3D Laparoscopic Ureterolysis for Retroperitoneal Fibrosis Secondary to Radical Hysterectomy and Radiation Treatment for Cervical Cancer: Results from the Oncological Institute, Cluj Napoca

Background: Ureterolysis represents the surgical treatment for retroperitoneal fibrosis. The aim of the study was to review the outcomes of patients who had undergone radical hysterectomy and radiotherapy for cervical cancer that later developed retroperitoneal fibrosis, for whom 3D laparoscopic ureterolysis was performed in our department and to review current published studies. Methods: We present a series of cases consisting of 6 patients with secondary retroperitoneal fibrosis. In all cases, the intervention was performed by the same surgeon from the Oncological Institute “Prof. Dr. Ion Chiricuț㔠Cluj-Napoca, Romania. We carried out a literature review, searching in the PubMed and MEDLINE studies published between 2000 and 2021 relevant to the matter and a total of 12 papers were selected. We reviewed the functional outcomes of patients that underwent minimally invasive ureterolysis. Results: 3D laparoscopic ureterolysis was performed in 6 patients. Mean operative time was 166 minutes and mean blood loss was 203 mL. No surgery required conversion. Five patients showed good functional results after ureteral stent removal. In one case, the patient developed acute pyelonephritis and the ureteral stents were kept. Conclusions: Laparoscopic ureterolysis for retroperitoneal fibrosis secondary to operated and radiation-treated cervical cancer represents one of the most complex and challenging surgeries in the urological field (AU)

Idiopathic Retroperitoneal Fibrosis: What Is the Optimal Clinical Approach for Long-Term Preservation of Renal Function?

BACKGROUND: The aim of this study was to investigate the long-term effects of ureteral stenting and the exact timing of stent removal in favor of surgery in patients with idiopathic retroperitoneal fibrosis (IRF). SUMMARY: Medline research terms of "idiopathic retroperitoneal fibrosis" AND " medical therapy" OR "ureteral stenting" OR "surgical treatment" were done. Systematic reviews and observational and clinical studies were analyzed to obtain indication regarding the objective of the study for a narrative review. Ninety-two papers were analyzed. The treatment of IRF includes the monitoring of retroperitoneal fibrotic process spread and the prevention of abdominal organs entrapment. Treatment of ureteral obstruction includes medical therapy and ureteral stenting (US) or percutaneous nephrostomy (PNS) to overcome the worsening of renal function. Up to now, the timing of US or PNS removal is not yet clear, both for the complexity of evaluating the efficacy of the medical therapy and demonstrating the resolution of obstructive nephropathy. Moreover, it is not yet clear if the long-term ureteral stent placement or PNS is able to maintain an efficient renal function. Ureterolysis with a laparoscopic robot-assisted approach is now considered as an ultimate treatment for ureteral obstruction, limiting the progression of kidney impairment and improving the quality of life of patients, although nephrologists are generally abdicant regarding the potential switch toward the surgical approach. KEY MESSAGES: Prospective studies regarding the long-term effects of US on the renal function impairment in patients with IRF should be structured to obtain adequate information on the exact timing for the surgical approach.

Minimally invasive ureterolysis and intraperitonealization of ureter for idiopathic retroperitoneal fibrosis; single center analysis of an erratic disease.

