SEARCH · Search PubMed

Results for “FIBROSITIS”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

SourceResearch connectionFrom yearThrough year

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 235 records · Page 13Linked to original sources

PubMed2001

Nephrogenic fibrosing dermopathy.

This report details the histopathologic findings in a unique fibrosing disorder that recently emerged among patients with renal disease. The affected patients were initially identified among recipients of renal transplants at a single institution, but later cases at other centers were identified, and included patients receiving renal dialysis for a variety of different kidney diseases. The cutaneous changes consisted largely of indurated plaques and papules on the extremities and trunk. Systemic findings seen in scleromyxedema, which the condition resembles in some respects, were absent. By routine microscopy, the findings range from a very subtle proliferation of dermal fibroblasts in early lesions, to a florid proliferation of fibroblasts and dendritic cells in fully developed cases. Thick collagen bundles with surrounding clefts are a prominent finding, and a variable increase in dermal mucin and elastic fibers was usually evident with special stains. CD-34 positive dermal dendrocytes were floridly abundant, with dendritic processes aligned with elastic fibers and around collagen bundles in a dense network. Factor XIIIa and CD-68 positive mono-and multinucleated cells are also present in increased numbers. Electron microscopy highlighted increased elastic fibers closely apposed to dendritic cell processes. The entire dermis was commonly involved, with increased spindle cells, collagen, mucin, and elastic fibers extending through the subcutis along the septa of fatty lobules. In some instances, the process resembled a sarcoma on histopathologic examination. The recent emergence of this condition and the apparent clustering of cases in specific dialysis centers initially suggested a possible infectious and/or toxic agent. To date, however, no such agent has been identified. We propose the term "nephrogenic fibrosing dermopathy (NFD)" until a specific cause can be identified.

Adult↗

PubMed2003

Nephrogenic fibrosing dermopathy after liver transplantation successfully treated with plasmapheresis.

Nephrogenic fibrosing dermopathy (NFD) is a recently described cutaneous fibrosing disorder associated with renal dysfunction. It appears similar to scleromyxedema but with some notable exceptions, including the lack of involvement of the face and absence of plasma cells on histology, systemic involvement, and paraproteinemia. Patients can present with thickened or edematous skin with indurated papules and plaques involving the extremities and the trunk. We report the first three cases of NFD after liver transplantation successfully treated with plasmapheresis. Two patients underwent liver transplantation for hepatitis C virus-induced cirrhosis and one for hepatitis B virus-induced cirrhosis. All the patients had encephalopathy, refractory ascites, and malnutrition prior to transplantation. Like those patients with NFD, all three of our patients had renal dysfunction and required hemodialysis before and after transplantation. Two were not dependent on dialysis at the time of diagnosis, however. These patients had excellent liver allograft function, but the other patient had allograft failure secondary to recurrent hepatitis C. Immunosuppression therapy consisted of basiliximab, mycophenolate mofetil, calcineurin inhibitor, and prednisone. The patients developed "woody" skin induration of the distal extremities, erythematous papules, and contractures at 1, 2, and 120 months after transplantation. Skin biopsies resembled NFD. No paraproteinemia was evident. One to three 5-day courses of plasmapheresis resulted in moderate to marked clinical improvement. The improvement of the kidney function in two of our patients did not appear to correlate with that of the skin disorder, because the kidney function was improving at the time the diagnosis of NFD was made. In conclusion, we report the first three cases of NFD after liver transplantation. Plasmapheresis was moderately successful in resolving the skin-indurated papules, severe skin induration, and associated joint contractures. Preliminary studies (unpublished data) show that decreasing plasma levels of transforming growth factor-beta1 after plasmapheresis appear to correlate with the amelioration of this clinical condition.

Adult↗

PubMed1983

Angiographic and scintigraphic findings in fibrosing mediastinitis.

The clinical and morphologic findings in the case of a 47-year-old man with fibrosing mediastinitis, most probably due to histoplasmosis, are described. Radionuclide angiography demonstrated obstruction of the superior vena cava with collateral vascularization. Computed tomography demonstrated a large calcific mass interposed between the pulmonary artery and superior vena cava suggesting potential pulmonary vasculature involvement. For this reason, pulmonary scintigraphy was performed which showed right lung perfusion and ventilation defects. Radionuclide angiography and pulmonary scintigraphy complement each other in determining the extent of vascular involvement with fibrosing mediastinitis. However, contrast venography is necessary to correctly delineate the anatomy of the obstructed superior vena cava and its collaterals.

Gallium Radioisotopes↗

PubMed1993

Fibrosing mediastinitis causing nonvisualization of one lung on pulmonary scintigraphy.

An interesting case of fibrosing mediastinitis, which is a rare cause of total nonvisualization of one lung on pulmonary scintigraphy, is presented. Scintigraphy with Tc-99m MAA showed a severe deficit of perfusion in the right lung, with normal perfusion of the left lung. Ventilation images were normal. CT and MRI each demonstrated the mediastinal nodal enlargement and MRI demonstrated the "flow void" signal phenomenon in the right pulmonary artery indicating its patency. Confirmation of actual patency without pulmonary artery emboli and with poor venous opacification was documented with angiography. Multiple complementary imaging modalities were helpful in correctly diagnosing fibrosing mediastinitis from an extensive list of differential possibilities for unilateral non-visualization of the lung on perfusion scanning.

Adult↗

PubMed2001

Tuberculous fibrosing mediastinitis: CT and MRI findings.

Fibrosing mediastinitis is a rare entity usually caused by granulomatous disease. Most cases develop as a complication of histoplasmosis. Mediastinal involvement caused by tuberculosis most often produces focally enlarged lymph nodes, with diffuse mediastinal involvement a very unusual manifestation of disease. The authors describe a patient with extensive tuberculous fibrosing mediastinitis, in whom fibrotic masses produced compression of airways and vascular structures with resultant parenchymal changes.

Adult↗

PubMed1983

The treatment of capillary-venous malformations using a new fibrosing agent.

We present our early experience in the treatment of cutaneous capillary-venous malformations using a new fibrosing agent called Ethibloc. Superselective angiography was used to delineate the vascular anomaly and effect temporary occlusion. Whenever possible, compression of venous outflow also was used, while the sclerosing agent was injected directly into the malformation by percutaneous puncture. Sixteen patients with capillary-venous malformations have been treated with this agent since 1979. A mild local inflammatory reaction was noted in some cases; there were no instances of skin necrosis. In 10 patients, the fibrosed vascular malformation was excised, and in 6 patients, the area of vascular anomaly decompressed to such a degree that resection was unnecessary.

Adolescent↗

PubMedDate not supplied

Musculosketal symptoms and non-REM sleep disturbance in patients with "fibrositis syndrome" and healthy subjects.

In sleep studies of (a) patients with the "fibrositis syndrome" and (b) healthy subjects undergoing stage 4 sleep deprivation, we observed in both groups the anomalous presence of alpha-rhythms in the non-rapid-eye-movement (NREM) sleep EEG. This phenomenon has been termed alpha-delta sleep. In the healthy subjects stage 4 deprivation was accompanied by the temporary appearance of muscoloskeletal and mood symptoms comparable to the symptoms seen chronically in the patients. It is suggested that the external arousing stimulus, which induced alpha-delta sleep in the subjects, is paralleled in the patients by an internal arousing mechanism. Such a mechanism, acting in competition with the NREM sleep system, would impair the presumed restorative function of NREM sleep and lead to the development of symptoms. It is proposed that the "fibrositis" symptom complex be considered a "non-restorative sleep syndrome". Evidence froms presented in support of the hypothesis that a disorder of serotonin metabolism serves as a basis for both the EEG sleep disturbance and the symptoms.

Adult↗

PubMed1996

Fibrosing cholestatic hepatitis in renal transplant recipients.

Fibrosing cholestatic hepatitis in a specific histologic manifestation of hepatitis B virus infection consisting of periportal fibrosis, hepatocyte ballooning, cholestasis, a relatively scant inflammatory infiltrate, and marked overexpression of hepatitis B viral antigens in hepatocytes. Until recently, fibrosing cholestatic hepatitis had been reported only in recipients of liver allografts. In this report, we present two patient in whom this lesion developed following renal transplantation. Both patients had previous liver biopsies showing relatively mild histologic changes. In one patient, the lesion developed early after retransplantation, during the period of maximal immunosuppression. However, in the second patient this lesion developed after withdrawal of immunosuppression. In both cases, death occurred within a few months because of progressive liver disease. Since this lesion can develop in "relatively healthy" hepatitis B carriers following transplantation of organs other than liver, these patients should have careful monitoring of their liver disease. Moreover, since the disease may progress despite withdrawal of immunosuppression, these patients would clearly benefit from the development of more effective therapies for posttransplant hepatitis B.

