Autoimmune pancreatitis metachronously associated with retroperitoneal fibrosis with IgG4-positive plasma cell infiltration

Retroperitoneal fibrosis is an uncommon disorder characterized by the formation of a dense plaque of fibrous tissue in the retroperitoneum, and its etiology remains unknown. Autoimmune pancreatitis is a rare type of chronic pancreatitis characterized by fibrosis with abundant infiltration of IgG4-positive plasma cells and lymphocytes and obliterative phlebitis in the pancreas. We present a case of autoimmune pancreatitis that developed 10 mo after the occurrence of retroperitoneal fibrosis. Histological findings of the resected retroperitoneal mass were marked periureteral fibrosis with abundant infiltration of IgG4-positive plasma cells and lymphocytes and obliterative phlebitis. These findings suggest a common pathophysiological mechanism for retroperitoneal fibrosis and autoimmune pancreatitis in this case. Some cases of retroperitoneal fibrosis might be a retroperitoneal lesion of IgG4-related sclerosing disease.     

Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis

Sclerosing pancreatitis is associated with raised concentrations of IgG4. We treated 22 patients with sclerosing pancreatitis, and identified and followed-up three with concomitant hydronephrosis caused by ureteral mass, later diagnosed as retroperitoneal fibrosis. We histologically examined the ureteral and pancreatic lesions of these patients and noted abundant infiltration of IgG4-bearing plasma cells in both tissues. Treatment with corticosteroids lowered serum concentrations of IgG4. IgG4 might also have a pathological role in a systemic fibrosing process that includes pancreatic and retroperitoneal lesions.     

Autoimmune pancreatitis with retroperitoneal fibrosis which responded to steroid therapy but was complicated with refractory renal dysfunction

A 58-year-old male had been diagnosed as having autoimmune pancreatitis (AIP) from the results of serological examinations and image findings. He was treated with prednisolone (PSL) for 3.5 months. Fifteen months later, follow-up CT revealed the main pancreatic duct (MPD) dilatation in the pancreas body to tail and right hydronephrosis caused by complicated retroperitoneal mass. We diagnosed him as having recurrent AIP with retroperitoneal fibrosis, and restarted PSL treatment. After one month, Examinations indicated amelioration of the MPD dilatation and right hydronephrosis, but not the right renal failure. This case indicates the importance of maintenance of PSL treatment.     

Autoimmune pancreatitis associated with biliary stricture,immune thrombocytopenic purpura and lung fibrosis

We report an autoimmune pancreatitis case with diverse manifestation of biliary stricture, lung ?brosis and immune thrombocytopenic purpura. The patient was a 70‐year‐old man who presented with jaundice. Abdominal ultrasonography and computed tomography revealed enlargement of the entire pancreas and endoscopic retrograde pancreatography showed diffuse irregular narrowing of the main pancreatic duct. Endoscopic retrograde cholangiography revealed a band‐like stricture of the bile duct in the hilar hepatic region with intrahepatic duct dilation. Chest X‐rays and computed tomography revealed interstitial ?brosis of the bilateral lower lungs. Thrombocytopenia and purpura appeared. Antinuclear antibody, anti‐Ro (SS‐A) and anti‐La (SS‐B) antibodies and rheumatoid factor were positive. All manifestations responded well to steroid therapy. From the present case it would appear that a multitude of manifestations are linked within one syndrome. If we encounter patients with autoimmune pancreatitis, we should not focus on a disease but rather on the possibility of a syndrome complex.     

Panhypopituitarism associated with severe retroperitoneal fibrosis

A 43-year-old man, with a history of central diabetes insipidus diagnosed 3 years previously, complained about reduced libido. An MRI scan showed a suprasellar lesion just below the supraoptic recess of the third ventricle. A stereotactically guided biopsy revealed fibrous glia, but no other specific tissue and no inflammatory cells. Two months later the patient presented with fatigue and muscular weakness. Tertiary adrenal failure and hypothyroidism were diagnosed by endocrine function tests and therapy with levothyroxine and hydrocortisone was started. Another 2 months later the patient was admitted with giddiness, nausea, peripheral oedema and oliguria. Radiological imaging and an open transperitoneal kidney exploration showed severe fibrosis around both ureters. Histological examination confirmed the diagnosis of idiopathic retroperitoneal fibrosis. Presumably the suprasellar tumour was the first manifestation of retroperitoneal fibrosis. Once the diagnosis 'idiopathic retroperitoneal fibrosis' is confirmed, fibrotic manifestations and complications involving extra-retroperitoneal tissues including the endocrine system, should be sought.     

Autoimmune pancreatitis associated with a large pancreatic pseudocyst

INTRODUCTIONAutoimmune pancreatitis (AIP) is a benign disease that responds well to steroid treatment. Characteristics in-clude radiological evidence of an irregular narrowing of the pancreatic main duct and a diffuse enlargement of the pancreas, together…     

Primary biliary cirrhosis associated with idiopathic retroperitoneal fibrosis

Idiopathic retroperitoneal fibrosis (IRF) and primary biliary cirrhosis (PBC) are distinct clinical disorders which rarely occur in the same patient. We report a 79-year-old man with the coexistence of both conditions. The patient had antibodies to both centromere and mitochondria, as indicated by indirect immunofluorescence. Diagnoses of IRF and PBC were confirmed histologically. Although the association between IRF and PBC is obscure, IRF may be involved in many autoimmune diseases associated with PBC. Received: September 3, 1999 / Accepted: December 17, 1999     

Autoimmune pancreatitis with pseudocysts

A 47-year-old woman was admitted to our hospital with complaints of fever, upper abdominal pain, and back pain. The serum amylase, C-reactive protein (CRP), and IgG (especially IgG₄) were elevated, and abdominal computed tomography (CT) revealed diffuse enlargement of the pancreas and pseudocysts. Endoscopic retrograde pancreatography (ERP) revealed diffuse irregular narrowing of the main pancreatic duct. Histopathological examination of the pancreatic tissue showed fibrotic change with lymphocytic infiltration. Based on these findings, we diagnosed this case as a case of autoimmune pancreatitis. This case also fully satisfied the diagnostic criteria for autoimmune pancreatitis established by the Japan Pancreas Society in 2002. Few reports have been published on cases of autoimmune pancreatitis complicated by the formation of pseudocysts in the pancreas. We, therefore, report this case here to emphasize that cases of autoimmune pancreatitis can be complicated by the development of pseudocysts.     

Hashimoto's thyroiditis and Graves' disease associated with retroperitoneal fibrosis.

Retroperitoneal fibrosis is a rare disease of uncertain pathogenesis. However, its possible association with several immunopathologic conditions, the possibility of systemic involvement by the fibrous process, the presence of various autoantibodies, and the frequent response to immunosuppressive treatment all support an autoimmune pathogenesis. Riedel's thyroiditis is a rare disease the pathogenesis of which is also thought to be immune-mediated based on its optimal response to steroids; Riedel's thyroiditis is also frequently reported in association with retroperitoneal fibrosis. We describe here two cases of autoimmune thyroid disease associated with retroperitoneal fibrosis, the first with features of primary myxedema, the second of primary thyrotoxicosis. Histology of retroperitoneal fibrosis is documented and it is compatible with an immunopathologic condition. Thus, these two cases add further support to the hypothesis of an autoimmune pathogenesis of retroperitoneal fibrosis and indicate the importance of carefully monitoring for the development of other autoimmune disorders, i.e., of the thyroid gland, in patients with retroperitoneal fibrosis.