ABSTRACT
Immunoglobulin G4 (IgG4)-related disease (IgG4RD) is a relatively recently recognized entity that is histopathologically characterized by an extensive infiltration of lymphocytes and IgG4-positive plasma cells with dense fibrosis. IgG4RD is now known to affect any organ system, and a few cases of gastrointestinal lesions have also been reported. However, solitary IgG4RD of the stomach is still very rare. Furthermore, as it can mimic malignant conditions, it is important to recognize this disease to avoid unnecessary surgery. Herein, we present a case of IgG4RD presenting as an isolated subepithelial mass in the stomach.
ABSTRACT
Immunoglobulin G4 (IgG4)-related disease (IgG4RD) is a relatively recently recognized entity that is histopathologically characterized by an extensive infiltration of lymphocytes and IgG4-positive plasma cells with dense fibrosis. IgG4RD is now known to affect any organ system, and a few cases of gastrointestinal lesions have also been reported. However, solitary IgG4RD of the stomach is still very rare. Furthermore, as it can mimic malignant conditions, it is important to recognize this disease to avoid unnecessary surgery. Herein, we present a case of IgG4RD presenting as an isolated subepithelial mass in the stomach.
Subject(s)
Fibrosis , Granuloma, Plasma Cell , Immunoglobulins , Lymphocytes , Plasma Cells , Stomach , Unnecessary ProceduresABSTRACT
Immunoglobulin G4 (IgG4)-related kidney disease (IgG4-KD) has recently been demonstrated to be an important part of IgG4-related sclerosing disease (IgG4-SD). However, since IgG4-KD is still relatively unfamiliar to radiologists and physicians as compared to IgG4-SD involving other organs, it could, therefore, be easily missed. In this article, we present a comprehensive pictorial review of IgG4-KD with regards to the imaging spectrum, mimickers, and clinicopathologic characteristics, based on our clinical experience with 48 patients during the past 13 years, as well as a literature review. Awareness of the broad imaging spectrum of IgG4-KD and differential diagnosis from its mimickers will thus facilitate its early diagnosis and treatment.
Subject(s)
Immunoglobulin G/metabolism , Kidney Diseases/pathology , Adult , Aged , Aged, 80 and over , Autoimmune Diseases/diagnostic imaging , Autoimmune Diseases/pathology , Female , Humans , Kidney Diseases/diagnostic imaging , Kidney Diseases/drug therapy , Magnetic Resonance Imaging , Male , Middle Aged , Steroids/therapeutic use , Tomography, X-Ray ComputedABSTRACT
Immunoglobulin G4 (IgG4)-related kidney disease (IgG4-KD) has recently been demonstrated to be an important part of IgG4-related sclerosing disease (IgG4-SD). However, since IgG4-KD is still relatively unfamiliar to radiologists and physicians as compared to IgG4-SD involving other organs, it could, therefore, be easily missed. In this article, we present a comprehensive pictorial review of IgG4-KD with regards to the imaging spectrum, mimickers, and clinicopathologic characteristics, based on our clinical experience with 48 patients during the past 13 years, as well as a literature review. Awareness of the broad imaging spectrum of IgG4-KD and differential diagnosis from its mimickers will thus facilitate its early diagnosis and treatment.
Subject(s)
Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Autoimmune Diseases/pathology , Immunoglobulin G/metabolism , Kidney Diseases/drug therapy , Magnetic Resonance Imaging , Steroids/therapeutic use , Tomography, X-Ray ComputedABSTRACT
It is well known that immunoglobulin G4 (IgG4)-related sclerosing disease usually occurs in the pancreas, bile duct and gall bladder, but not in the mediastinum, trachea or superior vena cava (SVC). In this case, a patient underwent mediastinal mass excision and trachea resection and repair for a mediastinal and intratracheal mass 15 years ago. This mass was diagnosed postoperatively as an inflammatory pseudotumour (plasma cell granuloma). Subsequently, a mass was found to have recurred in the SVC. We performed a mass excision and innominate vein to the right atrium auricle bypass operation. The mass was diagnosed as IgG4-related sclerosing disease. This patient is now disease and recurrence free.
Subject(s)
Immunoglobulin G , Mediastinal Neoplasms , Mediastinum , Sclerosis , Trachea , Aged , Female , Humans , Mediastinal Neoplasms/diagnostic imaging , Mediastinal Neoplasms/pathology , Mediastinal Neoplasms/surgery , Mediastinum/diagnostic imaging , Mediastinum/pathology , Mediastinum/surgery , Radiography , Sclerosis/diagnostic imaging , Sclerosis/pathology , Sclerosis/surgery , Trachea/diagnostic imaging , Trachea/pathology , Trachea/surgery , Vena Cava, Superior/diagnostic imaging , Vena Cava, Superior/pathology , Vena Cava, Superior/surgeryABSTRACT
Immunoglobulin G4 (IgG4)-related sclerosing disease is a systemic disease characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration in various organs. We described the imaging findings of an IgG4-related inflammatory pseudotumor in the urethra. The urethral mass showed isoattenuation on unenhanced CT images, delayed enhancement on enhanced CT images, iso- to slight hyper-intensity on T1 and T2 weighted magnetic resonance images, diffusion restriction on diffusion weighted images, and heterogeneously low echogeneity on ultrasonography.
Subject(s)
Granuloma, Plasma Cell/diagnosis , Immunoglobulin G/immunology , Urethral Diseases/diagnosis , Aged , Autoimmune Diseases/diagnosis , Female , Humans , Pancreatitis/diagnosis , Pancreatitis/immunology , Sclerosis , Urethral Diseases/immunologyABSTRACT
Immunoglobulin G4 (IgG4)-related sclerosing disease is a systemic disease characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration in various organs. We described the imaging findings of an IgG4-related inflammatory pseudotumor in the urethra. The urethral mass showed isoattenuation on unenhanced CT images, delayed enhancement on enhanced CT images, iso- to slight hyper-intensity on T1 and T2 weighted magnetic resonance images, diffusion restriction on diffusion weighted images, and heterogeneously low echogeneity on ultrasonography.
