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Clinical trials in fibrositis: a critical review and future directions.

A critical appraisal of the design of clinical trials which examined the effectiveness of various interventions in fibrositis was conducted. Therapeutic interventions included physical fitness, biofeedback, acupuncture, dothiepin, imipramine, cyclobenzaprine, S-adenosylmethionine and amitriptyline. The design and analysis of the randomized, controlled studies render their results reliable. Major areas for methodologic improvement in future trials were identified. Standardized, validated and reliable diagnostic and outcome criteria need to be established. Factors which could predict response need to be identified to enable the selection of the most appropriate patient population for inclusion in future studies. Finally, the incorporation of measures of patient function will result in more clinically meaningful outcome evaluation.

Fibromyalgia↗

Fibrositis syndrome: a historical perspective.

This paper traces the history of disturbed sleep, deep pain and exhaustion from biblical times to the present. This historical review provides the stage for the papers that follow in this volume on fibrositis syndrome.

Fibromyalgia↗

Sleep-wake mechanisms in fibrositis.

Patients with fibrositis experience chronophysiologic changes during sleep and when they are awake. A wide variety of factors contribute to this phenomenon. Chronobiologic research in the area of sleep-wake mechanisms need to be undertaken.

Biomechanical Phenomena↗

[Fibrosing mediastinitis].

Fibrosing mediastinitis is a rare entity either idiopathic or caused by granulomatous disease. Plain film findings mainly show widening of the upper half of the anterior mediastinum, whereas CT delineates more clearly the exact location of the mediastinal mass and the extent of compromise of mediastinal structures. Although vessels surrounded by the fibrous mass are typically smoothly bordered and only rarely displaced, diagnosis can only be suspected together with the patients history and clinical course.

Adult↗

The distribution of phenotypically distinct macrophage subsets in the lungs of patients with cryptogenic fibrosing alveolitis.

Monoclonal antibodies that identify phenotypically distinct macrophage subsets were used to analyse the macrophages in lung biopsy specimens and bronchoalveolar lavage fluid from patients with cryptogenic fibrosing alveolitis. Among the antibodies were RFD1, an interdigitating cell marker, RFD7, a marker for mature tissue macrophages, and RFD9, which identifies epithelioid cells as well as germinal centre macrophages. The lavage fluid was found to contain abnormally high numbers of cells staining with each of the antibodies, a finding that could be explained, at least in part, by an increased frequency of cells expressing more than one marker. In lung tissue macrophage phenotypes within the interstitium were found to differ significantly from those in the alveolar space. Most strikingly, cells bearing the antigen recognized by RFD9 were entirely absent from the interstitial macrophage population, whereas the vast majority in the alveolar lumen were RFD9-positive. The discrete compartmentalization of phenotypically different macrophages within the lung suggests that macrophages may contribute differently to lung pathology in each microenvironment. The functional capacity of the unusual RFD9-positive alveolar macrophages remains to be determined, but their close association with the process of alveolar occlusion indicates a role in the fibrotic process.

Aged↗

Circulating immune complexes in patients with cryptogenic fibrosing alveolitis.

Increased Clq binding levels have been obtained in serum from twenty-one (50%) of forty-two patients with cryptogenic fibrosing alveolitis (CFA) suggesting the presence of circulating immune complexes. There was a low frequency of positive results using a number of other tests for circulating immune complexes. The increased Clq binding levels were observed in six (35%) out of seventeen patients with lone lung involvement and in fifteen (60%) out of twenty-five patients with extrapulmonary connective tissue disorders. There was an especially close correlation between arthritis and elevated Clq binding. A strong correlation between Clq binding levels and levels of circulating rheumatoid factor (RF) and IgG, and enhancement in macrophage radiobioassay tests using RF-containing sera, suggested that RF might be involved in the circulating immune complexes in these patients. DNAase pre-treatment of sera did not influence the findings, and there was no correlation between Clq binding and levels of immunofluorescent ANA, C-reactive protein levels, or platelet counts. A weak correlation between Clq binding and erythrocyte sedimentation rates, and slightly lower binding levels in treated than untreated patients with 'lone' CFA suggested that binding levels may give some indication of disease activity and may in some instances be influenced by treatment.

