Case Of Idiopathic Retroperitoneal Fibrosis Research Articles

A case of idiopathic retroperitoneal fibrosis (Ormond’s disease) diagnosed by transduodenal EUS-guided fine-needle aspiration/biopsy

A case of idiopathic retroperitoneal fibrosis (Ormond’s disease) diagnosed by transduodenal EUS-guided fine-needle aspiration/biopsy

Клинический случай развития ретроперитонеального фиброза (болезни Ормонда) в практике ревматолога

Забрюшинный (ретроперитонеальный) фиброз (РПФ) — редкое заболевание, характеризующееся разрастанием воспалительной и фиброзной ткани в забрюшинном пространстве. Обычно эти разрастания локализуются вокруг инфраренальной части брюшной аорты и подвздошных артерий, часто покрывая мочеточники или другие органы брюшной полости. Идиопатические заболевания составляют 70% случаев. РПФ может быть ассоциирован с иммуноглобулином G4 (IgG4), что является причиной двух третей случаев идиопатического РПФ. Вторичный РПФ может развиться вследствие инфекций, злокачественных новообразований, приема лекарственных препаратов, забрюшинного кровотечения или других заболеваний. Описан клинический случай идиопатического РПФ, вероятно, ассоциированного с IgG4, у пациента, прошедшего симптоматическое оперативное лечение в урологической клинике и продолжившего лечение у ревматолога патогенетической терапией (метилпреднизолон и мофетила микофенолат) со значительным улучшением. Для ревматологов данная патология представляет безусловный клинический интерес, поскольку, с одной стороны, в основе данных изменений лежат процессы, аналогичные таковым при системных заболеваниях соединительной ткани, а с другой стороны — это редкая патология, встречаемость РПФ составляет от 0,1 до 1,3 случая на 100000 человек в год. Однако чаще такие пациенты сразу попадают на прием к врачам смежных специальностей (урологам, нефрологам, хирургам, сосудистым хирургам), что подчеркивает важность разбора такого клинического случая.

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.

Idiopathic Retroperitoneal Fibrosis Associated with Rheumatoid Arthritis in a Patient with Concomitant Chronic B Viral Hepatitis

Retroperitoneal fibrosis (RPF) is a rare, progressive disease characterized by chronic non specific inflammation of the retroperitoneum. Although the pathogenesis of idiopathic retroperitoneal fibrosis (IRF) remains unclear, IRF has been reported in association with autoimmune disorders. However, few cases of IRF associated with rheumatoid arthritis (RA) have been reported. We experienced a rare case of IRF in a patient with RA and chronic B viral hepatitis. A 39-year-old Korean man with RA and hepatitis B was referred to our hospital due to left hydronephrosis. An abdominal computed tomography (CT) scan and magnetic resonance imaging (MRI) showed a diffuse infiltrating retroperitoneal mass around the abdominal aorta and left ureter. The patient underwent intraureteral stent insertion and was treated with corticosteroid. Three months later, the follow up abdominal CT showed that the retroperitoneal mass had decreased in size. Herein, we report the first case of coexistent IRF, RA, and chronic B viral hepatitis with a literature review. (J Rheum Dis 2015;22:123-126)

Idiopathic retroperitoneal fibrosis mimicking periprosthetic hip infection

Total hip arthroplasty (THA) provides pain relief for those suffering from severe osteoarthritis, inflammatory arthritis, post-traumatic arthritis, developmental dysplasia, osteonecrosis of the femoral head, and end stages of several other hip diseases [1]. Despite the high rates of long-term success, THA is not without failure [1]. Periprosthetic hip joint infection is one of the frustrating complications for the patient. Although there are no ‘‘gold standard’’ diagnostic tools, proper diagnosis is essential for successful treatment [2]. Idiopathic retroperitoneal fibrosis (IRF) is a rare syndrome characterized by the development of fibrosclerotic tissue in the retroperitoneum, which often leads to encasement of the ureters [3]. Ureteral obstruction causing acute or chronic renal insufficiency is the most common and severe complication of IRF [4, 5]. Serum markers of inflammation are usually, but not invariably, elevated [6]. It is known that involvement of the upper urinary tract may produce referred pain to right and left lower abdominal quadrants, testis/labia and even to the groin area, mimicking hip origin pain [7]. In this report, a case of IRF combined with osteolysis of the acetabular component is presented. Referred groin pain from IRF with elevated inflammatory laboratory findings can lead to misdiagnosis of periprosthetic hip joint infection. Revisional THA was performed for osteolysis and steroid therapy was used for IRF.

