Open research questions in Vasculitis and related conditions
398 gap statements mined from Vasculitis and related conditions papers in our 7.9M-paper local library, which holds 1,881 papers on the topic — drawn mostly from each paper's own stated research gap, future-work, challenge and limitation notes, and its abstract. The ones listed below are a selection still marked open; each names the study that raised it, with a DOI link where the paper has one.
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What the literature leaves open
The study faces challenges in interpreting the results due to the limited number of cases and the potential for bias in the included case reports. The paper notes that some clinical, laboratory, therapeutic, and prognostic features seemed more frequent in patients with different LTRA types, Masi-Hamilos categories, steroid-tapering status, and ANCA positivity.
Eosinophilic Granulomatosis with Polyangiitis Cases Associated with Leukotriene Receptor Antagonist Use with a Focus on Drug Type, Temporal Drug–Disease Relationship, Steroid-Tapering Status, and Antineutrophil Cytoplasmic Antibody Positivity: A Systemati · 2026 · DOIThere is a lack of understanding of the association between LTRA use and EGPA cases. The clinical features of LTRA-associated EGPA cases are not well characterized.
Eosinophilic Granulomatosis with Polyangiitis Cases Associated with Leukotriene Receptor Antagonist Use with a Focus on Drug Type, Temporal Drug–Disease Relationship, Steroid-Tapering Status, and Antineutrophil Cytoplasmic Antibody Positivity: A Systemati · 2026 · DOIThe current evidence for infectious triggers of IgA vasculitis in children is limited. There is a need to distinguish epidemiological associations from incidental detection and infectious mimics. The immunopathogenic mechanisms of IgAV are not fully understood.
Infectious triggers in IgA vasculitis in children: current evidence, causal attribution and immunopathogenic mechanisms · 2026 · DOIThe study was a cross-sectional cohort, and long-term progression to renal failure was not evaluated. The study did not investigate the effects of hUC-MSCs on other immune cells or pathways. The study used a limited number of patients and healthy controls. The study did not evaluate the optimal dosage and treatment duration of hUC-MSCs.
hUC-MSCs regulating the balance of Th17/Treg in Henoch Schönlein purpura via STAT3 pathway · 2026 · DOIOne challenge is the heterogeneity in disease presentation, which makes it difficult to identify patients at high risk. Another challenge is the need for simple biomarkers that can facilitate early risk stratification. A technical challenge is the use of inverse probability weighting to adjust for baseline disease severity and treatment intensity.
Pretreatment neutrophil-to-lymphocyte ratio is associated with clinical phenotypes and infection-related mortality in microscopic polyangiitis: the multicentre REVEAL cohort study · 2026 · DOIOne of the challenges identified is the complexity of ANCA-associated vasculitides, which makes management challenging. Another challenge is the need for individualized treatment approaches, which requires a better understanding of the disease. The editorial also highlights the challenge of determining which biological signals meaningfully change clinical decisions and improve outcomes.
Editorial: Comprehensive insights into ANCA vasculitis: from immunopathology to treatment strategies · 2026 · DOIThe authors address the concern of limited histopathological confirmation. The research highlights the challenge of differential diagnosis from idiopathic inflammatory myopathy.
Muscle symptoms and MRI findings in newly diagnosed ANCA-associated vasculitis: implications for muscle-dominant ANCA-associated vasculitis · 2026 · DOIThe 2017 guideline-recommended gating strategy (RGS) for antineutrophil cytoplasmic antibody (ANCA) testing for ANCA-associated vasculitis (AAV) diagnosis has not been evaluated in Australia, and the optimal gating strategy remains unclear.
A classification criteria-based gating strategy improves anti-neutrophil cytoplasmic antibody (ANCA) testing performance characteristics for the diagnosis of ANCA-associated vasculitis · 2026 · DOIReliable tools for monitoring disease activity in Takayasu arteritis (TAK) are lacking, and ultrasonography (US) may represent a valuable option.
Definitions and online reliability assessment of elementary ultrasound lesions in Takayasu arteritis: a study from the OMERACT Ultrasound Working Group · 2026 · DOIBACKGROUND/AIMS: Giant cell arteritis (GCA) causes significant morbidity from disease burden and treatment complications, yet Australian data on healthcare utilisation are lacking.
Emergency department presentations and hospital admissions for patients with biopsy-positive giant cell arteritis in South Australia · 2026 · DOIAlthough indirect immunofluorescence (IIF) has traditionally been the reference technique, its diagnostic value in contemporary real-world practice remains uncertain.
ANCA testing in real-world clinical practice: diagnostic performance and predictive value in a Spanish cohort · 2026 · DOIThe Polymyalgia Rheumatica Activity Score (PMR-AS) is the best validated composite disease activity measure in PMR to date, but its reliability, measurement error and discriminative validity have not been studied.
Measurement properties of the Polymyalgia Rheumatica Activity Score (PMR-AS): reliability, measurement error and discriminative validity · 2026 · DOICurrent guidelines lack specific recommendations for SGS management and evidence is limited.
Clinical outcomes of subglottic stenosis in granulomatosis with polyangiitis: results of an international multicentre observational study · 2026 · DOIMost studies on renal outcomes in ANCA-associated vasculitis (AAV) focus on GN; however, the prognosis of patients without clinically apparent kidney involvement is understudied.
Renal outcomes in ANCA-associated vasculitis without clinically apparent kidney involvement: a multicentre REVEAL cohort study · 2026 · DOIReal-world practice patterns of eosinophilic granulomatosis with polyangiitis (EGPA) remain poorly defined.
