Immunoglobulin G4-related retroperitoneal fibrosis / Fibrosis retroperitoneal relacionada con IgG4

Abstract Some patients diagnosed with idiopathic retroperitoneal fibrosis could be reclassified as IgG4-related disease (IgG4-RD). Classification criteria have not been uniform and prevalence of IgG4-related retroperitoneal fibrosis (IgG4-RPF) is unknown in our region. We aimed to describe IgG4-RPF frequency relying on criteria published recently and comparing clinical, histopathologic and radiologic features with non-IgG4-RPF. From January, 2005 to December, 2020, nineteen adults with histopathologic diagnosis of idiopathic retroperitoneal fibrosis were included in a dynamic retrospective cohort at Hospital Italiano de Buenos Aires. Pathology slides were reviewed and immunohistochemistry was performed and assessed for each case. We used classification criteria described in 2019 American College of Rheumatology/European League Against Rheumatism to identify IgG4-RD cases. Ten of 19 patients met criteria for IgG4-RPF. Median age was similar in two subsets (61 versus 55, p = 0.2) and both had male predominance. Three out of 10 patients (p = 0.2) featured other manifestations of IgG4-RD in the IgG4-RPF group, and periaortic fibrosis was the most significant finding in images (p = 0.01). Corticosteroids were mostly used as therapy, followed by azathioprine and rituximab. Most patients did not receive specific treatment. IgG4-RPF patients had dense lymphocytic infiltrate and 8 out of 10 showed storiform fibrosis (p = 0.02). IgG4+ cells/hpf and IgG4/IgG ratio were significantly higher (p = 0.01). Over half of the patients in our cohort met the criteria of IgG4-RPF. New criteria may harmonize the identification of IgG4-RD. As IgG4-RD may be reversible at initial stages, these findings may lead to early recognition, treatment and integral follow-up.

Resumen Muchos pacientes con diagnóstico de fibrosis retroperitoneal idiopática (FRI) pueden ser reclasificados como enfermedad relacionada con IgG4 (ER- IgG4). Los criterios diagnósticos no han sido uniformes y la frecuencia de fibrosis retroperitoneal relacionada con IgG4 en nuestra región es desconocida. El objetivo fue describir la frecuencia de ER-IgG4 en pacientes clasificados como FRI y comparar características clínicas, histopatológicas y de laboratorio con aquellos que no reunían criterios de la enfermedad. Se incluyeron 19 adultos en un estudio de cohorte retrospectiva dinámica con diagnóstico anatomopatológico de FRI, en el Hospital Italiano de Buenos Aires, desde enero de 2005 hasta diciembre de 2020. Se revisaron las biopsias y se realizó inmun ohistoquímica en cada una. Se consideró caso al paciente que reunía los criterios de la American College of Rheumatology/European League Against Rheumatism 2019. Diez pacientes reunieron criterios de ER-IgG4. La mediana de edad fue similar en ambos grupos (61 vs. 55, p = 0.2) y en ambos hubo predominio masculino. Tres de 10 pacientes (p = 0.2) tuvieron otras manifestaciones de ER-IgG4 y la fibrosis periaórtica fue el hallazgo más significativo en los estudios por imágenes (p = 0.01). Los corticoides fueron las drogas más utilizadas seguidos por azatioprina y rituximab, pero la mayoría no reci bió tratamiento específico. Todos los pacientes con fibrosis retroperitoneal relacionada con IgG4 presentaron infiltrado linfocitario denso y 8/10 fibrosis estoriforme (p = 0.01), así como las células IgG4+/hpf y ratio IgG4/ IgG fueron significativamente mayores (p = 0.01). Más de la mitad de los pacientes con FRI cumplieron criterios de ER-IgG4. Los nuevos criterios diagnósticos podrían contribuir a homogeneizar la identificación de ER-IgG4. Dado que esta enfermedad puede ser reversible en estadios tempranos, estos resultados promueven aumentar el conocimiento de la entidad para tratamiento precoz y seguimiento integral.

