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PubMed1977

[Fibrosing alveolitis--a contribution to the ultrastructure and pathogenesis of interstitial pulmonary diseases (author's transl)].

Considering the functional anatomy of the lung parenchyma the pathomorphological response pattern of the alveolus has been described. Hereby, the fibrosing alveolitis is conceived as an uniform concept of a disease, to which many different aetiological factors are associated. The ultrastructural pathology of early changes in fibrosing alveolitis has been described in the bleomycin lung as an example, which pathogenesis (as a chemically induced pulmonary damage) has a character of a model for the process of fibrosing alveolitis.

Alveolitis, Extrinsic Allergic↗
PubMed1995

[Non-steroidal antirheumatic ointments in the treatment of primary periarticular and intramuscular fibrositis].

The author presents his experience assembled in a short-term (two-week) therapeutic trial with Mobilisin ointment, which contains flufenamic acid, in 50 patients with primary localized periarticular and muscular fibrositis. The preparation was used locally three times per day-2-3 cm of ointment pressed from a tube. The patients were given a form in which they recorded every night the effect classified in four grades from the best to zero effect. In 49 the tolerance of the ointment was very good. In 45 patients a therapeutic effect was achieved (90%), comprising 21 (42%) where the effect was excellent, in 24 it was good or satisfactory (48%) and only in 5 patients no effect was recorded or the patents' statement was vague. In the first group of 21 patients 16 were able to abandon analgetic and anti-inflammatory treatment with non-steroid antirheumatics. Patients with the muscular form of fibrositis practically agreed in favour of the ointment. Gels according to these patients tissues. The authors remind that according to their experience gels, incl. Mobilisin, are suited for periarticular forms of fibrositis. It is also useful to rub the ointment in micro-massage to achieve a greater initial hyperaemia and better resorption. Mobilisin ointment may prove useful also in other disciplines, in particular orthopaedics, surgery, traumatology, neurology, rehabilitation and sports medicine.

Adult↗
PubMed1994

Epidemiology of cryptogenic fibrosing alveolitis in Moravia and Silesia.

Cryptogenic fibrosing alveolitis in Moravia and Silesia (4 million inhabitants) was evaluated using standard epidemiological and clinical parameters. During 1981-1990 the incidence of cryptogenic fibrosing alveolitis ranged from 0.74 to 1.28/100,000. The trend of incidence was increasing slightly. The prevalence rose from 6.5 to 12.1/100,000. The male/female ratio was 1:1.2; 54% of patients were less than 40 years old. Biopsy (mostly open lung biopsy) supported the diagnosis in 38% of cases. During 1981-1990 years 379 new cases were detected, 488 cases altogether were registered up to 1990. The death rate calculated by incidence and prevalence was 13% and 10% resp. The mean regional incidences varied from 0.34 to 2.69/100,000, regional prevalences varied from 4.1 to 27.6/100,000. No correlation with the regional incidences of sarcoidosis and tuberculosis was found. No significant differences in coal mining areas with a high occurrence of pneumoconiosis or in areas with developed agriculture were detected. Significantly higher values were found in the regions of the four major diagnostic centres: a mean incidence of 1.58/100,000 and a mean prevalence of 20.8/100,000 (p < 0.05). The results suggest that the level of diagnostic possibilities and efforts dominates over ecological and environmental factors in the detecting of cryptogenic fibrosing alveolitis.

Adult↗
PubMed1996

Fibrosing inflammatory pseudotumors involving the skull base: MR and CT manifestations with histopathologic comparison.

PURPOSE: To describe the MR and CT features of fibrosing inflammatory pseudotumors of the skull base region, and to document the MR signal intensity of the lesions with histopathologic comparison. METHODS: We reviewed the MR and CT studies of five patients with pathologically proved fibrosing inflammatory pseudotumor involving the skull base. Unenhanced spin-echo T1- and T2-weighted and contrast-enhanced T1-weighted MR images were obtained at 0.5 T in three patients and at 1.5 T in two patients. MR findings were correlated with histopathologic findings in all five cases, and the enhancement pattern was compared with CT findings in three cases. RESULTS: In three cases, the cavernous sinus was involved unilaterally, with adjacent extracranial infiltrative masses. In one case, both orbits, the cavernous sinuses, and the tentorium were involved with diffuse infiltrative lesions. One patient had an infiltrative nasopharyngeal mass; and in all five patients, MR images showed localized involvement of the skull base, with bone marrow replaced by tumor. The soft-tissue lesions were hypointense on T2-weighted images in all five cases and showed homogeneous contrast enhancement. Histopathologic studies revealed scanty inflammatory cell infiltration with densely fibrotic background in all cases. The hypointensity of the lesions on T2-weighted images seemed to be related to the degree of fibrosis. CONCLUSION: Fibrosing inflammatory pseudotumor shows characteristic MR findings of infiltrative lesion with bone destruction and hypointensity on T2-weighted images. The lack of mobile protons due to the fibrotic background and/or high cellularity of the lesions may be the reason for their hypointensity and weaker enhancement on MR images.

