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?

Microhematuria en atención primaria, no siempre es lo que parece. La importancia de la ecografía como método diagnóstico / Microhaematuria in primary health care: It is not always what it seems. The importance of ultrasound as a diagnostic method

No disponible

Clinical features of hydronephrosis induced by retroperitoneal fibrosis: 17 cases reports / 北京大学学报(医学版)

OBJECTIVE@#To investigate the clinical features and outcome of hydronephrosis induced by retroperitoneal fibrosis (RPF), and to evaluate the effect of corticosteroid based therapy combined with surgical intervention of ureteral obstruction.@*METHODS@#A total of 17 RPF patients with hydronephrosis hospitalized in Peking University International Hospital from May 2016 to December 2019 were analyzed retrospectively.@*RESULTS@#The median age was 56 (53, 65) years, the male to female ratio was 2.4 : 1, and the disease duration was 4.00 (0.83, 8.00) months. The initial symptoms included back pain (9 cases), abdominal pain (6 cases), oliguria (2 cases) and lower limb edema (3 cases). Eight patients presented left hydronephrosis, 1 right hydronephrosis and 8 bilateral hydronephrosis. C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were both elevated in 13 patients (76.5%, n=17). Immunoglobin (Ig) G4 increased in 5 cases (29.4%, n=17). IgG, IgE and IgA increased in 4 cases (30.8%, n=13), 4 cases (30.8%, n=13) and 1 case (7.7%, n=13), respectively. Among 12 patients who underwent biopsy, 3 patients were diagnosed with IgG4-relate disease. The level of IgG4 in the tissues varied, 6 cases expressed less than 10 per high power field (HPF) or no expression (50.0%). Only 2 cases expressed 10-30/HPF (16.7%), and 4 cases revealed more than 30/HPF (33.3%). Among the 17 patients with ureteral obstruction, no urinary drainage procedure was needed in 4 patients who had mild ureteral obstruction, whereas, ureteral stenting was carried out in the other 13 cases before drug treatment. Time was too short to evaluate the effect of urinary drainage procedures in 4 patients. For the rest, ureterolysis had to be performed in 3 cases after failed ureteral stent insertion. Successful drain removal was accomplished in all of these 9 patients and the mean time to drain removal was (6.7±3.0) months. In addition, 10 patients had complete medical records after an average follow-up time of 5 (3-13) months. Levels of ESR, CRP, IgG4, IgG, IgE, IgA were 54.0 (36.3, 98.5) mm/h, 26.8 (8.7, 53.0) mg/L, 1.34 (0.55, 3.36) g/L, 16.3 (13.0, 21.1) g/L, 40.5 (31.4, 203.0) IU/mL, 2.51 (1.82, 3.25) g/L at baseline, which all decreased predominantly after treatment. ESR, CRP, IgG4, IgG, IgE and IgA dropped by 38.5 (23.5, 54.3) mm/h (P < 0.01), 23.0 (5.5, 52.0) mg/L (P < 0.05), 0.92 (0.40, 2.85) g/L (P < 0.01), 6.5 (1.7, 9.1) g/L (P < 0.05), 23.7 (4.8, 162.0) IU/mL (P < 0.05) and 0.77 (0.32, 1.26) g/L (P < 0.05), respectively. Size of mass measured by CT/MRI imaging became smaller significantly and hydronephrosis relieved.@*CONCLUSION@#Onset of RPF is insidious and lack of specific initial symptoms. Corticosteroid based therapy combined with surgical intervention of relieving obstruction is effective.

Fibrosis retroperitoneal nodular asociada a IgG4 como diagnóstico diferencial en tumores retroperitoneales. Reporte de un caso / Retroperitoneal fibrosis associated with immunoglobulin IgG4-related disease in the differential diagnosis in retroperitoneal tumors. Case report

