Surviving complications of retroperitoneal fibrosis with intermittent relapses over a period of 20 years: case report and literature review / Sobrevivir a las complicaciones de la fibrosis retroperitoneal con recaídas intermitentes durante un período de 20 años: reporte de un caso y revisión de la literatura

Idiopathic retroperitoneal fibrosis is a rare fibro-inflammatory disease of varied etiology which usually originates around aorta and spreads caudally along Iliac vessels into adjacent retroperitoneum causing ureteral obstruction as the most frequent complication.A 53-year-old male patient presented with complaint of mild pain in both the legs off and on. On investigating further, we found that he had been struggling with intermittent relapses every 3-4 years for last 20 years since he was first diagnosed with Idiopathic Retroperitoneal Fibrosis. He was 33-year-old when he first developed the symptoms of anuria for 48 hours and was diagnosed with Idiopathic retroperitoneal fibrosis. This was followed by atrophy of left kidney and hypertension 6 years later, then hypothyroidism after another 3years and finally involvement of Inferior Vena Cava and acute Deep Vein Thrombosis of lower limbs after another 3-4 years. His deep vein thrombosis was well managed in time. He was put on glucocorticoids everytime he had a relapse and a complication.We did a review of literature to understand recent advances about its pathogenesis, diagnosis, investigations and management. We searched in PubMed using terms like retroperitoneal fibrosis alone and in combination with related terms such as Inferior Vena Cava thrombosis, Deep Vein Thrombosis, Tamoxifen, Methotrexate. This case is unique as it is very rare to find acute Deep Vein Thrombosis in Idiopathic retroperitoneal fibrosis without development of any collaterals when Inferior Vena Cava lumen is compromised to almost complete obstruction.After a follow up of 20 years patient is doing well in terms of physical activity and psychological wellbeing with anti-hypertensives, thyroxine and anti-coagulants. Is the disease-free interval actually free of the disease or it just subsided with immunosuppressants to become active after some time?

La fibrosis retroperitoneal idiopática es una enfermedad fibroinflamatoria rara, de etiología variada que generalmente se origina alrededor de la aorta y se propaga caudalmente a lo largo de los vasos ilíacos en retroperitoneo adyacente causando obstrucción ureteral como la complicación más frecuente.Reportamos el caso de un paciente varón de 53 años que se presentó con un dolor leve en ambas piernas. Al investigar más a fondo, descubrimos que había estado luchando con recaídas intermitentes cada 3-4 años durante los últimos 20 años desde que se le diagnosticó por primera vez fibrosis retroperitoneal idiopática. Tenía 33 años cuando desarrolló por primera vez los síntomas de anuria durante 48 horas y se le diagnosticó fibrosis retroperitoneal idiopática. Esto fue seguido por atrofia del riñón izquierdo e hipertensión 6 años después, luego hipotiroidismo después de otros 3 años y finalmente afectación de la vena cava inferior y trombosis venosa profunda aguda de las extremidades inferiores después de otros 3-4 años. Su trombosis venosa profunda se controló bien a tiempo. Le recetaron glucocorticoides cada vez que tenía una recaída y una complicación.Hicimos una revisión de la literatura para comprender los avances recientes sobre su patogenia, diagnóstico, investigaciones y manejo. Se realizaron búsquedas en PubMed utilizando términos como fibrosis retroperitoneal sola y en combinación con términos relacionados como trombosis de la vena cava inferior, trombosis venosa profunda, tamoxifeno, metotrexato. Este caso es único, ya que es muy raro encontrar trombosis venosa profunda aguda en fibrosis retroperitoneal idiopática sin desarrollo de colaterales cuando la luz de la vena cava inferior está comprometida hasta una obstrucción casi completa.Después de un seguimiento de 20 años, el paciente se encuentra bien en términos de actividad física y bienestar psicológico con antihipertensivos, tiroxina y anticoagulantes. ¿El intervalo libre de enfermedad está realmente libre de la enfermedad o simplemente disminuyó con inmunosupresores para activarse después de algún tiempo?

Research advances in retroperitoneal fibrosis / 中华老年医学杂志

Retroperitoneal fibrosis (RPF) is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal organs.RPF can be divided into two types:idiopathic and secondary.Some of the previously diagnosed idiopathic retroperitoneal fibrosis cases belong to the IgG4-related disease category.In order to help clinicians to achieve better diagnostic accuracy,the article reviews current national and international research on this disorder,and summarizes the epidemiological characteristics,the pathogenesis,the clinical manifestations,the microscopicand imaging features,and the most recent advances in the management of RPF.