Adult↗
PubMed2000

Fibrosing alopecia in a pattern distribution: patterned lichen planopilaris or androgenetic alopecia with a lichenoid tissue reaction pattern?

BACKGROUND: Androgenetic alopecia is characterized by a defined area of progressive nonscarring alopecia. The clinical and histological findings in 15 women and 4 men with progressive scarring alopecia in a pattern distribution were studied. The results were evaluated and compared with clinicopathologic entities that feature scarring of the central scalp area, specifically, lichen planopilaris, pseudopelade, and follicular degeneration syndrome. OBSERVATIONS: Patients developed progressive fibrosing alopecia of the central scalp, without the multifocal areas of involvement typical of lichen planopilaris and pseudopelade. Perifollicular erythema, follicular keratosis, and loss of follicular orifices were limited to a patterned area of involvement. Biopsy specimens of early lesions demonstrated hair follicle miniaturization and a lichenoid inflammatory infiltrate targeting the upper follicle region. Advanced lesions showed perifollicular lamellar fibrosis and completely fibrosed follicular tracts indistinguishable from end-stage lichen planopilaris, pseudopelade, or follicular degeneration syndrome. CONCLUSIONS: Some patients with androgenetic alopecia might have additional clinical and histological features of inflammation and fibrosis limited to the area of androgenetic hair loss. In these patients, the histological findings of early lesions are identical to those seen in lichen planopilaris. The lichenoid tissue reaction leading to follicular destruction in these patients might be pathogenetically related to the events underlying androgenetic alopecia.

Adult↗
PubMed1997

Patterns of use of pancreatic enzyme supplements in fibrosing colonopathy: implications for pathogenesis.

Sixteen pathologically confirmed and 14 suspected cases of a new disease entity, fibrosing colonopathy, have been described in the UK and the US in children with cystic fibrosis since 1991. The patterns of use of pancreatic enzyme supplements in cases were compared with use in controls and in the market, in the 2 years prior to surgery for fibrosing colonopathy or the equivalent date in controls. The disease was only found in children with cystic fibrosis who had received brands of pancreatic enzyme supplement coated with methacrylic acid copolymer, for a period of at least 6 months. The risk was dose-related. No cases could be identified in children who had only received other pancreatic enzyme formulations, irrespective of the strength of the formulation or the dose.

Journal Article↗
PubMed1999

Pathogenesis of fibrosing colonopathy: the role of methacrylic acid copolymer.

Fibrosing colonopathy is a serious bowel disorder which was first seen in 1991 in patients with cystic fibrosis treated with high doses of pancreatic enzyme supplements. Epidemiological evidence from two case-control studies, temporal data and direct experimental evidence support the conclusion that there is a causal relationship between the disease and intake of high doses of the excipient, methacrylic acid copolymer, which is used to coat certain brands of pancreatic enzyme. A high intake of lipase, the active ingredient in pancreatic enzymes, is not a risk factor for fibrosing colonopathy in the absence of methacrylic acid copolymer. Excipients may be toxic if used in new patient populations such as children and in doses exceeding the safe levels predicted by animal toxicology.

Journal Article↗
PubMed1986

Evaluation of amitriptyline in primary fibrositis. A double-blind, placebo-controlled study.

Seventy patients with primary fibrositis satisfying Smythe's criteria were studied in a 9-week double-blind trial comparing 50 mg amitriptyline with placebo. Fifty-nine patients completed the trial: 27 were treated with amitriptyline, and 32 took a placebo. The patients who received amitriptyline improved significantly in their morning stiffness and pain analog scores at 5 and 9 weeks, compared with baseline scores, whereas no changes were noted in these parameters in the placebo group. Fibrocytic point tenderness did not improve significantly in either of the treatment groups. When compared with the placebo group, the amitriptyline group improved significantly with respect to sleep pattern and patient and physician global assessments. Our data indicate that amitriptyline has some therapeutic benefit in patients with primary fibrositis.