Paciente de 55 años de edad con cuadro de dolor en flanco izquierdo irradiado a región lumbar de 4 meses de evolución, con tomografía axial computarizada que reporta tumoración quística en región retroperitoneal que comprime uréter y sistema pielocaliceal izquierdo en contacto con cuerpo y cola de páncreas. Es intervenida quirúrgicamente y se encuentra en reporte patológico definitivo fibrosis retroperitoneal nodular asociada a IgG4; con Ki67 positivo en centros germinales (5%) e IgG4 positivo (40 células plasmáticas en 3 campos de 40x) por inmunohistoquímica. La fibrosis retroperitoneal nodular es una enfermedad poco frecuente, de evolución paulatina con excelente respuesta al manejo con esteroides. El tratamiento quirúrgico se reserva para casos que comprometen estructuras adyacentes, por lo que el identificarlo al estudiar una tumoración retroperitoneal conlleva un mejor pronóstico y sobrevida

The patient was a 55-year-old woman with a 4-month history of pain in left flank that irradiated to the lumbar region. Computed tomography revealed a cystic tumor in the retroperitoneal region that compresses the ureter and left renal pelvis in contact with pancreatic body and tail. Surgery was performed and the definitive pathological report diagnosed nodular retroperitoneal fibrosis associated with IgG4; Ki67-positive in germinal centers (5%) and IgG4-positive (40 plasma cells in 3 fields of 40x) by immunohistochemistry. Retroperitoneal fibrosis is a rare disease, that develops gradually and has an excellent response to steroid management. Surgical treatment is reserved for cases that compromise adjacent structures. Thus, identifying it when studying a retroperitoneal tumor leads to better prognosis and survival

Hipofisitis linfoplasmocitaria y fibrosis retroperitoneal asociadas a un síndrome poliglandular autoinmune: enfermedades relacionadas con IgG4. Caso clínico / Hypophysitis and retroperitoneal fibrosis associated with autoimmune polyglandular syndrome and IgG4-related disease: report of one case

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.

Actualización del diagnóstico y tratamiento de los aneurismas de aorta abdominal inflamatorios / Update in diagnosis and treatment of inflammatory abdominal aortic aneurysm

Los aneurismas de aorta abdominal inflamatorios son una entidad diferente de los aneurismas de aorta ateroscleróticos. Esta entidad se caracteriza por el engrosamiento de la pared aórtica, está asociada a fibrosis retroperitoneal y a adherencias de los órganos circundantes, como los uréteres y la vena cava. Suele afectar a varones sexagenarios y fumadores, y la clínica típica es una tríada consistente en dolor abdominal, fiebre y elevación de la velocidad de sedimentación eritrocitaria. El angio-TC es el gold standard en el diagnóstico, ya que permite la visualización del aneurisma, con una hipertrofia de tejidos blandos periaórticos. En los últimos años se ha propuesto el tratamiento endovascular como una opción eficaz. Sin embargo, la cirugía abierta realizada por cirujanos expertos puede ser una buena solución en casos seleccionados, ya que además permite tratar el atrapamiento ureteral. Con este artículo queremos hacer un repaso sobre la última evidencia en diagnóstico y tratamiento de esta entidad

Inflammatory abdominal aortic aneurysm is a distinct type of abdominal aortic aneurysm. This disease usually affects men in their 60's, with tobacco use as usual finding. Periaortic wall thickness with retroperitoneal fibrosis, and adhesion to adjoining organs such as ureters or inferior vena cava, are also common findings. Clinical symptoms found in this disease are as follows: abdominal or back pain, fever, and elevated erythrocyte sedimentation rate. Computed tomography scan with contrast enhancement is the gold standard for the diagnosis, showing an infrarrenal aortic aneurysm with a soft tissue inflammatory rind. In the past few years endovascular treatment has been proposed as an effective option. However, open surgery performed by expert surgeons may be a good solution in selected cases, since it also enables ureteral entrapment to be treated. A review of the latest evidence in the diagnosis and treatment of this condition is presented in this article

Retroperitoneal IgG4-related sclerosing disease mimics renal pelvic cancer on 18F-FDG PET/CT / Enfermedad esclerosante retroperitoneal relacionada con IgG4 simulando un cáncer de pelvis renal en PET/TC 18F-FDG

No disponible

Síndrome de Erdheim-Chester con periaortitis / Erdheim-Chester syndrome with periaortitis

No disponible

Tumor solitario fibroso extrapleural / Extrapleural solitary fibrous tumor

No disponible

Aneyaculación como síntoma inicial atípico en un paciente con fibrosis retroperitoneal idiopática / Anejaculation as an atypical initial symptom in a patient with retroperitoneal idiopathic fibrosis

