1/8. Multifocal fibrosclerosis presenting as Grave's orbitopathy. Bilateral exophthalmos associated with retroperitoneal and sellar fibrosis.BACKGROUND: Multifocal fibrosclerosis (MF) is a rare disease that may be misdiagnosed as Graves' orbitopathy. The combination of localisations of MF presented here has not been reported before. CASE REPORT: A 44-year-old man was referred with progressive bilateral exophthalmos. CT of his chest and abdomen revealed an intrathoracic and retroperitoneal peri-aortal soft-tissue process with hydronephrosis. Histological examination of orbital masses showed a fibrous process with low inflammatory activity. Later the sella was found to be involved as well. A diagnosis of MF was made. CONCLUSION: MF should be considered in the differential diagnosis of bilateral exophthalmos.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/8. Obstructive nephropathy as a result of retroperitoneal fibrosis: a review of its pathogenesis and associations.retroperitoneal fibrosis is a rare disease, typically with an insidious clinical course. It is thought that this disease process is perhaps an exaggerated reaction to an inciting inflammatory event. In this study, a case of retroperitoneal fibrosis is reported, in which the patient presented with typical symptoms of retroperitoneal fibrosis, along with some atypical vasculitic symptomatology. retroperitoneal fibrosis is a disease process with an unknown etiology, which has been observed to be associated with a number of different possible inciting factors. Two factors that have been documented in the literature as being associated with retroperitoneal fibrosis include the use of beta-blocking agents, and connective tissue disease processes such as systemic lupus erythematosus. The patient discussed was using beta-blocker medication and also had signs and symptoms suggestive of a lupus syndrome. There are no reported cases of the combined association of beta-blocker usage, lupus, and retroperitoneal fibrosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/8. Imaging findings in idiopathic pelvic fibrosis.Two patients presented with ureteric obstruction, and voiding symptoms and constipation, respectively, and were examined by means of intravenous urography and computed tomography. One patient was additionally examined by means of MR tomography. After CT (performed in both patients) and MRT (performed in one patient) had shown a diffuse, contrast-enhancing, infiltrating process in the small pelvis with infiltration of adjacent organs and vessels, surgical biopsy proved the diagnosis of idiopathic pelvic fibrosis. Extension of retroperitoneal fibrosis below the pelvic rim is very rare. Clinical symptoms of pelvic fibrosis are variable and imaging findings may lead to a broad list of differential diagnoses. We present two patients with idiopathic pelvic fibrosis and discuss radiological findings and differential diagnoses of this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/8. retroperitoneal fibrosis with duodenal stenosis.retroperitoneal fibrosis is a rare disease characterized by the formation of dense plaque of fibrous tissue covering the retroperitoneal structures. This disease is commonly presented as ureteral obstruction, but the involvement of duodenum is rare. We report a case of retroperitoneal fibrosis which was complicated with duodenal stenosis and was successfully treated with corticosteroids. A 58-yr-old man, who had history of aorto-iliac bypass graft due to arteriosclerosis obliterans with infrarenal aortic occlusion was admitted to the hospital with abdominal pain and a mass. Abdominal CT scan revealed the periaortic soft tissue mass encircling grafted aorta and stenosis of duodenal third portion. retroperitoneal fibrosis with duodenal stenosis was diagnosed and prednisolone therapy was initiated. Follow-up CT scan showed that the patient responded to prednisolone therapy with eased pain, shrinking periaortic mass, and reduced duodenal stenosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/8. retroperitoneal fibrosis during the course of ulcerative colitis. A simple coincidence?retroperitoneal fibrosis has been described as a rare occurrence during the course of inflammatory bowel disease, mainly Crohn's disease. This is the third report on retroperitoneal fibrosis occurring during the course of ulcerative colitis. A 62-year-old male patient with a 5-year history of ulcerative colitis developed stenosis of the left ureter due to retroperitoneal fibrosis. Treatment consisted in surgically releasing the ureter from the mass and steroids. During a 2.5-year follow-up, renal function was stable and ulcerative colitis in remission. Important aspects of this case are the moderate course of ulcerative colitis, ultrasound confirmation of normal kidney structure before manifestation of fibrosis, hypertension diagnosed four years before retroperitoneal fibrosis, a non-functioning kidney at diagnosis, and reduction of retroperitoneal mass after steroid treatment. retroperitoneal fibrosis, although a rare disease entity should be considered when a patient with ulcerative colitis develops otherwise unexplained renal insufficiency.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/8. 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.- - - - - - - - - - ranking = 2keywords = rare disease (Clic here for more details about this article) |
7/8. A rare case of Riedel's thyroiditis, 6 years after retroperitoneal fibrosis: two diseases with one pathogenesis?We describe a 70-yr-old female patient in whom both a retroperitoneal fibrosis and 6 years later a Riedel's thyroiditis were diagnosed. Both diseases belong to the group of fibrotic diseases called "multifocal fibrosis". retroperitoneal fibrosis is now known to be an auto-allergic reaction to lipid components of the atherosclerotic process in the abdominal aorta, resulting in a fibrotic reaction around it. We hypothesize that similarly Riedel's thyroiditis is in fact a periarteritis of the carotid artery, resulting in cervical fibrosis, secondarily involving the thyroid. This hypothesis would account for the occurrence of these two rare diseases in one patient.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/8. Human fibroblasts in idiopathic retroperitoneal fibrosis express hla-dr antigens.Idiopathic retroperitoneal fibrosis (IRF) is a rare human disease characterized by non-neoplastic fibroblastic proliferation associated with chronic inflammatory cells; its pathogenesis is obscure. We undertook an immunohistochemical study for the expression of hla-dr antigens and other immune-related markers by retroperitoneal proliferating fibroblasts and inflammatory cells from 2 IRF patients. Patterns of immunoreactivity were compared with those expressed by human nodular fasciitis (NF) and granulation tissue. In IRF, most fibroblasts immunostained strongly for hla-dr antigens, whereas fibroblasts in NF and granulation tissue did, not immunostain at all. The fibroblasts did not immunostain for interleukin 2 receptor, C3b receptor, CD-4, CD-8, or Leu-M1 in any of the tissue studied. Most macrophages and lymphocytes in IRF and NF immunostained Strangly for hla-dr antigens. In IRF, the CD-4 and CD-8 immunostained t-lymphocytes appeared equally distributed. The expression of hla-dr antigens by fibroblasts in IRF indicates that this rare disease may indeed be an immune-associated hypersensitivity disorder.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |