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PubMed1978

Fibrosing alveolitis.

Fibrosing alveolitis is a disease of unknown cause mainly involving the gas-exchanging portions of the lungs. It may occur in isolation and be called cryptogenic or idiopathic, in which case the clinical manifestations are mainly respiratory, or it may be associated with other disorders, such as rheumatoid arthritis. The histopathologic abnormalities of the pulmonary tissue are identical in either instance. Other names used for the disease have included usual interstitial pneumonia, desquamative interstitial pneumonia and the Hamman-Rich syndrome; these terms may describe different stages of the same pathologic process. Many authors in North America and those in the United Kingdom favour the term fibrosing alveolitis when describing chronic interstitial pneumonias. There may be accompanying nonspecific Immunologic abnormalities, which may denote that fibrosing alveolitis is part of the wide spectrum of diseases known as connective tissue disorders. Recently immune complexes have been found in the lung parenchyma; they probably result in the granulocyte destruction and reticuloendothelial proliferation seen in the acute phase of the disease.There are no specific diagnostic tests for the disease apart from lung biopsy, which can be performed at the time of thoracotomy or transbronchially, with the use of a flexible fibreoptic bronchoscope. Lavaged cells from the alveoli have also been obtained via the bronchoscope; in persons with fibrosing alveolitis a high proportion of these cells are neutrophils, and after corticosteroid treatment the proportion decreases. The progress of the disease can be followed by examination of these washings as well as by lung scanning with gallium-67 citrate. Newer methods of treatment using combinations of corticosteroids and immunosuppressant drugs are being evaluated and are initially proving to be successful.

Adult↗
PubMed1978

[Sonographic representation of fibroses (a contribution to the problem of tissue differentiation) (author's transl)].

Twelve patients with radiogenic fibroses were submitted to a sonographic examination by means of a grey-scale compound-scanner. As these skin fibroses are well suited for inspection and palpation, they are qualified to serve as a model with regard to the problem of sonographic tissue differentiation. Fibroses of medium and high degree are determined by characteristic properties in the B picture, whereas fibroses of low degree cannot be recorded by this technique.

Adult↗
PubMed1980

Alveolar macrophage lysosomal enzyme and C3b receptors in cryptogenic fibrosing alveolitis.

Levels of the lysosomal enzyme N-acetyl-beta-glucosaminidase were measured in alveolar macrophage extracts and lung lavage fluid obtained from patients with cryptogenic fibrosing alveolitis and a control group. Evidence is presented which suggests that macrophages from patients with cryptogenic fibrosing alveolitis secrete this enzyme in vivo. To investigate one of the possible mechanisms of stimulating this secretion, an assessment was made of the C3b receptor sites on alveolar macrophages. While there were fewer receptor sites on the macrophages from patients with cryptogenic fibrosing alveolitis, which might indicate that they were occupied in vivo, this difference did not reach statistical significance. It is suggested that the alveolar macrophage is not merely a phagocyte but that it also has a more active role in the pathogenesis of cryptogenic fibrosing alveolitis.

Acetylglucosaminidase↗
PubMed1993

Clearance of inhaled 99mTc-DTPA predicts the clinical course of fibrosing alveolitis.

The aim of this study was to determine whether the speed of technetium-labelled diethylene-triamine-pentacetate (99mTc-DTPA) clearance from the lung is predictive of disease progression in fibrosing alveolitis, as judged by changes in respiratory function tests. 82 nonsmoking patients with fibrosing alveolitis were studied (progressive systemic sclerosis, n = 53; lone cryptogenic fibrosing alveolitis, n = 29). Normal 99mTc-DTPA clearance at initial measurement predicted stable disease; rapid 99mTc-DTPA clearance identified patients at risk of deterioration. Repeat measurement of clearance, approximately 12 months later, enabled the definition of a subgroup at higher risk, with persistently abnormal 99mTc-DTPA clearance, and a smaller subgroup in whom reversion of clearance to normal was associated with a sustained improvement in respiratory function indices. These findings were not attributable to differences in treatment between subgroups. We conclude that the speed of 99mTc-DTPA clearance discriminates between stable and progressive disease in fibrosing alveolitis.

Administration, Inhalation↗
PubMed2000

Distribution of novel polymorphisms of the interleukin-8 and CXC receptor 1 and 2 genes in systemic sclerosis and cryptogenic fibrosing alveolitis.