La fibrosis retroperitoneal es una enfermedad rara de causa desconocida. Fue descrita por primera vez en la literatura inglesa en 1948 por Ormond. 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. En algunas ocasiones se han documentado trastornos de la eyaculación, siendo este un síntoma poco frecuente. La histología parece necesaria para un diagnóstico de certeza, no siendo imprescindible por el alto rendimiento de las pruebas diagnósticas. Su importancia radica en su diagnóstico precoz e inicio de tratamiento para impedir una evolución tórpida y sus complicaciones asociadas. Presentamos el caso de un paciente varón de 46 años con el diagnóstico de fibrosis retroperitoneal idiopática, que comienza con aneyaculación e hidronefrosis bilateral como síntomas principales (AU)

Retroperitoneal fibrosis is an uncommon disease of unclear cause. It was described for the first time in English literature by Ormond in 1948. It is characterised by chronic non-specific inflammation of the retroperitoneum that can entrap and obstruct retroperitoneal structures, notably the ureters. In some rare instances, it has been coupled with ejaculatory disorders. Histology would appear to be required for accurate diagnosis, but it does not affect the efficiency of diagnostic tests and procedures. Its importance lies in early diagnosis and early treatment to prevent a negative prognosis and further complications. We present the case of a 46-year-old male patient with a diagnosis of idiopathic retroperitoneal fibrosis, whose first symptoms were anejaculation and bilateral hydronephrosis (AU)

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.

Fibrosis idiopática como causa de uropatía obstructiva / Idiopathic fibrosis as the cause of obstructive uropathy

No disponible

Aneurismas inflamatorios de la aorta abdominal / Inflammatory abdominal aortic aneurysms

Los aneurismas inflamatorios de la aorta abdominal constituyen una entidad diferenciada de etiología desconocida que se caracteriza por una gruesa pared, superficie nacarada y la presencia de tejido fibrótico perianeurismático que atrapa las estructuras vecinas, especialmente los uréteres. Característicamente, aparecen en varones, muy fumadores y con edad media de 65 años y son mucho más sintomáticos que los aneurismas no inflamatorios, siendo lo más frecuente la aparición de dolorabdominal, pérdida de peso y elevación de la VSG. La prueba de referencia para su diagnóstico es el TAC, con una imagen típica en cuatro capas (luz aórtica, trombo mural, pared engrosada y masa periaórtica retroperiteneal). Su tratamiento se basa en tres pilares: exclusión del aneurisma (cirugía abierta o endovascular), actuación frente la fibrosis (corticoides einmunosupresores) y manejo de las complicaciones por afectación de órganos adyacentes. La cirugía abierta presenta grandesdificultades técnicas con una alta morbimortalidad, en cambio, el tratamiento endovascular constituye una opción no sólo posible, sino efectiva y segura, con unos resultados similares al tratamiento de los aneurismas no inflamatorios. El pronóstico de estos pacientes depende de la evolución de la fibrosis retroperitoneal, la cual es muy variable tanto en la cirugíaabierta como endovascular. Las series de que disponemos son aún pequeñas y de cortos períodos de seguimiento, por lo que es necesario hacer un seguimiento a largo plazo de la evolución de la fibrosis post EVAR para llegar a conclusiones más definitivas (AU)

Inflammatory abdominal aortic aneurysms constitute a distinct entity of unknown etiology characterized by a thick wall, awhite glistening surface and the presence of inflammatory retroperitoneal scar tissue that traps neighbour structures, especially the ureters. Tipically appear in males and heavy smokers with a mean age of 65 years. They are much more symptomaticthan non-inflammatory aneurysms, especially with the presence of abdominal pain, weigth loss and elevated ESR. The gold standard for diagnosis is TAC, with a typical image in four layers (aortic lumen, intraluminal thrombus, calcified andthickened wall and periaortic retroperitoneal mass). Their treatment is based on three pillars: exclusion of the aneurysm (open surgery and EVAR), treatment of fibrosis (corticosteroids and immunosuppressors) and anagement of complicationsof the neighbour organs involvement. Open surgery presents major technical challenges with high morbidity and mortality.EVAR appears as an option not only feasible but safe and effective, with similar results to the treatment of non-inflammatory aneurysms with EVAR. The prognosis of these patients depends on the development of retroperitoneal fibrosis, which is highly variable in both open and endovascular surgery. The series we have are still small and short periods of follow up, so it is necessary to follow the long-term evolution of fibrosis post EVAR to reach more definitive conclusions (AU)

