Open research questions in Vasculitis and related conditions
103 unresolved questions extracted from the limitations and future-work sections of 355 Vasculitis and related conditions papers in our library. Each links back to the study that raised it.
What the literature leaves open
The limited efficacy of glucocorticoid therapy in severe abdominal HSP. The high relapse rate after withdrawal of glucocorticoid therapy. The lack of reported use of telitacicept in HSP.
Addition of telitacicept to glucocorticoid reduces abdominal pain duration and relapse rate in children with severe abdominal Henoch-Schönlein purpura: a single-center retrospective matched cohort study · 2026 · DOIThe study is a single-center retrospective study - The sample size is limited to 74 patients - The study only included patients with biopsy-confirmed ANCA-associated glomerulonephritis - The study did not include a control group
Histological grading of tertiary lymphoid structures correlates with renal impairment and adverse outcomes in ANCA-associated glomerulonephritis · 2026 · DOIFurther studies are needed to validate the prognostic nomogram in larger cohorts - Research is needed to investigate the mechanisms by which tertiary lymphoid structures contribute to renal impairment - Studies should explore the potential therapeutic targets related to tertiary lymphoid structures in ANCA-associated glomerulonephritis
Histological grading of tertiary lymphoid structures correlates with renal impairment and adverse outcomes in ANCA-associated glomerulonephritis · 2026 · DOIfurther studies are needed to confirm the effectiveness of 18F-FDG-PET/MR in detecting pre-stenotic cTAK, - research on the long-term outcomes of children with pre-stenotic cTAK diagnosed using 18F-FDG-PET/MR
FDG-PET/MR in paediatric pyrexia of unknown origin: early detection of Takayasu arteritis—a single-centre experience · 2026 · DOIThere is a need for effective detection of pre-stenotic Takayasu arteritis in children with pyrexia of unknown origin. Conventional angiographic imaging has limitations in detecting the disease in its early phase.
FDG-PET/MR in paediatric pyrexia of unknown origin: early detection of Takayasu arteritis—a single-centre experience · 2026 · DOIConsidering the more vessels involved, more severe inflammation and higher mortality, aggressive treatment is warranted in infants.
A retrospective study of infantile-onset Takayasu arteritis: experience from a tertiary referral center in China · 2024 · DOIObjective: Takayasu artery (TAK) is a chronic inflammatory disease that mainly affects the aorta and its major branches and is rarely reported in infants.
A retrospective study of infantile-onset Takayasu arteritis: experience from a tertiary referral center in China · 2024 · DOIBACKGROUND AND OBJECTIVES: Peripheral neuropathy is a common manifestation of systemic and nonsystemic vasculitides; however, there is limited literature on vasculitic myopathy.
Galactose-deficient IgA1 on the immunoglobulin hinge region and its immune complexes are thought to play a central pathogenetic role in IgAV, however, an association between such molecules and specific GI mucosal damage remains unclear.
Gastrointestinal manifestations and pathogenesis in childhood immunoglobulin A vasculitis · 2024 · DOIThe prognostic value of NLR in AAV patients is unclear. There is a need for accessible and robust biomarkers for early stratification of high-risk AAV patients.
The neutrophil-to-lymphocyte ratio is associated with adverse outcomes in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis · 2026 · DOIAlthough a numerical trend was observed whereby infection-related mortality was higher in the high NLR group, the lack of statistical significance may be attributed to the limited number of fatal events; thus, larger cohort studies are warranted to further elucidate the association between NLR and specific causes of death.
The neutrophil-to-lymphocyte ratio is associated with adverse outcomes in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis · 2026 · DOIThe majority of clinical studies demonstrated moderate to high methodological quality, but small sample sizes and lack of blinding in outcome assessment were common limitations. The certainty of evidence for S100A4 was evaluated as very low due to extreme indirectness and imprecision.
S100 proteins in IgA vasculitis and other systemic vasculitides – from pathogenic mechanisms to clinical biomarkers: a systematic review · 2026 · DOIThe specific pathogenic role of S100 proteins in IgA vasculitis remains unclear. There is a lack of direct clinical or experimental data on the role of S100 proteins in systemic vasculitis cohorts.
S100 proteins in IgA vasculitis and other systemic vasculitides – from pathogenic mechanisms to clinical biomarkers: a systematic review · 2026 · DOIDiagnosing microscopic polyangiitis in seniors may be challenging due to multiple comorbidities. There is a need for tailored therapy to balance disease control with vulnerability to treatment toxicity.
Microscopic Polyangiitis in a Nonagenarian: Diagnostic and Therapeutic Challenges in Geriatric Vasculitis · 2026 · DOIThe paper highlights that older patients often present with nonspecific symptoms and reduced physiological reserve, delaying diagnosis, but there is insufficient evidence on early diagnostic strategies or screening protocols specifically designed for geriatric AAV presentation.
Microscopic Polyangiitis in a Nonagenarian: Diagnostic and Therapeutic Challenges in Geriatric Vasculitis · 2026 · DOIThe diagnosis of PAN is challenging due to its heterogeneous presentations. There is a lack of specific diagnostic criteria for PAN. Biopsies can sometimes be inconclusive.