Lymphedema and retroperitoneal fibrosis / 中华风湿病学杂志

Objective:To improve the understanding of the relationship between lymphedema and retroperitoneal fibrosis (RPF).Methods:Four cases with lymphedema and RPF in Beijing Shijitan Hospital Capital Medical University were reported. The diagnosis and treatment were analyzed and discussed.Results:All four patients had lymphedema onset and imaging showed suspicious RPF. One case of non-Hodgkin's lymphoma was confirmed by lymph node biopsy. The malignant lesions were excluded in the other two cases by pathology or positron emission tomography (PET)/computerized tomography (CT). They were proved to be idiopathic retroperitoneal fibrosis after treatment with glucocorticoid combined immunosuppressive agents. Another case was systemic amyloidosis mimicking retroperitoneal fibrosis.Conclusion:Lymphatic involvement in RPF is relatively rare, and the possibility of RPF should be considered when patients develop lymphedema. Even if the initial diagnosis is RPF, we should be wary of tumors or other diseases. Imaging examination should be performed, and tissue biopsy should be used if necessary, so as to facilitate early diagnosis and treatment and improve the prognosis of patients.

Clinical characteristics of retroperitoneal fibrosis:an analysis of 28 cases / 中华全科医师杂志

Clinical data of 28 patients diagnosed with retroperitoneal fibrosis (RPF) in Shanxi Bethune Hospital from September 2014 to September 2020 were retrospectively analyzed. There were 16 males and 12 female with a mean onset age of (56±11) years. The clinical manifestations were lumbago (16/28, 57.14%), abdominal pain (9/28, 32.14%) and back pain (8/28, 28.57%). Inflammatory indexes were elevated in 25 cases (89.29%), and 3 cases had renal insufficiency. Serum IgG4 was elevated in 3 cases (10.71%). Scheel imaging evaluation showed that proportion of type Ⅰ combined with type Ⅲ (abdominal aorta and/or iliac vascular involvement combined with unilateral or bilateral ureteral involvement) was the highest(50.00%, 14/28). followed by. Twenty-seven patients (96.43%) were treated with glucocorticoids and immunosuppressants; 14 patients (50.00%) underwent surgical intervention for ureteral obstruction or hydronephrosis; 6 patients (21.43%) relapsed during hormone withdrawal; 6 patients (21.43%) recovered from the disease and terminated medication; and 13 patients (46.43%) successfully removed the double J tube after lifting ureteral obstruction and hydronephrosis. It is suggested that RPF is more likely to occur in middle-aged and elderly men, with low back pain as the main clinical feature, and ureteral involvement as the main imaging feature. Glucocorticoid combined with immunosuppressive therapy is the conventional treatment. Surgical intervention can relieve acute obstruction and effectively improve the prognosis of patients.

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?

Chronic Periaortitis – Review and Presentations

Chronic periaortitis is a rare inflammatory condition predominantly affecting the abdominal segment of the aorta. This can present as IgG4 related inflammatory disease, idiopathic retroperitoneal fibrosis, perianeurysmal retroperitoneal fibrosis andinflammatory abdominal aortic aneurysm (IAAA). Aortitis can also be a manifestation of a number of rheumatological large vessel vasculitides such as Takayasu arteritis and giant cell arteritis (GCA). We present three interesting cases of chronic periaortitis and a literature review. The first case showsa classic picture ofIgG4 periaortitis. The second case illustrates periaortitis with retroperitoneal fibrosis, ureteric involvement and hydronephrosis, following abdominal aortic aneurysmal stenting. The final case presents as widespread periaortitis due to Takayasu's disease involving the entire aorta including the arch and root of the subclavian artery

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.

