Cardiac manifestations of IgG4-related disease: a case series

Abstract

Background IgG4-related disease (IgG4-RD) is an autoimmune condition affecting almost every organ system, with an early inflammatory phase and later fibrotic consequences. Vascular manifestations, particularly, large-vessel involvement in IgG4-RD, are well described. However, important IgG4-related effects on medium-sized arteries and the pericardium are less well recognized. These less frequently reported cardiovascular effects of IgG4-RD include coronary artery stenosis, pericardial disease, cardiac masses, and valvular heart disease. Case Summary This case series focuses on three patients that demonstrate the cardiovascular effects of IgG4-RD and the pitfalls and importance of early diagnosis. Cases 1 and 2 presented with cardiac manifestations prior to more typical organ systems being affected which led to a delay in diagnosis. Case 1 presented with an acute myocardial infarction secondary to IgG4-RD of the coronary arteries and Case 2 presented with pericarditis which progressed to pericardial constriction due to IgG4-RD. Case 3 already had a diagnosis of IgG4-RD from a prior renal biopsy which raised the index of suspicion that his pericardial disease and thoracic mass was also related to IgG4-RD. Discussion Cardiac manifestations of IgG4-RD remain under-recognized and include coronary artery and pericardial disease. These manifestations often precede more typical manifestations in other organ systems. Recognizing cardiac manifestations of IgG4-RD on cardiac imaging can raise clinical suspicion and act as a catalyst to ascertain a confirmatory diagnosis. Early diagnosis and treatment are crucial to prevent potentially fatal outcomes and irreversible fibrosis.

IGG4‐related giant lymphoproliferative‐inflammatory left atrial mass

“…In addition, masses associated with inflammatory diseases are rare. 6 The IgG4-related disease is a clinical condition that results in the vant organ biopsy should be shown. 7 IgG4-related disease can lead to cardiovascular system involvement due to coronary arteries, heart valves, pericardium, myocardium, or peripheral vessel involvement.…”

“…The most common cause of cardiac mass is thrombus; while primary tumors are rare, secondary tumor metastases can be seen, especially in breast and lung cancers. In addition, masses associated with inflammatory diseases are rare 6 …”

Coronary artery involvement in a patient with IgG4-related disease

“…Coronary CTA provides detailed visualization of the coronary arteries and displays the spectrum of IgG4-related coronary artery disease (CAD) such as stenosis, soft tissue formation, tumor-like lesion, coronary ectasia, and aneurysmal formation. In the context of IgG4-related CVD, CTA is particularly useful in identifying diffuse or partial arterial wall thickening (>2 mm) and homogeneous wall enhancement [1 , 2 , 4 , 7 , 8 , 12 , 13] . The present case showed no aneurysmal formations or tumor-like lesions, but stenosis and periarterial wall thickening.…”

“…The CMR findings revealed several characteristic features associated with IgG4-RD, including myocardial thickening, patchy or diffuse enhancement (indicating the presence of fibrotic changes), and pericardial thickening or effusion. Furthermore, CMR has the ability to detect and characterize cardiac tumors, which can be an important manifestation of IgG4-RD [1 , 14] . In our present case, CMR shows evidence of severe LV systolic dysfunction and akinesia of the septal, and lateral walls with infarction in the septal and lateral wall mirroring the fibrotic lesions that are an essential indicator of ischemic myocardial injury and dysfunction.…”

“…Its common manifestations include autoimmune pancreatitis (AIP), sclerosing cholangitis, sialadenitis, orbital region disease, retroperitoneal fibrosis, and chronic periaortitis. It most commonly affects middle-aged and older adults; however, it has also been reported in women and children [1] , [2] , [3] .…”