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PubMedDate not supplied

Fibrosing alveolitis in childhood. A long-term follow-up.

A retrospective analysis of 14 pediatric cases with fibrosing alveolitis, proved by lung biopsy, was carried out. The children were initially 6-16 years old (mean: 9.8 years) and were observed for 2 to 7 years. Symptoms had been present prior to diagnosis for 1 to 156 weeks (median: 11 weeks). The most prominent clinical findings were dyspnoea (100%), dry cough (91%), tachypnoea (85%), and weight loss (70%). Open (n = 9) or transbronchial (n = 5) lung biopsy specimens had predominantly cellular appearances in 8 children (group A) and showed predominantly fibrotic changes in 6 cases (group B). Lung function tests revealed a marked ventilatory impairment in all children. Mean vital capacity was 44% (SD 16%), and total lung capacity 58% (SD 14%) of the predicted normal values. Specific lung compliance was significantly lower in children with fibrotic changes (mean: 0.016 vs. 0.030 ml/cmH2O/ml in patients of group A, p less than 0.05). Results from 200 lung function measurements were evaluated during the follow-up. Corticosteroid treatment improved vital capacity in all children after 6 and 12 months of treatment to 64% and 62% of the predicted normal, respectively. Deterioration of pulmonary function was observed in 7 children when prednisone was tapered to below 10 mg per day. Only 4 children had a normal vital capacity at their last visit to the clinic. We conclude that children with fibrosing alveolitis experience severe alterations in lung function, and that the overall outlook is quite unfavorable.

Adolescent↗
PubMed2006

Corporoscopic excavation of the fibrosed corpora cavernosa for penile prosethesis implantation: optical corporotomy and trans-corporeal resection, Shaeer's technique.

INTRODUCTION: Implantation of a penile prosthesis in cases of neglected or resistant ischemic priapism, or delayed re-implantation following prosthesis infection and extraction, is usually a difficult and risky procedure due to fibrosis of the corpora cavernosa. Among the common complications are perforation of the urethra, tunica albuginea, and infection. The complications are usually due to the use of blind force against resistance. AIM: We propose the techniques of Trans-Corporeal Resection and Optical Corporotomy as adjuvant measures for excavating the fibrosed corpora cavernosa under vision, without the use of force against resistance. METHODS: Six patients with diffuse fibrosis of the corpora cavernosa were operated on. The instruments and technique are the same as for optical urethrotomy and transurethral resection. Optical Corporotomy was started with, where the corpora are incised from within. After establishment of a satisfactory passage, Trans-Corporeal Resection followed to scrape the fibrous tissue. Implantation of penile prosthesis was completed as usual. The procedure was performed through 1.5 cm incision in the tunica albuginea. MAIN OUTCOME MEASURES: Length, girth, and straightness in the erect position, as well as the incidence of complications. RESULTS: Operative time was an average of 90 minutes. No difficulty was encountered during the procedure. No complications were noted through 1 year of follow-up. CONCLUSION: Optical Corporotomy and Trans-Corporeal Resection allow for force-free, visually monitored excavation of the fibrosed corpora cavernosa, aiming at safer penile prosthesis implantation.

Erectile Dysfunction↗
PubMed1970

Fibrosing alveolitis associated with renal tubular acidosis.

The discovery of a case of renal tubular acidosis and fibrosing alveolitis led to the investigation of 19 further patients. Abnormal pulmonary function tests were found in a further four patients with overt renal tubular acidosis and in four out of eight patients with "incomplete" renal tubular acidosis. The response to an ammonium chloride test in seven patients with cryptogenic fibrosing alveolitis was normal. Those patients with a defect of both renal acidification and pulmonary gas transfer had concurrent autoimmune diseases such as Sjögren's syndrome and primary biliary cirrhosis. It is suggested that the renal and pulmonary abnormalities may be part of a systemic disorder capable of affecting many organs. Moreover, hyperglobulinaemia and autoantibodies in these patients further suggests that immunological mechanisms are concerned in the pathogenesis of these abnormalities.

Acidosis, Renal Tubular↗
PubMed1995

Antiphospholipid syndrome in a patient with rapidly progressive fibrosing alveolitis.

