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Puseljic, M; Schmid, J; Igrec, J; Hatzl, S; Scholz, L; Woelfler, A; Fuchsjäger, M; Talakic, E.
Pulmonary manifestations of VEXAS syndrome with acute interstitial pneumonia and diffuse alveolar hemorrhage: a case report and literature review
ARP RHEUMATOL. 2024; 3(2): 151-156. Doi: 10.63032/EVGB1553 (- Case Report)
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Führende Autor*innen der Med Uni Graz
Igrec Jasminka
Puseljic Marijan
Co-Autor*innen der Med Uni Graz
Fuchsjäger Michael
Hatzl Stefan
Schmid Johannes
Scholz Laura
Talakic Emina
Wölfler Albert
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Abstract:
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is an emerging adult-onset systemic autoinflammatory disorder affecting multiple organ systems. While lung involvement is common in this syndrome, literature regarding specific patterns is sparse. In this report, we present a case description of a patient with VEXAS syndrome who presented at the emergency department on two separate occasions with acute interstitial pneumonia (AIP) and diffuse alveolar hemorrhage (DAH). A literature review with a comparison of our observed findings to the general findings of VEXAS syndrome, AIP, and DAH is provided. This report underscores the rarity of specific pulmonary manifestations associated with VEXAS syndrome, contributing valuable insight to the limited literature available on this topic.
Find related publications in this database (using NLM MeSH Indexing)
Humans - administration & dosage
Lung Diseases, Interstitial - complications, diagnostic imaging, diagnosis
Hemorrhage - administration & dosage
Pulmonary Alveoli - pathology
Male - administration & dosage
Genetic Diseases, X-Linked - genetics, complications, diagnosis
Lung Diseases - pathology
Vacuoles - pathology
Middle Aged - administration & dosage
Syndrome - administration & dosage
Ubiquitin-Activating Enzymes - administration & dosage

Find related publications in this database (Keywords)
Autoinflammatory disease
Interstitial lung disease
Diffuse alveolar hemorrhage
Acute interstitial
pneumonia
Computed tomography
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