Research advances in retroperitoneal fibrosis / 中华老年医学杂志

Retroperitoneal fibrosis (RPF) is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal organs.RPF can be divided into two types:idiopathic and secondary.Some of the previously diagnosed idiopathic retroperitoneal fibrosis cases belong to the IgG4-related disease category.In order to help clinicians to achieve better diagnostic accuracy,the article reviews current national and international research on this disorder,and summarizes the epidemiological characteristics,the pathogenesis,the clinical manifestations,the microscopicand imaging features,and the most recent advances in the management of RPF.

Fibrosis retroperitoneal: presentación de un caso y revisión del tema / Retroperitoneal fibrosis: case report and literature review

Introducción: la primera descripción de obstrucción ureteral extrínseca por un proceso de fibrosis retroperitoneal se efectuó en 1905. En poco más de un siglo sólo se han reportado unos 800 casos de esta patología. Caso clínico: se reporta el caso de un paciente femenino de 55 años, que cursa con dolor abdominal difuso de larga evolución, acompañado de náusea, vómito, fiebre de 38°C e hipertensión de 160/100 mmHg. Se realiza tomografía axial computarizada observándose masa retroperitoneal que produce obstrucción de uréteres. Se realiza laparotomía exploradora con toma de biopsia y liberación de uréteres. El análisis histopatológico demuestra la presencia de fibrosis retroperitoneal, una entidad patológica poco frecuente, cuyo diagnóstico requiere un alto índice de sospecha en base a los hallazgos clínicos, de laboratorio y de gabinete. Revisión de la literatura: no se han formulado definiciones claras de los diferentes trastornos que se incluyen en el espectro de la fibrosis retroperitoneal, debido a lo infrecuente de esta enfermedad. Por esta razón hoy se carece de criterios diagnósticos y de una clasificación coherente de las diferentes formas que puede adoptar la enfermedad. Sin embargo, ante la sospecha de una fibrosis retroperitoneal se debe distinguir entre una forma idiopática y una secundaria, por las diferentes implicaciones para el tratamiento. Conclusión: la fibrosis retroperitoneal es un diagnóstico diferencial ante la presencia de dolor abdominal difuso asociado a síntomas de compresión ureteral o de grandes vasos.

Introduction. The first description of extrinsic uretheral obstruction by retroperitoneal fibrosis occurred in 1905. In little more than a century, about 800 cases of this disease have been reported. Case description. We report the case of a female 55 year-old patient who presents with diffuse abdominal pain of long duration, nausea, vomiting, fever of 38°C and hypertension of 160/100 mmHg. A CT scan is performed that shows a retroperitoneal mass that obstructs the urethers. Exploratory laparotomy was performed, urethers were released and biopsy was taken. Pathology analysis showed the presence of retroperitoneal fibrosis, a rare pathological entity whose diagnosis requires a high index of suspicion based on clinical, imaging and laboratory workup. Literature review. There are no clear definitions of the variety of disorders that are included in the spectrum of retroperitoneal fibrosis, due to the rarity of this condition. Consequently, we lack diagnostic criteria and a consistent classification of the different forms that it may adopt. However, when there is suspicion of retroperitoneal fibrosis, the first step is to establish whether it is idiopathic or secondary, as there will be treatment implications. Conclusion. Retroperitoneal fibrosis should be considered in the differential diagnosis whenever diffuse abdominal pain is associated with uretheral or great vessels compression.

A Case of Swelling of Lower Extremity due to Iliac Vein Occlusion with IgG4-Related Idiopathic Retroperitoneal Fibrosis / 日本心臓血管外科学会雑誌

A 62-year-old man presented with a chief complaint of swelling of the left lower extremity. Idiopathic retroperitoneal fibrosis had been diagnosed 6 years previously. Enhanced computed tomography demonstrated occlusion of the left common iliac vein, but without deep vein thrombosis, and a thick dense fibrous layer around the abdominal aorta and in front of the sacrum. After we administered steroid and anticoagulant therapy, remission of the swelling of the left lower extremity was obtained. Presently steroids are being gradually tapered, and he has remained free of recurrence of the swelling of his left lower extremity.

Fibrosis retroperitoneal idiopática: un enigma médico / Retroperitoneal fibrosis: a medical enigma

La fibrosis retroperitoneal es una enfermedad rara de causa desconocida. Fue descrita por primera vez en la literatura inglesa en 1948 por Ormond. Sin embargo, la primera descripción conocida de esta enfermedad fue realizada por el urólogo francés Albarran en 1905. Se caracteriza por una inflamación crónica e inespecífica del retroperitoneo, la cual puede atrapar y obstruir las estructuras que allí se encuentran, particularmente a los uréteres. Es frecuente que la enfermedad se detecte en etapas avanzadas, cuando se manifiesta por falla renal crónica. Los avances en las técnicas imaginológicas y el mayor conocimiento de la entidad han permitido que el diagnóstico se realice con mayor frecuencia en etapas más tempranas, cuando los pacientes aún no se encuentran obstruidos. Como resultado de las observaciones que muestran que los aneurismas inflamatorios de la aorta abdominal difieren de la enfermedad de Ormond sólo en el diámetro de la aorta inflamada, se ha sugerido que ambos síndromes representan variaciones de la misma patología, la cual se ha llamado "periaortitis crónica".

