Immunoglobulin G4-Related Aortic Disease

Uchida et al. published an article in 24 (2018) issue of the journal entitled “Immunoglobulin G subclass 4-related lymphoplasmic thoracic aortitis in a patient with acute type A aortic dissection,” in which they presented the case of a patient who was referred for surgical treatment.¹⁾ Histopathologic examination of his aortic tissue confirmed the diagnosis, showing marked adventitial thickening with fibrosis and an IgG4-positive plasma cell infiltrate.

Early in 2018, we also reported the case of a 65-year-old male patient with an enlarged ascending aorta and type B aortic dissection.²⁾ The patient had experienced retroperitoneal fibrosis, membranous nephropathy, and acute coronary syndrome. Serum IgG4 levels were not measured and a pathological examination did not indicate that the retroperitoneal fibrosis and membranous nephropathy were IgG4 related. A 2-year course of steroid therapy was prescribed and contributed to recovery from the retroperitoneal fibrosis and nephropathy. The patient’s IgG4 level after the therapy was 19.8 mg/dL. He did well for several years without steroid therapy but then developed type B aortic dissection 3 years ago, at which point his serum IgG4 level had risen to 190 mg/dL. Chest computed tomography showed the dissection associated with thickened periaortic changes.

Despite its importance for postoperative disease management, a definitive diagnosis of IgG4-related disease is not easy to obtain preoperatively.³⁾ IgG4-related inflammation can induce morphological changes in the aorta. For example, Lindsay et al.⁴⁾ presented a case of periaortitis with periaortic soft tissue surrounding the abdominal aorta. We wondered whether Fig. 1 of Uchida et al. actually shows periaortic thickness suggesting IgG4related disease. It would therefore be informative to know the perioperative serum IgG4 levels of their patient for IgG4-related diseases, steroid therapy is often prescribed for IgG4-related diseases, but the required therapeutic dose differs on a case-by-case basis. Tajima et al.⁵⁾ have suggested that higher doses of corticosteroids might be required for the treatment of IgG4-related cardiovascular diseases compared with other IgG4-related diseases. We are prompted to ask: did Uchida et al. consider steroid therapy for their patient?

In conclusion, IgG4-related disease is a new clinical entity that can affect a variety of organs. IgG4-related aortic diseases cause various morphological changes; however, clinical features and pathological findings can aid in obtaining definitive diagnoses when different types of aortopathy are implicated.

Disclosure Statement

None declared.