Tumor retroperitoneal en el espectro de enfermedades asociadas a igg4 / Retroperitoneal tumor in the clinical spectrum of igg4 related disease

Presentamos el caso de una mujer de 55 años sin antecedentes, que acude por dolor abdominal, se realiza una tomografía abdominopélvica evidenciando un tumor retroperitoneal de aspecto quístico en la periferia de la aorta abdominal, que afecta el riñón izquierdo. Se le realizó una laparotomía exploratoria, nefrectomía izquierda, tumorectomía retroperitoneal y una resección de un fragmento de la aorta abdominal con reporte histopatológico, corroborado por una prueba inmunohistoquímica de fibrosis retroperitoneal y aortitis por IgG4. El espectro de enfermedades relacionadas con IgG4 es de origen inmunológico con la capacidad de involucrar casi cualquier órgano. La epidemiología no es precisa, aunque se observa mayor predilección por la raza asiática, siendo más común durante la séptima década de vida. El diagnóstico radica en la coexistencia de varios parámetros clínicos, laboratoriales e histopatológicos, sin ser ninguno de estos patognomónicos. El objetivo del presente artículo es exponer el reporte de un caso con manifestación inusual de fibrosis retroperitoneal y dar a conocer que la importancia del diagnóstico radica en la decisión terapéutica, ya que cursa con buena respuesta al tratamiento inmunosupresor.

We present the case of a 55-year-old woman, with noclinical pathological history, who presented with abdominal pain, abdominopelvic tomography showing retroperitoneal tumoral in the periphery of the abdominal aorta, which affects the left kidney producing hydronephrosis. Exploratory laparotomy, left radical nephrectomy, retroperitoneal tumorectomy and resection of a fragment of abdominal aorta were performed, with histopathological report, corroborated by immunohistochemistry of retroperitoneal fibrosis and IgG4 aortitis. The spectrum of diseases related to IgG4, are of immunological origin that can involve almost any organ. The epidemiology is not precise, although a greater predilection for the Asian race is observed, being more common in the seventh decade of life, it should be suspected in patients with unexplained pain in one or more organs.The diagnosis lies in the coexistence of several clinical, laboratorial and histopathological parameters, without being any of these pathognomonic. We present a case with unusual presentation of retroperitoneal fibrosis and the importance of the diagnosis lies in the therapeutic decision, since it responds well to immunosuppressive treatment, without requiring surgical management.

Fibrosis retroperitoneal como causa de uropatía obstructiva. Reporte de caso / Retroperitoneal fibrosis as a cause of obstructive uropathy. Case report

Resumen La fibrosis retroperitoneal es una patología rara, en la mayoría de los casos idiopática, aunque se ha asociado a medicamentos, neoplasias y otras enfermedades de tejido conectivo. Histopatológicamente se evidencia inflamación y depósito de tejido fibrótico en el retroperitoneo y se caracteriza por cubrir los uréteres provocando lesión renal aguda obstructiva siendo ésta, la manifestación más frecuente; el diagnóstico definitivo se obtiene únicamente con biopsia y la base del tratamiento es la corticoterapia, aunque en casos severos y en resistencia a los corticoides se han usado otras terapias como los inmunomuladores. En ocasiones son necesarias las intervenciones quirúrgicas para el manejo de las complicaciones. Se presenta el caso de un hombre de 50 años que ingresó al servicio de urgencias del Hospital San José por dolor abdominal, los paraclínicos demostraron elevación de los azoados y en las imágenes diagnósticas hidronefrosis izquierda con componente de tejido blando interaortocava y periaórtico, se realizó biopsia retroperitoneal y se hizo diagnóstico de fibrosis retroperitoneal idiopática, se instauraron nefrostomías bilaterales y se inició manejo con corticoide.

