Akademik Veri Yönetim Sistemi
Balkan Radiology Congress 2024, İzmir, Türkiye, 23 - 26 Ekim 2024, (Özet Bildiri)
Introduction
Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated disease which can affect almost all organs of the body. Awareness is important because the disorder is treatable. Coronary artery involvement is a rare condition in IgG4-RD. Coronary artery anomalies (CAAs) are disorders that can vary widely in their presentation, ranging from benign variants with no clinical significance to potentially life-threatening conditions. Interarterial course anomaly is known as the most hemodynamically serious coronary artery anomaly.
Case Report
A 38-year-old male patient without any disease was admitted to the cardiology outpatient clinic with chest pain that had persisted for 2 months. ECG showed normal sinus rhythm. Cardiac biomarkers were normal. To further evaluate the chest pain, coronary CT angiography (CCTA) was performed. CCTA revealed diffuse and continuous periarterial soft tissue thickening surrounding the coronary arteries, particularly RCA and LA. There was significant luminal stenosis in distal LAD and acute marginalis branch of RCA. There were also ectatic segments involving both arteries. RCA was running between ascending aorta and the pulmonary trunk showing interarterial course.Invasive coronary angiography ( ICA ) didn’t show any adventitial changes. It only revealed luminal coronary ectatic changes. IgG4 level increased by 216 mg/dL. Sedimentation, WBC count and neutrophil ratio were also increased.With appropriate laboratory values accompanying typical radiological findings, coronary artery involvement due to IgG4-related disease was initially considered in the patient. The coronary anomaly was accompanied by unrelated IgG4 disease.
Discussion
Coronary artery involvement is a rare condition of IgG4-RD. Diffuse and continuous periarterial soft tissue thickening surrounding the coronary arteries is called "pigs-in-a-blanket" appearance on CCTA and it is typical for this disease. Also partial aneurysmal dilatation of the affected coronary segments may be observed. ICA may show coronary aneurysms but is limited in detecting the typical adventitial changes. Interarterial course coronary artery anomaly is considered malignant due to its association with an increased risk of sudden cardiac death. In the interarterial course, the coronary artery is located between aorta and pulmonary artery.
Conclusion
The combination of these two conditions in this patient increases the risk of life-threatening complications. Prompt recognition and appropriate management are crucial this complex patient.