Radiologic Findings of IgG4-Related Disease

Autoimmune pancreatitis (AIP), characterized by an autoimmune phenomenon of prominent lymphocytes, IgG4-bearing plasma cell infiltration and storiform fibrosis, has been widely reported as a specific type of chronic pancreatitis. Typical image findings of this disease are reported as diffuse pancreatic swelling and a capsule-like rim on CT or MRI. However, AIP presents with a variable morphology, such as focal, segmental and multifocal swellings. Because imaging findings for AIP can look like those of pancreatic cancer, AIP has often been treated with unnecessary surgical resection. In addition, AIP is complicated by the involvement of various other organs besides the pancreas that show lymphoplasmacytic infiltration and fibrosis. These are frequently misdiagnosed as inherent lesions of corresponding organs. Furthermore, these extra-pancreatic lesions show systemic distribution and share common features of IgG4- bearing plasma cell infiltration as well as favorable responses to corticosteroid, indicating the presence of systemic condition, IgG4-related diseases. AIP is now recognized as an IgG4-related disease. Detailed evaluations of imaging findings of CT, MRI and Gallium-67 (Ga-67) scintigraphy for the involvement of these various organs are useful for a correct diagnosis of this systemic disease.ArticleCurrent Immunology Reviews. 7(2):186-203 (2011)journal articl

Radiologic Findings of IgG4-Related Disease

Autoimmune pancreatitis (AIP), characterized by an autoimmune phenomenon of prominent lymphocytes, IgG4-bearing plasma cell infiltration and storiform fibrosis, has been widely reported as a specific type of chronic pancreatitis. Typical image findings of this disease are reported as diffuse pancreatic swelling and a capsule-like rim on CT or MRI. However, AIP presents with a variable morphology, such as focal, segmental and multifocal swellings. Because imaging findings for AIP can look like those of pancreatic cancer, AIP has often been treated with unnecessary surgical resection. In addition, AIP is complicated by the involvement of various other organs besides the pancreas that show lymphoplasmacytic infiltration and fibrosis. These are frequently misdiagnosed as inherent lesions of corresponding organs. Furthermore, these extra-pancreatic lesions show systemic distribution and share common features of IgG4- bearing plasma cell infiltration as well as favorable responses to corticosteroid, indicating the presence of systemic condition, IgG4-related diseases. AIP is now recognized as an IgG4-related disease. Detailed evaluations of imaging findings of CT, MRI and Gallium-67 (Ga-67) scintigraphy for the involvement of these various organs are useful for a correct diagnosis of this systemic disease.ArticleCurrent Immunology Reviews. 7(2):186-203 (2011)journal articl

Characteristic findings in images of extra-pancreatic lesions associated with autoimmune pancreatitis

Purpose: Autoimmune pancreatitis is a unique form of chronic pancreatitis characterized by a variety of extra-pancreatic involvements which are frequently misdiagnosed as lesions of corresponding organs. The purpose of this study was to clarify the diagnostic imaging features of extra-pancreatic lesions associated with autoimmune pancreatitis. Materials and methods: We retrospectively analyzed diagnostic images of 90 patients with autoimmune pancreatitis who underwent computer-assisted tomography, magnetic resonance imaging, and/or gallium-67 scintigraphy before steroid therapy was initiated. Results: AIP was frequently (92.2%) accompanied by a variety of extra-pancreatic lesions, including swelling of lachrymal and salivary gland lesions (47.5%), lung hilar lymphadenopathy (78.3%), a variety of lung lesions (51.2%), wall thickening of bile ducts (77.8%), peri-pancreatic or para-aortic lymphadenopathy (56.0%), retroperitoneal fibrosis (19.8%), a variety of renal lesions (14.4%), and mass lesions of the ligamentum teres (2.2%). Characteristic findings in CT and MRI included lymphadenopathies of the hilar, peri-pancreatic, and para-aortic regions; wall thickening of the bile duct; and soft tissue masses in the kidney, ureters, aorta, paravertebral region, ligamentum teres, and orbit. Conclusions: Recognition of the diagnostic features in the images of various involved organs will assist in the diagnosis of autoimmune pancreatitis and in differential diagnoses between autoimmune pancreatitis-associated extra-pancreatic lesions and lesions due to other pathologies.ArticleEUROPEAN JOURNAL OF RADIOLOGY. 76(2):228-238 (2010)journal articl

A case of complete atrioventricular block: The use of magnetic resonance imaging conditional pacemakers for diagnosing cardiac sarcoidosis

A 50-year-old man presented to the emergency department with repeated episodes of faintness and exertional dyspnea, and was found to have an atrioventricular (AV) block. Chest radiography and transthoracic echocardiography results were normal, without any evidence of heart failure, wall motion abnormalities, interventricular septum thinning, or bilateral hilar lymphadenopathy. A temporary pacemaker was implanted, followed by a permanent pacemaker. Chest computed tomography with contrast enhancement did not show abnormalities, including patent coronary arteries, lymph node adenopathy, and pulmonary abnormalities. Thus, an MRI conditional dual chamber pacemaker and leads were implanted. Six weeks following the implant, a cardiac MRI was performed to test for cardiac sarcoidosis. Although cine imaging showed normal left and right ventricular function, late gadolinium enhancement demonstrated multiple enhanced uptakes. Based on the results of the cardiac MRI, PET, and gallium scintigraphy, the most likely diagnosis was cardiac sarcoidosis. Although no abnormal findings were found on physical examination, blood work, chest radiography, and transthoracic echocardiogram, multiple regions of delayed enhancement were observed in the cardiac MRI. Thus, MRI conditional pacemakers are a useful tool for diagnosing cardiac sarcoidosis and early therapeutic intervention