scholarly journals Clinical Characteristics of an Internet-Based Cohort of Patient-Reported Diagnosis of Granulomatosis With Polyangiitis and Microscopic Polyangiitis: Observational Study

10.2196/17231 ◽  
2020 ◽  
Vol 22 (7) ◽  
pp. e17231
Author(s):  
Jason Michael Springer ◽  
Tanaz A Kermani ◽  
Antoine Sreih ◽  
Dianne G Shaw ◽  
Kalen Young ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Clinical Characteristics ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Renal Involvement ◽  
Diagnostic Testing ◽  
Research Network ◽  
Joint Involvement ◽  
Patient Reported ◽  
Prospective Longitudinal

Background Utilizing the traditional centers of excellence approach to conduct clinical trials involving rare diseases remains challenging. Patient-based registries have been shown to be both feasible and valid in several other diseases. Objective This report outlines the clinical characteristics of a large internet registry cohort of participants with a self-reported diagnosis of granulomatosis with polyangiitis or microscopic polyangiitis. Methods Patients with a self-reported diagnosis of granulomatosis with polyangiitis or microscopic polyangiitis in an internet-based prospective longitudinal cohort (from the Vasculitis Patient-Powered Research Network) were included. Data on symptoms, diagnostic testing, and treatment were collected using standardized questionnaires. Results The study compared patients with granulomatosis with polyangiitis (n=762) and patients with microscopic polyangiitis (n=164). Of the cohort, 97.7% (904/925) reported the diagnosis had been confirmed by a physician. Compared to microscopic polyangiitis, patients with granulomatosis with polyangiitis reported significantly more ear, nose, and throat manifestations (granulomatosis with polyangiitis: 641/723, 88.7%; microscopic polyangiitis: 89/164, 54.3%; z=10.42, P<.001), fevers (granulomatosis with polyangiitis: 325/588, 55.3%; microscopic polyangiitis: 64/139, 46.0%; z=1.96, P=.05), joint involvement (granulomatosis with polyangiitis: 549/688, 79.8%; microscopic polyangiitis: 106/154, 68.8%; z=2.96, P=.003), and pulmonary involvement (granulomatosis with polyangiitis: 523/734, 71.3%; microscopic polyangiitis: 90/154, 58.4%; z=3.13, P=.002). Compared to microscopic polyangiitis, patients with granulomatosis with polyangiitis reported significantly less renal involvement (granulomatosis with polyangiitis: 457/743, 61.5%; microscopic polyangiitis: 135/163, 82.8%; z=–5.18, P<.001) and renal transplantation (granulomatosis with polyangiitis: 10/721, 1.4%; microscopic polyangiitis: 7/164, 4.3%; z=–2.43, P=.02). Antineutrophil cytoplasmic antibody positivity was reported in 94.2% (652/692) of patients with granulomatosis with polyangiitis and 96.1% (147/153) of patients with microscopic polyangiitis. A biopsy showing vasculitis was reported in 77.0% (562/730) of patients with granulomatosis with polyangiitis and 81.9% (131/160) of patients with microscopic polyangiitis. Conclusions In this large, internet-based cohort of patients with a self-reported diagnosis of granulomatosis with polyangiitis or microscopic polyangiitis, disease manifestations were consistent with expectations for each type of vasculitis. Given the rarity of these and other vasculitides, conducting some types of research through internet-based registries may provide an efficient alternative to inperson, center-of-excellence clinical trials.

scholarly journals Clinical Characteristics of an Internet-Based Cohort of Patient-Reported Diagnosis of Granulomatosis With Polyangiitis and Microscopic Polyangiitis: Observational Study (Preprint)

2020 ◽  
Author(s):  
Jason Michael Springer ◽  
Tanaz A Kermani ◽  
Antoine Sreih ◽  
Dianne G Shaw ◽  
Kalen Young ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Clinical Characteristics ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Renal Involvement ◽  
Diagnostic Testing ◽  
Research Network ◽  
Joint Involvement ◽  
Patient Reported ◽  
Prospective Longitudinal

BACKGROUND Utilizing the traditional centers of excellence approach to conduct clinical trials involving rare diseases remains challenging. Patient-based registries have been shown to be both feasible and valid in several other diseases. OBJECTIVE This report outlines the clinical characteristics of a large internet registry cohort of participants with a self-reported diagnosis of granulomatosis with polyangiitis or microscopic polyangiitis. METHODS Patients with a self-reported diagnosis of granulomatosis with polyangiitis or microscopic polyangiitis in an internet-based prospective longitudinal cohort (from the Vasculitis Patient-Powered Research Network) were included. Data on symptoms, diagnostic testing, and treatment were collected using standardized questionnaires. RESULTS The study compared patients with granulomatosis with polyangiitis (n=762) and patients with microscopic polyangiitis (n=164). Of the cohort, 97.7% (904/925) reported the diagnosis had been confirmed by a physician. Compared to microscopic polyangiitis, patients with granulomatosis with polyangiitis reported significantly more ear, nose, and throat manifestations (granulomatosis with polyangiitis: 641/723, 88.7%; microscopic polyangiitis: 89/164, 54.3%; <i>z</i>=10.42, <i>P</i>&lt;.001), fevers (granulomatosis with polyangiitis: 325/588, 55.3%; microscopic polyangiitis: 64/139, 46.0%; <i>z</i>=1.96, <i>P</i>=.05), joint involvement (granulomatosis with polyangiitis: 549/688, 79.8%; microscopic polyangiitis: 106/154, 68.8%; <i>z</i>=2.96, <i>P</i>=.003), and pulmonary involvement (granulomatosis with polyangiitis: 523/734, 71.3%; microscopic polyangiitis: 90/154, 58.4%; <i>z</i>=3.13, <i>P</i>=.002). Compared to microscopic polyangiitis, patients with granulomatosis with polyangiitis reported significantly less renal involvement (granulomatosis with polyangiitis: 457/743, 61.5%; microscopic polyangiitis: 135/163, 82.8%; <i>z</i>=–5.18, <i>P</i>&lt;.001) and renal transplantation (granulomatosis with polyangiitis: 10/721, 1.4%; microscopic polyangiitis: 7/164, 4.3%; <i>z</i>=–2.43, <i>P</i>=.02). Antineutrophil cytoplasmic antibody positivity was reported in 94.2% (652/692) of patients with granulomatosis with polyangiitis and 96.1% (147/153) of patients with microscopic polyangiitis. A biopsy showing vasculitis was reported in 77.0% (562/730) of patients with granulomatosis with polyangiitis and 81.9% (131/160) of patients with microscopic polyangiitis. CONCLUSIONS In this large, internet-based cohort of patients with a self-reported diagnosis of granulomatosis with polyangiitis or microscopic polyangiitis, disease manifestations were consistent with expectations for each type of vasculitis. Given the rarity of these and other vasculitides, conducting some types of research through internet-based registries may provide an efficient alternative to inperson, center-of-excellence clinical trials.

scholarly journals CLINICAL AND LABORATORY FEATURES OF ANCA ASSOCIATED VASCULITIS: EXPERIENCE AT A TERTIARY CARE HOSPITAL IN LAHORE, PAKISTAN

2021 ◽  
Vol 71 (1) ◽  
pp. 29-33
Author(s):  
Muhammad Zeeshan Aslam ◽  
Asadullah Kakkar ◽  
Haseeb Ahmed Khan ◽  
Saira Elaine Anwar Khan
Keyword(s):  
Respiratory Symptoms ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Renal Involvement ◽  
Tertiary Care ◽  
Care Hospital ◽  
X Rays ◽  
Neurologic Manifestation ◽  
Anca Associated Vasculitis ◽  
Laboratory Features