Current management of eosinophilic granulomatosis with polyangiitis across Europe: insights from a multinational expert survey · 2026 · DOIPrior studies examining the malignancy risk in polymyalgia rheumatica (PMR) have reported conflicting results, with some indicating an increased risk within 6-12 months after diagnosis.
Lifetime risk of malignancy in polymyalgia rheumatica: a population-based matched cohort study from southern Norway · 2026 · DOIAlthough standard induction therapy includes cyclophosphamide or rituximab with glucocorticoids, the role of therapeutic plasma exchange (TPE) in paediatric AAV remains uncertain.
Therapeutic plasma exchange in paediatric ANCA-associated vasculitis: a multicentre real-world experience · 2026 · DOIRapid access strategy (RAS) clinics for individuals with suspected PMR may reduce GC initiation before rheumatological evaluation, but evidence is limited.
Impact of a rapid access strategy on referral rates, diagnosis, and glucocorticoid initiation in suspected polymyalgia rheumatica · 2025 · DOIWhile F-18 FDG PET/CT is well-established in large vessel vasculitis, its role in MVV remains unclear.
The interplay of +RhF and +MPO antibodies and AAV in patients with RA+ANCA warrants further investigation.
ANCA-associated vasculitis in patients with rheumatoid arthritis: A single-center cohort study · 2025 · DOIPrior studies demonstrated that vascular enhancement on cranial vessel wall magnetic resonance imaging (vw-MRI) decreases with treatment of GCA, but whether enhancement increases during relapse is not well known.
Longitudinal changes on cranial magnetic resonance imaging in relapsing giant cell arteritis · 2025 · DOIInvestigate the effects of hUC-MSCs on other immune cells or pathways in HSP. Evaluate the optimal dosage and treatment duration of hUC-MSCs in HSP patients. Examine the long-term efficacy and safety of hUC-MSCs treatment in HSP patients. Explore the potential of hUC-MSCs as a therapeutic strategy for other autoimmune diseases.
hUC-MSCs regulating the balance of Th17/Treg in Henoch Schönlein purpura via STAT3 pathway · 2026 · DOIBased on these promising findings, FDG PET/CT may progressively emerge as a biomarker of PAN-spectrum vasculitis, particularly when other tests are inconclusive.
The role of patient-reported outcomes measures (PROMs) in polymyalgia rheumatica (PMR) remains scarcely studied.
Patient-reported outcome measures in incident Polymyalgia Rheumatica: Changes after treatment initiation, short-term glucocorticoid discontinuation, and at one-year follow-up · 2026 · DOITAK is an important and underrecognized cause of ocular inflammation and ischemia in young patients.
Ocular involvement in Takayasu arteritis – A case series from a tertiary eye center in South India · 2026 · DOI
Most-cited papers in Vasculitis and related conditions
- Rituximab versus Cyclophosphamide for ANCA-Associated Vasculitis · New England Journal of Medicine · 2010 · 2,371 citations
- AUTOANTIBODIES AGAINST NEUTROPHILS AND MONOCYTES: TOOL FOR DIAGNOSIS AND MARKER OF DISEASE ACTIVITY IN WEGENER'S GRANULOMATOSIS · The Lancet · 1985 · 1,270 citations
- 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides · Carolina Digital Repository (University of North Carolina at Chapel Hill) · 2020 · 1,255 citations
- RETRACTED: Avacopan for the Treatment of ANCA-Associated Vasculitis · New England Journal of Medicine · 2021 · 982 citations
- Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis · New England Journal of Medicine · 2020 · 796 citations
- Pathophysiology of polytrauma · Injury · 2005 · 702 citations
- 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis · Arthritis & Rheumatology · 2021 · 591 citations
- 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis · Arthritis & Rheumatology · 2021 · 574 citations
- 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis · Annals of the Rheumatic Diseases · 2022 · 476 citations
- 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis · Annals of the Rheumatic Diseases · 2022 · 444 citations
Most recent work
- Retraction: Jayne DRW et al. Avacopan for the Treatment of ANCA-Associated Vasculitis, N Engl J Med 2021;384:599-609. · New England Journal of Medicine · 2026
- Current and Future Treatments for Takayasu Arteritis: Toward Cardiovascular Risk Modification · Circulation · 2026
- A phase 2, randomised, placebo-controlled study of guselkumab in adults with new-onset or relapsing giant cell arteritis · Annals of the Rheumatic Diseases · 2026
- 2025 EULAR recommendations for the management of polymyalgia rheumatica and primary large vessel vasculitis · Annals of the Rheumatic Diseases · 2026
- Establishing C-X-C motif chemokine receptor 4 as a novel imaging target in giant cell arteritis · Arthritis Research & Therapy · 2026
- Interstitial Lung Disease in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis: A European Multicenter Study · Arthritis & Rheumatology · 2026
- Targeting Immunologic Pathways in Eosinophilic Granulomatosis With Polyangiitis: Translating Emerging Evidence Into Clinical Practice · Allergy · 2026
- OCT AND OCT-A FINDINGS IN GIANT CELL ARTERITIS · Retina · 2026
- Treatment of Eosinophilic Granulomatosis With Polyangiitis (EGPA): Do We Need Immunosuppressives? · The Journal of Allergy and Clinical Immunology In Practice · 2026
- Definitions and online reliability assessment of elementary ultrasound lesions in Takayasu arteritis: a study from the OMERACT Ultrasound Working Group · Seminars in Arthritis and Rheumatism · 2026
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