OBJECTIVE: To search for single-nucleotide polymorphisms in the interleukin-8 (IL-8) and IL-8 receptor CXCR-1 and CXCR-2 genes, and to compare their distribution among patients with systemic sclerosis (SSc) with fibrosing alveolitis (FASSc) or without fibrosing alveolitis (NFASSc), or patients with cryptogenic fibrosing alveolitis (CFA), and normal healthy subjects. METHODS: Fifty control subjects were screened for potential polymorphisms by using polymerase chain reaction in association with sequence-specific primers incorporating mismatches at the 3' end. The novel polymorphisms were subsequently examined in British Caucasian subjects, including 194 healthy controls, 71 patients with CFA, and 128 patients with SSc who were further subdivided into 78 FASSc patients and 50 NFASSc patients. RESULTS: Three novel biallelic polymorphisms were identified in the IL-8 gene (all in noncoding areas of the gene), 1 was found in the CXCR-1 gene (resulting in a conservative amino acid change), and 3 were observed in the CXCR-2 gene, of which the first resulted in a silent codon change and the others were in the 3' untranslated area of exon 3. Compared with controls, a significant increase in the frequency of the CXCR-2 +785 CC homozygote and of the CXCR-2 +1208 TT homozygote was found in the SSc patients (37% versus 22% [P = 0.01] and 33% versus 17% [P = 0.003], respectively). A subgroup analysis revealed this association to be significant both in the FASSc patients and in the NFASSc patients. CONCLUSION: This report describes an association between SSc and 2 polymorphisms occurring close to each other in the CXCR-2 gene. This finding and its functional significance need to be confirmed and analyzed in future studies.

Antigens, CD↗
PubMed1995

Fibrosing vasculitis in Wegener's granulomatosis: ultrastructural and immunohistochemical analysis of the vascular lesions.

This study of two cases of pulmonary Wegener's granulomatosis (WG) focuses on the ultrastructural aspects of the vascular wall injury and on the immunohistochemical characterization of the perivascular connective matrix. The iterative waves of endothelial cell necrosis and regeneration are demonstrated by the multilamellar appearance of the basal lamina. Neutrophils infiltrate the vessel wall and myofibroblasts are recruited to injured vessels. The perivascular connective matrix associates basement-membrane like and fibrillar material with fibrin deposits. The initiation of the fibrosing process was assessed by the visualization of matrix molecules involved in targeting (p-fibronectin), organizing (cellular fibronectin and tenascin) and stabilizing (lysyl-oxidase) the fibrogenic activity. These elementary lesions affect different levels of the vascular tree, and capillaritis is involved in the extension of the pathological process. Lysyl-oxidase labelling reveals the fibrosing front which is located on the border of dense fibrosis. The markers of fibrosing activity disappear in the areas of fibrosis following vasculitis and/or ischaemic necrosis and/or granulomatosis. Vasculitis plays a major role in both the genesis and progression of the fibrosis observed in the late stage of WG.

Arteries↗
PubMed1998

[Lichen planopilaris simulating postmenopausal frontal fibrosing alopecia (Kossard)].

A 68-year old woman presented with a frontal fibrosing alopecia and lesions of the buccal mucous membranes typical for lichen planus. Postmenopausal frontal fibrosing alopecia (PFFA) has recently been described by Kossard as a distinct entity characterized by progressive recession of the frontotemporal and parietal hair margins leading to permanent alopecia in the form of a symmetrical band-like area of scanning in postmenopausal women. The histology (perifollicular lymphocytic infiltration and fibrosis, increase of apoptosis of hair follicle keratinocytes) is indistinguishable from that of lichen planopilaris. The localization and age- and sex-related characteristics of PFFA are not sufficient to delineate it as a discrete entity from lichen planopilaris. Our observation of oral lichen planus in a postmenopausal woman with frontal fibrosing alopecia points to the possibility that PFFA actually may represent a variant of lichen planopilaris with a predilection for the frontotemporal hairline. Other variants of lichen planopilaris include multifocal lichen planopilaris, disseminated lichen planopilaris (Lassueur-Graham-Little syndrome), lichenoid pseudopelade, and any combination of these ("mixed type"). An effective therapy of PFFA is not known. Also, treatment of lichen planopilaris forms in which fibrosis predominates over inflammation is similarly problematic, but the natural course of these diseases seems to be self-limited.

Aged↗
PubMed2002

[Kossard frontal fibrosing alopecia in a man].

In 1994 Steven Kossard described a new and peculiar type of hair loss that he named postmenopausal frontal fibrosing alopecia. In 6 elderly women he observed a symmetric regression of the frontal hair line. Often the eyebrows were also affected. Histology showed lichen planopilaris. There were no clinical signs of lichen planus on the rest of the body. Since the original description by Kossard, several cases of frontal fibrosing alopecia have been described--almost all of them in elderly women. We report a man with frontal fibrosing alopecia of the Kossard type.

