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PubMed1990

Autoantibody and idiotype profile of lung involvement in autoimmune rheumatic disease.

Several reports have linked the presence of certain serum autoantibodies with particular clinical manifestations of autoimmune disease. For example, the Jo-1 antibody is now established as a marker for fibrosing alveolitis in polymyositis. To investigate the possible association of further autoantibodies or idiotypes with fibrosing alveolitis in autoimmune rheumatic disease a panel of autoantibodies was measured in serum samples from 28 patients with systemic lupus erythematosus (SLE) (10 with and 18 without lung involvement), 21 patients with scleroderma (12 with fibrosing alveolitis and nine without), and 41 patients with 'lone' fibrosing alveolitis. Antibodies measured were IgM and IgG anti-dsDNA and anti-ssDNA antibody; IgG and IgM anticardiolipin antibody; anti-poly (ADP-ribose) antibody; antibodies to two common idiotypes of anti-DNA antibodies, designated 134 and 16/6; and IgM, IgG, and IgA isotypes of rheumatoid factor. None of these antibodies was specifically associated with lung involvement in SLE or scleroderma, but a trend was found towards an increase in all autoantibodies in association with lung disease in SLE, while the reverse trend was seen in scleroderma.

Antibodies, Antinuclear↗
PubMed2006

Fibroblasts of recipient origin contribute to bronchiolitis obliterans in human lung transplants.

RATIONALE: The participation of circulating precursor cells in the development of experimental pulmonary fibrosing lesions in mice has been recently demonstrated. OBJECTIVES: This study analyzes whether circulating, bone marrow-derived, fibroblastic precursor cells contribute to the development of fibrosing lesions in human lungs, especially bronchiolitis obliterans. METHODS: The occurrence of in situ microchimerism in bronchiolitis obliterans lesions of human lung allografts (n = 12) as well as of autologous lung tissue from patients post-bone marrow transplantation (n = 2) was analyzed using laser-assisted microdissection after immunohistochemical labeling of leukocytes followed by short tandem repeat-polymerase chain reaction-based genotyping. Combined immunofluorescence and fluorescence in situ hybridization for sex chromosomes was performed for independent confirmation in cases with appropriate sex mismatch (n = 2). MEASUREMENTS AND MAIN RESULTS: The bronchiolitis obliterans lesions of all 12 lung transplant patients contained considerable numbers of recipient-derived fibroblasts (mean, 32%). The fibrosing pulmonary lesions of the two bone marrow-transplanted patients also displayed clear in situ microchimerism. The in situ detection methodology confirmed these results, although to a lower degree (6-16%). CONCLUSIONS: These data clearly demonstrate the involvement of circulating fibroblastic precursor cells in the development of human fibrosing lung lesions and provide evidence that these cells are most probably bone marrow derived. These results may open new venues regarding the prevention of fibrosis in lung transplants and potentially in other organs.

Adult↗
PubMed1996

Inheritance of susceptibility to bleomycin-induced pulmonary fibrosis in the mouse.

Based on the range of patient responses to treatment, and on animal studies, it is hypothesized that individual variation in sensitivity to bleomycin-induced pulmonary fibrosis is controlled genetically. A genetic model has been developed by (a) establishing a distinct difference in bleomycin-induced lung damage in two inbred strains of mice [parental generation: C57BL/6J (fibrosis-prone phenotype) and C3Hf/Kam (fibrosis-resistant phenotype)] and (b) characterizing inheritance of the fibrosing phenotype in the F1 (first filial) and F2 (F1 intercross; second filial) generations derived from the parental strains. Male mice received 100 mg/kg and female mice 125 mg/kg of bleomycin via s.c. osmotic minipump. The animals were sacrificed 8 weeks after treatment or when their breathing rate indicated respiratory distress. The percentage of lung with fibrosis for each mouse was quantified with image analysis of a histological section of the left lung. The mean percentage of fibrosis for the C57BL/6J males was 8.4 +/- 0.8% (SE) and 4.4 +/- 0.8% for females, and the C3Hf/Kam mice of either sex did not present the fibrosing lesion (mean score, 0%). Significant difference (P = 6 x 10(-6)) was measured in percentage of fibrosis between the two strains of F1 males, but not F1 females (P = 0.38), suggesting the presence of an X-linked factor associated with the fibrosing phenotype. From an ANOVA the X-linked factor is estimated to contribute 19% of the fibrosis phenotype. A genetic model of two or three loci controlling the fibrosing phenotype is proposed from the data of the parental, F1, and F2 generations. The mouse model demonstrates that susceptibility to bleomycin-induced pulmonary fibrosis is a heritable trait controlled by a few genetic loci.