The antiphospholipid syndrome is a thrombotic disorder which can occur in a primary form or more classically in systemic lupus erythematosus. An association between cryptogenic fibrosing alveolitis and the antiphospholipid syndrome has not previously been reported. We describe a patient with severe cryptogenic fibrosing alveolitis who developed pulmonary embolism and myocardial infarction in the presence of antiphospholipid antibody. The case also illustrates that worsening respiratory function may represent superimposition of one lung pathology on another, and may not simply be a deterioration of the pre-existing disease.

Antiphospholipid Syndrome↗
PubMed2000

Adult familial cryptogenic fibrosing alveolitis in the United Kingdom.

BACKGROUND: Familial cases of cryptogenic fibrosing alveolitis (CFA) have previously been reported; however, the prevalence and genetic background of this disorder are not known. The clinical and epidemiological findings of 25 families identified within the UK are reported. METHODS: Adult pulmonary physicians in the UK were asked to identify all families under their care in which two or more individuals had been diagnosed with fibrosing alveolitis of unknown cause. A detailed structured questionnaire was sent to each proband to delineate possible environmental/occupational exposures and to obtain complete pedigree data. Physicians were also asked to provide clinical and diagnostic information. RESULTS: Twenty five families were identified comprising 67 cases. Suitable data for analysis were available for 21 families (57 cases). The male:female ratio was 1. 75:1 (p<0.05). A high resolution computed tomographic (HRCT) scan was performed in 93% and a diagnosis of CFA confirmed on biopsy specimens in 32%. The mean age at diagnosis was 55.5 (2.5) years. Fifty percent of cases were ever smokers and 18% had been diagnosed as asthmatic. Exposure to known fibrogenic agents was recorded by 36% of patients. Clinical signs/symptoms and histological findings were indistinguishable from non-familial cases. CONCLUSIONS: This study represents the largest cohort of familial CFA cases reported to date and confirms a prevalence of 1.34 cases per 10(6) in the UK population. Although rare, such cases represent an important subgroup in which a genetic susceptibility to pulmonary fibrosis is particularly evident. Familial patients are younger at diagnosis but otherwise indistinguishable from non-familial cases. The mode of inheritance is as yet unclear but a number of genetic loci are likely to be involved and are the subject of ongoing studies.

Adult↗
PubMed1978

"Blue bodies" in a case of cryptogenic fibrosing alveolitis (desquamative type) an ultra-structural study.

A patient with cryptogenic fibrosing alveolitis, with both mural and desquamative features, had two lung biopsies at the times of coronary artery surgery. These lung specimens were studied, using light and electron microscopy, with immunofluorescence techniques and electron microanalysis. In addition to the typical changes of cryptogenic fibrosing alveolitis previously reported, we found "blue-staining bodies" within alveolar macrophages and giant cells. These bodies were 15--25 micrometer in diameter with an iron rich outer rim and core of connective tissue mucin--possibly chondroitin sulphate or dermatan sulphate. It seems unlikely that these "blue bodies" were due to fibreglass dust to which the patients had had a trivial exposure, but their exact nature and significance remains unclear.

Dust↗
PubMed1980

Response to methotrexate in fibrosing alveolitis associated with connective tissue disease.

Methotrexate by intermittent intramuscular injection was used to treat three patients with fibrosing alveolitis complicating connective tissue diseases. All three patients had improvement of symptoms, two had radiological improvement, and one had significant improvement of pulmonary function. Two patients whose pulmonary symptoms, function, and chest radiographs had deteriorated on penicillamine improved when treated with methotrexate. Methotrexate may be a useful treatment for fibrosing alveolitis complicating connective tissue diseases.

Adult↗
PubMed1983

Cryptogenic fibrosing alveolitis: prediction of fibrogenic activity from immunohistochemical studies of collagen types in lung biopsy specimens.

Collagen immunofluorescence studies were performed on biopsy specimens from 25 patients with cryptogenic fibrosing alveolitis. We studied the relationship of these results to the clinical, radiological, and physiological assessments of disease activity during six-month periods before and after the lung biopsy; to the appearances on routine histological examination; to the inflammatory cell proportions in bronchoalveolar lavage fluid; and to the response to treatment. Positive associations were observed between the presence of type III collagen and disease activity before (p less than 0.015) and after (p less than 0.02) lung biopsy. These were independent of clinical and routine histological features. The type-III-positive group also contained nine of the 11 responding to treatment. None of the type-III-positive patients had a quiescent or stable course. In contrast, most patients with no type-III-collagen fluorescence had a stable course over the time of this study and did not improve on treatment. It is suggested that collagen typing may be an additional useful method of assessing potentially reversible disease in cryptogenic fibrosing alveolitis.