Adult↗

[Prevention of recurrence in the treatment of flap fibroses].

Compared with the results of former follow-up examinations, the use of modern techniques of plastic surgery has resulted in reducing considerably the recurrence rate of flap fibroses. They permit to apply the principle of preventive transplantation of skin or mucosa to the whole of the denture-supporting area in danger of insult. The use of free mucosa grafts offers a number of advantages. Further reduction of the recurrence rate by this new technique may be expected from an improvement in prosthetic after-care and from a more generous excision of the mucosal areas in danger of insult.

Dentures↗

[Diagnostic bronchoalveolar lavage in assessing the efficacy of the treatment of idiopathic fibrosing alveolitis].

A study of the results of repeated diagnostic bronchoalveolar lavage in 27 patients with idiopathic fibrosing alveolitis (IFA) has shown that the investigation of cellular composition of the bronchoalveolar content (BAC) made it possible to specify IFA activity and its therapy-related change. Normalization or a decrease below 6% of the neutrophil content in BAC was typical of achieving low activity and remission of disease. A relatively high count of neutrophils in BAC (over 10%) during therapy was observed in preserved significant IFA activity resistant to drug therapy.

Adult↗

Fibrositis.

Fibrositis is a common clinical syndrome characterized by diffuse musculoskeletal pain and multiple tender points on physical examination. The symptoms wax and wane but do not produce severe disability. Although no single treatment is curative, a comprehensive program of patient education, drug therapy and appropriate physical activity is beneficial in many patients.

Diagnosis, Differential↗

[Morphologic studies in fibrosing alveolitis. I. Content of alveolar macrophages and lymphocytes in lung parenchyma in correlation with the duration of the disease].

In 50 patients with fibrosing alveolitis the content of lymphocytes and alveolar macrophages was semiquantitatively estimated and correlated to the duration of the disease. Increasing duration of the disease was correlated with a decrease of the content of lymphocytes and macrophages. The findings demonstrate close correlations between the duration of the disease and the infiltration of lung tissue with macrophages and lymphocytes.

Adult↗

[Alveolitis and lung fibroses caused by drugs].

The causes for alveolitis and lung fibroses are very multifarious. In the scale of the contaminant factors the medicaments occupy only a small sector. Nevertheless, the knowledge of such a side effect is significant on account of the often unfavourable prognosis of the alveolitis. When medicaments with wellknown ability of impairment are administered (e.g. cytostatic drugs, antiarrhythmic drugs etc.) a narrow-meshed observation with suitable control parameters is to be demanded. Only the timely recognition of a side effect of a medicament in the lung structure can prevent the fatal change into a pulmonary fibrosis. In 801 alveolitis and lung fibrosis diagnosed in our clinic 10 times a medicament was established as cause.

Drug-Related Side Effects and Adverse Reactions↗

A controlled and blinded study of immunoreactant deposition at the dermal-epidermal junction of patients with primary fibrositis syndrome.

The prevalence of immunoreactant deposition at the dermal-epidermal junction (DEJ) in clinically normal, sun exposed skin was assessed by direct immunofluorescence (IF) in 36 patients with primary fibrositis syndrome (PFS) and 12 clinically healthy control subjects. A granular or homogeneous deposition of IgG was seen at the DEJ in 52.8% (19/36) of patients with PFS and in 16.7% (2/12) of controls. The difference in IgG deposition between patients with PFS and controls was statistically significant (p = 0.030). Patients with PFS and controls did not demonstrate significant IF staining for IgA, IgM, or C3. Albumin deposition at the DEJ appeared to parallel IgG deposition in patients with PFS and control subjects who were positive for this immunoglobulin. No relationship was noted between IgG deposition and demographic or clinical characteristics of these patients' disease. These data suggest that IgG and albumin deposition at the DEJ is frequent in PFS and that further study of this skin phenomenon is warranted.

Adult↗

Cryptogenic fibrosing alveolitis in children.