Animals↗
PubMed1990

Evaluation of alveolitis by studies of lung biopsies.

Immunocytochemical studies of lung biopsies from patients with cryptogenic fibrosing alveolitis and those having fibrosing alveolitis associated with scleroderma show that numerous T-cells occur in the alveolar walls and septa of all patients with these diseases, implying that cell-mediated hypersensitivity reactions are involved in the pathogenesis. However, B-lymphocytes, mainly within lymphoid follicles, are also present and, in the CFA group, such patients, and those with less than 50% T-suppressor/cytotoxic cells in the diffuse T-cell infiltrates, may have a poorer prognosis. Interleukin-2 receptor expression indicates that T-cell activation is occurring within the lungs, and there is evidence that interstitial monocytes/macrophages and alveolar epithelial cells may play a more important role in antigen presentation than alveolar macrophages. These observations on the features of the inflammatory response in the interstitial tissues of patients with fibrosing alveolitis need to be incorporated into the existing hypothesis on pathogenetic mechanisms based mainly on lavage findings of alveolar macrophages and granulocytes.

Antibodies, Monoclonal↗
PubMed2002

Non-invasive pulsed ultrasound quantification of the resolution of basal cell carcinomas after photodynamic therapy.

The probability of local control of basal cell carcinomas (BCC) treated by photodynamic therapy (PDT) depends strongly on lesion thickness, thicker lesions often requiring two treatments. We examine the utility of 20 MHz pulsed ultrasound (US) for the non-invasive measurement of thickness and rate of regression after PDT treatment. PDT was by topically applied 20% aminolaevulinic acid, followed at 6 h by a standard 100 J/cm(2) of 630 nm light. Patients ( n=60) were selected as being difficult to treat with existing modalities for reasons of likely poor quality of healing or of cosmesis in this very largely elderly population. Ultrasound 'A' scans were made immediately before treatment, and at first and subsequent follow-ups. Parameters measured non-invasively for BCC, adjacent normal skin, and for fibroses after previous conventional therapies, were (a) thickness of skin or lesion, (b) linear density of ultrasound echoes and (c) linear density of high-amplitude echoes. Prior to treatment, median skin thickness (to the dermal/subcutaneous boundary) was 2.6 mm (range 1.2-5.7), fibroses 2.5 mm (1.4-5.6) and BCC 1.5 mm (0.5-4.4). Median linear density of echoes for normal skin, fibroses and BCC plus underlying tissue were 5.6, 5.5 and 4.5, respectively, the BCC values being significantly lower ( p=0.002). The corresponding medians for high-amplitude echoes were 1.9, 1.9 and 1.1 (skin or fibrosis versus BCC, p=0.001). Patients whose BCCs appeared clinically to be controlled at up to 220 days after a single treatment, all had values of ultrasound parameters corresponding to skin/fibrosis and were significantly different from measurements on the same site prior to treatment. Patients whose tumours appeared to be reverting to the original BCC ultrasound pattern were subsequently found to be recurring as judged clinically. Non-invasive pulsed ultrasound indicates that rates of resolution vary widely between BCC of similar initial thickness and that the probability of clearance of BCC by PDT is determined largely by the deepest, sometimes small, regions within a lesion, with the overall area being relatively unimportant.

Adult↗
PubMed2000

Gelatinase B is required for alveolar bronchiolization after intratracheal bleomycin.