Adult↗

PubMed1997

Hepatitis C virus-related fibrosing cholestatic hepatitis after renal transplantation.

Fibrosing cholestatic hepatitis is a well-described syndrome in patients with immunodeficiency and chronic hepatitis B. It is clinically, biologically, and histologically characterized by rapidly progressive hepatic failure, a mildly elevated serum aminotransferase level, an extensive periportal fibrosis associated with intense cholestasis, mild inflammatory cellular infiltrate, no cirrhosis, and a high hepatocellular level expression of B viral antigens. This syndrome reflected a direct hepatocytopathic injury linked to high intrahepatic viral antigen expression. Because the syndrome of fibrosing cholestatic hepatitis has not been described in chronic hepatitis C, we report the first well-characterized case in a renal transplant patient with chronic hepatitis C and discuss the clinical and pathogenic implications of such a syndrome in this setting.

Adult↗

PubMed2006

Nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy).

PURPOSE OF REVIEW: Nephrogenic systemic fibrosis, also known as nephrogenic fibrosing dermopathy, was first documented in 1997 as a scleroderma-like fibrosing entity of the skin in association with renal insufficiency. Rheumatologists, along with other specialists, may be the first to encounter these patients; and both a familiarity with the disorder and vigilance for its cardinal features is in order. This review provides an update and highlights recent theories, achievements and ongoing research in understanding this emerging and enigmatic disorder. RECENT FINDINGS: Clinical reports support the evidence of nephrogenic systemic fibrosis as a systemic disease and emphasize an increase in its recognition in the United States, Europe and Asia. The most recent work supports a model whereby circulating fibrocytes together with fibrogenic factors lead to the evolution of this disabling and sometimes fatal disorder. SUMMARY: While a specific cause of nephrogenic systemic fibrosis remains to be established, the pathogenesis seems to be multifactorial, with the postulated involvement of the circulating fibrocytes. Recent published data including information from the Yale University NSF Registry has shed light on the clinical spectrum, cause, pathogenesis and treatment options. Clinical awareness of nephrogenic systemic fibrosis is still emerging and future studies are warranted to clarify its etiopathogenesis and effective therapies.

Fibrosis↗

PubMed1984

Discontinuity of the basement membrane in fibrosing basocellular carcinomas and basosquamous carcinomas of the skin: an immunohistochemical study with human laminin and type IV collagen antibodies.

Thirteen basocellular carcinomas (BCC) of different histologic types and 5 basosquamous carcinomas (BSC) of the skin were stained for laminin and type IV collagen with rabbit antibodies against the human basement membrane (BM) proteins, using an immunoperoxidase technique. The BM around the tumor aggregates contained both laminin and type IV collagen, and was continuous and distinct in all the nonfibrosing BCCs but indistinct or interrupted in the fibrosing BCCs and BSCs. The BM was not influenced by the focal adnexal differentiation of the BCC cells. The disintegrity of the BM in the fibrosing BCCs and BSCs may reflect some kind of disturbance in the interaction between the neoplastic epithelium and the connective tissue stroma, and be connected with the more aggressive nature of these tumors compared with ordinary BCCs. Thus local aggressive behavior seems to be accompanied by defects in the BM.

Antibodies↗

PubMed2005

Osseous metaplasia in the setting of nephrogenic fibrosing dermopathy.

BACKGROUND: Nephrogenic fibrosing dermopathy (NFD) is a new skin-fibrosing disorder associated with renal dysfunction. It is marked by the acute onset of induration involving the upper and lower limbs, and it is characterized by distinctive histopathologic findings. METHODS: We report the case of a patient on hemodialysis who presented initially with the characteristic clinical and pathological features of NFD. The patient progressively developed painful hyperkeratotic spicules on both thighs and bone metaplasia was confirmed. RESULTS: Histological studies were performed at different stages of the disease showing an evolution from the well-known initial phase of NFD, characterized by thickening of the dermis, increased number of fibroblast-like cells, and mucin deposits, to an end stage characterized by the presence of dermal ossification. CONCLUSIONS: We report the exceptional finding of bone metaplasia in the setting of a case of NFD.

Adult↗

PubMed2005

Histopathologic comparison of nephrogenic fibrosing dermopathy and scleromyxedema.