INTRODUCTION: Idiopathic retroperitoneal fibrosis is a known cause of obstructive uropathy. Ureterolysis is done when medical management fails or the presentation is at an advanced stage. Conventionally ureterolysis without omental wrap has been considered incomplete. Our Institute has experience of laparoscopic or robotic ureterolysis with intraperitonealization of the ureter alone and no other adjunctive procedure. This study retrospectively assesses the result of the procedure with patients presenting with varying severity of disease. METHODS: From 2008, all patients who underwent laparoscopic or robotic ureterolysis were analyzed retrospectively for pre-operative management, operative findings, and post operative outcomes. RESULTS: We operated and released nine renal units in seven patients. Two of the nine cases were performed robotically completely and the rest was performed by laparoscopic approach. Median follow up was 60 months. All patients documented resolution of symptoms. The mean post-operative creatinine at 1 year was significantly decreased to 1.47 ± 0.49 mg/dl in comparison to preoperative creatinine (p < 0.05). The postoperative mean ESR decreased significantly from a preoperative value of 58.2 ± 19.41 mm to 15.8 ± 17.23. The nuclear scan revealed unobstructed drainage and radiological imaging revealed resolution of hydronephrosis and fibrosis in all. The mean GFR on the nuclear scan after 3 and 12 months of surgery was 36.3 ± 4.33 and 40 ± 3.77, respectively. Thus, there was significant increase noted in GFR at 3 and 12 months in comparison to preoperative GFR (p < 0.05). CONCLUSION: Laparoscopic/robotic ureterolysis with intraperitonealization alone is secure and durable procedure for idiopathic retroperitoneal fibrosis needing surgical release.

Idiopathic retroperitoneal fibrosis with endometrial cancer: a case report and literature review.

BACKGROUND: Retroperitoneal fibrosis is a rare disease characterized by chronic nonspecific inflammation, which leads to clinical compression manifestations of retroperitoneal organs especially ureter. Approximately 70 percent of retroperitoneal fibrosis cases are idiopathic which has no clear etiology. This study reported a rare case of a 48-year-old woman presented with idiopathic retroperitoneal fibrosis and endometrial cancer. CASE PRESENTATION: A 48-year-old woman presented with irregular vaginal bleeding without abdominal pain, bloating or discomfort. The patient was diagnosed iRPF after splenectomy 13 years ago. Then she took prednisone for 2 years and took tamoxifen for about 11 years. She stopped taking the medication from October 2019 to May 2020 and then started taking tamoxifen again until November 2020. Two weeks after she stopped taking tamoxifen, she presented with irregular vaginal bleeding. Gynecological ultrasound revealed a thick endometrium with uneven echo enhancement and blood flow signals. Then diagnostic curettage was performed with pathological examination showed endometroid carcinoma. Later, the patient was admitted to Peking University Third Hospital for surgery. Preoperative imaging examinations, including CT, MRI, and PET/CT, all showed pelvic enlarged lymph nodes and they were highly suspected to have lymph node metastasis. The patient underwent laparoscopic surgical staging and enlarged lymph nodes in the pelvic and aortic regions were removed. Finally, the pathology confirmed that endometrioid adenocarcinoma and fibrosis, but there was no tumor infiltration in these enlarged lymph nodes. The patient is now in good condition. CONCLUSION: This case report stressed the difficulty to distinguish between lymph node metastasis and inflammatory hyperplasia by common imaging methods. Due to increased surgical difficulty among retroperitoneal patients, lymphadenectomy should be carefully evaluated to avoid additional surgical complications and over-treatment.

Comparison of Laparoscopic and Open Ureterolysis for Retroperitoneal Fibrosis: Results from a Tertiary Referral Center.

