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Objectives: Retroperitoneal fibrosis (RPF) is an uncommon disease due an inflammatory condition and deposit of fibrotic tissue that involves the retroperitoneal area over the lower four lumbar vertebrae. Very few epidemiologic studies exist that accurately characterize the incidence and prevalence of the disease., Materials and Methods: A review of the English language literature was performed using the MEDLINE combining the keywords: "retroperitoneal fibrosis", "Ormond´ disease", "IG4 related disease". Additionally, hand search of bibliographies of included studies and previous reviews was also performed to include additional information., Results: RPF develops insidiously, because the initial symptoms are non-specific. Pain is the most common presenting symptom. Various radiological diagnostic methods are used in the diagnosis of retroperitoneal fibrosis. Contrast-enhanced computerized tomography (CT) is a useful method for diagnosing retroperitoneal pathologies. Magnetic resonance imaging (MRI) is an important radiological method especially in the diagnosis of fibrotic tissue and in the examination of the retroperitoneal organ relation with fibrous tissue. Nuclear imaging is also a method used in renal function evaluation and patient follow-up. Various medical and surgical treatments would be used in the treatment of retroperitoneal fibrosis. In general, immunosuppressive agents such as corticosteroids, tamoxifen, azothiopurine, cyclophosphamide, cyclosporine, progesterone, mycophenolate mofetil are used in medical treatment. Surgical treatment methods are recommended in cases where medical treatment is not efficient., Conclusion: Unfortunately, despite a recent surge in the number of publications on this topic, a few progress has been made in our understanding of the classification, pathophysiology, and, most importantly, the most appropriate treatment for this disease.

We report a 23 year old woman presenting with a nephrotic syndrome due to minimal change disease, central diabetes insipidus, primary hypothyroidism, vitiligo and universal alopecia. Eleven years later, she presented secondary amenorrhea due to hypogonadotropic hypogonadism, with mild hyperprolactinemia and central adrenal insufficiency. A magnetic resonance imaging of the sella turcica showed a pituitary mass with suprasellar extension that was resected using a transsphenoidal approach. Pathology confirmed the presence of a lymphoplasmacytic hypophysitis. She needed a second surgical resection due to mass growth and neuro-ophthalmologic impairment. One year later, systemic lupus erythematosus, arterial hypertension and type 2 diabetes mellitus were diagnosed. Two years later, due to back pain, constipation and renal failure, retroperitoneal fibrosis was found, satisfactorily treated with glucocorticoids and colchicine. Hence, this clinical vignette shows the coexistence of autoimmune polyglandular syndrome with retroperitoneal fibrosis and lymphoplasmacytic hypophysitis. Tissue analysis showed the presence of IgG4 producing plasma cells in the pituitary and retroperitoneum, which constitute a basis for the diagnosis of IgG4 related disease.

Introduction: Autoimmune pancreatitis is a kind of chronic pancreatitis characterized by the presence of lymphoplasmacytic infiltration and severely elevated serum IgG and IgG4, which has been associated to many extrapancreatic lesions and other autoimmune disorders, leading to the theory of an autoimmune mechanism involved in the pathogenesis of this disease., Case Report: We report the case of a man who simultaneously presented with autoimmune pancreatitis associated with retroperitonal fibrosis, and a lesion of the extrapancreatic bile duct, with total response to corticosteroid treatment for 4 months and absence of recurrence after 24 months of follow-up., Discussion: Autoimmune pancreatitis is a kind of chronic pancreatitis that is probably a part of a systemic autoimmune disease, with retroperitoneal fibrosis and extrapancreatic bile duct lesion being the most commonly associated extrapancreatic lesions. A correct diagnosis and early treatment of this disease may aid in the total resolution of lesions, especially in cases with a low activity grade.