Objective: To determine the clinical and laboratory features during the disease course in patients of anticytoplasmic antibody (ANCA) associated vasculitis in Pakistani patients presenting to a tertiary care center. Study Design: Case series. Place and Duration of Study: Fatima Memorial Hospital Shadman Lahore, from Dec 2018 to Mar 2019. Methodology: A collection of 20 patients regarding demographic data, constitutional symptoms, mucocutaneous symptoms and signs, upper respiratory symptoms, lower respiratory symptoms, orbital and ocular manifestation, cardiovascular, peripheral vascular manifestation, central and peripheral neurologic manifestation, abdominal manifestation and renal manifestation Results: Sixteen patients (80%) had a diagnosis of Granulomatosis with Polyangiitis, and 4 patients (20%) wereof microscopic polyangiitis. The most common systemic involvement in descending order were constitutionalsymptoms (75%), ear nose and throat symptoms (50%), renal (50%), respiratory (45%), ocular (40%) and neurologic (40%). Most common laboratory abnormalities in our patients included leukocytosis (45%), anemia (35%), hematuria (50%), proteinuria (45%), and elevated serum creatinine (45%). Cytoplasmic-anti cytoplasmic antibody (C-ANCA) was positive in 11 (55%), all cases were of granulomatosis with polyangiitis, P-ANCA was positive in 5 (25%) of all patients, with 4 (100%) in Microscopic polyangiitis. Analysis of Granulomatosis with Polyangiitis according to gender and cytoplasmic-anti cytoplasmic antibody status showed correlation of renal involvement with cytoplasmic-anti cytoplasmic antibody status with statistical significance of p=0.036. Plain chest X-rays showed infiltrates in 2 (10%), nodularity 2 (10%), cavitation in 2 (10%), effusion in 1 (5%), and reticulonodular showing in 1 (5%) patients. High-resolution computed tomography findings included ground-glass opacification in 5............

Elucidation of qualitative patient-reported outcome (PRO) data using latent dirichlet allocation (LDA) in patients (pts) undergoing radical cystectomy.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e18245-e18245
Author(s):  
Thomas Michael Atkinson ◽  
Bruce D. Rapkin ◽  
Elizabeth Schofield ◽  
Bernard H. Bochner ◽  
Yuelin Li
Keyword(s):  
Decision Making ◽  
Clinical Trials ◽  
Radical Cystectomy ◽  
Clinical Decision Making ◽  
Routine Care ◽  
Free Text ◽  
Post Surgery ◽  
Patient Reported ◽  
Latent Topics ◽  
Prospective Longitudinal

e18245 Background: As PROs become commonplace in clinical trials and routine care, we must identify efficient analytic methods to better understand and ultimately integrate this information into clinical decision-making. While PROs are often captured via qualitative methods to provide context and pt voice to traditional objective and/or quantitative assessments, the current analytic standard is resource intensive and impractical for timely delivery of this information to clinicians and trialists. To address this problem, we propose the use of LDA, a natural language processing technique that automates the distillation of vast amounts of free-text data into underlying topics. Methods: As part of a prospective longitudinal trial of pts ( N = 200; M age = 63.8; 20% female; 46%) undergoing radical cystectomy (52% stage T2-T4) and urinary diversion (49% continent) for bladder cancer (ClinicalTrials.gov identifier NCT00745355), pts completed 30 minute interviews at baseline and 6-months post-surgery where they were asked to characterize their quality of life with respect to goals or milestones they would like to reach, problems they would like to solve, situations they would like to prevent, things they would like to remain the same, and commitments they would be willing to let go. LDA was used to extract latent topics and themes, stratified by time. Results: LDA extracted 7 latent topics and showed shifting patient priorities. At baseline, pt primary concerns were related to surgery and recovery. Six months post-surgery, pt goals transitioned toward themes related to concerns about cancer recurrence, regaining a sense of normalcy, return to work, enjoyment of life and increased appreciation of friends and family. Conclusions: LDA was used as an automated tool to illustrate a shift in pt goals and concerns as they transition into survivorship. This novel data analytic technique offers the possibility of significantly reducing the resources required to summarize qualitative PRO data. As such, this information may now be more readily available for immediate inclusion in the decision-making process as part of routine care and clinical trials. Clinical trial information: NCT00745355.