Aged↗
PubMed1997

Contrast enema findings of fibrosing colonopathy.

PURPOSE: Our objective was to describe characteristic contrast enema findings of fibrosing colonopathy. MATERIALS AND METHODS: We performed barium enemas on 18 children with cystic fibrosis who had received greater than 4500 U of lipase per kg body weight per meal and who had distal intestinal obstruction syndrome unresponsive to medical management. RESULTS: Fifteen patients had abnormal enema results. The most common findings included stricture, loss of haustra, and colonic shortening. Ten patients required surgery, nine underwent colon resection, and one had lysis of adhesions. Pathologists confirmed fibrosing colonopathy in eight of nine speci- mens. CONCLUSION: Colonic stricture, abnormal haustra, and longitudinal shortening are characteristic barium enema findings of fibrosing colonopathy.

Barium Sulfate↗
PubMed2006

Successful conservative management of idiopathic fibrosing pancreatitis in children.

UNLABELLED: Idiopathic fibrosing pancreatitis, a rare cause of obstructive jaundice and abdominal pain in children, which has certain features in common with the emerging entity of autoimmune pancreatitis as described in adults, has frequently been managed surgically. We present our experience of successful conservative management of this condition in children. Three children (6-12 years; two girls, one boy) presented with a short history of abdominal pain followed by obstructive jaundice. Abdominal ultrasonographic examination in each case showed dilated intrahepatic and common bile ducts with a bulky pancreas, predominantly the head. These findings were confirmed by magnetic resonance imaging. In two cases, the diagnosis of fibrosing pancreatitis was made by exclusion after extensive investigation. The third case had a percutaneous ultrasound-guided pancreatic needle biopsy. Two patients were managed by supportive medical therapy alone, whilst the third, with symptomatic obstructive jaundice, underwent temporary endoscopic stenting of the common bile duct. Cases have been followed-up for 12-49 months. There was complete clinical and biochemical resolution of obstructive jaundice in all three cases. Plasma bilirubin concentrations decreased to normal within 3-8 weeks. Serial abdominal imaging showed a gradual resolution of biliary dilatation and abnormal pancreatic morphology with subsequent pancreatic atrophy. Two children developed steatorrhoea that responded to pancreatic enzyme supplements, and one patient developed diabetes mellitus. None of the cases needed invasive surgery for diagnosis or management. CONCLUSION: With careful radiological and biochemical assessment and monitoring, invasive surgery can be avoided in the management of fibrosing pancreatitis. The eventual outcome is no different from reported surgically treated cases.

Abdominal Pain↗
PubMedDate not supplied

Cystic fibrosis with fibrosing colonopathy in the absence of pancreatic enzymes.

Fibrosing colonopathy, characterized by dense submucosal fibrosis in the large bowel, is a disorder associated with bowel dysfunction in patients with cystic fibrosis who receive pancreatic enzyme supplementation. Most commonly, patients present with a distended abdomen and abdominal pain. Radiographs frequently demonstrate colonic wall thickening and luminal narrowing. Here I describe a neonate with cystic fibrosis who presented with both clinical and histological features of fibrosing colonopathy who had not received pancreatic enzymes. This report expands our understanding of the pathogenesis of fibrosing colonopathy.

Biopsy↗
PubMed1991

Fibrosing mediastinitis with coronary artery involvement.

This case report describes a patient with chronic fibrosing mediastinitis involving the entire intrapericardial aorta, innominate artery, and the base of the heart with involvement of the proximal segments of the coronary arteries. This finding was unsuspected before emergency coronary artery bypass grafting. Coronary stenosis due to fibrosing mediastinitis is a rare complication of fibrosing mediastinitis. Owing to the extensive fibrosis, the normal bypass, cardiac preservation, and revascularization techniques required alteration and are discussed.

Coronary Disease↗
PubMed1993

Increased hepatocyte expression of hepatitis B virus transcription in patients with features of fibrosing cholestatic hepatitis.