BACKGROUND: Nephrogenic fibrosing dermopathy (NFD) clinically presents as indurated plaques and papules in patients with renal dysfunction. The differential diagnosis generally includes scleromyxedema (SMX), an idiopathic systemic disorder with cutaneous manifestations, in which patients also develop indurated papules and plaques. The two entities can be extremely difficult to distinguish microscopically. Histopathologic differences with immunophenotypic comparison, to our knowledge, have not been thoroughly studied. We compared these two entities with an emphasis on immunohistochemistry. DESIGN: Nine biopsies diagnosed as NFD and seven biopsies diagnosed as SMX were retrospectively collected from the University of Pennsylvania Medical Center's surgical pathology and dermatopathology archives. Immunohistochemical staining for CD34, factor XIIIa, CD31, smooth muscle actin, CD68, and procollagen-I, as well as colloidal iron, were performed on each biopsy. Amount of expression for each of these markers, as well as degree of inflammation, for each biopsy was evaluated using a grading system of 0--3. RESULTS: Overall, NFD and SMX showed similar expression for all markers except procollagen-I, which showed increased expression in SMX. DISCUSSION: Although some immunophenotypic differences were found, our study did not demonstrate microscopic characteristics that can be easily used diagnostically to distinguish NFD from SMX. Clinical pathologic correlation is paramount in distinguishing these two entities. Kucher C, Xu X, Pasha T, Elenitsas R. Histopathologic comparison of nephrogenic fibrosing dermopathy and scleromyxedema.

Antigens, CD34↗

PubMed2005

Successful treatment of three cases of nephrogenic fibrosing dermopathy with extracorporeal photopheresis.

BACKGROUND: Nephrogenic fibrosing dermopathy (NFD) is a recently described cutaneous fibrosing disorder associated with renal dysfunction. Patients present with thickened skin or oedematous skin with indurated papules and plaques involving extremities and trunk, and often associated with disabling contracture of the adjacent joints. The aetiology and pathogenesis remain largely unknown. As a consequence, therapeutic measures with proven efficacy are nonexistent to date. OBJECTIVES: To consider treatment with extracorporal photopheresis (ECP) in three patients. Patients We report three new cases of NFD with the characteristic clinical and pathological features. Two patients required haemodialysis due to end-stage renal failure, despite prior renal transplantation. One patient had renal dysfunction but was never on dialysis, nor had she been transplanted. ECP treatment was administered at intervals of 2-4 weeks. RESULTS: All three patients showed a softening of the skin lesions and a marked improvement of the joint motility starting after four cycles of ECP. One patient developed a complete regression of her skin lesions after 16 cycles of ECP, and response to therapy was observed despite constantly elevated renal values. CONCLUSION: These data indicate that ECP may represent a valuable therapeutic option for NFD.

Adult↗

PubMed1981

Bronchoalveolar macrophages in sarcoidosis and cryptogenic fibrosing alveolitis.

Previous reports suggest that blood monocytes and tissue epithelioid cells in patients with sarcoidosis are "activated', but few studies have been undertaken on human alveolar macrophages. In the present study, bronchoalveolar lavage samples have been obtained from eighteen patients with sarcoidosis and these have been compared with twenty controls and twenty-nine patients with non-granulomatous chronic inflammatory interstitial lung disease (cryptogenic fibrosing alveolitis). Assessment of the functional state of the macrophages was made by measurements of C3b receptor sites on the cell membrane, intra and extracellular lysosomal enzyme (beta-D-glucosamidase) and the degree of spreading of macrophages on glass. C3b receptor sites and intracellular levels of lysosomal enzyme were significantly reduced in sarcoidosis compared to controls; levels of extracellular enzyme in the lavage supernatant fluid and macrophage spreading were similar to controls. These features suggest that alveolar macrophages from patients with sarcoidosis are not "activated'. By contrast, in cryptogenic fibrosing alveolitis, macrophages show a greater extracellular:intracellular ratio of lysosomal enzyme and more spreading, suggestive of "activation'.

Adult↗

PubMed1992

Distribution of the cytoskeletal protein beta-tubulin in normal lung, cryptogenic fibrosing alveolitis and lung tumours.

