Challenges in diagnosis of limited granulomatosis with polyangiitis

Zimba, Olena; Doskaliuk, Bohdana; Yatsyshyn, Roman; Bahrii, Mykola; Hrytsevych, Marta

doi:10.1007/s00296-021-04858-8

Cited by 10 publications

(7 citation statements)

References 43 publications

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“…Of them, 54 (51.4%) were males and 51 (48.6%) were females (8 chose not to specify their gender). The median number of years in medical practice was 13 (6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20). Most common among the responders were rheumatologists (72 [63.7%]), internal medicine specialists (14 [12.4%]), and general practitioners (8 [7.08%]).…”

Section: Resultsmentioning

confidence: 99%

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Physicians’ perceptions about antineutrophil cytoplasmic antibody-associated vasculitis: an online survey report in the time of the COVID-19 pandemic

et al. 2022

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Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are characterized by necrotizing inflammation of small and medium-size vessels that often manifest with devastating multi-organ effects. They present with a myriad of systemic features and require potent immunosuppression. Since they are uncommonly encountered in clinical practice, it is necessary to understand physicians’ knowledge and perceptions about this group of diseases. An online questionnaire was designed featuring 28 questions based on relevant global practice guidelines, recommendations, and previous online surveys on AAV. The questionnaire was validated by a core group of specialists with an interest in AAV. It was shared via social networking sites and entries were restricted to physicians. Only completed entries were analyzed with descriptive statistics. A total of 113 respondents from 21 different countries responded of whom the commonest were rheumatologists, internists, and general practitioners. Forty-five (40%) ran clinics dedicated to AAV patients as a part of their practice. They commented on organs involved in AAV; vasculitis secondary to infections, drugs or other rheumatic diseases; various tests useful for AAV diagnosis; and drug choices for induction and maintenance. They mentioned their experience regarding COVID-19 in AAV patients as well as vasculitic manifestations of COVID-19. Various methods to mitigate cardiovascular risks in AAV were mentioned. Finally, the respondents indicated how medical education needed to be strengthened to increase awareness and knowledge regarding AAV. This survey helped to inform about various perceptions regarding AAV across countries, including current practices and recent evolution of management. It also provided information on treatment of the COVID-19 in AAV patients. This survey showed that there is still a lack in understanding the prevalent definitions and there is gap between guidelines and current practice. Key Points • Perception about ANCA-associated vasculitis differ across countries. • The number of cases encountered across 21 different countries are limited implying a need for multi-national cooperation to study this disease further. • The COVID-19 pandemic has changed the approach towards ANCA-associated vasculitis by the various clinicians. Supplementary Information The online version contains supplementary material available at 10.1007/s10067-022-06452-0.

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Section: Resultsmentioning

confidence: 99%

“…This may be a reason that less than 50% of the respondents could identify all the organs that can be potentially affected by AAV. As a result, clinicians may underdiagnose organ-limited AAV [ 17 ]. Also, the number of respondents who know about vasculitides secondary to drugs, infection, or autoimmune diseases is less than optimal.…”

Section: Discussionmentioning

confidence: 99%

Physicians’ perceptions about antineutrophil cytoplasmic antibody-associated vasculitis: an online survey report in the time of the COVID-19 pandemic

et al. 2022

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“…Recent studies have suggested impaired immune responses to COVID-19 vaccine when rituximab, MMF and methotrexate are used, which needs to be considered and may require modification [17]. All rheumatic diseases have a different prognosis, varied responses to immunosuppression, especially severe untreated AAV has very high mortality [18][19][20], hence withholding IST treatment in the context of COVID-19 needs to balanced weighing the risk versus benefit ratio. Interestingly, about a third of physicians wanted to test the patient for COVID-19 before initiating therapy in organ/lifethreatening manifestations.…”