Increased expression of matrix metalloproteinases, particularly gelatinase B (MMP-9), has been described in the lungs in pulmonary fibrosis. Intratracheal bleomycin is often used experimentally to produce lesions resembling human fibrosing alveolitis. To assess the role of gelatinase B in bleomycin-induced fibrosing alveolitis, we instilled bleomycin intratracheally into gelatinase B-deficient mice and gelatinase B+/+ littermates. Twenty-one days after bleomycin the two groups of mice were indistinguishable in terms of pulmonary histology and total lung collagen and elastin. However, the lungs of gelatinase B-deficient mice showed minimal alveolar bronchiolization, whereas bronchiolization was prominent in the lungs of gelatinase B+/+ mice. Gelatinase B was identified immunohistochemically in terminal bronchiolar cells and bronchiolized cells 7 and 14 days after bleomycin in gelatinase B+/+ mice, and whole lung gelatinase B mRNA was increased at the same times. Many bronchiolized cells displayed Clara cell features by electron microscopy. Some bronchiolized cells stained with antibody to helix transcription factor 4, a factor associated with the ciliated cell phenotype. Thus, fibrosing alveolitis develops after intratracheal bleomycin irrespective of gelatinase B. However, gelatinase B is required for alveolar bronchiolization, perhaps by facilitating migration of Clara cells and other bronchiolar cells into the regions of alveolar injury.

Animals↗
PubMed1983

Chronic relapsing pancreatitis in childhood.

We report 10 children with chronic relapsing pancreatitis. These patients can be divided into three groups, based on their clinical history, manifestations, and radiographic findings. Group 1 includes four patients with hereditary pancreatitis; these patients have had recurrent abdominal pain since early childhood, and have a positive family history for pancreatitis. Group 2 includes two patients with clinical and radiographic findings similar to those in patients with hereditary pancreatitis but without a family history of pancreatitis. Group 3 includes four patients with fibrosing pancreatitis who had symptoms and signs of obstructive jaundice. Our report emphasizes three points: (1) that chronic pancreatitis does occur in young children and is most commonly caused by hereditary pancreatitis or fibrosing pancreatitis; (2) that endoscopic retrograde cholangiopancreatiography is a safe and valuable tool for the study of pancreatic and common bile ducts; and (3) that surgical intervention is indicated to drain the pancreatic duct in patients with hereditary pancreatitis, and sphincterotomy is an effective therapy for patients with fibrosing pancreatitis.

Adolescent↗
PubMed2003

Klatskin tumors and Klatskin mimicking lesions of the biliary tree.

AIM: We report on the preoperative capability of imaging modalities and clinical assessment to differentiate between Klatskin tumors and Klatskin mimicking lesions of the biliary tree. Adenocarcinomas of the hepatic ducts (Klatskin tumors) mimic benign fibrosing cholangitis. Extensive resections carry a substantial risk but offer the only chance for cure in patients with a Klatskin tumor. METHODS: Thirty-three consecutive patients who underwent resection for suspicion of a malignant tumor of the hepatic hilum were reviewed. All patients underwent preoperative ultrasonography, computed tomography, ERCP and angiography. The patients were divided into a group of true Klatskin tumors and a group of benign Klatskin mimicking lesions. RESULTS: Twenty-seven of the resected specimens were malignant tumors, and six lesions showed only fibrosing cholangitis. Preoperative clinical presentation and imaging modalities were very similar between Klatskin tumors and fibrosing cholangitis. CONCLUSIONS: Management of obstruction of the liver hilum is dictated by the suspicion of malignancy. Complete removal of the tumor remains the therapeutic aim but clinical presentation and imaging modalities cannot help to differentiate between Klatskin tumors and Klatskin mimicking lesions prior to surgery.

Aged↗
PubMed1996

Density gradient of the lung parenchyma at computed tomographic scanning in patients with pulmonary hypertension and systemic sclerosis.

