retroperitoneal fibrosis
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2022 ◽  
Author(s):  
Shoichiro Mukai ◽  
Naotaka Sakamoto ◽  
Hiroaki Kakinoki ◽  
Tadamasa Shibuya ◽  
Ryosuke Moriya ◽  
...  

Abstract Objectives: To evaluate the management and outcome of idiopathic retroperitoneal fibrosis (iRPF) in Japan, and identify its clinical biomarker. Methods: We retrospectively analyzed 129 patients with iRPF treated between January 2008 and May 2018 at 12 university and related hospitals. Patients treated with glucocorticoid were analyzed to identify a predictive biomarker. These patients were classified into three groups according to overall effectiveness (no change: NC, complete response: CR and partial response groups: PR), and each parameter was compared statistically.Results: Male-female ratio was 5: 1, and median age at diagnosis was 69 (33-86) years. Smoking history was reported in 59.6% of the patients. As treatment, 95 patients received glucocorticoid therapy with an overall response rate of 84%. As a result, serum concentration of IgG4 was significantly decreased in NC group compared with the other two groups (56.6mg/dL vs 255mg/dL, 206mg/dL, P=0.0059 and 0.0078). ROC analysis was performed between the non-responder (NC) and responder groups (CR+PR) to identify the cut-off value of serum IgG4 as a predictive marker. As a result, AUC was 0.793 and the values of sensitivity and specificity were 0.85 and 0.64, respectively, under the cut-off values of 67.6mg/dL. Conclusions: In the majority of iRPF patients, glucocorticoid therapy resulted in a favorable response. Pre-treatment serum IgG4 concentration may have potential as a predictive biomarker of steroid treatment.


2022 ◽  
Vol 4 (1) ◽  
Author(s):  
Madalina Nussberger ◽  
Olaf Chan-Hi Kim ◽  
Sergio Cogliatti ◽  
Gautier Müllhaupt ◽  
Thomas Neumann

AbstractThe etiology of solid retroperitoneal mass may be autoimmune or neoplastic and should be investigated by imaging and histology. The spectrum of differential diagnoses includes retroperitoneal fibrosis and retroperitoneal tumors. As treatment for these entities differs substantially, early and accurate diagnosis is essential. We present a case of a 54-year-old woman admitted to our hospital with stroke-like symptoms. Suspecting vasculitis, magnetic resonance imaging of the head and abdomen was performed, which revealed circular enhancement of the internal carotid artery as well as retroperitoneal and periaortic masses. In light of the radiographic findings, an autoimmune process, such as retroperitoneal fibrosis, was hypothesized. Steroid treatment was initiated but did not lead to significant remission. Re-evaluation of the mass with fine-needle aspiration did not show malignant cells while diagnostic surgery and histological assessment revealed neoplastic lymphoproliferation. The final diagnosis was a non-Hodgkin B-cell lymphoma. Chemo- and immunotherapy were initiated. Follow-up abdominal computed tomography revealed significant remission of the retroperitoneal mass. Initially, the retroperitoneal mass was highly suspicious for RF. While imaging can be useful, obtaining histology should always be considered when there is an uncertain clinical presentation. Without histology, we would have missed a non-Hodgkin B-cell lymphoma in this case. Minimally invasive techniques such as fine-needle aspiration may be practical but can give false-negative results.


Author(s):  
João Carvão ◽  
◽  
Carlota Vida ◽  
Luís Resende ◽  
Francisca Silva ◽  
...  

Retroperitoneal fibrosis is a rare condition defined by an overproduction of fibro-inflammatory tissue in the retroperitoneum, usually involving the abdominal aorta, but also managing to invade adjacent structures. We report a case of a 47-year-old female patient with an atypical radiological presentation of retroperitoneal fibrosis, involving predominantly the right kidney. Ultimately, the diagnosis was performed by an immunohistochemical study that identified IgG4+ plasmocytes after kidney nephrectomy, also known as Ormond’s disease. There are no universal guidelines for the treatment of retroperitoneal fibrosis, due to its rarity and lack of randomized controlled trials comparing different therapeutic strategies.


Author(s):  
Izabela Łoń ◽  
Monika Wieliczko ◽  
Jacek Lewandowski ◽  
Jolanta Małyszko