Tubulin is the major protein of microtubules. The immunocytochemical distribution of tubulin within the human respiratory tract has not been investigated in detail and no analysis of diseased lung or lung tumours has been undertaken. We therefore studied the distribution of beta-tubulin in formalin-fixed normal lung (n = 6), cryptogenic fibrosing alveolitis (n = 10) and lung tumours (n = 66), using a monoclonal antibody to beta-tubulin. In normal lung positive immunostaining was observed in all ciliated epithelium from the trachea down to bronchiolar level; blood vessel endothelium, vascular smooth muscle and nerve bundles were also strongly positive; pneumocytes, cartilage and airway smooth muscle gave weak staining. A similar distribution of beta-tubulin was seen in cryptogenic fibrosing alveolitis, but with strong tubulin immunostaining of fibroblasts in the interstitium and of the cytoplasm of ciliated respiratory epithelial cells. In lung tumours, six of 17 (35%) adenocarcinomas, one of two adenosquamous and one of 17 (5%) squamous cell carcinomas gave strong immunostaining. All six large cell carcinomas gave strong immunostaining for tubulin. In neuroendocrine tumours, two of seven (28%) carcinoids, two of seven (28%) atypical carcinoids and seven of 10 (70%) small cell carcinomas were strongly positive for tubulin. beta-Tubulin is widely distributed in the normal and diseased respiratory tract and is found in many lung tumours, particularly in large cell and small cell carcinomas which are highly aggressive in behaviour.

Humans↗

PubMed1994

Immunohistochemical localization of transforming growth factor-beta 1 in the lungs of patients with systemic sclerosis, cryptogenic fibrosing alveolitis and other lung disorders.

To study the role of transforming growth factor-beta 1 (TGF-beta 1) in the pathogenesis of pulmonary fibrosis we have examined lung biopsies from nine patients with systemic sclerosis and interstitial lung disease, eight with 'lone' cryptogenic fibrosing alveolitis, two with cystic fibrosis, two with extrinsic allergic alveolitis, two with Langerhans' cell histiocytosis, one with lymphangioleiomyomatosis, one with giant cell interstitial pneumonia, and one adenocarcinoma of the lung. In cryptogenic fibrosing alveolitis, both 'lone' and associated with systemic sclerosis alveolar macrophages, bronchial epithelium and hyperplastic type II pneumonocytes expressed intracellular TGF-beta 1. Extracellular TGF-beta 1 was found in the fibrous tissue immediately beneath the bronchial and hyperplastic alveolar epithelium. In normal lung, however, the alveolar epithelium and alveolar interstitium were negative for both forms of TGF-beta 1. There was strong expression of TGF-beta 1 in hyperplastic mesothelium and its underlying connective tissue and in Langerhans' cells in the two cases of histiocytosis. In the organizing pneumonia in cystic fibrosis, the intraalveolar buds of granulation tissue reacted strongly for the extracellular form of TGF-beta 1 and the overlying hyperplastic epithelium expressed the intracellular form. In lymphangioleiomyomatosis, the aberrant smooth muscle cells strongly expressed intracellular TGF-beta 1 and the extracellular form was expressed in the adjacent connective tissue. In giant cell interstitial pneumonia, the numerous alveolar macrophage including the multinucleate forms, expressed intracellular TGF-beta 1, as did the hyperplastic alveolar epithelium.(ABSTRACT TRUNCATED AT 250 WORDS)

Humans↗

PubMed1998

Nucleoside analogue therapy in fibrosing cholestatic hepatitis--a case report in an HBsAg positive renal transplant recipient.

A 45-year-old HBsAg carrier (HBeAb positive with normal liver function tests) underwent renal transplantation for mesangioproliferative glomerulonephritis. Sixteen months later he developed jaundice. Investigations showed he remained HBeAb positive, but HBV-DNA levels were 99 pg/ml, indicating active replication of a HBV pre-core mutant. He was commenced on lamivudine therapy with a subsequent rapid fall in HBV-DNA levels to 2.8 pg/ml, but liver function tests continued to deteriorate and he developed hepatorenal failure. Liver biopsy showed fibrosing cholestatic hepatitis. He underwent liver transplantation, which was complicated by lactic acidosis. Lamivudine was withdrawn and HBV prophylaxis with HB immunoglobulin was commenced. Unfortunately he died 38 days post-transplant of surgical complications with no evidence of HBV recurrence. We discuss the use of nucleoside analogues in fibrosing cholestatic hepatitis and review the literature.

Cholestasis, Intrahepatic↗