SynthesisRheumatology international2023
Pulmonary manifestations in VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome: a systematic review.
Synthesis in Rheumatology international, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers, 2 of them syntheses that pooled it.
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Who cites it
14 citing papers in PubMed, 2 syntheses or guidelines pooled it, 28 citations in OpenAlex.
- Emerging treatment approaches for VEXAS syndrome: a systematic review and meta-analysis.Annals of hematology · 2025Pooled it
- Clinical characteristics, disease trajectories and management of vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome: a systematic review.Rheumatology international · 2024Pooled it
- VEXAS syndrome unmasked from relapsing polychondritis and infection mimicry: a case-based review.Rheumatology international · 2026Review
- Exploring glucocorticoid dose-response patterns in VEXAS syndrome: a pilot retrospective study.Rheumatology international · 2026Review
- Refractory fever, pulmonary infiltrates, and auricular chondritis: a rare case of VEXAS syndrome following COVID-19 infection in the emergency department.World journal of emergency medicine · 2025Article
- An Atypical Presentation of VEXAS Syndrome: A Diagnostic Conundrum.The Ulster medical journal · 2025Article
- VEXAS: A review of current understandings and emerging treatment strategies.Frontiers in immunology · 2025Review
- Vexas Syndrome in a Moroccan Patient: The Story of a Two-Year Diagnostic Lag.European journal of case reports in internal medicine · 2025Article
- A vexing case of a 73-year-old man with fevers, orbital cellulitis, and asymptomatic interstitial lung disease.Respirology case reports · 2024Article
- A clinical phenotype of VEXAS syndrome with pleural effusion, infiltrates, and systemic inflammation in a 76-year-old patient: a case report.Journal of medical case reports · 2024Article
- VEXAS syndrome presenting as diffuse alveolar haemorrhage.BMJ case reports · 2024Article
- VEXAS and Myelodysplastic Syndrome: An Interdisciplinary Challenge.Journal of clinical medicine · 2024Article
- Case report: VEXAS as an example of autoinflammatory syndrome in pulmonology clinical practice.Frontiers in medicine · 2024Article
- VEXAS syndrome: a newly discovered systemic rheumatic disorder.Reumatologia · 2023Review
Corrections and comments
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Authors and funding
4 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundVEXAS (vacuoles, E1 enzyme, X-linked, auto-inflammatory, somatic) syndrome is a newly described auto-inflammatory disease. Many cases feature pulmonary infiltrates or respiratory failure. This systematic review aimed to summarize respiratory manifestations in VEXAS syndrome described to date.
methodsDatabases were searched for articles discussing VEXAS syndrome until May 2022. The research question was: What are the pulmonary manifestations in patients with VEXAS syndrome? The search was restricted to English language and those discussing clinical presentation of disease. Information on basic demographics, type and prevalence of pulmonary manifestations, co-existing disease associations and author conclusions on pulmonary involvement were extracted. The protocol was registered on the PROSPERO register of systematic reviews.
resultsInitially, 219 articles were retrieved with 36 ultimately included (all case reports or series). A total of 269 patients with VEXAS were included, 98.6% male, mean age 66.8 years at disease onset. The most frequently described pulmonary manifestation was infiltrates (43.1%; n = 116), followed by pleural effusion (7.4%; n = 20) and idiopathic interstitial pneumonia (3.3%; n = 9). Other pulmonary manifestations were: nonspecific interstitial pneumonia (n = 1), bronchiolitis obliterans (n = 3), pulmonary vasculitis (n = 6), bronchiectasis (n = 1), alveolar haemorrhage (n = 1), pulmonary embolism (n = 4), bronchial stenosis (n = 1), and alveolitis (n = 1). Several patients had one or more co-existing autoimmune/inflammatory condition. It was not reported which patients had particular pulmonary manifestations.
conclusionThis is the first systematic review undertaken in VEXAS patients. Our results demonstrate that pulmonary involvement is common in this patient group. It is unclear if respiratory manifestations are part of the primary disease or a co-existing condition. Larger epidemiological analyses will aid further characterisation of pulmonary involvement and disease management.
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