Idiopathic retroperitoneal fibrosis with particular perirenal and intrarenal extension associated with left renal artery stenosis. The atheromatous periaortitis with retroperitoneal fibrosis suggests a pathogenic relationship between atherosclerosis and fibrosis?

We present a case of idiopathic retroperitoneal fibrosis (RPF) in a female patient of 45 years, obese (BMI = 39 kg/sqm), hypertensive since 2005, with diabetes mellitus treated with diet and diabetes insipidus in whom, during a routine control, the following has been found: serum creatinine 1.74 mg/dl, and an inflammatory syndrome associated with fever. Spiral-CT (Multi-slice-Sensation 64) scan shows retroperitoneal fibrosis in relation with periaortitis that affects the thoracic and abdominal aorta. RPF is extending perirenally and at the level of the renal hilum with subsequent calyceal dilations (hydrocalycosis) associated with left renal artery stenosis. The particularity of the case is represented by the perirenal and intrarenal evolution of fibrosis with left renal artery stenosis with moderate impairment of renal function reversible under treatment with Tamoxifen. This case, with chronic periaortitis subsequent to an extended aortic atherosclerosis with retroperitoneal fibrosis can be representative for the pathogenic relationship between atherosclerosis and fibrosis.

Type 1 diabetes and idiopathic retroperitoneal fibrosis: Case report

Retroperitoneal fibrosis is characterized by the presence of a retroperitoneal tissue, consisting of chronic inflammation and marked fibrosis, which entraps the retroperitoneal organs. In two-thirds of cases, the retroperitoneal fibrosis is idiopathic. The pathogenic mechanism is not clearly identified. We report a case of idiopathic retroperitoneal fibrosis associated with type 1 diabetes mellitus. A 61-year-old woman with C peptide negative insulindependent diabetes developed retroperitoneal fibrosis revealed by bilateral hydronephrosis. Anti-GAD 65 antibodies were positive. There were no signs of autoimmune pancreatitis: no steatorrhea, normal IgG4 isotype levels, and absence of pancreas morphological abnormalities.

Association of idiopathic retroperitoneal fibrosis, rapidly progressive glomerulonephritis and antiproteinase 3 antineutrophil cytoplasmic antibodies (anti PR3-ANCA)

We report a case of idiopathic retroperitoneal fibrosis and rapidly progressive glomerulonephritis with serum antiproteinase 3 antineutrophil cytoplasmic antibodies (anti-PR3-ANCA), without clinical or histological signs of Wegener's granulomatosis, in a 46-year-old man. Our case and previously reported cases showing the same association support the hypothesis that the association is not fortuitous, but reflects a common immunological mechanism.

Idiopathic retroperitoneal fibrosis: a rare onset of the illness caused by haemorrhagic fever with renal syndrome

Sir, Idiopathic retroperitoneal fibrosis (IRF) is a collagen vascular disease of unknown aetiology. It is characterized by chronic, non-specific retroperitoneal inflammation, which may cause ureteric obstruction. Many authors believe that it is a type of immune disorder [1]. Bosnia and Herzegovina is known as a region where Hanta virus infection has been endemic for >50 years [2]. A case of IRF associated with haemorrhagic fever with renal syndrome (HFRS) has not been reported up to now. A 44-year-old, previously healthy man was hospitalized with acute renal failure. He was febrile 2 days before admission, had dull abdominal pain, decreased urine output, shortness of breath, diarrhoea and arterial hypertension (190/120mmHg). Blood tests showed metabolic acidosis (HCO3: 15.9mmol/l) and increased C-reactive protein (11.26mg/l), potassium (7.9mmol/l), serum creatinine (884 mmol/l), blood urea nitrogen (17.6mmol/l) and globulins (49.1 g/l) and decreased haemoglobin (7.4mmol/l). Urinanalysis showed proteinuria and leukocyturia. Urine culture was negative. Indirect immunofluorescene tests for Hanta viruses were positive for Pummala virus. Ultrasound showed acute renal parenchymal lesions with bilateral hydronephrosis, grades I–II, and widening of the wall of the abdominal aorta. The presence of a great number of rodents in the forest where the patient was working has been reported by the epidemiology service. After supportive, antihypertensive and diuretic therapy, the patient’s renal function stabilized, with serum creatinine at 187 mmol/l and potassium at 4.5mmol/l. Intravenous urography showed a functioning left kidney, with a suspected retrocaval ureter on the right side and dilation of the channels of the right kidney. A computed tomography contrast scan showed a solid retroperitoneal mass, in the form of thick plate of high density, extending from the level of the renal hilum down caudally to the bifurcation of the aorta (compatible with retroperitoneal fibrosis). A double-J stent was applied and steroids and androgens were administered (pronison 60mg plus tamoxifen 20mg 2). After 3 months, the stents were removed and medications were continued. After 6 months, the patient’s total DTPA clearance was 61.1ml/min (11.8ml/min in the left kidney and 49.3ml/min in the right kidney), measured by technetium marked by diethylaminoacid. Steroids and androgens were withdrawn after 12 months. The patient has normal blood pressure and stable renal function, with serum creatinine at 125mmol/l. It remains a mystery whether HFRS triggered an immune abnormality and acceleration of the symptoms of a latent IRF or whether the two diseases merely coincided. Adequate treatment of HFRS was certainly the reason that renal function recovered and the progression of the disorder caused by the chronic disease, IRF, was hampered.

