High resolution computed tomography as a predictor of lung histology in systemic sclerosis

Background The relative proportions of fibrosis and inflammation seen by open lung biopsy examination is a predictor of disease outcome in fibrosing alveolitis. This study was designed to assess the ability of high resolution computed tomography to predict the histological appearance of open lung biopsy specimens from patients with systemic sclerosis.     

Lingular and right middle lobe biopsy in the assessment of diffuse lung disease

It has been said that the lingula and right middle lobe should be avoided for open-lung biopsy because of nonspecific fibrosis and vascular changes. To determine if the diagnostic yields of lingular or right middle lobe biopsy specimens were unsatisfactory, we reviewed the results of open-lung biopsy in 73 adult patients; 26 were immunocompromised and 47, nonimmunocompromised. We found no evidence to suggest that these two sites were inherently inferior. In 20 of the nonimmunocompromised patients, computed tomography was performed prior to biopsy, and demonstrated no particular tendency for greater involvement of the lingula or right middle lobe. We conclude that lingular and right middle lobe biopsy is useful in the diagnosis of parenchymal lung disease and that these sites should not necessarily be avoided. Computed tomographic scanning prior to biopsy is helpful in guiding the surgeon to the appropriate sites from which to obtain biopsy specimens.     

The significance of parenchymal changes of acute cellular rejection in predicting chronic liver graft rejection

BACKGROUND: Chronic rejection (CR) in liver allografts shows a rapid onset and progressive course, leading to graft failure within the first year after transplantation. Most cases are preceded by episodes of acute cellular rejection (AR), but histological features predictive for the transition toward CR are not well documented. METHODS: We assessed the predictive value of centrilobular necrosis, central vein endothelialitis (CVE), central vein fibrosis, and lobular inflammation in the development of CR. One-week and one-month biopsy specimens of 12 patients with CR were compared with those of a control group consisting of 17 patients, who experienced AR without developing CR. The progress of the histological changes was further evaluated in follow-up biopsy specimens of the CR group taken at 2 months and beyond 3 months after transplantation. RESULTS: Centrilobular necrosis, CVE, central vein fibrosis, and lobular inflammation were common features in both groups at 1 week. At 1 month, the incidence declined in the control group. The CR group showed an increased incidence and persistence of these features in the follow-up biopsy specimens. The incidence and median grade of severity of CVE was significantly higher in the CR group (P=0.04 and P<0.001). The severity of portal and lobular inflammation was also more pronounced in the CR group (P=0.01 and 0.068). Conversely, in the control group, the incidence of the lobular features decreased and the severity of CVE declined significantly (P=0.03). CONCLUSION: The shift from a predominantly portal-based process toward lobular graft damage represents the early transition of AR to CR, for which a modification of immunosuppression might be necessary to prevent graft loss.     

Can early liver biopsies predict long-term outcome of the graft?

Background: Chronic rejection (CR) in liver allografts show a rapid onset and progressive course, leading to graft failure within the first year after transplantation. Most cases are preceded by episodes of acute cellular rejection (AR), but histological features predictive for the transition toward CR are not well documented. Method: We assessed the predictive value of centrilobular necrosis, central vein endothelialitis (CVE), central vein fibrosis, and lobular inflammation in the development of CR. One-week and one-month biopsy specimens of 12 patients with CR were compared with those of a control group consisting of 17 patients, who experienced AR without developing CR. The progress of the histological changes was further evaluated in follow-up biopsy specimens of the CR group taken at 2 months and beyond 3 months after transplantation. Result: Centrilobular necrosis, CVE, central vein fibrosis, and lobular inflammation were common features in both groups at 1 week. At 1 month, the incidence declined in the control group. The CR group showed an increased incidence and persistence of these features in the follow-up specimens. The incidence and median grade of severity of CVE was significantly higher in the CR group (p=0.04, and P<0.001). The severity of portal and lobular inflammation was also more pronounced in the CR group (P+0.01 and 0.069). Conversely, in the control group the incidence of the lobular features decreased and the severity of CVE declined significantly (P=0.03). Conclusion: The shift from a predominantly portal-based process toward lobular graft damage represents the early transition of AR to CR, for which a modification of immunosuppression might be necessary to prevent graft loss.     