Objectives: To compare the surgical outcomes of open and laparoscopic ureterolysis procedures in patients requiring surgical treatment for ureteral obstruction caused by retroperitoneal fibrosis (RPF). Materials: This study was designed retrospectively. The clinical records of patients who underwent ureterolysis between January 2005 and April 2019 because of ureteral obstruction caused by RPF were examined. According to the type of surgery, the patients were divided into two groups as Group 1 (open ureterolysis) and Group 2 (laparoscopic ureterolysis). Demographic features, preoperative-postoperative renal functions, duration of follow-up with ureteral stents, and perioperative-postoperative complications were examined. The requirement of ureteral stent placement during the follow-up period was accepted as unsuccessful ureterolysis. Results: Ureterolysis was performed in 13 patients and 23 renal units. Eleven of these patients were men and two were women. The median age of the patients was 54 (44-68) years. There were six patients and 12 renal units in Group 1 and seven patients and 11 renal units in Group 2. Postoperatively, a total of four patients (30%) had minor complications (Clavien-Dindo 1-2) and one patient had a major complication (Clavien-Dindo 3a). Ureterolysis was determined to be effective in 21 of the renal units (91%) [11/12 (92%) in Group 1 vs 10/11 (91%) in Group 2]. No statistically significant difference was found between the groups in terms of the success and complication rates (p = 1 and p = 0.529, respectively). Postoperative hospitalization length and recovery time to return to normal preoperative activities were significantly shorter in Group 2 than in Group 1 (p = 0.011 and p = 0.041, respectively). Conclusions: The success and complication rates were similar between the open and laparoscopic methods for ureterolysis. Laparoscopic approach was advantageous over open approach in terms of postoperative hospitalization length and recovery time to return to normal preoperative activities.

Retroperitoneal fibrosis as a postoperative complication following renal transplantation in cats.

OBJECTIVES: The aim of this report was to describe the clinical signs, diagnostic imaging findings, surgical management, histopathological findings, outcome and possible risk factors for cats that developed retroperitoneal fibrosis (RPF) following renal transplantation. METHODS: Medical records of cats that underwent renal transplantation and developed clinically significant RPF between 1995 and 2019 were reviewed. RESULTS: Eighty-one cats underwent 83 renal transplantations. Of these 81 cats, six developed clinically significant RPF. For all six cats, renal transplantation was performed using cold organ preservation solution and ureteral papilla implantation. Immunosuppression protocol included ciclosporin and prednisolone. All cats had at least one subtherapeutic trough ciclosporin level (<250 ng/ml) in the postoperative period. Cats presented with moderate-to-severe azotemia 39-210 days following renal transplantation. Abdominal ultrasonography and contrast pyelography revealed various degrees of hydroureter and hydronephrosis of the transplanted kidney. Surgical examination revealed a layer of dense fibrous tissue surrounding the transplanted kidney, ureter and bladder resulting in ureteral obstruction. Ureteral obstruction was managed by reimplantation of the proximal ureter or renal pelvis to the bladder. Histopathologic examination of the fibrous tissue and affected portion of the distal ureter revealed fibrous connective tissue with lymphoplasmacytic infiltration and perivascular inflammation suggestive of an autoimmune type reaction. Of the six cats, two died within 5 days after revision surgery, two developed signs consistent with recurrent partial ureteral obstruction (40 and 41 days after revision), one was euthanized 6 years later for an unrelated disease and one was lost to follow-up. CONCLUSIONS AND RELEVANCE: The incidence of RPF in this population of cats was relatively low (7%), but still represents a significant cause of morbidity and mortality. The cause of RPF remains unknown, although investigation into suboptimal immunosuppression as a potential cause for local rejection reaction is warranted.

An unusual case of periaortitis mimicing aortic aneurysm with coronary artery involvement.

We hereby describe a rare case of coronary artery involvement in a patient with periaortitis which was mimicking an aortic aneurysm on computed tomography (CT). This case also highlights the role of CT in differentiating aortic pathologies to guide the management and also the importance of coronary evaluation in such patients.

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

Retroperitoneal Liposarcoma Masquerading as an Impending Rupture of Inflammatory Abdominal Aortic Aneurysm.