Adult↗
PubMed1984

Serum type III procollagen peptide concentration in cryptogenic fibrosing alveolitis and its clinical relevance.

The serum concentrations of a metabolite produced during synthesis type III collagen, type III procollagen peptide (PCP), were measured in patients with cryptogenic fibrosing alveolitis, and the results were related to the course of the disease and its response to treatment. The mean PCP concentration was significantly higher for patients untreated at the time of sampling than for an age and sex matched control group (p less than 0.001). Seventeen of these patients subsequently started treatment and their response to treatment was assessed by measurement of changes in the transfer coefficient (KCO) and forced vital capacity (FVC) at six months and one year later. Those patients showing a response to treatment, indicated by an increase in the percentage predicted KCO or FVC of more than 10% one year after starting treatment, had a significantly higher mean PCP concentration (p less than 0.01 and p less than 0.001 respectively) than patients with stable or deteriorating physiological indices. When the PCP concentrations of these patients were compared with repeat measurements three to six months after the commencement of treatment 14 out of 17 showed a decrease in PCP levels. This percentage change in PCP concentrations correlated significantly with the change in the percentage predicted KCO and FVC at 12 months after the commencement of treatment (p less than 0.01). These results suggest that measurement of PCP concentrations in patients with cryptogenic fibrosing alveolitis may be useful in the assessment of response to treatment.

Adrenal Cortex Hormones↗
PubMed1985

Immunohistological analysis of lung tissue from patients with cryptogenic fibrosing alveolitis suggesting local expression of immune hypersensitivity.

Immunohistological analysis using monoclonal antibodies in conjunction with histochemical techniques has been applied to lung biopsy material from patients with cryptogenic fibrosing alveolitis. Subsets of lymphoid and non-lymphoid cells have been identified in situ. This analysis showed that the inflammatory cells present were predominantly mononuclear. Most of the lymphoid cells were B lymphocytes, organised into follicles with occasional germinal centre formation. IgM was the major class of immunoglobulin expressed. Both T4+ and T8+ lymphocytes were seen diffusely distributed in the interstitium. The T4+ positive cells were also seen within the B lymphoid follicles. Almost all non-lymphoid cells expressed the phenotype of inflammatory macrophages, but a few also expressed a phenotype characteristic of interdigitating cells. These results suggest that a local B lymphoid immune response is occurring in cryptogenic fibrosing alveolitis. The possibility that a cell mediated immune response is also emerging is discussed.

Adult↗
PubMed1989

Randomised controlled trial comparing prednisolone alone with cyclophosphamide and low dose prednisolone in combination in cryptogenic fibrosing alveolitis.

In a randomised, controlled study alternate day prednisolone with an initial high dose phase ("prednisolone only series") has been compared with cyclophosphamide plus alternate day low dose prednisolone ("cyclophosphamide-prednisolone series") in 43 patients with previously untreated fibrosing alveolitis (five patients had received prednisolone in minimal dosage). In the prednisolone only series prednisolone 60 mg daily was given for one month and then reduced by 5 mg a week to 20 mg on alternate days or the minimum dose to maintain early improvement. Patients in the cyclophosphamide-prednisolone series received 100, 110, or 120 mg cyclophosphamide daily (depending on body weight) plus 20 mg prednisolone on alternate days. Treatment was continued indefinitely, or changed to the alternative regimen if the patient deteriorated, failed to improve, or developed drug toxicity. For response to treatment (as judged by change in breathlessness score, radiographic appearance, and lung function) patients were classified as improved, stable, or deteriorating. Deaths from cryptogenic fibrosing alveolitis were also analysed. Improvement had occurred at one or more assessments in seven of the 22 patients in the prednisolone only series and in five of the 21 patients in the cyclophosphamide-prednisolone series. At three years, however, only two of the 22 patients in the prednisolone only series were still improved and three stable, compared with one and seven of the 21 patients in the cyclophosphamide-prednisolone series (three of the seven had stopped treatment because of toxicity). Life table analysis suggested better survival in patients in the cyclophosphamide-prednisolone series but this was not significant. At three years 10 of 22 patients in the prednisolone only series had died compared with three of 21 patients in the cyclophosphamide-prednisolone series. With death or failure of first treatment regimen as outcome there was a significant advantage to the patients having cyclophosphamide-prednisolone. This advantage was explained in part by the better lung volumes in this group on admission. After allowance had been made for total lung capacity (TLC), no other factor was predictive of outcome. Analyses of subgroups according to TLC on admission showed that patients with a TLC below 60% predicted did badly and those with a TLC of 80% or more predicted did well with both regimens. Patients with an initial TLC of 60-79% predicted did better with the cyclophosphamide-prednisolone regimen. Side effects were uncommon in both series and those due to cyclophosphamide resolved when treatment was stopped. The combination of cyclophosphamide with prednisolone may be an alternative to prednisolone alone with an initial high dose phase. Many patients, however, failed to respond to either treatment.

