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 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

scholarly journals Eosinophilic granulomatosis with polyangiitis in a Nigerian woman

2019 ◽  
Vol 12 (6) ◽  
pp. e228901
Author(s):  
Ngozi Lina Ekeigwe ◽  
Olufemi Adelowo ◽  
Ehiaghe Lonia Anaba ◽  
Hakeem Olaosebikan
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Rare Condition ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Systemic Autoimmune Disease ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
American College Of Rheumatology ◽  
Nigerian Woman ◽  
History Of ◽  
Perinuclear Anca ◽  
Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss Syndrome, is a systemic autoimmune disease that is usually associated with asthma and eosinophilia. It is a rare condition associated with antineutrophil cytoplasmic antibodies (ANCA). We report a case of a 52-year-old Nigerian woman who presented with bilateral leg swelling with multiple ulcers, background history of allergic rhinitis and chronic sinusitis since adolescence. There were also associated symptoms of peripheral neuropathy, weight loss, peripheral blood eosinophilia and positive perinuclear ANCA and she was previously managed for rheumatoid arthritis and asthma. She fulfilled the American College of Rheumatology criteria for EGPA and was treated with glucocorticoids and methotrexate which led to significant improvement of her symptoms.

Childhood-onset Eosinophilic Granulomatosis with Polyangiitis (formerly Churg-Strauss Syndrome): A Contemporary Single-center Cohort

2013 ◽  
Vol 40 (6) ◽  
pp. 929-935 ◽  
Author(s):  
Samantha Gendelman ◽  
Andrew Zeft ◽  
Steven J. Spalding
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Upper Airway ◽  
Initial Therapy ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Childhood Onset ◽  
Churg Strauss Syndrome ◽  
Organ Systems ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

Objective.To date only 38 cases of childhood-onset eosinophilic granulomatosis with polyangiitis (cEGPA; formerly Churg-Strauss syndrome) have been reported. Additional patients with cEGPA could enhance the understanding of this rare and life-threatening condition. Our objectives were (1) to determine the frequency of specific organ system involvement; (2) to examine initial therapeutic regimen; and (3) to document disease and therapy-related morbidity in a contemporary cohort of patients with cEGPA.Methods.Retrospective review of patients evaluated at the Cleveland Clinic between 2003 and 2011 who met either American College of Rheumatology or Lanham criteria for EGPA and whose age was < 18 years at symptom onset.Results.Nine patients (8 female; 7 white) were identified. Median age at onset of rhinitis/asthma symptom was 13 years and median age at diagnosis of cEGPA was 15 years. All patients demonstrated eosinophilia, upper airway disease (allergic rhinitis, chronic sinusitis, and/or nasal polyps), and pulmonary involvement. Other frequently involved organ systems included musculoskeletal (67%), gastrointestinal (67%), cutaneous (67%), neurologic (56%), and cardiac (44%). Antineutrophil cytoplasmic antibody (ANCA) serologies were negative in all patients. The medications used most frequently for initial therapy included oral (44%) or intravenous corticosteroids (56%) and azathioprine (67%). Disease or therapeutic complications occurred in half of the cohort and included heart failure, stroke, and sequela from longterm, high-dose steroids.Conclusion.Eosinophilia, in combination with upper airway, pulmonary, musculoskeletal, neurologic, and cardiac manifestations, is frequently observed in cEGPA. ANCA titers are often negative. Steroids are the mainstay of initial therapy but steroid-related side effects occur regularly.

scholarly journals Addison’s disease presenting with perimyocarditis

2018 ◽  
Vol 31 (1) ◽  
pp. 101-105
Author(s):  
Elisa Baranski Lamback ◽  
Grazia Morandi ◽  
Eleni Rapti ◽  
Georgi Christov ◽  
Paul A. Brogan ◽  
...  
Keyword(s):  
Systemic Inflammation ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Manifestations ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Polyglandular Autoimmune Syndrome ◽  
Case Presentation ◽  
Prompt Treatment ◽  
Autoimmune Syndrome ◽  
Life Saving ◽  
Eosinophilic Granulomatosis

AbstractBackground:Polyglandular autoimmune syndrome (PGA) and eosinophilic granulomatosis with polyangiitis (EGPA) do not seem to represent a coincidental association.Case presentation:A case of a 15-year-old boy is reported who presented with severe systemic inflammation, perimyocarditis and cardiogenic shock, in whom EGPA was initially suspected and later diagnosed with autoimmune adrenalitis with PGA.Conclusions:The severity of the systemic inflammation and perimyocarditis suggests a more widespread autoimmune-mediated process. Autoimmune adrenal insufficiency should be considered in all cases of pericarditis and perimyocarditis, especially when the severity of clinical manifestations exceeds the expected for the severity of the cardiac findings, as timely identification and prompt treatment may be life-saving.

scholarly journals Safety and Efficacy of Mepolizumab in Patients with Eosinophilic Granulomatosis with Polyangiitis

