Diffuse thymic fibrosis: Histologic pattern of injury or distinct entity?
Department of Pulmonary and Mediastinal Pathology, Armed Forces Institute of Pathology, Washington, DC 20306, USA.The American journal of surgical pathology (Impact Factor: 5.15). 02/2010; 34(2):211-5. DOI: 10.1097/PAS.0b013e3181c91301
Thymic fibrosis in the absence of another primary thymic lesion, such as a neoplasm or cyst, is unusual. We identified 6 cases of primary extensive thymic fibrosis that developed in 3 men and 3 women, aged 28 to 60 years, mean 48 years. Patients had limited or no symptoms. Two patients had dyspnea and 1 had cough and hemoptysis. One female patient had myasthenia gravis. The lesions measured 3.5 to 17 cm in greatest dimension, mean 8 cm, and were confined to the anterior mediastinum as determined by imaging, intraoperative notes, and/or gross examination. All cases showed diffuse fibrosis with variable collagen deposition, lymphoplasmacytic infiltrates, and involution/atrophy of thymus. One case had rare IgG4-positive plasma cells and focal obliterative phlebitis. The histology showed overlap with that of IgG4-related sclerosing disease, which to our knowledge has not been documented earlier in this location. Although the etiology of the lesions is undetermined, altered immunity and/or infection may play a role.
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Article: IgG4-related sclerosing disease[Show abstract] [Hide abstract]
ABSTRACT: Autoimmune pancreatitis (AIP) is a peculiar type of pancreatitis of presumed autoimmune etiology. AIP is diagnosed currently based on characteristic radiological findings (irregular narrowing of the main pancreatic duct and enlargement of the pancreas) in combination with serological findings (elevation of serum IgG, and IgG4, and presence of autoantibodies), clinical findings (elderly male preponderance, occasional extrapancreatic lesions, and favorable response to steroid therapy), and histopathological findings (dense infiltration of IgG4-positive plasma cells and T lymphocytes with fibrosis and obliterative phlebitis in the pancreas). Based on histological and immunohistochemical examination of various organs of AIP patients, a novel clinicopathological entity of "IgG4-related sclerosing disease" has been proposed. This is a systemic disease that is characterized by extensive IgG4-positive plasma cell and T lymphocyte infiltration of various organs. Clinical manifestations are apparent in organs such as the pancreas, bile duct, gallbladder, salivary or lacrimal gland, and retroperitoneum, where tissue fibrosis with obliterative phlebitis is pathologically induced. AIP is not simply a form of pancreatitis but rather a pancreatic lesion reflecting an IgG4-related sclerosing disease. This disease includes autoimmune pancreatitis, IgG4-related sclerosing cholangitis, IgG4-related cholecystitis, IgG4-related sialadenitis, IgG4-related retroperitoneal fibrosis. In some cases, only 1 or 2 organs are clinically involved, while in others 3 or 4 organs are affected. Serum IgG4 levels and immunostaining with anti-IgG4 antibody are useful in establishing the diagnosis. Since malignant tumors are frequently suspected on initial presentation, IgG4-related sclerosing disease should be considered in the differential diagnosis to avoid unnecessary surgery.Rinsho byori. The Japanese journal of clinical pathology 11/2009; 57(11):1113-9.
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