RATIONALE AND OBJECTIVES: Pulmonary hypertension may complicate systemic sclerosis in the absence of fibrosing alveolitis. Abnormal pulmonary perfusion is known to result in regional differences in lung density at computed tomographic (CT) scanning. We assessed possible disturbances in the normal CT density gradient in patients with systemic sclerosis and reduced gas transfer but no clinical or radiologic evidence of fibrosing alveolitis. METHODS: We analyzed the CT scans of 15 patients with systemic sclerosis. Regions of interest were drawn in the most dependent and nondependent zones in the upper and lower lobes of each lung. Average densities for the most dependent and nondependent zones were calculated for the upper and lower lobes, and a global lung density measurement was derived. Zonal and global density gradients were derived from mean density measures (mean density of dependent areas minus mean densities of nondependent areas). RESULTS: Five of the 15 patients had pulmonary hypertension, defined as an estimated systolic pressure in the pulmonary artery of greater than 30 mm Hg shown by Doppler echocardiography. Patients with pulmonary hypertension were characterized by higher global lung density for nondependent zones (median, -821 H versus -853 H; p = .07) but not for dependent zones (-800 H versus -815 H, p = .71). The lung density gradient was reduced globally and in the lower zones in patients with pulmonary hypertension (p = .05). CONCLUSION: In cases of systemic sclerosis without fibrosing alveolitis, we found a significantly smaller density gradient between dependent and nondependent lung regions in patients with pulmonary hypertension compared with patients without pulmonary hypertension. This finding suggests that the normal CT density gradient depends in part on normal compliance of the pulmonary vasculature.

Absorptiometry, Photon↗
PubMed1998

Indomethacin and pancreatic enzymes synergistically damage intestine of rats.

The use of high-dose pancreatic enzymes by patients with cystic fibrosis was associated with the development of fibrosing colonopathy. Preliminary studies indicated that the infusion of high-dose pancreatic enzymes alone did not cause intestinal damage. We hypothesized that cystic fibrosis patients that developed fibrosing colonopathy had increased intestinal permeability. Our goal was to develop a rat model for pancreatic enzyme-induced fibrosing colonopathy by increasing intestinal permeability with the use of indomethacin. Pancreatic enzymes, 150,000 units/kg/day, and indomethacin, 3 mg/kg/day, alone and in combination were administered via duodenal catheter to rats for 10 days. Indomethacin and pancreatic enzymes caused intestinal damage, resulting in significant increases in the total number of ulcers (P < 0.007), the number of severe ulcers (P < 0.003), and ulcers in the cecum and colon (P < 0.0007). We conclude that the combination of indomethacin and pancreatic enzymes acts synergistically to cause damage to the intestine.

Animals↗
PubMed2006

The course of hepatitis C virus infection in pretransplantation anti-hepatitis C virus-negative renal transplant recipients: a retrospective follow-up study.

BACKGROUND: The aim of this study is to evaluate the natural course of hepatitis C virus (HCV) infection in renal transplant recipients infected shortly before or after renal transplantation. METHODS: Seventeen renal transplant recipients with no detectable antibodies to HCV before renal transplantation either seroconverted after transplantation or developed cholestatic syndrome without seroconversion, but with HCV RNA positivity. They were followed up for a mean of 7.2 +/- 4.2 (SD) years after renal transplantation and underwent consecutive liver biopsies. RESULTS: Biochemical abnormalities initially were observed a median of 5.7 months (25th, 75th percentiles, 2.4, 13.9) after transplantation. Initial liver biopsies showed acute hepatitis in 5 patients and chronic hepatitis in 9 patients, whereas 3 patients had histological findings of fibrosing cholestatic hepatitis. During a median follow-up of 2.0 years (25th, 75th percentiles, 1.3, 4.6), the condition of 5 patients, initially with diagnoses of acute hepatitis, deteriorated rapidly, with a median fibrosis progression rate of 0.77 (25th, 75th percentiles, 0.56, 0.86) per year. Six patients with chronic hepatitis progressed with a median fibrosis progression rate of 0.35 (25th, 75th percentiles, 0.15, 0.69) per year in a median of 3.1 years (25th, 75th percentiles, 2.4, 3.5), whereas the other 3 patients with chronic hepatitis with elevated cholestatic liver enzyme levels developed early fibrosing cholestatic hepatitis (1 patient) or vanishing bile duct syndrome (2 patients). Genotype 1 was found in 7 of 9 patients with fibrosing cholestatic hepatitis or vanishing bile duct syndrome (78%; P = 0.049). Six of 17 patients died a median of 6.1 years (25th, 75th percentiles, 1.5, 7.1) posttransplantation; 4 of these 6 patients died of hepatic failure. CONCLUSION: HCV infection acquired shortly before or after renal transplantation frequently is associated with an adverse clinical outcome, characterized by rapid progression of fibrosis, development of cholestatic syndrome, and high mortality rate. Acute hepatitis occurring under maximal immunosuppression is of great prognostic significance, determining a specific high-risk group.