BACKGROUND: Recurrent hepatitis B after liver transplantation may be complicated by fibrosing cholestatic hepatitis. This syndrome is associated with rapid graft failure and is characterized by ballooning degeneration of hepatocytes and abundant viral antigen expression. METHODS: To study this disorder further, in situ hybridization studies were performed on 36 liver biopsy specimens from 14 transplanted patients with recurrent hepatitis B and 18 nontransplanted controls with chronic hepatitis B. Biopsy specimens were scored for histological features and intensity of riboprobe hybridization signal to hepatitis B virus (HBV) DNA and RNA. RESULTS: HBV DNA hybridization signals of 2+ to 3+ intensity were observed in 53% of the posttransplant biopsies but none of the nontransplanted samples (P < 0.001). HBV RNA signals of this intensity were found in 42% of the transplant biopsy specimens compared with 17% of the nontransplant specimens (P < 0.07). Features of fibrosing cholestatic hepatitis were noted in 12 biopsies; 11 of these displayed RNA signals of 2+ to 3+ intensity (92%) compared with 4 of 24 (17%) biopsy specimens without this diagnosis (P < 0.001). The level of hepatocyte RNA correlated with the extent of hepatocellular ballooning (P < 0.007). CONCLUSIONS: These data suggest that fibrosing cholestatic hepatitis is associated with enhanced hepatitis B virus transcription and support a cytopathic role for the virus in the development of this syndrome.

Adult↗
PubMed2005

Frontal fibrosing alopecia in postmenopausal women.

BACKGROUND: Frontal fibrosing alopecia is a variety of cicatricial alopecia characterized by a band of frontal/frontoparietal hair recession and marked decrease or a complete loss of the eyebrows, typically observed in women who are postmenopausal. OBJECTIVE: The purpose of this study was to report clinical and histopathologic findings and results of treatment in a group of women affected by the disease. METHOD: A total of 14 women with alopecia of the frontal hairline were evaluated from June 2000 through July 2003 in our outpatient consultation for hair disorders. RESULTS: Clinical examination revealed a band of symmetric recession of the frontoparietal hairline extending to the preauricular areas associated with loss of follicular orifices, mild skin atrophy, and perifollicular erythema at the scalp margin. In all, 9 patients also had partial or total loss of the eyebrows. The histologic features of the scalp specimens were similar in all our patients with a reduction of the number of hair follicles, and a high number of intermediate and velluslike follicles. Intemediate and velluslike follicles were more commonly affected than terminal follicles by the lymphocytic inflammatory infiltrate and perifollicular fibrosis. CONCLUSION: Frontal fibrosing alopecia is a cicatricial alopecia that follows destruction of hair follicles by an inflammatory lymphocytic infiltrate that is localized around the upper portion of the hair follicle. It differs from lichen planopilaris because the lymphocytic infiltrate and fibrosis affect selectively the intermediate and the velluslike follicles of the frontal margin and eyebrows. The reason for this selective involvement is still unknown. Frontal fibrosing alopecia may represent a variety of lichen planopilaris with selective involvement of certain androgen-dependent areas. The affected follicles may have typical biologic markers that could explain the clinical and histologic features found in the disease. It is interesting to note that some of the patients treated with finasteride (2.5 mg/d) showed an arrest in the progression of the disease. Even if there is no proof for a hormonal basis of the disease, the effectiveness of finasteride in some patients may indicate that androgens might be partially responsible of the pathogenesis of the disease.

Aged↗
PubMed1988

The value of high definition, narrow section computed tomography in fibrosing alveolitis.

Fibrosing alveolitis was studied by high definition, narrow section computed tomography (CT) in 50 patients, 30 of whom were suffering from systemic sclerosis and 20 from cryptogenic fibrosing alveolitis (CFA). Apart from changes in the mid and lower zones, abnormalities were also observed anteriorly in the upper lobes and in the pleura. Scanning in the prone position was important in seeking evidence of minimal pathological change. In systemic sclerosis CT was 24% more accurate than high kilovoltage radiography in demonstrating minimal evidence of fibrosing alveolitis; this had a characteristic distribution as a peripheral crescent of high attenuation in the lower lobes before any changes were visible on standard radiographs. The CT appearances in CFA were also characteristic, particularly when the disease appeared to be early or limited. We believe that high definition CT may be of value in suggesting the diagnosis of CFA in patients with non-specific abnormalities on the chest radiograph.

Humans↗
PubMed1998

Treatment of fibrosing cholestatic hepatitis with lamivudine.

