Title |
The many faces of IgG4-related disease: report of a case with inaugural recurrent aortic aneurism ruptures and literature review
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Published in |
Rheumatology International, May 2018
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DOI | 10.1007/s00296-018-4046-9 |
Pubmed ID | |
Authors |
Mariana Luís, Luísa Brites, Bruno Fernandes, Diogo Jesus, Tânia Santiago, Sara Serra, João Rovisco, Lina Carvalho, José António P. da Silva, Armando Malcata |
Abstract |
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. |
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Unknown | 1 | 100% |
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Members of the public | 1 | 100% |
Mendeley readers
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Unknown | 16 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
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Other | 3 | 19% |
Student > Bachelor | 3 | 19% |
Researcher | 2 | 13% |
Student > Postgraduate | 2 | 13% |
Student > Master | 1 | 6% |
Other | 2 | 13% |
Unknown | 3 | 19% |
Readers by discipline | Count | As % |
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Medicine and Dentistry | 11 | 69% |
Unknown | 5 | 31% |