Female↗
PubMed2001

Chylothorax in cats: is there a role for surgery?

Chylothorax is a complex disease with many identified underlying causes including cardiac disease, mediastinal masses, heartworm disease and trauma. Management of this disease should be directed at identifying the cause, if possible, and treating the underlying disorder. In cats with idiopathic chylothorax, medical management is recommended initially because the condition may resolve spontaneously. Owners should be made aware of the potential development of fibrosing pleuritis in affected cats. When medical management is impractical or unsuccessful, surgical intervention should be considered. Surgical options include mesenteric lymphangiography and thoracic duct ligation, pericardiectomy, omentalisation, passive pleuroperitoneal shunting, active pleuroperitoneal or pleurovenous shunting, and pleurodesis. Of these, only thoracic duct ligation and pericardiectomy are preferred by the author because, if successful, the result is complete resolution of the chylothorax, thereby reducing the risk of developing fibrosing pleuritis. Omentalisation may be beneficial in some animals as adjuvant therapy, but this procedure may still allow fibrosing pleuritis to occur. Until the aetiology of the effusion in cats with idiopathic chylothorax is understood, the treatment success rate will be less than ideal. Future research needs to be directed at determining the pathophysiologic mechanisms underlying this disease in cats.

Animals↗
PubMed2003

Evaluation and management of alveolitis and interstitial lung disease in scleroderma.

PURPOSE OF REVIEW: In the fibrosing alveolitis of systemic sclerosis, treatment decisions depend on prognostic evaluation, which continues to excite considerable interest and debate. Advances in the staging of fibrosing alveolitis of systemic sclerosis and recent therapeutic studies are discussed in this review. RECENT FINDINGS: The decision about whether to start treatment is often the most difficult clinical challenge, because many patients have limited pulmonary fibrosis that will not necessarily progress. The estimation of disease extent (using high-resolution CT) and disease severity (using pulmonary function tests) is pivotal. Factors reducing the threshold for treatment, in addition to severe disease, include evidence of recent deterioration, a short duration of systemic disease, antitopoisomerase antibody positivity, and, in some cases, bronchoalveolar lavage findings (although the role of bronchoalveolar lavage remains contentious). Histologic appearances at surgical biopsy have little prognostic value, with the great majority of patients having nonspecific interstitial pneumonia. Best current initial treatment consists of either oral or intravenous cyclophosphamide, usually administered with low-dose corticosteroid therapy, although the risk of scleroderma renal crisis with low-dose steroid therapy requires further evaluation. SUMMARY: Careful prognostic evaluation, including the staging of disease severity and the definition of longitudinal disease behavior (by serial imaging and pulmonary function tests), is central to the formulation of a logical management plan in fibrosing alveolitis of systemic sclerosis. Cyclophosphamide, the best initial treatment currently, is associated with significant toxicity, justifying therapeutic studies of other immunosuppressive agents and a wide range of anticytokine and antifibrotic agents.

Adrenal Cortex Hormones↗
PubMedDate not supplied

CT findings in malignant pleural mesothelioma related to nonoccupational exposure to asbestos and fibrous zeolite (erionite).

Endemic malignant pleural mesothelioma (MPM) in Turkey is related to two mineral fibers, tremolite asbestos and fibrous zeolite (erionite). Thirteen cases of MPM from the Cappadocian area, where the soil is rich in erionite, and 29 cases of MPM, from villages whose occupants have high asbestos exposure, were examined by CT. The CT findings of the two groups of MPM were compared with respect to the configuration of the pleural lesions, stage of disease, fissural involvement, pleural effusion, presence of calcified pleural plaques, and chronic fibrosing pleuritis. In erionite-related MPM the pleural lesions were flat and smooth in 69.1%; in asbestos-related MPM the lesions were nodular in 55.1%. Stage IV disease, calcified pleural plaques, and chronic fibrosing pleuritis were more common in the erionite-related MPM. The rest of the findings were similar in both groups. The early radiological diagnosis of erionite-related MPM may be even more difficult because of the similarity of the pleural lesions to chronic fibrosing pleuritis.