Tomography versus computed tomography for assessing step off in intraarticular distal radial fractures

Computed tomography scans have supplanted conventional tomography for many applications and often are considered the imaging study of choice for assessing intraarticular distal radial fractures. Concern about cost containment in healthcare delivery prompts the question of whether the two studies provide comparable information and at what cost. Common intraarticular distal radial fractures were created in 12 lightly embalmed cadaveric specimens. The fractures were fixed with radiolucent Kirschner wires. Articular step off was measured with a caliper. Plain radiographs, computed tomography scans, and trispiral tomograms were obtained of each specimen. Maximal step off was measured blindly by two musculoskeletal radiologists and four hand surgeons. The radiographic measurements were compared with the actual step off and expressed as a positive or negative deviation from the actual value. There was no statistically significant difference between computed tomography scans and tomograms in predicting step off. In addition, the difference between actual and radiographic measurements was insignificant in tomogram readings and different in one of the computed tomography measurements. In the authors' institution, a tomogram costs $200, and a computed tomography scan costs $562. Trispiral tomography is more accurate and cost effective than computed tomography, and thus when available should be considered the imaging modality of choice for assessing articular step off in distal radius fractures.     

Fluorescence as a guide to bronchial biopsy.

The presence of fluorescence was assessed in 34 unselected patients undergoing bronchoscopy, 11 of whom suffered from bronchial carcinoma. Bronchoscopic inspection was carried out with white light and then repeated with blue light after the injection of 2 ml of 10% sodium fluoresceinate. Fluorescence was graded visually. Seventy six pairs of biopsy specimens were obtained. Of 38 specimens from non fluorescent areas, histological appearances were normal in 34. Slight inflammation was observed in three biopsy specimens, and in one there was evidence of tumour even though the specimen was obtained some distance from the primary tumour. Of 38 biopsy specimens from fluorescent areas, histological examination showed appearances of moderate inflammation in 10, severe inflammation in 10, tuberculosis in one, and carcinoma in 11. In six instances histological appearances were normal. Five normal subjects who were smokers were also examined by the same technique. Appearances of metaplasia were found more often in areas showing fluorescence than in areas not showing fluorescence. Bronchial fluorescence is considered to be related to alteration in vascularity and is not specific for carcinoma. The most fluorescent areas are generally the most pathological and visible tumour is very fluorescent, but moderate degrees of fluorescence are difficult to interpret. Biopsy of every fluorescent area seems excessive.     

Chronic bird fancier's lung: histopathological and clinical correlation. An application of the 2002 ATS/ERS consensus classification of the idiopathic interstitial pneumonias

Background: Chronic bird fancier''s lung (BFL) has often been misdiagnosed as one of the idiopathic interstitial pneumonias (IIPs). Methods: To define the clinical and pathological characteristics of chronic BFL, 26 patients with chronic BFL from whom a surgical lung biopsy specimen was taken between October 1992 and June 2001 were evaluated. The histopathological characteristics of the surgical lung biopsy specimens were examined and correlations between the histopathology and clinical characteristics were analysed. The quality of chronic inflammatory and fibrotic changes was expressed according to the 2002 ATS/ERS consensus classification of IIPs. Results: Two patients were diagnosed as having bronchiolitis obliterans organising pneumonia (BOOP)-like lesions, five as having cellular non-specific interstitial pneumonia (NSIP)-like lesions, and eight as having fibrotic NSIP-like lesions. The other 11 patients were considered to have usual interstitial pneumonia (UIP)-like lesions because of the temporal heterogeneous appearances of the fibrotic changes. However, fibrosis in these patients had developed in centrilobular as well as perilobular areas, suggestive of hypersensitivity pneumonitis. Nineteen patients (73.1%) had multinucleated giant cells, often with cholesterol clefts, while only five patients (19.2%) had granulomas. Patients with BOOP-like or cellular NSIP-like lesions tended to have recurrent acute episodes, whereas patients with UIP-like lesions had an insidious onset. Patients with BOOP-like or cellular NSIP-like lesions had a more favourable outcome than those with fibrotic NSIP-like and UIP-like lesions. Conclusions: The qualities of chronic inflammatory and fibrotic lesions vary significantly among patients with chronic BFL but correlate with clinical features and prognosis.     

Computed tomography-guided bronchoalveolar lavage in idiopathic pulmonary fibrosis.