Abstract Retroperitoneal fibrosis is a rare, in most cases idiopathic, pathology, although it has been associated with medications, neoplasms and other connective tissue diseases. In terms of histopathology, inflammation and deposits of fibrotic tissue in the retroperitoneum are observed and, characteristically, this covers the urethra, provoking acute obstructive kidney damage, the most frequent manifestation of the disease. The definitive diagnosis is obtained solely via biopsy, and the basis of treatment is corticotherapy, although in severe cases, and where resistance to corticosteroids exists, other treatments have been used, such as immunomodulators. Occasionally, surgical interventions are necessary to manage complications. The case of a 50-year-old man who came to the Hospital emergency service due to abdominal pain is presented. Paraclinical studies showed azotemia, and diagnostic images showed left hydronephrosis with a component of interaortocaval and periaortic soft tissue. A retroperitoneal biopsy was conducted, and a diagnosis of idiopathic retroperitoneal fibrosis was made. Bilateral nephrostomies were put in place and treatment with corticosteroids was initiated.

Idiopathic Retroperitoneal Fibrosis with Bilateral Varicocele: A Rare Presentation of Very Rare Clinical Entity.

Retroperitoneal fibrosis is a very rare inflammatory disease leading to extensive fibrosis throughout the retroperitoneum. The majority of cases are idiopathic in which the exact cause is not known. The clinical presentation in retroperitoneal fibrosis is vague and nonspecific, and therefore the diagnosis relies mainly on radiological findings. Here we describe a rare case of idiopathic retroperitoneal fibrosis resulting in Grade 3 varicocele formation. To the best of our knowledge this is the first case report of its kind.

Immunoglobulin G4-Related Aortitis of the Abdominal Aorta / 대한흉부외과학회지

Noninfectious aortitis, inflammatory abdominal periaortitis, and idiopathic retroperitoneal fibrosis are chronic inflammatory diseases with unclear causes. Recent studies have shown that some cases of aortitis are associated with immunoglobulin G4 (IgG4)-related systemic disease. Herein, we report a case of IgG4-related aortitis (IgG4-RA) that was diagnosed after surgery. Our patient was a 46-year-old man who had experienced abdominal pain for several weeks. Preoperative evaluations revealed an area of aortitis on the infrarenal aorta. He underwent surgery, and histological examination resulted in a diagnosis of IgG4-RA.

Retroperitoneal fibrosis in the era of immunoglobulin G4-related disease

BACKGROUND: Retroperitoneal fibrosis (RPF) is a rare disease characterized by fibroinflammatory tissue in the periaortic or periiliac retroperitoneum, where it frequently encases ureters. There is emerging evidence that a subset of this disease is part of a spectrum of multisystemic autoimmune diseases collectively referred to as “immunoglobulin G4 (IgG4)-related disease”. METHODS: We retrospectively analyzed 27 idiopathic RPF patients and identified a subset as IgG4-related RPF, which we categorized according to recently published comprehensive diagnostic criteria. We compared clinical and laboratory characteristics and response to treatment between the two groups. RESULTS: Of 27 total patients, 16 (59.3%) were diagnosed as having IgG4-related RPF, and these were predominantly male. They were also significantly older and more likely to have other organ involvement, hydronephrosis, and postrenal acute kidney injury (AKI) compared to those with idiopathic RPF. However, there was no difference in response rate to systemic steroid treatment. CONCLUSION: IgG4-related RPF accounts for a substantial portion of RPF cases previously identified as “idiopathic RPF” in Korea. Clinical and laboratory characteristics of IgG4-related RPF are similar to those of idiopathic RPF except for a striking male predominance, older age, and higher incidence of postrenal AKI in IgG4-related RPF. More comprehensive, prospective studies are needed to clearly distinguish IgG4-related RPF from idiopathic RPF based on clinical manifestation and to further assess treatment response and long-term prognosis.

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.