Background: Retroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encircles abdominal organs including aorta and ureters. Data on the incidence of this disease are limited. Summary: The disease may be idiopathic or secondary to infections, malignancies, drugs or radiotherapy. Idiopathic form is an immune-mediated entity and a part of the broader spectrum of idiopathic diseases termed chronic periaortitis, characterized by a morphologically similar fibroinflammatory changes in aorta and surrounding tissues. Taking into account the dominant symptoms and clinical charac-teristics of patients with periaortitis, two subtypes of disease could be distinguished. Vascular subtype include patients with non-dilated aorta or with inflammatory abdominal aortic aneu-rysm, both with and without involvement of adjacent structures and with numerous risk factors for atherosclerosis. In renoureteral subtype obstructive uropathy manifesting with hydronephro-sis and acute kidney injury is predominant finding. Due to the variety of symptoms, diagnosis of RPF remains challenging, difficult and often delayed. A series of diagnostic tests should be performed, in order to confirm the diagnosis idiopathic RPF. Laboratory work-up include eval-uation of inflammatory indices and immunological studies. A biopsy and histopathological evaluation may be necessary to confirm diagnosis and differentiate the disease. Computed to-mography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET) are the modalities of choice for the diagnosis and follow-up of this disease. Management of ureteral obstruction, hydronephrosis, and aortic aneurysms often requires surgical evaluation and treatment. The pharmacological treatment of RPF has been evaluated in a few randomized trials and is mainly based on observational studies. Steroid therapy remains the gold standard of treatment. Key messages: Nowadays multidisciplinary team approach with clinical and diagnos-tic experience in both primary and secondary RPF as well as two major subtypes should be offered. Centers specialized in rare diseases with collaboration with other units and referral sys-tem yield the best possible outcomes.


Author(s):  
Fatos Mete ◽  
Tuba Mengeneci ◽  
Emre Albayrak ◽  
Yavuz AYAR ◽  
Melike Nalbant ◽  
...  

IgG4 related disease is an immune-mediated chronic inflammatory disease. It is a multisystemic disease that can lead to retroperitoneal fibrosis with involvement of the pancreas, glands, thyroid, lymph nodes, etc. and characterized histopathologically by lymphoplasmocytic cell infiltration. We evaluated a patient diagnosed with IGG4 related disease impaired kidney function.


2021 ◽  
Vol 13 (4) ◽  
pp. 369-375
Author(s):  
M Possover

Background: Endometriosis of the sciatic nerve (ESN) is considered a rare disease. How can endometriosis develop within the sciatic nerve; a structure which has nothing in common with the uterus either anatomically or functionally, and why it occurs in the absence of any retroperitoneal/parametric endometriosis, is unknown. A better understanding of the pathophysiology of this enigmatic disease may improve its diagnosis and therapy. Materials and Methods: From a pool of 452 patients operated for ESN, only patients with “isolated” endometriosis of the sciatic nerve” confirmed at laparoscopy were included in this study. Patients with suspicion of ESN by extension from a parametric, ovarian or other intraperitoneal deeply infiltrating endometriosis were excluded from this study. Main outcome measure: All information acquired during the preoperative patient’s medical history and clinical examination were collected and compared with the morphological aspects of the disease observed by the laparoscopic treatment. Patients were classified into three groups according to the time interval between the onset of sciatic pain and the time of surgery: less than 1 year (Group 1), between 1 and 3 years (Group 2), and more than 3 years (Group 3). Results: Two hundred sixty-seven consecutive patients were included in this study. In Group 1 (n=67), 76% of the patients presented with cyclical sciatica, without sensory or motor disorders of the lower limbs. Laparoscopic exploration found in the great majority of these patients only the presence of an isolated endometrioma in the nerve itself, the size of which was proportional to the time elapsed since the onset of pain. In Group 2 (n=83), pain had become constant in 91% of the patients with neurological disorders of the lower limb (foot drop, Trendelenburg gait, atrophied muscles) in about 30% of patients. Laparoscopic examination revealed, in addition to intraneural cystic lesions, a retroperitoneal fibrosis in more than 80% of the patients. In the third group (N=117), more than 80% of the patients presented with neurological disorders of the lower limb, with, on laparoscopic examination, massive retroperitoneal fibrosis with endometriomas in the nerve and adjacent pelvic wall muscles in all patients and an infiltration of the obturator nerve in 41% of patients. Conclusions: The different morphologic aspects of ESN do not correspond to different forms of the disease, but obviously to one single disease at different stages of its evolution. ENS starts first with the development of an endometrioma within the sciatic nerve, then develops in a second step a perineural fibrosis that expands into the whole retroperitoneal space and finally involves surrounding anatomical structures. The ESN is a very particular pathology because it induces a completely new aspect on the pathogenesis of endometriosis: all hypothesis of implanted endometrial cells following retrograde menstruation, angiogenic spread, lymphogenic spread or the metaplasia theory cannot explain the pathogenesis of this disease. ESN obviously does not develop from “genital metastatic cells”. A possible hypothesis for explanation the pathogenesis of ESN, could consist in the development of endometriosis of the nerve from progenitor stem cells present within the nerve, pluripotent cells which, for an as yet unknown reason (possibly in connection with iterative inflammations and micro-damages of the nerve itself), mutate and proliferate to form endometriosis.


Author(s):  
H. H. S. Kharagjitsing ◽  
J. van Vooren ◽  
E. G. Brilman ◽  
T. R. Hendriksz ◽  
T. van Gelder ◽  
...  

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