Presence Of Lymphoplasmacytic Infiltrate Research Articles

Differential diagnosis of IgG4-related disease

IgG4-related disease is a chronic autoimmune fibro-inflammatory disease characterized by the presence of lymphoplasmacytic infiltrate, storiform fibrosis, obliterating phlebitis, increased number of IgG4+ cells in tissue, and, in most cases, an elevated serum IgG4 level. This disease often affects the pancreas, salivary glands and lymph nodes, but can involve almost any tissue. Its etiology is still unclear, the central role in the pathogenesis belongs to B-lymphocytes, T2-helpers, interleukins 1-β, 4, 5, 10, 13 and tumor growth factor 1-β. The ambiguous clinical picture and frequent simultaneous involvement of several organs make it difficult to diagnose, so biopsy plays a leading role in making a diagnosis. The characteristic microscopic picture, the presence of certain populations of lymphocytes are key criteria in establishing the correct diagnosis.

Mediastinal mass in a patient with IgG4-related disease

Introduction: IgG4-related disease is now considered to result from an imbalance in the regulatory immune system, and it is diagnosed on the basis of clinical and histopathologic evidence such as the presence of lymphoplasmacytic infiltrates, especially increased IgG4-positive plasma cells and fibrotic sclerosing tissue. Case report: We report herein a 45-year-old male patient with mediastinal mass with IgG4-related disease. IgG4-related disease can involve any organ but the most frequently involved sites include pancreato-hepatobiliary tract, salivary glands, and kidneys, generally manifesting as a mass in one or more sites mimicking a neoplasia. In the thoracic region, IgG4-related disease manifests as interstitial lung disease and fibrosing mediastinitis. Mediastinal lesion in our patient was quite resemble to mediastinal tumor derived from anterior mediastinum. This is the first case with mediastinal mass in a patient with this disease. Conclusion: Although very rare, we should rule out this disease as differential diagnosis in patients with anterior homogeneous density mediastinal mass simulating mediastinal tumor.

Recurrent autoimmune hepatitis after orthotopic liver transplantation.

To determine the frequency, risk factors, and consequences of recurrent autoimmune hepatitis after liver transplantation, 41 patients with type 1 disease were monitored after surgery in accordance with a surveillance protocol. Tacrolimus or cyclosporine plus prednisone were administered to each patient, and liver biopsy examinations were performed at least annually according to protocol. Corticosteroid therapy was ultimately discontinued in only 2 patients. Recurrent disease was defined as the presence of lymphoplasmacytic infiltrates in liver tissue in the absence of other causes of allograft dysfunction. Autoimmune hepatitis recurred in 7 patients (17%), and the mean time to recurrence was 4.6 +/- 1 years. Recurrence was asymptomatic in 4 of 7 patients and detected only by surveillance liver biopsy assessment in 2 patients. Histological changes were mild, and there was no progression to cirrhosis during 4.9 +/- 0.9 years of observation. Five-year patient (86% v. 82%; P =.9) and graft (86% v. 67%; P =.5) survival rates were not statistically different between patients with and without recurrent disease. HLA-DR3 or HLA-DR4 occurred more commonly in patients with than without recurrence (100% v. 40%; P =.008) and healthy subjects (100% v. 49%; P =.01). Recurrent disease was unrelated to donor HLA status. In conclusion, recurrence after transplantation for type 1 autoimmune hepatitis is common. Its mild manifestations and favorable prognosis may reflect early detection by a surveillance protocol and/or continuous corticosteroid treatment. HLA-DR3- or HLA-DR4-positive recipients are at risk for recurrence regardless of donor HLA status.