Enfermedad relacionada con IgG4 a propósito de dos casos / Disease related to IgG4. Apropos of two cases

RESUMEN La enfermedad relacionada con IgG4 (ER-IgG4), es una enfermedad autoinmune multisistémica caracterizada por lesiones fibroesclerosantes inflamatorias formadoras de masas o pseudotumores, concentraciones elevadas de IgG4 séricas e infiltración tisular extensa por células plasmáticas IgG4 positivas. Incluye numerosas entidades que previamente se consideraban órgano-específicas. Puede afectar prácticamente a cualquier órgano, siendo los más afectados páncreas, glándulas salivales, glándulas lacrimales, ganglios linfáticos, retroperitoneo y riñones. El diagnóstico se realiza mediante una combinación de hallazgos clínicos, serológicos, imagenológicos e histopatológicos. Tiene pronóstico reservado pero con un diagnóstico precoz se logra el control y en ocasiones la regresión de la enfermedad, aunque tiende a presentar recaídas. Es una entidad infradiagnosticada debido en parte, a que fue caracterizada recientemente. Se presentan dos casos que forman parte del espectro que abarca esta entidad, la tiroiditis de Riedel y la fibrosis retroperitoneal, que una vez diagnosticados respondieron inicialmente a la terapéutica con altas dosis de corticoides (AU).

ABSTRACT The disease related to IgG4 (ER-IgG4 in Spanish) is a multi-systemic, autoimmune disease characterized by inflammatory fibro-sclerotizing lesions forming masses or pseudo tumors, high concentrations of serum IgG4 and extensive tissue infiltrations by IgG4-positive plasma cells. It includes several entities previously considered organ-specific ones. It can affect almost any organ, being pancreas, salivary glands, lacrimal glands, lymphatic ganglion, retroperineum and kidneys the most affected ones. The diagnosis is made through combining clinical, serologic, imaging and histopathological findings. It has a reserved prognosis but with an early diagnosis its control is reached, and occasionally also the disease’s remission, although it tends to have relapses. It is an underdiagnosed disease, partially due to its recent characterization. We present two cases entering the range covered by this disease, the Riedel’s thyroiditis and retroperitoneal fibrosis, both initially answering to therapy with high doses of corticoids after diagnosis (AU).

Cancer of Unknown Primary Site Mimicking Retroperitoneal Fibrosis / 대한내과학회지

Retroperitoneal fibrosis (RPF) is a rare disease characterized by marked fibro-inflammatory tissue in the retroperitoneum. Approximately 70% of cases of RPF are idiopathic, while the rest can be secondary to several other causes. The diagnosis is mainly obtained by imaging modalities such as computed tomography (CT). However, histological examination should be considered when the clinical manifestations and imaging studies suggest malignancy. In particular, in malignant diseases with retroperitoneal metastases, abnormal collagen plaques are formed from an exuberant desmoplastic response, which may not be distinguishable from RPF on CT scans. Therefore, even if CT suggests RPF, biopsy is essential to identify malignant disease because it typically results in a fatal prognosis. Here, we report a case of metastatic adenocarcinoma of unknown primary site that developed only in the retroperitoneum and was initially diagnosed as RPF based on CT findings.

Disruption of low density lipoprotein receptor pathway is involved in peritoneal fibrosis induced by high glucose peritoneal dialysis solution / 中华肾脏病杂志