BACKGROUND: High resolution computed tomography (HRCT) is now recognised as a sensitive tool for predicting the histological characteristics of the lung parenchymal abnormalities in patients with idiopathic pulmonary fibrosis (IPF). A reticular pattern on HRCT scanning is indicative of fibrotic histology while a ground glass pattern has been associated with inflammatory disease. The purpose of the present study was to investigate whether the cell population in the bronchoalveolar lavage (BAL) fluid from different lobes differs according to HRCT characteristics in patients with IPF. METHODS: Twenty six patients with IPF (18 men) of mean (SE) age 67 (2) years were included in the study. A semiquantitative analysis of the extent of the abnormalities on the HRCT scan was applied by summing the proportion of both reticular and ground glass patterns in each lobe (expressed as percentage of total area evaluated) and 100 ml double BAL was then randomly performed in the lobe with the most extensive involvement (lobe A) and that with the least extensive involvement (lobe B). RESULTS: Twenty three of the 26 patients (88%) had an abnormal cell count in the BAL fluid from lobe A compared with 18 patients (69%) with abnormalities in the BAL fluid from lobe B. The median (range) percentage of 8.5% (0-34%) and the absolute numbers of neutrophils (1.3 x 10(4)/ml, 0-14.6 x 10(4)/ml) in lobe A were significantly higher than those in lobe B (5% (0-26%) and 1.2 x 10(4)/ml (0-5 x 10(4)/ml), respectively). The percentage (3%, 0-19%) and absolute numbers (0.65 x 10(4)/ml, 0-4 x 10(4)/ml (0-4.8 x 10(4)/ml), respectively). For the group as a whole a correlation was found between the percentage and absolute numbers of neutrophils in the BAL fluid and the total score of abnormalities on the HRCT scan in the most involved lobe (lobe A). Multiple regression analysis indicated that both the percentage and absolute numbers of neutrophils were significantly and independently related to the extent of ground glass pattern. CONCLUSIONS: In patients with IPF the cell population in the BAL fluid is not homogeneous and seems to be related to the characteristics of the abnormalities on the HRCT scan present in the lavaged lobe.     

Pirfenidone reduces subchondral bone loss and fibrosis after murine knee cartilage injury

Pirfenidone is an anti‐inflammatory and anti‐fibrotic drug that has shown efficacy in lung and kidney fibrosis. Because inflammation and fibrosis have been linked to the progression of osteoarthritis, we investigated the effects of oral Pirfenidone in a mouse model of cartilage injury, which results in chronic inflammation and joint‐wide fibrosis in mice that lack hyaluronan synthase 1 (Has1^?/?) in comparison to wild‐type. Femoral cartilage was surgically injured in wild‐type and Has1^?/? mice, and Pirfenidone was administered in food starting after 3 days. At 4 weeks, Pirfenidone reduced the appearance, on micro‐computed tomography, of pitting in subchondral bone at, and cortical bone surrounding, the site of cartilage injury. This corresponded with a reduction in fibrotic tissue deposits as observed with gross joint surface photography. Pirfenidone resulted in significant recovery of trabecular bone parameters affected by joint injury in Has1^?/? mice, although the effect in wild‐type was less pronounced. Pirfenidone also increased Safranin‐O staining of growth plate cartilage after cartilage injury and sham operation in both genotypes. Taken together with the expression of selected extracellular matrix, inflammation, and fibrosis genes, these results indicate that Pirfenidone may confer chondrogenic and bone‐protective effects, although the well‐known anti‐fibrotic effects of Pirfenidone may occur earlier in the wound‐healing response than the time point examined in this study. Further investigations to identify the specific cell populations in the joint and signaling pathways that are responsive to Pirfenidone are warranted, as Pirfenidone and other anti‐fibrotic drugs may encourage tissue repair and prevent progression of post‐traumatic osteoarthritis. © 2017 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 36:365–376, 2018.

     

Retroperitoneal fibrosis secondary to different etiologies (hemilaminectomy and hypothyroidism): reports of two cases

Retroperitoneal fibrosis (RF) is a clinical entity characterized by the progressive proliferation of connective tissue that rarely forms a mass involving the periaortic area of the abdomen, which may be idiopathic as well as a result of an inflammatory process after aneurysmal dilatation of the aorta. This fibrotic tissue may cover both aorta and iliac arteries, reach the retroperitoneum and surrounding ureters, and cause serious obstructions and renal insufficiency in three-quarters of patients. Most of the patients are known to have atherosclerosis and local inflammation against the antigens of the plaques. A systemic autoimmune disease presenting with retroperitoneal fibrosis seems to be pronounced more frequently nowadays because of the elevated acute-phase reactant levels, positive autoantibodies, and concurrent autoimmune diseases affecting other organs in majority of the diagnosed patients. Ultrasonography, computed tomography, magnetic resonance imaging, positron emission tomography, and retroperitoneal biopsy are useful in diagnosing and assessing the full extent of the disease. Surgical interventions such as ureterolysis and aneurysm repair are frequently performed, but medical therapy including steroids and immunosuppressants is often needed because of the inflammatory and chronic-relapsing nature of the disease. In this paper, we described two cases diagnosed with RF secondary to hemilaminectomy and hypothyroidism, and we summarized the literature related to RF.     

