syndrome ◽  
Differential Diagnosis ◽  
Abdominal Pain ◽  
Treatment Options ◽  
Clinical Manifestations ◽  
Signs And Symptoms ◽  
Treatment Modalities ◽  
Dietary Advice ◽  
Emerging Therapies ◽  
Irritable Bowel

Irritable bowel syndrome (IBS) is a complex, functional gastrointestinal condition characterized by abdominal pain and alteration in bowel habits without an organic cause. One of the subcategories of this disorder is IBS with diarrhea (IBS-D). Clinically, patients who present with more than 3 months of abdominal pain or discomfort associated with an increase in stool frequency and/or loose stool form are defined as having IBS-D. This review addresses IBS-D, detailing the epidemiology, etiology and genetics, pathophysiology and pathogenesis, diagnosis, clinical manifestations and physical examination findings, differential diagnosis, treatment, emerging therapies, complications, and prognosis. Figures show potential mechanisms and pathophysiology of IBS, IBS-D suspected by clinical assessment and Rome III criteria, pharmacologic and nonpharmacologic treatment options, potential mechanisms of action of probiotics, and potential treatment modalities. Tables list the Rome criteria for IBS, alarm signs and symptoms suggestive of alternative diagnoses, IBS criteria, differential diagnosis of IBS-D, dietary advice options for IBS-D, and alternative and emerging therapies in IBS-D. This review contains 5 figures, 6 tables and 42 references KEYWORDS: IBS-D, eluxadoline, rifaximin, probiotics, bloating, antidepressants, bile acid malabsorption, microscopic colitis, celiac

scholarly journals Ranking Self-reported Gastrointestinal Side Effects of Pharmacotherapy in Sarcoidosis

Lung ◽  
2020 ◽  
Vol 198 (2) ◽  
pp. 395-403 ◽  
Author(s):  
M. Drent ◽  
V. L. J. Proesmans ◽  
M. D. P. Elfferich ◽  
N. T. Jessurun ◽  
S. M. G. de Jong ◽  
...  
Keyword(s):  
Weight Gain ◽  
Side Effects ◽  
Side Effect ◽  
Treatment Options ◽  
Clinical Manifestations ◽  
Future Research ◽  
Dietary Interventions ◽  
Cross Sectional ◽  
Organ Systems ◽  
Gastrointestinal Side Effects

Abstract Background Clinical manifestations of sarcoidosis vary widely, depending on the intensity of the inflammation and the organ systems affected. So far, no curative treatment exists; the disease can only be suppressed. All treatment options cause side effects affecting quality of life. The aim of this study was to establish and rank the prevalence of self-reported gastrointestinal side effects of drugs used in the treatment of sarcoidosis. Methods A cross-sectional web-based anonymous survey about complaints and side effects was conducted among sarcoidosis patients in the Netherlands, United Kingdom, and United States of America. Results Of the participants, 70% were being treated with one or more drugs. The most important reported side effect was weight gain, associated with increased appetite among prednisone users (as monotherapy as well as in combination with other drugs). Methotrexate (MTX) users especially experienced nausea, with monotherapy as well as combination therapy. Vomiting and weight loss were most prominent among azathioprine and mycophenolate mofetil (MMF) users, whereas diarrhoea was frequently mentioned by MMF and MTX users. The reported side effects of hydroxychloroquine were generally rather mild. Conclusion The current study ranked the gastrointestinal side effects associated with pharmacotherapy in sarcoidosis patients. Pharmacotherapy does have multiple gastrointestinal side effects. The strongest association between a reported side effect and drug use was that of weight gain associated with increased appetite among prednisone users. It would therefore be useful for future research to look further into dietary interventions to counter these side effects and reduce their burden.

scholarly journals Could COVID-19 Trigger Presentation or Exacerbation of Vasculitides? (A 14-Year-Old Girl with Newly Diagnosed GPA After COVID-19 Infection)

2021 ◽  
Vol In Press (In Press) ◽  
Author(s):  
Seyed Reza Raeeskarami ◽  
Seyyed Hosein Mousavi ◽  
Mohammad Ashouri ◽  
Parvin Akbariasbagh ◽  
Raheleh Assari ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Pediatric Population ◽  
Multiple Organ ◽  
Newly Diagnosed ◽  
Gi Tract ◽  
Case Presentation ◽  
Anca Associated Vasculitis ◽  
Organ Systems ◽  
Necrotizing Glomerulonephritis ◽  
St Elevation

Introduction: Granulomatosis with polyangiitis (GPA) is a kind of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), which involves small-to-medium-sized vessels. Besides, GPA usually involves the upper and lower respiratory tract, and also causes necrotizing glomerulonephritis. The involvement of the heart and gastrointestinal (GI) tract in GPA is unusual, and these are atypical places of this vasculitis. Case Presentation: A 14-year-old girl with a newly diagnosed GPA after afflicting with COVID-19 infection presented with ST-elevation Myocardial Infarction (MI), GI perforation, and intracranial hemorrhage. Conclusions: Although GPA is rare in the pediatric population, it might occur in this age group and could involve multiple organ systems.

scholarly journals Hemophagocytic Lymphohistiocytosis: A Life Threatening Cytopenic Condition

2021 ◽  
Vol 15 (2) ◽  
pp. 98-102
Author(s):  
Suranjit Kumar Saha ◽  
MM Shahin Ul Islam ◽  
Nasir Uddin Ahmed ◽  
Prativa Saha
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Clinical Manifestations ◽  
Specific Treatment ◽  
Gene Mutations ◽  
Multiple Organ ◽  
Treatment Modalities ◽  
Hematopoietic Stem ◽  
Organ Systems ◽  
Life Threatening ◽  
Poor Outcomes

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder that occurs in many underlying conditions in all age. This is characterized by unbridled activation of cytotoxic T lymphocytes, natural killer (NK) cells and macrophages resulting in raised cytokine level. Those cytokines and immune mediated injury occur in multiple organ systems. It may be primary and secondary. Primary HLH is familial, childhood presentation and associated with gene mutations. Secondary HLH is acquired, adulthood presentation that occurs in infections, malignancies inflammatory and autoimmune diseases etc. Clinical manifestations include fever, splenomegaly, lymphadenopathy, neurologic dysfunction, coagulopathy, features of sepsis etc. Laboratory investigation includes cytopenias, hypertriglyceridemia, hyperferritinemia, abnormal liver function, hemophagocytosis, and diminished NKcell activity. Treatment modalities include immunosuppressive, immunomodulatory agents, cytostatic drugs, T-cell antibodies, anticytokine agents and hematopoietic stem cell transplantation (HSCT). Besides those, aggressive supportive care combined with specific treatment of the precipitating factor can produce better outcome. With treatment more than 50% of children who undergo transplant survive, but adults have quite poor outcomes even with aggressive management. Faridpur Med. Coll. J. 2020;15(2): 98-102

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