Fibrosing cholestatic hepatitis is a histological variant of hepatitis B virus infection with a high rate of mortality. We describe a patient who acquired acute hepatitis B virus infection 8 months after renal transplantation. Clinical features of rapidly progressive liver failure, indicated by prolonged prothrombin time (57 seconds) and increased bilirubin (40.4 mg/dL) and ammonia (129 mumol/L) concentrations, were accompanied by an extremely high serum HBV DNA level (2.153 x 10(6) pg/mL). Liver biopsy specimen showed fibrosing cholestatic hepatitis with widespread balloon degeneration of hepatocytes, focal hepatocyte loss, bile stasis, periportal fibrosis, mild lymphocytic infiltration, and strongly positive immunohistochemical staining for hepatitis B surface antigen (HBsAg) and hepatitis B core antigen. Lamivudine therapy suppressed HBV DNA to < 10 pg/mL within 4 weeks, which was followed by gradual recovery of liver function from a state of hepatic precoma. Twenty-four months after the onset of hepatitis, the patient had normal prothrombin time and bilirubin, transaminase, and albumin levels. She remained HBsAg positive and hepatitis B e antigen negative. Renal allograft function was stable, with a creatinine level of 1.52 mg/dL. HBV DNA remained suppressed after 22 months of lamivudine therapy. Our experience shows that fibrosing cholestatic hepatitis and liver failure caused by HBV infection can be successfully treated with lamivudine.

Adult↗
PubMed1997

Fibrosing colonopathy in children with cystic fibrosis.

PURPOSE: Fibrosing colonopathy is a newly described entity seen in children with cystic fibrosis. The radiological hallmarks are foreshortening of the right colon with varying degrees of stricture formation. High-dose enzyme therapy has been implicated as the cause of this process. The purpose of this study is to review the author's experience with evaluation and treatment of these patients. METHODS: There are currently 380 patients being treated at our CF center. Fifty-five of these patients have been treated with high-dose enzyme therapy (> 5,000 units of lipase/kg). The medical records of these patients, who are at risk for developing fibrosing colonopathy, were reviewed for the presence of recurrent abdominal complaints, and the work-up and treatment of these symptoms. RESULTS: Chronic complaints of abdominal pain, distension, change in bowel habits, or failure to thrive were present in 24 of the 55 patients treated with high-dose enzymes. So far, 18 of these 24 patients have been evaluated by contrast enema. Thirteen of eighteen have been found to have fibrosing colonopathy characterized by foreshortening and strictures of the colon. Additional findings included focal strictures of the right colon (7 of 13), long segment strictures (5 of 13), and total colonic involvement (1 of 13). Nine patients with the most severe symptoms have undergone colon resection, including five segmental right colectomies, three extended colectomies (ileo-sigmoid anastomosis), and one subtotal colectomy with end-ileostomy. Pathological evaluation has shown submucosal fibrosis, destruction of the muscularis mucosa, and eosinophilia. No postoperative complications or deaths occurred. All nine postoperative patients have noted marked symptomatic improvement. Contrast enema follow-up results are available for six patients, and have documented no recurrent strictures to date. Three of four nonoperative patients have less severe symptoms and are currently being treated conservatively. The other family has refused surgery and the patient is being treated symptomatically. CONCLUSION: High-dose lipase replacement has been implicated as the etiology for FC and was present in all of our patients. Our cystic fibrosis center now routinely limits lipase to 2,500 U/kg per dose. We recommend the use of the contrast enemas to evaluate at-risk patients who have chronic abdominal complaints or who present with recurrent bowel obstruction. Colon resection should be performed in those with clinically and radiographically significant strictures with the expectation of a good outcome.

Child↗
PubMed1995

Fibrosing colonopathy in cystic fibrosis: results of a case-control study.

Fibrosing colonopathy was first described in cystic fibrosis (CF) children in 1994. We have done a nested case-control study to identify possible associations with this condition. A case ascertainment within the UK CF population to identify any cases that occurred between January, 1984, and April, 1994, found 14 cases, all under 14 years and confirmed by independent histopathological review. All had presented since April, 1993; 12 were boys and six had received some or all of their care in Liverpool. Each case was matched, by date of birth, with four controls from the UK CF Registry. Information was obtained about cases and controls from their case records and by a structured interview with the families. In the 12 months before surgery, there was an association between the occurrence of fibrosing colonopathy and use of high-strength pancreatic enzyme preparations. This association was dose related. Odds ratio per extra 1000 high-strength capsules was 1.45 (95% CI 1.14-1.84). For use of protease, the odds ratio per million extra units per kg was 1.55 (1.19-2.03). For usage of individual high-strength products at any time during the 12 months before surgery some differences were observed; for Creon 25000 the odds ratio was 0.38 (0.10-1.42), for Nutrizym 22 43.4 (2.51-751), and for Pancrease HL 8.4 (1.95-36.1). These last two confidence intervals are extremely wide and compatible with these two products having the same odds ratios. Laxative use was independently predictive (odds ratio 2.42 [1.20-4.94]). We conclude that there is a dose-related association between high-strength pancreatic enzyme preparations and fibrosing colonopathy.

Adolescent↗