Pathological changes in yearly protocol liver biopsy specimens from healthy pediatric liver recipients

Many centers perform biopsies on transplanted livers annually to assess allograft function because serum biochemical tests do not always correlate with histological findings. Although criteria exist for diagnosing acute cellular rejection, no similar criteria exist to describe the histopathological changes observed in the "normal" liver of an immunosuppressed but healthy child. The purpose of this study was to define the histopathological changes in the allografted livers of healthy children who have undergone transplantation and to evaluate them during long-term follow-up. One hundred fifty-eight yearly protocol liver biopsy specimens of 54 children who received transplants between January 1988 and March 1996 and at least 1 year of follow-up were reviewed, and the biopsy findings were correlated with those of serum tests of liver function performed concomitantly. Thirty-three biopsy specimens were excluded because serum transaminase levels were abnormal, the biopsy specimen was abnormal and diagnostic for a specific lesion, or follow-up showed progression of a specific disease process. In addition, time zero biopsy specimens from 21 of the 54 children were available for comparison. In the protocol biopsy specimens, portal and/or parenchymal mononuclear inflammatory infiltrates were frequent findings (48% and 25%, respectively). Other less common features were mild fibrosis (8%) and focal pericholangitis (6%). Findings in both protocol and time zero biopsy specimens included minimal to mild bile ductular proliferation (15% and 9.5%, respectively) and rare hepatocyte necrosis (1.6% and 5%). No yearly protocol biopsy specimens resulted in any patient benefit. Transplanted livers in immunosuppressed children who are clinically healthy and have normal transaminase levels commonly show histological changes consisting of scattered, mild to moderate, portal and/or parenchymal mononuclear infiltrates that are clinically insignificant. Yearly protocol biopsies in healthy pediatric recipients have been abandoned by the investigators after 3 years of follow-up. (Liver Transpl Surg 1997 Nov;3(6):559-62)     

The histology of interstitial cystitis

Several studies have reported histologic findings in interstitial cystitis (IC) bladder biopsy specimens. However, these studies used a variety of criteria to define IC, which may explain the variation noted in the histologic changes. Clinical experience shows that these biopsy specimens are often not helpful in confirming the diagnosis. Our study was designed to examine the histologic features identified in bladder biopsy specimens from patients with IC and compare them with biopsy specimens from a control population. Although IC patients as a group had a higher incidence and degree of denuded epithelium, ulceration, and submucosal inflammation, none of these findings was pathognomonic. In addition, these findings occurred only in interstitial cystitis patients with pyuria or small bladder capacity. The inflammatory infiltrate seen in IC was composed predominantly of lymphocytes, with increasing numbers of plasma cells as the degree of inflammation increased. There was no specific predilection for the inflammatory infiltrate to be perineural. Submucosal inflammation was associated with denuded epithelium, ulceration, pyuria, and a clinical response to therapy suggesting a pathophysiologic relationship. Epithelial and basement membrane thickness, submucosal edema, vascular ectasia, fibrosis, and detrusor muscle inflammation and fibrosis were not significantly different in the IC and control patients. These findings suggest that IC is a chronic submucosal inflammatory disease, at least in those patients with small bladder capacities or pyuria. IC is best diagnosed from its clinical features; the histologic changes identified in the bladder biopsy play a supportive role in this diagnosis. Mast cells play a limited role in the diagnosis of IC.     

Cerebral tumors: Specific features in children

Brain tumors are the second leading cause of cancer in children. Primary tumors predominate and are of very varied histological types. Their prognosis and treatment depend on the histological type and grade. The diagnostic approach to these includes analysis of the site of the lesion and appearances on computed tomography and MR, and taking account of the age and clinical features of the child. CT is used to diagnose the tumor in an emergency situation. Conventional MR provides a morphological approach and allows a staging assessment to be carried out before surgery. Advanced MR techniques (diffusion-weighted and perfusion imaging, MR spectroscopy) provide further information for the differential diagnosis, presumptive diagnosis of type and grade and to guide biopsy towards the most malignant areas in the lesion.     