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 POS0823 ARTHRITIS IN ADULT IGA VASCULITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 664.2-665
Author(s):  
A. Hočevar ◽  
V. Jurčić ◽  
M. Tomsic ◽  
Z. Rotar
Keyword(s):  
Gastrointestinal Tract ◽  
Clinical Characteristics ◽  
Renal Involvement ◽  
Elevated Serum ◽  
Joint Involvement ◽  
P Value ◽  
Iga Vasculitis ◽  
Upper And Lower Extremities ◽  
Tract Involvement ◽  
Metatarsophalangeal Joints

Background:Arthritis is one of the main clinical features of IgA vasculitis (IgAV). Joint involvement represents the second most common manifestation in childhood IgAV with the predilection for the knees and ankles.Objectives:We aimed to describe the characteristics of arthritis in adult IgAV cohort.Methods:We analysed medical records of histologically proven adult IgAV cases, diagnosed between January 2010 and December 2020 at our secondary/tertiary rheumatology centre. The frequency, temporal occurrence and the localization of arthritis was recorded. In addition, we searched for potential differences in other IgAV features between patients with and without arthritis.Results:During the 132-month observation period we identified 328 new IgAV cases (59.5% males, median (IQR) age 64.3 (45.1; 76.1) years). Forty-eight (14.6%) patients developed arthritis. Arthritis was the first IgAV manifestation in 16 (4.9%) patients. Arthritis was mono-, oligo- and poly- articular (involving up to 15 joints) in 13 (4.0%), 25 (7.6%) and 10 (3.0%) patients, respectively. Arthritis was most common in wrists and ankles (each in 18 (37.5%) patients); metacarpophalangeal joints and knees (each in 11 (22.9%)); proximal interphalangeal joints (9 (18.8%)); elbows (8 (16.7%)) and metatarsophalangeal joints (4 (8.3%)). Clinical differences between IgAV patients with and without arthritis are presented in table 1. Patients with arthritis were significantly younger, more commonly developed gastrointestinal tract involvement compared to those without arthritis. Arthritis remitted in all with immunomodulatory treatment (given predominantly for necrotic skin purpura or visceral involvement). Follow up (FU) data accessible for 42/48 (87.5%) patients with arthritis showed that IgAV relapsed in 10 (23.8%) patients during a median (IQR) 24.5 (12.9; 40.7) month FU. Relapses were limited to skin and/or kidneys, there were no relapses of arthritis.Table 1.Clinical characteristics of IgA vasculitis patients with and without arthritisClinical characteristicsArthritis IgAV(48)Non-arthritis IgAV(280)P valueMale gender (%)56.360.00.636Age (years)*49.9 (36.1-66.9)65.4 (48.4;77.5)<0.001Ever smoker (%)50.043.60.435Prior infection (%)43.830.40.094Generalized purpura50.050.01.0Skin necroses (%)35.449.60.085GI involvement (%)47.925.00.002Renal involvement (%)47.946.81.0Elevated serum IgA (%)33.3 (11/33)52.0 (115/221)0.061Legend: * median and IQR; GI gastrointestinal;Conclusion:Arthritis in adult IgAV was frequently oligoarticular, involved large and small joints of both upper and lower extremities, and was not prone to chronic course and recurrence. In addition, we found an association between arthritis and gastrointestinal tract involvement.Disclosure of Interests:None declared

scholarly journals Clinical Characteristics and Outcome of ANCA-Associated Vasculitis; Experience of A Single Reference Vasculitis Center

Acta Medica ◽  
2020 ◽  
Vol 51 (4) ◽  
pp. 22-32
Author(s):  
İlim Irmak ◽  
Silam Bas ◽  
Maide Gözde Inam ◽  
Fatih Tekin ◽  
Berkay Yesilyurt ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Clinical Characteristics ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Demographic Data ◽  
Pulmonary Involvement ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Proteinase 3 ◽  
Thoracic Computed Tomography