Section: Discussionmentioning

confidence: 99%

Managing ANCA-associated vasculitis during the COVID-19 pandemic: results from an online survey

et al. 2021

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Management of ANCA-associated vasculitis (AAV) during the COVID-19 pandemic poses unique therapeutic challenges. An online survey was conducted to understand physician’s choices for treating AAV during the COVID-19 pandemic. Web-based survey featuring nineteen questions was circulated amongst physicians across various specialties. The responses regarding immunosuppressive therapy for remission induction and maintenance, COVID-19 testing, and preventive measures were recorded. A total of 304 responses were recorded. Most of the respondents were from India (83.9%) and comprised rheumatologists (66%) in practice for ≥ 5 years (71%). Though a majority preferred Rituximab or intravenous cyclophosphamide (CYC) as a remission induction agent, a significant proportion opted for oral CYC and mycophenolate mofetil (MMF) also. Only one-third wanted to test for COVID-19 before initiating immunosuppressive therapy in patients with organ/life-threatening manifestations. Rituximab was the most favored maintenance therapy (47%), followed by azathioprine, MMF, and methotrexate. The results of this focused survey of managing AAV patients depict the real-world dilemmas and physicians’ choices in this setting.

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“…After institutional ethics approval, we recruited 254 patients (age > 20 years) diagnosed with GPA and were under treatment at Alzahra Hospital affiliated to Isfahan University of Medical Sciences, Isfahan, Iran (from 2013 to 2020) and Amir Alam Hospital affiliated to Tehran University of Medical Sciences, Tehran, Iran (from 2020 to 2022) to plan a retrospective multi-center study. The diagnosis was established based on American College of Rheumatology and Revised International Chapel Hill Consensus criteria 2012 for the classification of GPA [32][33][34]. Inclusion criteria consisted of patients diagnosed as GPA using the criteria we mentioned earlier, who were followed more than 12 months.…”

Section: Methodsmentioning

confidence: 99%

Predictors of relapse in granulomatosis with polyangiitis: a multi-center study

Safari

Alesaeidi²,

Pakzad³

et al. 2022

Egypt Rheumatol Rehabil

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Background Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis. Its severity ranges from indolent disease to fulminant that may cause death. With treatment, remission is seen in more than 80% of cases, although relapse is still common. There have been studies showing that there may be factors to predict relapse in GPA. Based on relapses, the decision to start treatment and/or to monitor the patients more closely is made. Therefore, predicting the relapse of GPA can be effective in controlling the disease. Our aim was to investigate possible factors for relapse in GPA. We recruited 254 patients diagnosed with GPA who were under treatment at Alzahra hospital affiliated to Isfahan University of Medical Sciences (from 2013 to 2020) and Amir Alam Hospital affiliated to Tehran University of Medical Sciences (from 2020 to 2022) to plan a retrospective study. Chi-squared or Fisher’s exact tests were performed to compare categorical variables, while the Mann–Whitney U test was used to compare continuous variables. Results Analysis of our patients (aged 20–80,122 females) showed that 147 patients (57.9%) experienced relapse. Mean age in relapse group was 43.8 ± 16.6 and in no-relapse group was 45.6 ± 15.1 (P > 0.05). Among many potential predictors, we observed in multivariate analysis that positive PR3-ANCA (proteinase 3-antineutrophilic cytoplasmic antibodies) (P = 0.007, OR:2.62,CI:1.29–5.31),nose manifestations (P = 0.004, OR 3.00, CI 1.43–6.26), mucosal membranes involvement (P = 0.009, OR 4.21, CI 1.43–12.38), and gastrointestinal tract (GI) complications (P = 0.03, OR 5.64, CI 1.14–27.90) were significant predictors of GPA relapse. Conclusion Among clinical and laboratory features we studied, positive PR3-ANCA, nose manifestations, mucosal membranes involvement, and GI complications were independent predictors of relapse in patients with GPA.

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Challenges in diagnosis of limited granulomatosis with polyangiitis

Cited by 10 publications

References 43 publications

Physicians’ perceptions about antineutrophil cytoplasmic antibody-associated vasculitis: an online survey report in the time of the COVID-19 pandemic

Physicians’ perceptions about antineutrophil cytoplasmic antibody-associated vasculitis: an online survey report in the time of the COVID-19 pandemic

Managing ANCA-associated vasculitis during the COVID-19 pandemic: results from an online survey

Predictors of relapse in granulomatosis with polyangiitis: a multi-center study

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