BACKGROUND: We present a unique case scenario of a periaortic liposarcoma masquerading as an impending rupture of an inflammatory abdominal aortic aneurysm (AAA). METHODS AND RESULTS: A 57-year-old man was referred to our unit for an emergency endovascular repair of "an inflammatory AAA with computed tomography (CT) features of impending rupture." He underwent an uneventful endovascular repair with a bifurcated endograft (C3; Gore, Flagstaff, AZ). Seven weeks later, CT showed that the periaortic "mass" grew larger and asymmetric, and a CT-guided needle biopsy suggested the presence of a high-grade malignant mesenchymal tumor. He underwent laparotomy and excision of the retroperitoneal tumor en bloc with the anterior wall of the infrarenal aorta. The endograft acted as an excellent "safety net" providing adequate hemostatic control and obviating the need for aortic cross-clamping and repair of the aortic defect with a patch or tube graft. CONCLUSIONS: The learning point from the present case is that when faced with an inflammatory AAA and/or retroperitoneal fibrosis, the rare possibility of a retroperitoneal neoplasm should be kept in mind.

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

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.

Retroperitoneal fibrosis-clinical presentation and outcome analysis from urological perspective.

PURPOSE: To study clinical presentation, laboratory results, imaging findings and treatment options and outcomes of retroperitoneal fibrosis (RPF). To determine whether it follows the same natural course and response to treatment in the Asian population as in the Western world. MATERIALS AND METHODS: Medical records of patients diagnosed with RPF on imaging and histopathology between February 2010 and April 2016 were reviewed. RESULTS: Of the 21 patients analyzed, mean age at presentation was 50.81 years. The male to female ratio was 0.9:1. Pain was most common presenting complaint (95.23% cases), almost 85% cases were idiopathic and rests were postradiation induced. The median creatinine level was 1.8 mg/dL. The mean erythrocyte sedimentation rate (ESR) was 53.2 mm/h. Hydronephrosis was present in all patients and 47.6% had atrophic kidneys. Diffuse retroperitoneal mass was present in 61.1%. Ureterolysis with lateralization, omental wrapping or gonadal pedicle wrap was done in 17 cases. Two patients underwent uretero-ureterostomy. One patient underwent ileal replacement of ureter, and one ileal conduit. Eighteen patients received concurrent medical treatment, 11 were given tamoxifen, 2 steroids (Prednisolone), and five were given both. Of the 20 patients with follow-up, 70% had complete symptomatic relief; ESR improvement was seen in 77.8%. Follow-up ultrasound showed resolved and decreased hydronephrosis in 20% and 55% respectively. One patient had treatment failure and 17.65% had disease recurrence. CONCLUSIONS: RPF is a rare disease with varied presentation and outcomes. The male to female ratio may be equal in Asians and smoking could be lesser contributing factor. More Asian cohort studies are required to support same.

[Laparoscopic treatment of retroperitoneal fibrosis.] / Tratamiento laparoscopico de la fibrosis retroperitoneal.

OBJECTIVES: Retroperitoneal fibrosis is a disease that may condition a severe involvement of various organs, mainly upper urinary tract, even causing renal insufficiency. It was first described by Albarran in 1905 and it is also known as Ormonds disease. The correct diagnosis includes, in many cases, the performance of one or more tests: CT scan, MRI, renal scan, etc. It is often necessary the insertion of double J catheters on percutaneous nephrostomy tubes with the aim to preserve renal function. Initial treatment is medical, based on corticoids, and , less frequent, immunosuppressive or chemotherapy drugs. Surgery is the treatment of choice when ureteral entrapment by the fibrous plaque is not solved with medical treatment. Such operation may be performed with a conventional open approach (laparotomy) or by pure, hand assisted laparoscopic surgery, or robotic surgery. In all cases, the technique involves freeing the ureters from the fibrous plaque that entraps them, leaving them intraperitoneal, and it is recommendable to wrap them with an omental flap. The implantation of minimally invasive techniques has made that, in groups with experience in laparoscopy, open surgery is being abandoned and the laparoscopic approach indication is increasing. Our group has performed 10 laparoscopic ureterolysis from 2005. In two patients, it was bilateral. Despite surgical repair, two renal units were lost, keeping the rest with different levels of renal function depending on the preoperative level of disease. We did not have major complications and the mean hospital stay was 5.5 days. Although, there is not important published scientific evidence about this technique and it is unlikely we will have it in an immediate future, due to the rarity of this disease, and the different degree of involvement that conditions. It is not unreasonable to propose that, based on the literature reviewed and our own experience, laparoscopic approaches, despite being complex, may solve the ureteral entrapment with similar results to open surgery but less morbidity and shorter hospital stay.