Retroperitoneal Fibrosis Secondary to Different Etiologies (Hemilaminectomy and Hypothyroidism): Reports of Two Cases

Retroperitoneal fibrosis (RF) is a clinical entity characterized by the progressive proliferation of connective tissue that rarely forms a mass involving the periaortic area of the abdomen, which may be idiopathic as well as a result of an inflammatory process after aneurysmal dilatation of the aorta. This fibrotic tissue may cover both aorta and iliac arteries, reach the retroperitoneum and surrounding ureters, and cause serious obstructions and renal insufficiency in three-quarters of patients. Most of the patients are known to have atherosclerosis and local inflammation against the antigens of the plaques. A systemic autoimmune disease presenting with retroperitoneal fibrosis seems to be pronounced more frequently nowadays because of the elevated acute-phase reactant levels, positive autoantibodies, and concurrent autoimmune diseases affecting other organs in majority of the diagnosed patients. Ultrasonography, computed tomography, magnetic resonance imaging, positron emission tomography, and retroperitoneal biopsy are useful in diagnosing and assessing the full extent of the disease. Surgical interventions such as ureterolysis and aneurysm repair are frequently performed, but medical therapy including steroids and immunosuppressants is often needed because of the inflammatory and chronic-relapsing nature of the disease.

In this paper, we described two cases diagnosed with RF secondary to hemilaminectomy and hypothyroidism, and we summarized the literature related to RF.     

Transurethral implantation of silicone polymer for stress incontinence: evaluation of a porcine model and mechanism of action in vivo

OBJECTIVE: To evaluate the anatomical location, histological appearances and mechanism of action of sili-cone polymer (Macroplastique(R)) after transurethral implantation. MATERIALS AND METHODS: An in vivo pig model was developed to investigate the organization of Macroplastique 6 weeks after injection. The results were compared with the immediate appearance of injected Macroplastique in a surgical training device (using isolated pig urethrae) and with the histological appearances of Macroplastique in an explanted human specimen. RESULTS: Macroplastique was subserosal in all specimens; it provoked a marked foreign-body inflammatory reaction, producing firm nodules 6 weeks after implantation. The histological appearances in the in vivo pig model and the human specimen were indistinguishable. CONCLUSIONS: These findings indicate that the mechanism of action of Macroplastique is to augment pressure transmission to the proximal urethra by producing focal but firm expansion of periurethral tissues.     

Chronic hypersensitivity pneumonitis

Hypersensitivity pneumonitis (HP) is traditionally divided on clinical grounds into acute, subacute, and chronic stages. Most biopsy specimens come from patients in the subacute stage, in which there is a relatively mild, usually peribronchiolar, chronic interstitial inflammatory infiltrate, accompanied in most cases by poorly formed interstitial granulomas or isolated giant cells. However, the pathologic features in the chronic, ie, fibrotic stage, are poorly defined in the literature. These features are important to recognize because the chronic stage of HP is often associated with a poor prognosis. We reviewed 13 cases of chronic HP. Where information was available, exposures to the sensitizing agent had generally occurred over a long period of time. Three patterns of fibrosis were seen: 1) predominantly peripheral fibrosis in a patchy pattern with architectural distortion and fibroblast foci resembling, microscopically, usual interstitial pneumonia (UIP); 2) relatively homogeneous linear fibrosis resembling fibrotic nonspecific interstitial pneumonia (NSIP); and 3) irregular predominantly peribronchiolar fibrosis. In some instances, mixtures of the UIP-like and peribronchiolar patterns were found. In all cases, the presence of scattered poorly formed granulomas, or isolated interstitial giant cells, or sometimes only Schaumann bodies indicated the correct diagnosis. In 7 cases, areas of typical subacute HP were present as well. High-resolution CT scans showed variable patterns ranging from severe fibrosis, in some instances with an upper zone predominance, to predominantly ground glass opacities with peripheral reticulation. We conclude that, at the level of morphology, chronic HP may closely mimic UIP or fibrotic NSIP. If no areas of subacute HP are evident, the presence of isolated giant cells, poorly formed granulomas, or Schaumann bodies is crucial to arriving at the correct diagnosis, and the finding of peribronchiolar fibrosis may be helpful. Despite the presence of extensive fibrosis, some patients responded to removal from exposure and steroid therapy.     