[18F]FDG PET/CT value in the diagnostic evaluation of patients with polyarteritis nodosa · 2026 · DOIFurther studies are needed to evaluate the diagnostic performance of [18F]FDG PET/CT in PAN. The 'ant-farm' uptake pattern should be further investigated. The correlation between [18F]FDG PET/CT findings and clinical, biological, and histopathological data should be further explored.
[18F]FDG PET/CT value in the diagnostic evaluation of patients with polyarteritis nodosa · 2026 · DOIThere is a need for a more accurate and reliable imaging biomarker for PMR. The current diagnosis and monitoring of PMR rely primarily on clinical features and elevated acute-phase reactants.
Superior correlation with clinical activity and unique detection patterns: a prospective study of FAPI versus FDG-PET in polymyalgia rheumatica and giant cell arteritis · 2026 · DOIThe association between IgA nephropathy and DAH is not well understood. There is a lack of reports on the occurrence of DAH in patients with dialysis-dependent IgA nephropathy.
Diffuse Alveolar Hemorrhage in Dialysis-Dependent IgA Nephropathy Suggesting Persistent Systemic Vasculitis: A Case Report and Literature Review · 2026 · DOIThere is a need for alternative therapies for IgA vasculitis nephritis. Current treatments have limitations, and some patients do not respond well to them. The study aims to address this gap by investigating the efficacy and safety of telitacicept.
Efficacy and safety of telitacicept in IgA vasculitis nephritis: a single-center retrospective study · 2026 · DOIThe case report is limited to a single patient. The diagnosis of Takayasu arteritis can be challenging due to its rarity and nonspecific symptoms. The overlap with premature atherosclerosis and lack of advanced inflammatory imaging represent important limitations.
Beyond Classic Territories: Atypical Iliofemoral Vascular Involvement in a Suspected Takayasu Arteritis Presenting as Peripheral Arterial Disease Mimic in Young Age · 2026 · DOIThere is a lack of awareness about the atypical presentations of Takayasu arteritis. The diagnosis of Takayasu arteritis can be challenging due to its rarity and nonspecific symptoms. There is a need for high clinical suspicion and multimodality imaging in patients with unexplained claudication and uncontrolled hypertension.
Beyond Classic Territories: Atypical Iliofemoral Vascular Involvement in a Suspected Takayasu Arteritis Presenting as Peripheral Arterial Disease Mimic in Young Age · 2026 · DOIThe association between Takayasu arteritis and Down syndrome is not yet fully understood. The pathophysiological mechanism of Takayasu arteritis has not yet been fully elucidated.
The diagnostic accuracy of ultrasound and cranial MRI for giant cell arteritis is not well established. The influence of cardiovascular risk on diagnostic accuracy is not well understood.
Diagnostic accuracy of ultrasound versus cranial MRI for giant cell arteritis: dependence on cardiovascular risk for ultrasound only. · 2026 · DOIThere is a lack of awareness of the potential coexistence of microscopic polyangiitis, systemic lupus erythematosus, and transthyretin amyloidosis in patients with splenic marginal zone lymphoma.
Coexistence of Microscopic Polyangiitis, Systemic Lupus Erythematosus, and Transthyretin Amyloidosis in an Elderly Japanese Patient With Splenic Marginal Zone Lymphoma: An Autopsy Case Report · 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
- Pathophysiology of polytrauma · Injury · 2005 · 702 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
- 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis · Annals of the Rheumatic Diseases · 2022 · 329 citations
- Dietary “Fibre” and Pregnancy Toxaemia · BMJ · 1953 · 271 citations
- Large-vessel vasculitis · Nature Reviews Disease Primers · 2022 · 240 citations
- Benralizumab versus Mepolizumab for Eosinophilic Granulomatosis with Polyangiitis · New England Journal of Medicine · 2024 · 192 citations
- Diagnosis and management of ANCA-associated vasculitis · The Lancet · 2024 · 190 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
- Regression of vascular wall thickening with upadacitinib in a patient with clinically silent pediatric-onset takayasu arteritis and ulcerative colitis: case-based review · Clinical Journal of Gastroenterology · 2026
- Large Vessel Vasculitis: Recent Advances in Pathophysiology and Targeted Therapies · Drugs · 2026
- S100 proteins in IgA vasculitis and other systemic vasculitides – from pathogenic mechanisms to clinical biomarkers: a systematic review · Frontiers in Immunology · 2026
- In newly diagnosed polymyalgia rheumatica, weekly methotrexate improved glucocorticoid-free remission rates at 1 y · Annals of Internal Medicine · 2026
- [18F]FDG PET/CT value in the diagnostic evaluation of patients with polyarteritis nodosa · European Journal of Nuclear Medicine and Molecular Imaging · 2026
- Phase 3 Trial of Secukinumab in Polymyalgia Rheumatica · New England Journal of Medicine · 2026
- Secukinumab for Giant Cell Arteritis · NEJM Evidence · 2026
- Renal Involvement in Pediatric Small-Vessel Vasculitis: A Comprehensive Review of Clinical Impact, Diagnosis, and Management · Medical Sciences · 2026
- Large Vessel Vasculitis Revisited: Mechanistic Insights and Advances in Diagnosis and Management · Journal of the American Heart Association · 2026
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