Tratamiento laparoscopico de la fibrosis retroperitoneal / Laparoscopic treatment of retroperitoneal fibrosis

OBJETIVOS: La fibrosis retroperitoneal es una enfermedad que puede condicionar una afectación severa de distintos órganos, fundamentalmente del aparato urinario superior, pudiendo llegar incluso a ser la causa de una insuficiencia renal. Fue descrita por Albarrán en 1905 y se le conoce también como enfermedad de Ormond. El diagnóstico correcto que incluye, en muchos de los casos, la realización de una o varias pruebas: TAC (tomografía axial computarizada), RNM (resonancia nuclear magnética), gammagrafía renal, etc, obliga con relativa frecuencia, a la colocación de catéteres doble J o nefrostomías percutáneas con el fin de preservar la función de los riñones. El tratamiento inicial es médico, basado en la utilización de corticoides y, con menos frecuencia inmunosupresores o quimioterápicos. La cirugía es el tratamiento de elección cuando el atrapamiento ureteral por la placa fibrosa no queda resuelto con el tratamiento médico indicado en cada paciente. Dicha cirugía puede hacerse mediante un abordaje convencional (laparotomía) o mediante cirugía laparoscópica pura, manoasistida o robótica. En todos los casos la técnica consiste en liberar el o los uréteres de la placa fibrosa que los atrapa, dejarlos situados a nivel intraperitoneal y es recomendable utilizar un manguito de epiplon para envolverlos. La implantación cada vez mayor de las técnicas menos invasivas ha hecho que, en los grupos con experiencia laparoscópica, se vaya abandonando la cirugía abierta y sea cada vez más frecuente la indicación del abordaje laparoscópico. Nuestro grupo ha realizado 10 ureterolisis laparoscópicas desde el año 2005 en el que la indicamos por primera vez. En dos pacientes la técnica se realizó en los dos uréteres a la vez. Se perdieron, a pesar de la cirugía, dos unidades renales, manteniéndose el resto con distintos niveles de función renal dependiendo del grado de afectación que presentaban previamente. No tuvimos complicaciones mayores siendo la estancia media hospitalaria de 5,5 días. Aunque la literatura actual no tiene publicaciones sobre estas técnicas con importante evidencia científica y, por la rareza de la enfermedad y el distinto nivel de afectación que condiciona, es poco probable que las tengamos en un futuro inmediato, no es descabellado plantear, en base a la literatura revisada y a nuestra propia experiencia que, aunque complejos, los abordajes laparoscópicos pueden solucionar el atrapamiento ureteral con resultados similares a la cirugía abierta pero con menor morbilidad y menor estancia hospitalaria

OBJECTIVES: Retroperitoneal fibrosis is a disease that may condition a severe involvement of various organs, mainly upper urinary tract, even causing renal insufficiency. It was first described by Albarran in 1905 and it is also known as Ormonds disease. The correct diagnosis includes, in many cases, the performance of one or more tests: CT scan, MRI, renal scan, etc. It is often necessary the insertion of double J catheters on percutaneous nephrostomy tubes with the aim to preserve renal function. Initial treatment is medical, based on corticoids, and, less frequent, immunosuppressive or chemotherapy drugs. Surgery is the treatment of choice when ureteral entrapment by the fibrous plaque is not solved with medical treatment. Such operation may be performed with a conventional open approach (laparotomy) or by pure, hand assisted laparoscopic surgery, or robotic surgery. In all cases, the technique involves freeing the ureters from the fibrous plaque that entraps them, leaving them intraperitoneal, and it is recommendable to wrap them with an omental flap. The implantation of minimally invasive techniques has made that, in groups with experience in laparoscopy, open surgery is being abandoned and the laparoscopic approach indication is increasing. Our group has performed 10 laparoscopic ureterolysis from 2005. In two patients, it was bilateral. Despite surgical repair, two renal units were lost, keeping the rest with different levels of renal function depending on the preoperative level of disease. We did not have major complications and the mean hospital stay was 5.5 days. Although, there is not important published scientific evidence about this technique and it is unlikely we will have it in an immediate future, due to the rarity of this disease, and the different degree of involvement that conditions,. It is not unreasonable to propose that, based on the literature reviewed and our own experience, laparoscopic approaches, despite being complex, may solve the ureteral entrapment with similar results to open surgery but less morbidity and shorter hospital stay