Objective: The aim of the study is to describe the clinical characteristics of Antineutrophil cytoplasmic antibodies-associated vasculitis and to analyze the parameters affecting the outcome. Materials and Methods: The study is a retrospective cohort study. Totally 130 patients with Antineutrophil cytoplasmic antibodies-associated vasculitis (18 years and over) who were followed up between October 2014 and October 2019 were analyzed. Demographic data, laboratory values, clinical course, thorax computed tomography findings and treatment approaches were noted from the charts of patients. Patients were divided into two groups as those with pulmonary involvement and non-pulmonary involvement. Results: We retrospectively reviewed the medical records of 130 patients with Antineutrophil cytoplasmic antibodies-associated vasculitis; 111 with granulomatosis with polyangiitis, 15 with microscopic polyangiitis, 1 with eosinophilic granulomatosis with polyangiitis, and 3 with other types of vasculitis. The ratio of having the abnormality in thoracic computed tomography was 72.2%. There were 84 cases with pulmonary involvement and 46 cases with non-pulmonary involvement. The frequency of microscopic polyangiitis was significantly higher (p=0.034) in non-pulmonary involvement cases. There were 67 cases with proteinase 3 Antineutrophil cytoplasmic antibodies and 39 cases with myeloperoxidase Antineutrophil cytoplasmic antibodies positivity. Most of the cases with proteinase 3 Antineutrophil cytoplasmic antibodies positivity were classified as granulomatosis with polyangiitis, this was statistically significant. Recovery was referenced for the outcome. Any of the variables were found statistically significant effective on outcome. Conclusions: Cases with pulmonary involvement were more than the cases without pulmonary involvement in our study. microscopic polyangiitis was significantly higher in non- pulmonary involvement cases. We studied on a large group, and these significant findings may have important implications for the investigation, pathogenesis, and treatment of Antineutrophil cytoplasmic antibodies-associated vasculitis.

scholarly journals Use of Rituximab for the Treatment of Antineutrophil Cytoplasm Antibody–associated Vasculitis in Canada, 2010–2020

2021 ◽  
pp. jrheum.210537
Author(s):  
Arielle Mendel ◽  
Christian Pagnoux
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Research Network ◽  
Remission Induction ◽  
First Line ◽  
First Line Therapy ◽  
Anca Associated Vasculitis ◽  
Line Therapy

Rituximab (RTX) is the only biologic approved to date for the treatment of antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV).1,2,3 The Canadian Vasculitis Research Network (CanVasc) has recommended RTX as first-line therapy for remission induction,4 and recently, as the preferred maintenance agent in severe granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).5

scholarly journals A case report of microscopic polyangiitis presenting as diffuse pulmonary symptoms with no renal involvement

2020 ◽  
Vol 8 (4) ◽  
pp. 1578
Author(s):  
Shreyans D. Singhvi ◽  
Preksha Singh ◽  
Rafe Khan ◽  
Monila Patel ◽  
Pavan Acharya
Keyword(s):  
Case Report ◽  
Renal Dysfunction ◽  
Microscopic Polyangiitis ◽  
Systemic Disease ◽  
Renal Involvement ◽  
Rapidly Progressive Glomerulonephritis ◽  
Pulmonary Hemorrhage ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Immune Disorder ◽  
Patient Reported

Microscopic polyangiitis (MPA) is a small blood vascular disorder, but with presence of no granulomas. This is a pauci- immune disorder vessels which lead to vascular inflammation and then later, it can lead to organ microscopic polyangiitis. This disorder affects multiple organs as it is a systemic disease, but usually presents with renal dysfunction - rapidly progressive glomerulonephritis. There is a possible association of p- ANCA (antineutrophil cytoplasmic antibodies) and can help with the diagnosis of the disorder. This case report is of a 70-year-old female, who presented with symptoms of diffuse pulmonary hemorrhage. After routine blood examination, radiological examination- X ray (chest) and HRCT- thorax, routine urine examination, ANA and ANCA test, the final diagnosis of microscopic polyangiitis with no renal involvement was made. The case was given immunosuppressant therapy and the patient reported of mitigation of symptoms. This case report is unique as MPA usually presents with renal dysfunction, but in this case, the patient had particularly pulmonary involvement with completely normal renal function. This case report will help future physicians for quick diagnosis of similar cases and treatment for the same.

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