RESUMEN
Immunoglobulin G (IgG)4-related pancreatitis, also named as type Ⅰ autoimmune pancreatitis, is recognized as a pancreatic manifestation of IgG4-related disease. It is very hard to differentiate it from pancreatic cancer due to similar clinical manifestations with pancreatic cancer. Diagnosis of IgG4-related pancreatitis mainly relies on pathological examination and imaging examination. Corticosteroids are the main treatment for IgG4-related pancreatitis, and the risk of relapse is still high with steroid taper or following withdrawal of steroids. Based on researches on IgG4-related pancreatitis and our clinical experience, the authors have analyzed and summarized pathogenesis, clinical manifestations, clinical diagnosis, differentiation from pancreatic cancer, clinical treatment of IgG4-related pancreatitis, and suggested that elucidation of its mechanism and induction of immunotherapy may improve its efficacy.
RESUMEN
Immunoglobulin G (IgG) 4-related pancreatitis,also named as type I autoimmune pancreatitis,is recognized as a pancreatic manifestation of IgG4-related disease.It is very hard to differentiate it from pancreatic cancer due to similar clinical manifestations with pancreatic cancer.Diagnosis of IgG4related pancreatitis mainly relies on pathological examination and imaging examination.Corticosteroids are the main treatment for IgG4-related pancreatitis,and the risk of relapse is still high with steroid taper or following withdrawal of steroids.Based on researches on IgG4-related pancreatitis and our clinical experience,the authors have analyzed and summarized pathogenesis,clinical manifestations,clinical diagnosis,differentiation from pancreatic cancer,clinical treatment of IgG4-related pancreatitis,and suggested that elucidation of its mechanism and induction of immunotherapy may improve its efficacy.
RESUMEN
Progressively transformed germinal centers (PTGC) is a benign process characterised by a morphological variant of reactive follicular hyperplasia in lymph nodes. It was recently shown that some cases of PTGC are associated with IgG4-related disease (IgG4-RD) or increased IgG4 plasma cells. Five years ago, a 57-year-old woman presented with enlargement of multiple lymph nodes in the left parotid, submandibular, and neck areas, pathologically diagnosed as PTGC after excisional biopsy. Since then, she has experienced numbness in her extremities, especially the left shoulder and arm, pruritus on the left side of the face and intermittent facial palsy, for which she has been receiving regular symptomatic treatment. Recently the patient developed diabetes mellitus (approximately seven months ago). In routine follow-up scans, a mass was detected in left kidney and magnetic resonance imaging of the abdomen prior to surgery revealed a slightly enhanced bulky mass replacing the pancreatic tail and uncinate process. The mass in left kidney was diagnosed as clear cell renal cell carcinoma, and the pathological features of the pancreatic lesion were those of IgG4-related chronic fibrosing pancreatitis. Retrograde examination of the neck lymph node diagnosed as PTGC showed increased deposition of IgG4-positive plasma cells.