Objective To explore the potential mechanisms of low density lipoprotein receptor (LDLr) in high glucose peritoneal dialysis solution (PDS)-induced peritoneal fibrosis.Methods Human peritoneal mesothelial cells (PMCs) were applied.In pre-experiment,human PMCs were cultured with 1.5％ PDS,2.5％ PDS and 4.25％ PDS for 6 h,12 h and 24 h.4.25％ mannitol was used as high osmotic pressure control.In formal experiment,PMCs were divided into the control group (treated with phosphate buffer saline) and the high glucose PDS group (treated with 4.25％ PDS for 24 h).Morphological change of PMCs was observed by inverted microscope.The mRNA and protein expressions of extracellular matrix proteins such as α-smooth muscle actin (α-SMA),fibroblast specific protein-1 (FSP-1) and collagen Ⅰ in PMCs were respectively measured by real-time PCR and Western blotting.The lipid accumulation was observed by oil red O staining and filipin staining,and the content of intracellular cholesterol ester was detected by high-performance liquid chromatography.The co-expression of the sterol regulatory element-binding protein (SREBP) cleavage-activating protein (SCAP) with golgin was observed with immunofluorescent staining.The mRNA and protein expressions of LDLr,SREBP-2 and SCAP were respectively detected by real-time PCR and Western blotting.The mRNA and protein expressions of mammalian target of rapamycin (mTOR),eukaryotic initiation factor 4E-binding protein 1 (4EBP1),and p70 S6 kinase (S6K1) were respectively detected by real-time PCR and Western blotting.Results (1) Compared with the 1.50％ PDS stimulation,4.25％ PDS for 24 h intervention significantly increased the expression of LDLr in PMCs (P ＜ 0.05),and high osmotic pressure control at 6 h,12 h and 24 h had no statistical difference (P ＞ 0.05).(2) Compared with those in the control group,in high glucose PDS group PMCs showed notable elongation consistent with the morphology of myofibroblasts,the expressions of α-SMA,FSP-1 and collagen Ⅰ were increased (all P ＜ 0.05),and the intracellular cholesterol were enhanced (P ＜ 0.05).Meanwhile,the co-expression of SCAP with golgin was enhanced,and the mRNA and protein expressions of LDLr,SREBP-2 and SCAP were up-regulated in high glucose PDS group (all P ＜ 0.05).Further,the mRNA and protein phosphorylation of mTOR,4EBP1 and S6K1 were increased (all P ＜ 0.05).Conclusions The disruption of LDLr feedback regulation is involved in high glucose PDS-mediated cholesterol accumulation in PMCs by mammalian target of rapamycin complex 1 (mTORC1) pathway,which promotes the accumulation of extracellular matrix and peritoneal fibrosis.

18F-FDG PET/CT in diagnosis of retroperitoneal fibrosis / 中国介入影像与治疗学

Objective To explore the value of 18F-FDG PET/CT in diagnosis of retroperitoneal fibrosis (RPF).Methods Totally 12 RPF patients underwent 18 F-FDG PET/CT scanning,and the characteristics of metabolic activity,distribution of retroperitoneal lesions and maximum standardized uptake value (SUVmax) were analyzed retrospectively.Results Among 12 patients,7 were initial evaluation patients,5 were post-treatment patients.Of 7 initial evaluation patients,there were 4 patients with secondary RPF,including prostate carcinoma,breast cancer and IgG4-related disease.Retroperitoneal mass isodense with muscle surrounding the abdominal aorta or the iliac arteries were found in 12 patients,and ureteral involvement were found in 11 paients (11/12,91.67％).SUVmax in initial evaluation patients (4.21±1.76) was higher than that in patients after treatment (1.46±0.25,P＜0.05).According to PET/CT,3 idiopathic RPF patients as initial evaluation with high metabolic activity lesions received immunosuppressive therapy including corticosteroids and tamoxifen,and 4 secondary RPF patients with high metabolic activity lesions received treatment based on causes.Among 5 post-treatment patients,3 continued to accept current corticosteroids maintenance dose,while 2 did not receive any other treatment.Conclusion PET/CT may be used to evaluate the activity and extension of lesions in patients with RPF.

A case of retroperitoneal fibrosis responding to steroid therapy

ABSTRACT A 69-year-old man presented at the hospital with complaints of prolonged stomach pain extending from the week prior. Enhanced computed tomography (CT) revealed a low density area in the retroperitoneal space. A radiologist diagnosed the patient with retroperitoneal fibrosis. One week later, an enhanced CT revealed an exponential increase of the low density area and slight right hydronephrosis. Upon admission, prednisolone administration was initiated at a dose of 40mg/day. The size of the retroperitoneal soft tissue mass decreased gradually. Although the dose of prednisolone was tapered to 5mg, the patient is doing well without any sign of recurrence.

