Rheumatology International

, Volume 38, Issue 8, pp 1565–1570 | Cite as

The many faces of IgG4-related disease: report of a case with inaugural recurrent aortic aneurism ruptures and literature review

  • Mariana Luís
  • Luísa Brites
  • Bruno Fernandes
  • Diogo Jesus
  • Tânia Santiago
  • Sara Serra
  • João Rovisco
  • Lina Carvalho
  • José António P. da Silva
  • Armando Malcata
Cases with a Message


Vascular involvement in IgG4-related disease (IgG4-RD), is a well-recognized feature and large vessel commitment, especially the aorta, can be the only manifestation of the disease. Being a newly recognized disease, its diagnosis and workup still represents a challenge in clinical practice. A 47-year-old-man with two aortic aneurysms ruptures, one at abdominal and the other at thoracic level, was referred to our rheumatology department. The initial analysis of the surgical specimen obtained 3 years earlier revealed a nonspecific aortitis. Re-evaluation of the biopsy with immunohistology now demonstrated the presence of IgG4 deposits. Evidence-based recommendations regarding diagnosis, treatment and follow-up of IgG4-related large-vessel involvement are lacking. In this particular case, histopathology were crucial. The authors review and discuss vascular involvement in IgG4-RD and respective treatment options.


IgG4 related-disease Aortitis Large-vessel disease Treatment 


Author contributions

M. L. and L.B. conceived of the presented idea; D.J., T.S., S.S. and J.R. participated in the literature review; B.F. and L.C. performed the histological work and contributed to the histopathological-related literature review; J.A.P.S. and A.M. supervised the findings of this work; All authors discussed the results and contributed to the final manuscript.

Compliance with ethical standards

Conflict of interest

Mariana Luís, Luisa Brites, Bruno Fernandes, Diogo Jesus, Tânia Santiago, Sara Serra, João Rovisco, Lina Carvalho, José António P. da Silva and Armando Malcata declare that they have no conflict of interest.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Rheumatology DepartmentCentro Hospitalar e Universitário de CoimbraCoimbraPortugal
  2. 2.Anatomical PathologyCentro Hospitalar e Universitário de CoimbraCoimbraPortugal

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