Adult↗
PubMed1997

Cryptogenic fibrosing alveolitis: lack of association with Epstein-Barr virus infection.

BACKGROUND: Cryptogenic fibrosing alveolitis (CFA) is a well defined clinical entity of unknown aetiology. An association between CFA and the presence of protein indicating Epstein-Barr virus (EBV) replication within epithelial cells of the respiratory tract has recently been suggested, leading to speculation for a role for EBV in the pathogenesis of CFA. METHODS: Lung tissue was obtained from patients in three groups: those with cryptogenic fibrosing alveolitis, either lone or associated with systemic sclerosis; patients with other pulmonary disorders; and patients with normal lung. Paraffin blocks were stained using three antibodies raised against well defined EBV antigens. In addition, EBER-1 and EBER-2 anti-sense nucleotide probes were used in an attempt to identify EBV RNA. DNA was also extracted from the tissue sections and evaluated for evidence of EBV DNA using the polymerase chain reaction. RESULTS: Immunohistochemistry showed inconsistent focal positive staining with anti-EBV antibodies in all three groups, but there was no evidence of EBV RNA using in situ hybridisation. None of the samples from patients with pulmonary fibrotic disorders was found to contain EBV DNA following gene amplification. CONCLUSION: Contrary to an earlier report, these results do not support the hypothesis that EBV has a role in the pathogenesis of CFA.

DNA, Viral↗
PubMed1977

Fibrosing alveolitis in patients with neurofibromatosis.

Fibrosing alveolitis, or interstitial pulmonary fibrosis, is a common manifestation of neurofibromatosis, and was observed in 7 of 70 patients with the disease. Though neurofibromatosis is congenital, fibrosing alveolitis does not appear until adulthood, and occurs in 20% of patients with the disease who are over 30 years old. Characteristic radiographic findings include linear, interstitial density, and large upper lobe bullae; this combination limits the differential diagnosis. Pathological examination demonstrates alveolar wall thickening progressing to fibrosis and lung destruction. Pulmonary function tests can show obstructive or restrictive lung disease.

Adolescent↗
PubMed1986

Fibrosing alveolitis: CT-pathologic correlation.

Computed tomography (CT) was performed within 10 days of open lung biopsy in nine patients with fibrosing alveolitis. One-centimeter collimation contiguous scans through the chest were obtained in all patients. Additional 1.5-mm collimation scans were obtained in the area in which lung biopsy was later performed in six patients. In seven patients, CT demonstrated patchy involvement of the lung parenchyma, areas with a reticular pattern being intermingled with areas of normal lung. The reticular pattern was associated with cystic spaces 2-4 mm in diameter and was more severe in the lung periphery. Histologically, the reticular pattern corresponded to areas of irregular fibrosis. One patient had diffuse honeycombing (2-20-mm cysts), and one had honeycombing only in the lung periphery. In all patients, CT clearly defined the architectural changes seen on open lung biopsy. These changes were much better seen on the 1.5-mm than on the 10-mm collimation scans. CT may be helpful in determining the pattern and distribution of lung involvement in patients with fibrosing alveolitis and in guiding the surgeon to the most appropriate area(s) for biopsy.

Adult↗
PubMed1992

Fibrosing alveolitis: prognostic implication of ground-glass attenuation at high-resolution CT.

To evaluate the prognostic implication of ground-glass attenuation at high-resolution computed tomography (HRCT) in assessing response to treatment in fibrosing alveolitis, the authors correlated HRCT findings with the improvement in pulmonary function, as represented by the increase in percentage predicted values on pulmonary function tests after corticosteroid therapy. Nineteen patients underwent HRCT before treatment and pulmonary function testing before and after treatment. The HRCT scans were reviewed by two independent observers. Areas of ground-glass attenuation were quantified subjectively by using a 0%-100% scale with 10% increments. The extent of ground-glass attenuation at HRCT was significantly correlated with improvement in diffusing capacity for carbon monoxide (r = .67, P = .0019), forced vital capacity (r = .71, P = .0007), and forced expiratory volume in 1 second (r = .64, P = .0034) after steroid treatment. These results suggest that ground-glass attenuation at HRCT is a good predictor of response to treatment in fibrosing alveolitis.