Adult↗
PubMed1988

A comparison of cyclobenzaprine and placebo in the management of fibrositis. A double-blind controlled study.

The efficacy of cyclobenzaprine (Flexeril), as compared with placebo, was tested in a 12-week, double-blind, controlled trial of 120 patients with fibrositis. Of the patients who received placebo, 52% dropped out due to lack of efficacy of the drug, compared with 16% of patients taking cyclobenzaprine. The dropout rate due to adverse reactions was similar in the 2 groups. Patients taking cyclobenzaprine experienced a significant decrease in the severity of pain and a significant increase in the quality of sleep. There was a trend toward improvement in the symptoms of fatigue, but morning stiffness was not alleviated. These improvements in symptoms were associated with a significant reduction in the total number of tender points and in muscle tightness. Our findings indicate that cyclobenzaprine is a useful adjunct in treating patients with the fibrositis syndrome.

Adult↗
PubMed2004

Dialysis-associated systemic fibrosis (nephrogenic fibrosing dermopathy): study of inflammatory cells and transforming growth factor beta1 expression in affected skin.

OBJECTIVE: Nephrogenic fibrosing dermopathy (NFD) is a newly recognized cutaneous fibrotic disorder occurring in individuals with end-stage renal disease (ESRD). The aim of the present study was to describe the clinical and histopathologic features of 9 new cases and to characterize the inflammatory cells and expression of transforming growth factor beta1 (TGFbeta1) in affected skin. METHODS: Clinical and laboratory assessments, including serology and pulmonary function studies, were performed in 9 patients undergoing long-term dialysis (8 hemodialysis; 1 peritoneal dialysis) for ESRD of diverse etiologies. Skin, fascia, striated muscles, lungs, and heart were examined by histopathology. Inflammatory cells were characterized by immunophenotyping using specific monoclonal antibodies. TGFbeta1 expression was determined by in situ hybridization. RESULTS: All patients displayed cutaneous features resembling both systemic sclerosis and diffuse fasciitis, with severe loss of motion and flexion contractures in multiple joints. Six patients displayed woody induration of the muscles of the legs, thighs, and forearms. Five of the 6 patients with lung involvement had a reduced diffusion capacity for carbon monoxide on pulmonary function testing. Marked elevations of the erythrocyte sedimentation rate and/or C-reactive protein level were found in 6 patients. Antinuclear antibodies were present at low titers in 4 patients. Histopathologic studies indicated that in addition to the dermis, the fibrotic process affected the subcutaneous tissue, fascia, striated muscles, lungs, and myocardium. Large numbers of CD68+/factor XIIIa+ dendritic cells and increased expression of TGFbeta1 were found in affected skin and muscle. CONCLUSION: Our findings indicate that the fibrotic process of NFD affects not only the dermis, but also the subcutaneous tissues, fascia, and other organs, including striated muscles, heart, and lungs. We therefore believe this is a systemic fibrosing process, and we suggest that dialysis-associated systemic fibrosis would be a better term for the condition.

Adult↗
PubMedDate not supplied

Role of cellulose in wood dust-induced fibrosing alveo-bronchiolitis in rat.

Our experiments suggest that in the development of plant dust-induced fibrosing alveobronchiolitis--Scadding's fibrosing alveolitis--the cellulose content of plant dusts has a decisive aetiological role. Namely, the wood dust (pine) and the cellulose induced morphologically identical granulomatous inflammation and fibrosis, whereas the fibre-free extract of wood dust did not cause pathological changes in the lungs. The induction of H2O2 and superoxide anion production, shown in vitro in leucocytes, probably has an important role in the development of fibrosis.

Animals↗
PubMed1982

The ultrastructure of human fibrosing alveolitis.

This report describes the ultrastructural findings in 37 patients who underwent open lung biopsy which yielded diagnoses of fibrosing alveolitis. A spectrum of lesions are categorized for the capillary endothelium and its basement membrane, the interstitial space and its fibrocellular components, and the alveolar epithelium and its basement membrane. The findings typify the different pulmonary cellular reactions to injury. Evidence for cellular regeneration and death in both epithelial and endothelial cell populations include atypical epithelial cell proliferation, capillary basement membrane multilamination, decrease in capillary lumen size and prominent pericytic ensheathment of pulmonary capillaries. Within the interstitium of the lung, proliferation of collagen and elastic fibers are documented, but in addition, abundant myofibroblasts and smooth muscle cells are present. No ultrastructural evidence of immune complex deposition was found in this study. The morphologic findings of fibrosing alveolitis further support the widespread concept that the lung responds to various injuries in a similar manner and undergoes a common reparative response regardless of etiology.