Uropatía obstructiva recurrente en arteritis de Takayasu / Recurrent upper urinary tract obstruction in takayasu arteritis

La arteritis de Takayasu (AT) es una enfermedad inflamatoria infrecuente de vasos grandes. A menudo, el daño crónico originado en las grandes arterias requiere de revascularización mediante prótesis vasculares. Excepcionalmente la implantación de las prótesis origina lesiones en otros órganos, como los uréteres, los cuales pueden obstruirse por compresión extrínseca por el tejido fibroso retroperitoneal originado como reacción a la presencia de la prótesis vascular.Se comunica el caso de una mujer que presentó hidronefrosis bilateral en forma asincrónica después de los 2 años de la instalación de una prótesis aorto-ilíaca con insuficiencia renal aguda, la cual pudo revertirse mediante. (AU)

Takayasu's arteritis is a rare inflammatory disease of large vessels. Often, chronic damage resulting in large arteries stenosis requires revascularization with vascular prostheses. Exceptionally, the presence of prostheses causes lesions in other organs, such as ureters, which are subject to extrinsic compression caused by retroperitoneal fibrous tissue originated as a reaction to the presence of vascular prostheses. We report the case of a woman who presented bilateral asynchronous hydronephrosis after 2 years of the installation of an aorto- iliac graft with acute renal failure, which could be reversed by releasing the ureter from the periprosthetic fibrous retroperitoneal tissue.(AU)

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.

Protective effect and its mechanism of Shenkang injection on peritoneal mesothelial cells in CAPD mice / 天津医药

Objective To study the protective effect of Shenkang injection on peritoneal mesothelial cells (PMCs) of continuous ambulatory peritoneal dialysis (CAPD) mice, and explore the possible mechanism. Methods Forty ICR mice were randomly divided into blank control (A) group, peritoneal dialysis (B) group, low dose of Shenkang (C) group ( 2.5%dialysate+5 mL/kg Shenkang injection), medium (D) group (2.5% dialysate+10 mL/kg Shenkang injection) and high (E) group (2.5% dialysate+ 20 mL/kg Shenkang injection). Mice were observed for 4 weeks. Fasting blood glucose, total cholesterol, triglyceride and C-reactive protein (CRP) were detected by biochemical assay. Tumor necrosis factor-alpha (TNF-α), transforming growth factor-beta 1 (TGF-β1), vascular endothelial growth factor (VEGF) and connective tissue growth factor (CTGF) levels of serum and dialysate were detected by ELISA. Pathological changes of peritoneal tissue were observed by HE staining. Expression and mRNA transcription levels of these four cytokines in the peritoneal tissue were detected by immunohistochemical staining and real-time PCR respectively. Results There were no significant differences in body weight, fasting blood glucose, total cholesterol and triglyceride between 5 groups of mice (P>0.05). Compared with group B, there was no significant difference in CRP level between group C and group E, but which was significantly decreased in group D (P<0.05). The serum and dialysate levels of TNF-α, TGF-β1, VEGF and CTGF were decreased in group C and group D. The serum and dialysate levels of TNF-αand TGF-β1 were significantly increased in group E (P<0.05), but there was no significant difference between VEGF and CTGF in group E. Compared with group E, except for CTGF in dialysate of group C, the serum and dialysate levels of TNF-α, TGF-β1, VEGF and CTGF were significant decreased in group C and group D (P<0.05). Damaged PMCs were found in group B, which were improved in various degrees in group C, group D and group E. Compared with group B, the protein expression and mRNA relative transcription levels of TNF-α, TGF-β1, VEGF and CTGF tended to decrease gradually in group C, group D and group E (P<0.05). Conclusion A certain concentration of Shenkang injection can protect PMCs by inhibiting the expression of TNF-α, TGF-β1, VEGF and CTGF in CAPD mice, so as to control the occurrence and development of peritoneal fibrosis.