2019 ◽  
Vol 2019 ◽  
pp. 1-6 ◽  
Author(s):  
Ravi Ranjan Pradhan ◽  
Gaurav Nepal ◽  
Shobha Mandal
Keyword(s):  
Side Effects ◽  
Granulomatosis With Polyangiitis ◽  
Multiple Organ ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Sustained Remission ◽  
Organ Systems ◽  
Frequent Relapses ◽  
Systemic Glucocorticoids ◽  
Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of vasculitis disorder which involves multiple organ systems and is characterized by asthma, pulmonary infiltrates, sinusitis, neuropathy, and peripheral eosinophilia. It also has an effect on the heart, skin, kidneys, and gastrointestinal tract. Interlukin-5 (IL-5) is involved in maturation and activation of eosinophil, the production of which is increased in the EGPA. Treatments of EGPA are limited to systemic corticosteroids and immunomodulators. These drugs are associated with significant side effects. Besides this, the response of patients to these drugs may be disappointing. Frequent relapses, the need for long-term medium-to-high-dose glucocorticoid therapy, and failure to achieve remission are not uncommon findings. There is a need for noble agents that could reduce frequent relapses and the dose of systemic glucocorticoids and maintain a sustained remission without significant side effects. Mepolizumab is IL-5 antagonist and may have value in treating patients with EGPA. Therefore, we did a systematic review to evaluate the efficacy and safety of mepolizumab in patients with EGPA.

ANCA-associated vasculitis

2020 ◽  
pp. 4556-4569
Author(s):  
David Jayne
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Multiple Organ ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Histological Lesion ◽  
Considerable Heterogeneity ◽  
Organ Systems ◽  
Strauss Syndrome ◽  
Acute And Chronic Inflammation ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

The ANCA-associated vasculitides (AAV) are a grouping of three syndromes of acute and chronic inflammation characterized by their clinical and histological phenotypes, which are associated with circulating antineutrophil cytoplasm autoantibodies (ANCA). They comprise: granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis); microscopic polyangiitis (MPA); and eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome). The defining histological lesion is a microscopic vasculitis affecting arterioles, capillaries, or venules associated with few or no deposits of immunoglobulin or complement. Granulomata, involving or close by blood vessels, are commonly present in GPA. These diseases involve multiple organ systems with considerable heterogeneity in extent and severity of organ involvement between patients, and overlapping clinical and histological features between syndromes.

scholarly journals Pulmonary Vasculitides: A Radiological Review Emphasizing Parenchymal HRCT Features

Diagnostics ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. 2318
Author(s):  
Stefano Palmucci ◽  
Corrado Inì ◽  
Salvatore Cosentino ◽  
Luigi Fanzone ◽  
Stefano Di Pietro ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Case Series ◽  
Interstitial Lung Diseases ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Immune Mediated ◽  
Antineutrophil Cytoplasmic Autoantibody ◽  
Interstitial Disease ◽  
Eosinophilic Granulomatosis

Vasculitides represent a heterogeneous group of immune-mediated disorders, characterized by a systemic inflammatory destructive process of the blood vessels resulting either in ischemia or hemorrhage. The organ involved and vessel size influence the pattern of presentation of the pathology. The lung is commonly involved in systemic vasculitides, with heterogeneous clinical, radiological, and histopathological presentations. Primary vasculitides most commonly associated with lung parenchymal involvement include small-vessel antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides, such as granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). Several studies have reported cases of interstitial lung diseases (ILDs) associated with systemic vasculitis, particularly those positive for ANCA associated vasculitis/vasculitidis: AAV. We have selected from our case series different radiological features of pulmonary vasculitis (i.e., solitary or multiple nodules, cavitary lesions, nodules with centrilobular or peribronchial distribution, airspace consolidations, “crazy paving” appearance, interstitial disease), including cases with interstitial lung alterations. Therefore, the aim of this review is to describe the typical clinical manifestations of vasculitides and their main radiologic features (especially AAV).

scholarly journals Peripheral Neuropathy in ANCA Vasculitis

2021 ◽  
Author(s):  
Mouna Snoussi ◽  
Faten Frikha ◽  
Zouhir Bahloul
Keyword(s):  
Peripheral Neuropathy ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Vasculitis ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

Antineutrophil cytoplasmic antibodies (ANCA)-associated diseases are necrotizing systemic vasculitides that affect small blood vessels (arterioles, capillaries and venules). This entity represents three main systemic vasculitides: granulomatosis with polyangiitis (GPA; formerly Wegener’s granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss’ syndrome). Their clinical manifestations are polymorphous, being the most frequent respiratory, oto-laryngo-pharyngeal and renal involvement. Peripheral neuropathy (PN) is reported in almost 50% of the patients. The aim of this chapter is to discuss the prevalence, clinical presentation, treatment and prognosis of PN in ANCA-associated vasculitis.

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