Aktuelle Rheumatologie 2023; 48(03): 212-215
DOI: 10.1055/a-1887-5341
Kasuistik

Vexas Syndrome Presenting As Long Covid-19

Vexas-Syndrom zeigt sich als Long Covid-19
Derya Yildirim
1   Rheumatology, Gazi Universitesi Tip Fakultesi, Ankara, Turkey
,
Mehmet Arda Inan
2   pathology, Gazi Universitesi Tip Fakultesi, Ankara, Turkey
,
Nalan Akyurek
2   pathology, Gazi Universitesi Tip Fakultesi, Ankara, Turkey
,
Abdurrahman Tufan
1   Rheumatology, Gazi Universitesi Tip Fakultesi, Ankara, Turkey
› Author Affiliations

Abstract

Objective VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently described systemic inflammatory syndrome caused by somatic mutations of UBA1. COVID-19 is a viral infection that was described in 2019 and spread widely and quickly all around the world. Constitutional, thrombotic and pulmonary symptoms of these two conditions are similar, which is why cases of VEXAS syndrome may be misdiagnosed as a COVID infection.

Case report We introduced a case report of a 72-year-old male patient with VEXAS syndrome who had fever, fatigue, deep vein thrombosis and a cough and was thought to have a long COVID-19 infection for one year. Then we diagnosed him with VEXAS syndrome with vacuoles in myelomonocytic cells, skin lesions and a mutation of the UBA-1 gene.

Conclusion VEXAS and long COVID are two new conditions with overlapping clinical presentations. Physicians must be aware of these clinical conditions because of their different treatment strategy and prognosis.



Publication History

Article published online:
20 September 2022

© 2022. Thieme. All rights reserved.

Georg Thieme Verlag
Rüdigerstraße 14, 70469 Stuttgart, Germany

 
  • References

  • 1 Beck DB, Ferrada MA, Sikora KA. et al. Somatic Mutations in UBA1 and Severe Adult-Onset Autoinflammatory Disease. N Engl J Med 2020; 383: 2628-2638
  • 2 Georgin-Lavialle S, Terrier B, Guedon AF. et al. French VEXAS group; GFEV, GFM, CEREMAIA, MINHEMON. Further characterization of clinical and laboratory features in VEXAS syndrome: large-scale analysis of a multicentre case series of 116 French patients. Br J Dermatol 2022; 186: 564-574
  • 3 Tufan A, Matucci-Cerinic M. Immune dysfunction in COVID-19 and judicious use of antirheumatic drugs for the treatment of hyperinflammation. Turk J Med Sci 2021; 17 51(SI-1) 3391-3404
  • 4 Guarnera A, Podda P, Santini E. et al. Differential diagnoses of COVID-19 pneumonia: the current challenge for the radiologist – a pictorial essay. Insights Imaging 2021; 12: 34
  • 5 Deer RR, Rock MA, Vasilevsky N. et al. Characterizing Long COVID: Deep Phenotype of a Complex Condition. EBioMedicine 2021; 74: 103722
  • 6 Sisó-Almirall A, Brito-Zerón P, Conangla Ferrín L. et al. Long Covid-19: Proposed Primary Care Clinical Guidelines for Diagnosis and Disease Management. Int J Environ Res Public Health 2021; 18: 4350
  • 7 Koster MJ, Kourelis T, Reichard KK. et al. Clinical Heterogeneity of the VEXAS Syndrome: A Case Series. Mayo Clin Proc 2021; 96: 2653-2659
  • 8 Huang H, Zhang W, Cai W. et al. VEXAS syndrome in myelodysplastic syndrome with autoimmune disorder. Exp Hematol Oncol 2021; 10: 23
  • 9 Obiorah IE, Beck DB, Wang W. et al. Myelodysplasia and Bone Marrow Manifestations of Somatic UBA1 Mutated Autoinflammatory Disease. Blood 2020; 136: 20-21
  • 10 Bourbon E, Heiblig M, Gerfaud Valentin M. et al. Therapeutic options in VEXAS syndrome: insights from a retrospective series. Blood 2021; 137: 3682-3684
  • 11 Loschi M, Roux C, Sudaka I. et al. Allogeneic stem cell transplantation as a curative therapeutic approach for VEXAS syndrome: a case report. Bone Marrow Transplant 2022; 57: 315-318
  • 12 Comont T, Heiblig M, Rivière E. et al. French VEXAS study group, Groupe Francophone des Myélodysplasies (GFM) and MedecineINterne, HEmato et ONco (MINHEMON) group. Azacitidine for patients with Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic syndrome (VEXAS) and myelodysplastic syndrome: data from the French VEXAS registry. Br J Haematol 2022; 196: 969-974