Adrenal Cortex Hormones↗
PubMed2004

Nephrogenic fibrosing dermopathy treated with extracorporeal photopheresis.

Nephrogenic fibrosing dermopathy (NFD) is a rare fibrosing skin disease of unknown etiology occurring in patients with terminal renal disease. It was first described in the year 2000. The histology of NFD shows an increased number of dendritic cells, fibroblasts and thickened collagen fibers resembling scleromyxedema. It can be distinguished from scleromyxedema by a different distribution pattern of the skin lesions with indurated plaques mainly on the extremities and the absence of paraproteinemia. As yet, no treatment for NFD has been proven to be uniformly efficient. We describe the case of a 40-year old patient with renal insufficiency who was treated with hemodialysis and who had undergone kidney transplantation. Two years after transplantation, she developed sclerodermiform brownish plaques on her extremities. The induration improved significantly after 4 cycles of extracorporeal photopheresis.

Adult↗
PubMed1995

Interleukin-8. Differential expression in lone fibrosing alveolitis and systemic sclerosis.

Fibrosing alveolitis may occur alone (CFA) or in association with systemic sclerosis (FASSc). FASSc was recently shown to have a prognostic advantage over CFA. Because interleukin-8 (IL-8) is likely to be a major determinant of neutrophil alveolitis, we evaluated IL-8 expression in patients with CFA and FASSc and compared it with that in normal individuals and sarcoidosis and systemic sclerosis patients without pulmonary involvement (SSc no FA). IL-8 protein in bronchoalveolar lavage fluid (BALF) was assessed by immunoassay, and IL-8 mRNA expression was assessed using Northern analysis and reverse transcription-polymerase chain reaction (RT-PCR) and in situ hybridization of lung parenchyma. Compared with normal subjects, IL-8 concentration was significantly greater in both CFA (p < 0.001) and FASSc groups (p < 0.05) but no different in sarcoidosis. The IL-8 concentration in CFA was higher than in FASSc (p < 0.01) and was related to BAL % neutrophils (rs = 0.48, p < 0.01). IL-8 mRNA expression evaluated by Northern analysis was seen only in patients with CFA and FASSc and was related to BAL % neutrophils (rs = 0.63, p < 0.01). We suggest that IL-8 is a key factor in the pathogenesis of fibrosing alveolitis and that the poorer prognosis of CFA compared with FASSc is related to higher levels of IL-8 within the lower respiratory tract.

Adult↗
PubMed1991

Pathogenetic significance of reactive oxygen species in diffuse fibrosing alveolitis.

Excessive release of reactive oxygen metabolites (ROM) from lung inflammatory cells has been claimed to be of major pathogenetic significance in diffuse fibrosing alveolitis. In the present study, the content of oxidized methionine residues [Met(O)] as a percentage of total methionine (Met) in BAL-derived proteins was used to assess the biologic effect of ROM. In addition, procollagen-III-peptide was measured in BAL fluid as a marker of fibroblast activation. We investigated bronchoalveolar lavage (BAL) samples from seven control patients without evidence of interstitial lung disease and from 42 patients with fibrosing alveolitis caused by idiopathic pulmonary fibrosis (IPF), n = 20, or by collagen vascular disease (CVD), n = 22. Met(O) was elevated in the patients with IPF or CVD compared with that in the control subjects (8.86 +/- 1.26 and 8.13 +/- 1.44% versus 3.36 +/- 0.49%, p less than 0.01 and p less than 0.05, respectively; mean +/- SEM). A positive correlation was found between percentage of neutrophils in BAL and Met(O) in both groups separately and combined (IPF, r = 0.84; p less than 0.001; CVD, r = 0.44; p less than 0.05; IPF and CVD, r = 0.60; p less than 0.001), whereas an inverse relationship existed between Met(O) and the percentage of alveolar macrophages in BAL (IPF, r = -0.59; p less than 0.01; CVD, r = -0.24; NS; IPF and CVD, r = -0.41; p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