Retroperitoneal fibrosis is an uncommon disease of unclear cause. It was described for the first time in English literature by Ormond, in 1948. However, the first known description of this disease was done by the French urologist Albarran, around 1905. It is characterized by chronic nonspecific inflammation of the retroperitoneum that can entrap and obstruct retroperitoneal structures, notably the urethers. This disease frequently is detected only after severe renal failure is present. However, advances in imaging techniques and increased awareness of the disease have resulted in an earlier diagnosis in unobstructed patients. As a result of the observations that showed that the only difference between an inflammatory aneurysm of the abdominal aorta and Ormond's disease is the diameter of the vessel, it had been suggested that both syndromes represent variations of the same pathology, which has been named "chronic periaortitis".

Idiopathic Retroperitoneal Fibrosis with Myofascial Pain Syndrome: A case report

Idiopathic retroperitoneal fibrosis (IRF) is a rare condition in which a mass of fibrous tissue encompasses the abdominal aorta and the common iliac arteries. Although the histologic findings of IRF are mostly benign, its diagnosis is often delayed, leading to significant physiologic deteriorations, namely renal insufficiency, and poor treatment prognosis. Back pain, which is a common presenting symptom, may lead to confusion in determining the diagnosis of IRF. This report presents a patient with retroperitoneal fibrosis whose diagnosis was delayed due to the concomitant presence of myofascial pain syndrome and lumbar intervertebral disc herniation.

Idiopathic Retroperitoneal Fibrosis Associated with Hashimoto's Thyroiditis in an Old-aged Man

Idiopathic retroperitoneal fibrosis (IRPF) is a rare disease characterized by a retroperitoneal inflammatory proliferative fibrosing process. Hashimoto's thyroiditis is the most common inflammatory condition of the thyroid gland; and is a frequently-occurring autoimmune disorder manifesting predominantly in middle-aged women. We report a rare association of IRPF with Hashimoto's thyroiditis in a 67-year-old man demonstrating good response to steroid therapy.

Efficiency of positron-emission tomography in two cases of idiopathic retroperitoneal fibrosis / 대한내과학회지

Idiopathic retroperitoneal fibrosis is a relatively rare disease that is characterized by the proliferation of fibrous tissue with an inflammatory process in the retroperitoneal cavity. It can cause an obstruction and compression of the ureter, abdominal aorta, and finally progress to renal failure. During the initial stages, the histology shows active inflammation. However, in the late stages, fibrous scarring occurs and the tissue becomes relatively avascular and acellular. Increased 18F-FDG accumulation was observed in our patient at the time of disease onset, which was attributed to the presence of inflammatory cells and actively metabolizing fibroblasts. We describe two patients with idiopathic retroperitoneal fibrosis, who were examined by 18F-FDG-PET, and discuss the efficiency of positron emission tomography in the diagnosis and management of idiopathic retroperitoneal fibrosis patients.

Idiopathic Retroperitoneal Fibrosis Mimicking a Pelvic Tumor: a Case of Pericystitis Plastica

Retroperitoneal fibrosis was first described in 1905 by Albarran, a French urologist, who performed ureterolysis for ureteral compression produced by the disease. However, this disease became an established clinical entity by Ormond's account in the English literature in 1948. Pericystitis plastica has been used the define an extremely rare type of Idiopathic retroperitoneal fibrosis (IRF) constricting the bladder. In this study, we discussed the recovery of 29-year-old woman with pericystitis plastica who was misdiagnosed as pelvic malignancy or a chronic/subacut pelvic inflammation at the first evaluation.

A case of idiopathic retroperitoneal fibrosis with chronic renal failure undergoing maintenance hemodialysis / 대한내과학회지

The idiopathic retroperitoneal fibrosis (IRF) is an inflammatory and fibrosing process that can be complicated by periureteral encasement, ureteral obstruction and subsequent chronic renal failure if left untreated. Unfortunately, treatment is often delayed due to the nonspecific nature of the presenting signs and symptoms. The treatment of IRF is controversial. Recently, surgical correction of IRF attempted open surgical treatment or using laparoscopy before severe renal damage.We experienced a case of IRF with chronic renal failure. Serum creatinine on admission was 22.1 mg/dL, and he was treated with percutaneous nephrostomy. Due to uremic symptom and progressive elevated serum creatinine, he was started hemodialysis. We present the case with a review of literature.

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.