Fibrosis retroperitoneal postraumática como causa de dolor lumbar / Post traumatic retroperitoneal fibrosis as a cause of low-back pain

We present the case of a male patient with posttraumatic retroperitoneal fibrosis whose main clinical expression was low-back pain. Diagnosis was establis hedusing CAT-scan and MRI, which revealed a largemass of soft tissue that almost entirely enveloped the abdominal aorta. Treatment with 40 mg of prednisone every 24 hours was established. This dose was reduced gradually, and progressive remission of clinical sign sand symptoms was achieved, with a significant improvement of subsequent imaging-test results. Treatment was continued for one year. Two and a half years later the patient remains symptom-free, with no recurrence of his condition (AU)

Se presenta el caso de un paciente varón afecto de una fibrosis retroperitoneal postraumática, a cuyo diagnóstico se llegó a partir de dolor lumbar como síntoma principal. El diagnóstico se efectuó en base a los estudios mediante CT y RM, los cuales demostraron una gran masa de tejido de partes blandas que rodeabanla aorta. El tratamiento consistió en dosis de prednisonaque inicialmente se instauró a 40 mg cada 24 horas, y posteriormente se fue reduciendo de forma gradual hasta la remisión de los signos y síntomas, y consecuentemente de los estudios de imagen. El tratamiento con corticoides se mantuvo durante un año. Dos años y medio el paciente está libre de síntomas sin recidivade su proceso (AU)

A Case of Cap Polyposis Complicated with Idiopathic Retroperitoneal Fibrosis / 대한소화기학회지

An optimal treatment for cap polyposis has not been established. Several treatment approaches, including anti-inflammatory agents, antibiotics, immunomodulators, and endoscopic therapy have been described. Surgical resection of the affected colon and rectum may be indicated for patients with persistent disease. Repeat surgery is indicated in cases of recurrence after surgery. However, symptomatic polyposis may still recur, and spontaneous resolution of cap polyposis is possible. We report a case of recurrent cap polyposis complicated with retroperitoneal fibrosis after inadequate low anterior resection with a positive resection margin. Surgical approaches for the treatment of cap polyposis should be carefully considered before treatment.

A Case of Idiopathic Sclerosing Mesenteritis with Retroperitoneal Fibrosis / 대한소화기학회지

Sclerosing mesenteritis is a rare inflammatory disease of the bowel mesentery. It produces tumor-like masses of the mesentery composed of varying degrees of fibrosis, chronic inflammation, and fat necrosis. It has been described variously as fibrosing mesenteritis, retractile mesenteritis, mesenteric Weber Christian disease, and systemic nodular panniculitis. The etiology and pathogenesis of the disease are as yet unknown, but autoimmune disorder, previous abdominal surgery, trauma, and ischemia could play a role. The clinical features include abdominal pain, vomiting, diarrhea, and constipation. Occasionally, patients with this condition may present with bowel obstruction. Rarely, It can be associated with other idiopathic inflammatory disorders such as retroperitoneal fibrosis, sclerosing cholangitis, and orbital pseudotumors. We report a case of idiopathic sclerosing mesenteritis with retroperitoneal fibrosis in a 58-year-old man.

Síndrome de compresión de vena cava inferior secundario a fibrosis retroperitoneal / Inferior vena cava compression syndrome secondary to retroperitoneal fibrosis

Se estudió un paciente de 57 años de edad, de tez negra, con edemas en miembros inferiores relevantes por su volumen y extensión. Se realizó la discusión clínica la cual orientó hacia la búsqueda de una lesión o tumor retroperitoneal asociado a un síndrome de compresión u obstrucción de vena cava inferior y se comprobó imagenológicamente. La confirmación histológica se obtuvo por vía quirúrgica

We studied a 57 year-old patient of black complexion, with swelling of the lower limbs which was relevant due to its mass and extension. The clinical discussion guided us to search for a lesion or retroperitoneal tumor associated with a compression syndrome or obstruction of the inferior vena cava, which was determined radiologically. Histological confirmation was obtained through surgery