Aluminum Silicates↗
PubMed1998

The effect of intestinal permeability on pancreatic enzyme-induced enteropathy in the rat.

BACKGROUND: Fibrosing colonopathy in cystic fibrosis occurs in children 2 to 7 years old and is associated with excess doses of high and regular strength lipase pancreatic enzymes, given for more than 6 months. A rat model was developed to study the effects of excessive doses of pancreatic enzymes equivalent to those producing fibrosing colonopathy in cystic fibrosis patients. METHODS: Five groups of animals were studied after administration of different combinations of pancreatic enzymes, oleic acid, and reserpine. RESULTS: Pancreatic enzymes alone produced minimal damage to the intestine and none to the liver. Excessive doses of pancreatic enzymes in combination with agents that increased intestinal permeability (oleic acid, reserpine) were associated with intestinal eosinophilia and necrosis of the jejunoileal muscle layer and inflammatory nodules in the liver, which increased with duration of insult. CONCLUSIONS: Increased intestinal permeability potentiates damage to the intestine caused by excessive pancreatic enzyme dosage. It is a characteristic of cystic fibrosis that may increase vulnerability to fibrosing colonopathy.

Animals↗
PubMed1984

Anti-Jo-1 antibody: a marker for myositis with interstitial lung disease.

An autoantibody known as anti-Jo-1 antibody is found in 25% of patients with myositis. Its prevalence in patients with both myositis and cryptogenic fibrosing alveolitis was 68% (13 out of 19 patients), compared with 7.5% in patients with myositis alone (four of 53) and 3% in patients with cryptogenic fibrosing alveolitis alone (two of 62). Anti-Jo-1 antibody may be useful in indicating patients with myositis and cryptogenic fibrosing alveolitis. Raynaud's phenomenon, the sicca syndrome, and mild arthritis are also often part of the syndrome.

Autoantibodies↗
PubMed1970

Lung function in patients receiving busulphan.

An attempt was made to achieve earlier detection of busulphan lung (fibrosing alveolitis) and to determine its incidence by means of serial studies during life, including measurement of the gas transfer factor. Twenty-three patients were investigated over an average period of nearly two years of busulphan treatment. One case of busulphan lung was detected and subsequently confirmed at necropsy, but in the remainder there was no clinical, radiological, or physiological evidence of fibrosing alveolitis. It is concluded that the development of fibrosing alveolitis may be related to individual genetic or immunological factors rather than to busulphan dosage.

Adolescent↗
PubMed1997

Colonic wall thickness measured by ultrasound: striking differences in patients with cystic fibrosis versus healthy controls.

BACKGROUND: Colonic strictures represent an advanced stage of fibrosing colonopathy in patients with cystic fibrosis. AIMS: To clarify whether ultrasonography can identify patients with an early stage of fibrosing colonopathy and to determine clinical factors that influence bowel wall thickening. PATIENTS: Ninety patients with cystic fibrosis, median age 10 years, and 46 healthy controls, median age 13 years, were investigated. METHODS: Bowel wall thickness was measured by ultrasound in a prospective study. RESULTS: In cystic fibrosis, wall thickness of both small intestine and colon was significantly (p < 0.0001) higher than in controls; 81% of patients with cystic fibrosis had a maximum colon wall thickness at any site of 2 mm or more, a value that was never reached by controls. The maximum colon wall thickness was 6.5 mm. Bowel wall thickness was unchanged at re-examination after one year. There was no progression even with high dose pancreatic supplements. There was no association between bowel wall thickness and clinical features such as previous meconium ileus, intestinal resection, distal intestinal obstruction syndrome, abdominal pain, or pancreatic enzyme dose. CONCLUSIONS: There is genuine intestinal involvement in cystic fibrosis; in a few cases this could lead to fibrosing colonopathy.

Adolescent↗