Adult↗
PubMed1989

Relapsing poly(peri)chondritis associated with fibrosing alveolar disease and antibodies to pneumocytes type II and Clara cells.

A 62-year-old man with histological confirmed relapsing polychondritis showed chondritis of ears and nose, arthritis, keratitis and a hemolytic anemia. The bronchoalveolar lavage, computed tomography of the thorax and pulmonary function tests disclosed findings compatible with fibrosing alveolar disease. IgG antibodies to alveolar pneumocytes type II and bronchiolar Clara cells were detected by indirect immunofluorescence of human lung tissue. To our knowledge this is the first report of fibrosing alveolar disease in relapsing polychondritis and detection of antibodies to human pneumocytes type II and Clara cells.

Autoantibodies↗
PubMed1978

Fibrosing alveolitis (pulmonary interstitial fibrosis) evoked by experiemntal inhalation of Gasoline vapours.

Rats exposed to an atmosphere contaminated with gasoline vapour for 6--12 weeks exhibit progressive focal interstitial fibrosis of the lung, which is associated with irregular alveolar collapse. It is suggested that this experimental model of diffuse fibrosing lung disease constitutes a useful tool for the study of the dynamics of pulmonary reactivity to atmospheric pollutants and which may facilitate an understanding of the pathogenesis of fibrosing alveolitis in man.

Air Pollutants↗
PubMed1988

Idiopathic fibrosing pancreatitis: a cause of obstructive jaundice in childhood.

Idiopathic fibrosing pancreatitis is a chronic process of unknown etiology characterized by extensive infiltration of the pancreatic parenchyma by fibrous tissue. This disease process is uncommon in the pediatric patient and is consequently rarely considered in the differential diagnosis of abdominal pain and jaundice in the child. The sonographic demonstration of a dilated biliary tree and common bile duct compressed by an enlarged pancreas may be the first suggestion of this entity. Two patients with idiopathic fibrosing pancreatitis and obstructive jaundice are reported with a review of the clinical, radiographic, and pathologic findings.

Biopsy↗
PubMed1988

Primary biliary cirrhosis with fibrosing alveolitis.

A 65-year-old case diagnosed as primary biliary cirrhosis without definite signs of Sjögren's syndrome at age 62 developed interstitial lung disease, which was clinically, histologically, radiographically, and scintigraphically compatible with fibrosing alveolitis. Analysis of the cells in bronchoalveolar lavage fluid revealed, however, increased proportions of not only neutrophils but also lymphocytic cells, which were predominant. This case should focus attention on the association of primary biliary cirrhosis and fibrosing alveolitis.

Aged↗
PubMed1992

Familial cryptogenic fibrosing alveolitis: a case report.

A family of seven siblings is described, all of whom, developed finger clubbing during their third decade. Three of the seven developed cryptogenic fibrosing alveolitis (CFA). Of the remaining four siblings, two have died prematurely from conditions possibly associated with cryptogenic fibrosing alveolitis. The youngest two siblings remain asymptomatic at present. This paper reports one of the most concentrated incidences of the rare familial form of CFA.

Biopsy↗
PubMed2003

Fibrosing mediastinitis with superior vena cava obstruction as the initial presentation of Langerhans' cell histiocytosis in a young child.

We present a 2-year-old girl with an unusual presentation of Langerhans' cell histiocytosis (LCH). Five months prior to admission to our hospital, she received IV steroids for bronchial obstruction. On admission, clinical signs of SVC obstruction were evident and a mediastinal mass was evident on the chest radiograph and MRI. Biopsy revealed fibrosing mediastinitis. Five months later, osteolysis was present on a skull radiograph. Surgical biopsy of the skull lesion revealed LCH. This case is unique because it demonstrates a rare initial manifestation of LCH that has not been previously reported. Furthermore, the primary, solitary mediastinal manifestation without calcifications was histologically interpreted as fibrosing mediastinitis, and the final diagnosis of LCH was only made after identifying the skull lesion.

Child, Preschool↗