痛風発症に特発性後腹膜線維症の関与が疑われた一例

患者は64歳男性で, 腹部CTスキャンで偶然に特発性後腹膜線維症(IRF)が見出される半年前に, 痛風発作を初発. IRFによる自覚症状はないが, 血中の可溶性インターロイキン2受容体( sIL-2R ) は4510U/mlの異常高値であった. プレドニゾロン投与にて約4週後に, 後腹膜の線維化はほぼ消退し, sIL-2Rは994U/mlへ減少した. 治療前後で尿酸代謝の各指標(平均±SD, n=4)を比較すると, 血清尿酸値(7.3±0.2vs.5.9±0.4mg/dl, P =0.0003) と24時間尿中尿酸排泄量(528±78vs.384±55mg/day, P=0.02)は, 治療後に有意に低下したが, 尿酸クリアランス(5.0±0.8vs.45±05ml/min, P=0.29)や尿酸・クレアチニンクリアランス比(5.0±0.3vs.5.0±1.0%, P=0.89)は有意差がなかった. IRFでは, 浸潤する炎症性細胞と線維芽細胞の増生に基づく核酸代謝回転の亢進やサイトカイン分泌によって痛風を招来する可能性が示唆され, 二次性痛風の原因疾患の一つとして鑑別を要すと考えられる.

A CASE REPORT OF IDIOPATHIC RETROPERITONEAL FIBROSIS FORMING A LOCALIZED TUMOR

We report a case of idiopathic retroperitoneal fibrosis forming a localized tumor, which is relatively rare. A 55-year-old man was found to have a retroperitoneal tumor by abdominal ultrasonogram at a medical check up and was admitted to our hospital. On admission, he was asymptomatic and no physical abnormalities were seen. Abdominal CT scans revealed a mass in the left retroperitoneal space. The mass was confirmed to show low intensity on both Tl and T2 weighted images in MRI, which suggested fibrous component. Since a possibility of malignant tumor could not be still ruled out, the tumor was removed for the purpose of examination and treatment. The pathological diagnosis was idiopathic retroperitoneal fibrosis. The patient is strictly followed in the out patient clinic and no signs of recurrence have been observed. It is necessary to consider idiopathic retroperitoneal fibrosis in case of retroperitoneal localized mass.

Long-term follow-up of low-dose methotrexate therapy in one case of idiopathic retroperitoneal fibrosis

Idiopathic retroperitoneal fibrosis (RPF) is characterized by the development of a fibrotic mass surrounding the abdominal aorta and its branches, of unknown aetiology. Several immunological mechanisms can be operative in the pathogenesis of RPF. Based on this assumption we treated a patient affected by idiopathic RPF with low-dose methotrexate (MTX) therapy. To our knowledge this is the first example of the effectiveness and safety of a long-term low-dose MTX treatment in the post-surgical management of RPF.

Idiopathic retroperitoneal fibrosis infiltrating ureteral wall

We describe the eighth case of idiopathic retroperitoneal fibrosis infiltrating the ureter to cause intrinsic obstruction. Discovery of this situation intraoperatively necessitates a change in treatment plan, as ureterolysis is not sufficient.

高齢発症の特発性後腹膜線維症の１例

An elderly case of idiopathic retroperitoneal fibrosis (IRPF) with bilateral ureteral obstruction was reported. A 74-year-old man was admitted with complaints of general fatigue and loss of appetite. An elastic hard mass with a smooth surface was palpated in the left upper quadrant of the abdomen. Abnormal ESR and CRP were noticed. Abdominal ultrasonography showed both a homoechoic mass which surrounded the aorta in the retroperitoneal space and bilateral hydronephrosis. Computed tomography revealed a retroperitoneal mass involving bilateral ureters. Magnetic resonance imaging demonstrated a mass with a sharp image and signal intensity. Furthermore the morphological relationship between the mass and the ureters and major vessels clearly indicated the characteristics of benign retroperitoneal fibrosis. Based upon the above findings, a diagnosis of IRPF was made. After confirming the histological diagnosis by biopsy, treatment consisting of bilateral ureterolysis, intra-abdominal transposition of ureters and oral administration of prednisolone was performed, resulting in a normalization of laboratory findings. Analysis of 86 previously cases of IRPF reported up to 1990 in Japan, revealed the peak age to be in the 7th decade with predominance in males (males: females = 57:29).

