Pictorial review of computed tomography and magnetic resonance imaging findings of cardiovascular manifestations of IgG4-related disease

Abstract

This study includes a series of 5 cases demonstrating cardiovascular manifestations of findings of IgG4-related disease. Two cases demonstrate peri-aortic soft tissue thickening in the infrarenal abdominal aorta and bilateral ostio--proximal common iliac artery. In 2 cases there were circumferential soft tissue lesions around the arch of the aorta. One of the cases showed coexistent, biopsy-proven Riedel’s thyroiditis and infiltrative soft tissue along the right atrial wall and interatrial septum. In one case there was a partly calcified mass in the left hemi-thorax consistent with a diagnosis of IgG4-related fibrosing mediastinitis.

Reshaping the Concept of Riedel’s Thyroiditis into the Larger Frame of IgG4-Related Disease (Spectrum of IgG4-Related Thyroid Disease)

“…Some studies enrolled patients with non-thyroid manifestations of IgG4-RD, but further on, the authors identified that subgroups of these subjects were associated with IgG4-mediated thyroid features consistent with IgG4-RTD. We identified three such studies [ 61 , 62 , 63 ] ( Table 4 ).…”

“…This was a 49-year-old female who presented complications at the carotid, subclavian, and pulmonary arteries, and a small increase in serum IgG4 without hypothyroidism. The general IgG4-related condition responded well to glucocorticoid therapy [ 61 ]. We also mention one case report of Pacella et al [ 47 ] that identified a 53-year-old male with retroperitoneal fibrosis diagnosed 4 years after an episode of RT requiring decompression surgery at that moment, with both thyroid and retroperitoneal findings being elements of IgG4-RD [ 47 ].…”

“…Currently, despite being an unusually rare entity, RT has been added to the larger register of IgG4-RD, thus many practitioners other than endocrinologists and surgeons might have to take into consideration thyroid involvement due to IgG4 connections [ 6 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 54 , 57 , 60 , 61 , 62 , 63 ]. Nowadays, it is essential to assess serum IgG4 levels but mostly to perform an immunohistochemistry exam in order to point out the IgG4 burden to have RT confirmation.…”

“…Other drugs such as tamoxifen and rituximab might prove useful in selected cases. Surgery, although not a first-line option unless an emergency, still represents a strong player in overall management from open biopsy to tracheal intervention [ 6 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 54 , 57 , 60 , 61 , 62 , 63 ]. Awareness of various issues is essential: from not being able to perform a complete thyroid removal due to massive fibrosis/stony consistency [ 44 , 54 ], to intra-operatory pneumothorax requiring thoracentesis, as seen in similar procedures for other etiological events [ 6 , 64 , 65 ].…”

“…A total of 66 subjects with RT are included in our analysis based on published cases. The rate of serum IgG4 evaluation remained low; normal values do not exclude RT [ 6 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 54 , 57 , 60 , 61 , 62 , 63 ].…”