Clinical signs of fibrosis in small intestinal neuroendocrine tumours.

BACKGROUND: In patients with small intestinal neuroendocrine tumours (SI-NETs), serotonin and other cytokines released from tumour cells may induce fibrosis, leading to carcinoid heart disease and abdominal fibrotic reactions. The aim of this study was to assess the prevalence, clinical complications and management of this reaction in the abdomen. METHODS: This was a retrospective cohort study of patients with SI-NETs diagnosed between 1985 and 2015. Clinical data, outcomes, radiological findings, and surgical and radiological interventions were reviewed. RESULTS: A total of 824 patients were diagnosed with SI-NETs in the study interval. Clinically significant abdominal signs and symptoms of fibrosis occurred in 36 patients. Of these, 20 had critically symptomatic central mesenteric fibrosis causing obstruction of mesenteric vessels, and 16 had retroperitoneal fibrosis causing obstructive uropathy with hydronephrosis. Extensive fibrosis causing mesenteric vessel obstruction and/or obstructive uropathy was more often associated with symptomatic and advanced disease encompassing lymph node metastases in the mesenteric root, para-aortic lymph node metastases, as well as liver metastases and peritoneal carcinomatosis. Palliative intervention in terms of superior mesenteric vein stenting or resection of central mesenteric metastases and/or percutaneous nephrostomy and J stent treatment was beneficial in the majority of the patients. CONCLUSION: Extensive abdominal fibrosis associated with clinically significant symptoms of intestinal ischaemia and/or obstructive uropathy was linked to advanced disease in patients with SI-NETs. Prompt recognition and minimally invasive intervention was effective in disease palliation.

A Case of Idiopathic Retroperitoneal Fibrosis Associated With Sjögren's Syndrome.

After presenting with nonspecific symptoms, a 52-year-old female was found to have a 3.4 × 2.6 cm retroperitoneal mass resulting in hydronephrosis on abdominal computed tomography scan. After extensive workup including surgical debulking, excisional biopsy, and autoimmune panel, she was found to have idiopathic retroperitoneal fibrosis (IRF) with negative staining for immunoglogulin G4 (IgG4). Two years after presentation, the patient developed dyspnea, cough, and xerostomia. Repeat anti-Sjögren Syndrome A and anti-Sjögren Syndrome B titers and labial gland biopsy confirmed the diagnosis of concomitant Sjögren's syndrome (SS). The patient was treated with mycophenolate mofetil with improvement in her symptoms. Most cases of retroperitoneal fibrosis are associated with IgG4-related disease or other autoimmune disease. To our knowledge, this is only the second reported case of SS associated with IRF. Because symptoms of IRF are nonspecific, there is often a delay in diagnosis resulting in end-organ disease such as ureteral obstruction or hydronephrosis. Although IRF is uncommon, it should be considered in patients presenting with abdominal or flank pain, especially in patients with concomitant autoimmune disorders. Early recognition of disease can prevent end-organ damage and, as more cases are diagnosed, its relationship to SS may be elucidated leading to further advances in treatment and surveillance.