High resolution computed tomographic assessment of asbestosis and cryptogenic fibrosing alveolitis: a comparative study.

BACKGROUND: The aim of this study was to compare the distribution and configuration of lung opacities in patients with cryptogenic fibrosing alveolitis and asbestosis by high resolution computed tomography. METHODS: Eighteen patients with cryptogenic fibrosing alveolitis and 24 with asbestosis were studied. Two independent observers assessed the type and distributions of opacities in the upper, middle, and lower zones of the computed tomogram. RESULTS: Upper zone fibrosis occurred in 10 of the 18 patients with cryptogenic fibrosing alveolitis and in six of the 24 patients with asbestosis. A specific pattern in which fibrosis was distributed posteriorly in the lower zones, laterally in the middle zones, and anteriorly in the upper zones was seen in 11 patients with cryptogenic fibrosing alveolitis and in four with asbestosis. Band like intrapulmonary opacities, often merging with the pleura, were seen in 19 patients with asbestosis but in only two with cryptogenic fibrosing alveolitis. Areas with a reticular pattern and a confluent or ground glass pattern were the commonest features of cryptogenic fibrosing alveolitis (15 and 14 patients respectively) but were uncommon in asbestosis (four and three patients). Pleural thickening or plaques were seen in 21 patients with asbestosis and in none with cryptogenic fibrosing alveolitis. CONCLUSION: Apart from showing pleural disease high resolution computed tomography showed that confluent (ground glass) opacities are common in cryptogenic fibrosing alveolitis and rare in asbestosis whereas thick, band like opacities are common in asbestosis and rare in cryptogenic fibrosing alveolitis.     

Bronchoscopic findings in children with non-cystic fibrosis chronic suppurative lung disease

BACKGROUND: Published data on the frequency and types of flexible bronchoscopic airway appearances in children with non-cystic fibrosis bronchiectasis and chronic suppurative lung disease are unavailable. The aims of this study were to describe airway appearances and frequency of airway abnormalities and to relate these airway abnormalities to chest high resolution computed tomography (cHRCT) findings in a cohort of children with non-cystic fibrosis chronic suppurative lung disease (CSLD). METHODS: Indigenous children with non-cystic fibrosis CSLD (>4 months moist and/or productive cough) were prospectively identified and collected over a 2.5 year period at two paediatric centres. Their medical charts and bronchoscopic notes were retrospectively reviewed. RESULTS: In all but one child the aetiology of the bronchiectasis was presumed to be following a respiratory infection. Thirty three of the 65 children with CSLD underwent bronchoscopy and five major types of airway findings were identified (mucosal abnormality/inflammation only, bronchomalacia, obliterative-like lesion, malacia/obliterative-like combination, and no macroscopic abnormality). The obliterative-like lesion, previously undescribed, was present in 16.7% of bronchiectatic lobes. Structural airway lesions (bronchomalacia and/or obliterative-like lesion) were present in 39.7% of children. These lesions, when present, corresponded to the site of abnormality on the cHRCT scan. CONCLUSIONS: Structural airway abnormality is commonly found in children with post-infectious bronchiectasis and a new bronchoscopic finding has been described. Airway abnormalities, when present, related to the same lobe abnormality on the cHRCT scan. How these airway abnormalities relate to aetiology, management strategy, and prognosis is unknown.     

Bilateral ureteral stricture due to ureteral fibrosis. Case report

A case of bilateral ureteral fibrosis is reported. The patient, a 32-year-old man, was admitted as a result of acute renal failure. Ultrasonography showed bilateral hydronephrosis, so that in-dwelling percutaneous nephrostomy tubes were immediately inserted in the bilateral kidneys. Since retrograde and antegrade pyelograms revealed bilateral midureteral stricture, idiopathic retroperitoneal fibrosis at first suspected, but no mass in the retroperitoneal space was found by a computed tomogram. An exploratory laparotomy showed no fibrous mass in the retroperitoneal space either, but that the wall of the ureter was thickened. Biopsy of the ureter led to a histological diagnosis of fibrosis. Since evidence of inflammation such as elevation of the erythrocyte sedimentation rate and cross-reacting protein was found, 50 mg of prednisolone has been administered every other day, with the result that inflammation is now under control. This report deals with specific ureteral fibrosis related to idiopathic systemic fibrosis and collagen disease.