The acute renal failure is a grave pathology, of rapid establishment and relatively frequent in the hospital environment. We can describe three etiological groupS, which are responsible for it, amongst which are emphasized the pre-renal reasons. The obstructive pathology, of minor incidence, increases with the age. It is described the case of a 67-yr-old patient who was admitted in the Nephrology Service because of abrupt decline of the renal function. Among the initial symptoms, he presented arterial hypertension (190/90) and preserved diuresis. Blood analysis: urea 199 mg/dl, creatinine 7.7 mg/dl, without proteinuria. Sonography reported a bilateral ureteral hydronephrosis with simple cyst of possible ischemic origin. In view of the absence of previous biochemical data of renal failure, we considered possible reasons which start with an acute pattern. In initial evaluation, pre-renal etiology was not seen (high blood pressure, right cardiac systole function). The absence of prostatic syndrome and sonography discovery did not justify a diagnosis of urinary tract obstruction. Finally, abdominal-pelvic scan showed a periaortic retroperitoneal mass which included both ureters and appeared to trigger the obstruction. Combined efforts were pursued with the Urology Service, which implanted a bilateral "double J" catheter and later operated surgically on the patient, carrying out an alternating ureterolysis of both ureters. The biopsy manifested a retroperitoneal fibrosis, and the renogram showed a residual renal function of 20% in the right kidney and 80% in the left kidney. Due to the failure of the previous measures and as a last therapeutic recourse when one year had passed from the diagnosis, a continuous regimen with tamoxifen (anti-estrogen drug) in dose of 20 mg/dl each 12 hours was started, which began a progressive remission in the size of the observed mass by scan (CT) and magnetic resonance (MR). The treatment was completed during 12 months and in this time, the levels of blood urea nitrogen and creatinine were reduced gradually too. Finally, at the end of the treatment, the magnetic resonance demonstrate the complete disappearance of the fibrosis.

Retroperitoneal fibrosis is a rare inflammatory disease, in which the fibrosis plate leads to the compression of the affected structures. There are several causes of retroperitoneal fibrosis like specific and unspecific inflammatory diseases. In the specific group of retroperitoneal fibrosis we find the one due to pancreatitis. We do present a rare case of retroperitoneal fibrosis caused by a pancreatitis in a HIV+ patient.

Immediately after ureterolysis in the treatment of idiopathic retroperitoneal fibrosis, we covered the ureter with a large epiploon, forming a sheath with omentum covering the entire ureter from the renal pelvis to the juxtavesical area of the same. This technique revitalizes the ischemic ureter resulting from ureterolysis and protects against the reinclusion of the ureter mobilized inside the sclerolipomatous mass.

Based on 8 cases observed the author attempted to specify the possiblities of diagnosis and therapy of the idiopathic retroperitoneal fibrosis. If the pathogeny is very dark this illness is accompanied by an unspecific chronic inflammatory process. The radiology (U.I.V. and U.P.R.) does permit to affirm the affection. The ureterolysis with bilateral intraperitonization or, in unilateral cases, the extraperitonization of the ureter fixing the peritoneum to the psoas are the most effective surgical methods. They can be combined with the cortico-therapy which permits a recovery of the renal function and improves the ureteral transit.

We report on six patients with with retroperitoneal fibrosis (RPF) who were evaluated preoperatively by CT. Patient scans disclosed a retroperitoneal fibrous layer localized between L4-S2, S3 in four of the six cases (65%). Similarly, varying degrees of uni-or bilateral utero-hydronephrosis were observed in five patients, and venous involvement with collateral abdominal circulation was observed in two. The surgical findings coincided with those of the CT scans, and disclosed that in the two patients with no evidence of a fibrous layer on the scan, this was due to the its being localized to an area in one or both sides of the pelvic cavity. The diagnosis was histologically confirmed as idiopathic retroperitoneal fibrosis in all six cases. We consider CT to be the preoperative method of choice in the diagnosis of RPF because it not only provides information on the status of the urinary system, the involvement of other retroperitoneal structures and the extent of the fibrous layer, it also provides orientation as to the possible etiology of the retroperitoneal fibrosis.