IDIOPATHIC RETROPERITONEAL FIBROSIS

Abstract A case of idiopathic retroperitoneal fibrosis of an unusual site, at the upper pole of the right kidney, is reported. The case was confirmed histologically and treated with glucocorticoid for nine months. This resulted in definite clinical improvement and complete normalization of the elevated ESR and abnormal serum electrophoresis. Compression of the inferior vena cava, demonstrated by cavography, was significantly less marked after the corticoid medication. The patient has been followed for eight months after discontinuation of the treatment, and throughout this period he has remained symptom‐free and fully capable of working. As a conclusion from this case of idiopathic retroperitoneal fibrosis, and from reports in the literature, the author feels that all cases of idiopathic retroperitoneal fibrosis should be treated with glucocorticoid in high doses over a long period, also when surgery is indicated.

IDIOPATHIC RETROPERITONEAL FIBROSIS SIMULATING ADVANCED PELVIC CARCINOMA.

SUMMARYA case of idiopathic retroperitoneal fibrosis, in which the fibrotic process was completely confined to the pelvis, is described. The first presenting symptom was dyspareunia but the condition was not diagnosed until serious ureteric obstruction had occurred with threatening renal failure. The physical findings then were those of a “frozen pelvis” simulating advanced carcinoma. The ureteric obstruction was relieved by uretero‐ileocystostomy, and after this operation a striking regression of the fibrosis occurred.Addendum.‐Some weeks after the completion of this paper, this case developed in an unexpected direction. The patient began to complain of dyspepsia and at the same time experienced increasing difficulty in emptying the bladder. Renal function remained unimpaired. Barium meal showed a small lesion of the stomach near the cardia with the characteristic appearance of a leiomyoma. Pelvic examination showed a recurrence of the induration and fibrosis of the pelvic floor. At cystoscopy the urethra was narrowed by the mechanical strangulation of the extrinsic fibrosis, but the bladder and bladder neck appeared normal. Repeated dilatation of the urethra failed to give relief and the establishment of an external urinostomy appeared to be the only way round the difficulty. This was done on 7th May 1964, the previously constructed ileal loop being disconnected from the bladder and brought out as a stoma in the left iliac fossa.Dyspepsia persisted, and a repeat barium meal showed such an increase in size of the lesion as to suggest a malignant process. The urinostomy functioned well, and she managed it with little inconvenience: but the bladder, although isolated now from the urinary tract, continued to fill up with infected mucus. By July the pelvic floor felt as solid and “frozen” as it had done when the patient was first seen two years earlier. Mr R. de Vere kindly saw her again and, to provide drainage of the now completely obstructed bladder, established a deliberate vesicovaginal fistula. The vesicovaginal septum at the operation was more than a centimetre thick, and tissue was taken for histological examination. This now showed an entirely different picture, with widespread infiltration of the connective tissue by an undoubted undifferentiated sarcoma.Soon after this, she became jaundiced and rapidly deteriorated, dying at home on 16th August. Permission for post‐mortem examination was unfortunately withheld.Many writers on this subject have stated that the histological diagnosis rarely offers any difficulty. Trever (1958), however, has described a case which presented as retroperitoneal fibrosis, but which ended fatally as a sarcoma, and 1 have seen a similar case. The dramatic resolution and remission seen in the present case does not accord well with a diagnosis of sarcoma ab initio, though Dr J. H. O. Earle, who has kindly reviewed the original sections including a biopsy taken at another hospital in 1961, feels that he would have been unwilling to exclude the possibility of malignancy even in the first section taken.Whether or not the lesion in this case was indeed a sarcoma from the onset cannot be established with certainty. A further lesson to be learnt from it is that cases of retroperitoneal fibrosis should be kept under close and critical review even though a long‐standing remission, either with or without treatment, seems to have been established.

A case of idiopathic retroperitoneal fibrosis presenting as a systemic collagen disease

A case of idiopathic retroperitoneal fibrosis is reported, with concomitant Raynaud's disease, pulmonary fibrosis, keloid scars and the presence of antinuclear factors in the blood. It is suggested that these unusual features are evidence in favor of regarding idiopathic retroperitoneal fibrosis as a collagen or an autoimmune disease.