Cryptogenic fibrosing alveolitis (CFA) in children is supposedly a rare diffuse lung disease characterized clinically by tachypnoea, cough, poor weight gain, and cyanosis. Histologically, it is characterized by thickening of alveolar walls and infiltration of the alveolar septae with mononuclear cells and fibrous tissue. The condition is more acute in children, with fulminant course. Ten children were diagnosed as having CFA out of three hundred children seen over a period of 3 years. Diagnosis was established by characteristic clinical findings and laboratory evaluation. All the children had tachypnoea at rest and bilateral crepitations in the absence of fever. Diffuse bilateral persistent irregular shadows were noted on the chest X-ray of these children. Pulmonary function studies revealed a restrictive type of abnormality with low transfer factor for carbon monoxide in six children. Open lung biopsy was done in eight children. The biopsy material was graded according to nine morphologic variables. Administration of steroids early in the course of illness resulted in amelioration of symptoms in 60% of the patients.

Adolescent↗

[Primary fibrosing cholangitis (author's transl)].

Primary fibrosing cholangitis is a rare and grave disorder. It consists of an inflammatory stenosis, localised or extensive, of the extra or intra-hepatic system, with intramural fibrous thickening, leaving the mucosa and the serosa undamaged. It has many similar features to primary biliary cirrhosis, and would seem to be the same response of the biliary system to an agression which remains of unknown aetiology. The resection of localised stenoses, bilio-digestive by-pass procedures or external drainage of the common duct can only delay progression towards fatal cholangitis.

Bile Ducts, Intrahepatic↗

Expression of class II MHC antigens on alveolar epithelium in fibrosing alveolitis.

Lung biopsy specimens from eight patients with fibrosing alveolitis with or without connective tissue disease were investigated for the presence of immune deposits and the expression of class II histocompatibility antigens on the alveolar epithelium. Immune deposits were not detected. The alveolar epithelium stained strongly with anti-Ia (HLA-DR) monoclonal antibodies in seven out of eight biopsy specimens from the patients, but not in control lung tissue. Sub- and intra-epithelially localized mononuclear cell infiltrates consisted predominantly of T8-positive (cytotoxic/suppressor) lymphocytes. The expression of class II antigens on the alveolar epithelium and its infiltration with T8 positive cells is consistent with local presentation of self-antigens and an ensuing local immune response.

Adult↗

Common bile duct obstruction secondary to chronic fibrosing pancreatitis: treatment by use of cholecystoduodenostomy in the dog.

Six small to medium-sized, middle-aged, female dogs with histories of acute pancreatitis developed clinical signs of extrahepatic biliary obstruction. Clinical findings were similar in the 6 dogs and included icterus. Serum biochemical analyses indicated high concentrations of total bilirubin and cholesterol and high alkaline phosphatase and alanine transaminase activities. Exploratory abdominal surgery was performed in each dog. Each dog had a firm mass involving the body of the pancreas, with obstruction of the distal portion of the common bile duct, marked peripancreatic inflammation, and omental adhesions. Cholecystoduodenostomy, using an open mucosal appositional technique for biliary redirection, was performed in each dog. Clinically, results of surgery were good to excellent (ie, lack of postoperative icterus, anorexia, lethargy, or weight loss and absence or infrequency of vomiting). The mean postoperative evaluation period for the 6 dogs was 35 months (range, 20 to 48 months); 5 dogs were alive and healthy at the end of the study. Histologic examination of tissue specimens of the pancreatic mass indicated chronic active fibrosing pancreatitis in the 6 dogs.

Animals↗

[Early detection of fibrosing alveolitis in collagenoses].

Lung function tests during a five-year follow-up were performed on 29 patients with various collagen diseases. At the beginning of the study none of the patients had signs of any pulmonary dysfunction. The onset of fibrosing alveolitis was accompanied by a marked reduction of the diffusing capacity for CO and a more minor alteration of the static specific compliance. The vital capacity and the pO2 at rest changed only in cases of advanced pulmonary fibrosis.

Airway Resistance↗

[Retrospective study of 50 cases of idiopathic fibrosing alveolitis with catamnesis].

44 out of 50 patients with idiopathic fibrosing alveolitis were followed up from 1 month to 11 years (mean 2 years 9 months). One patient out of 13 (8%) with 4 symptoms or fewer (mean follow-up 55 months) died. 23 out of 31 (74%) with 5 symptoms or more (mean follow-up 24.3 months) died, and 68% of fatalities occurred within 2 years. Lung function tests were performed in 47 cases and histological diagnosis in 38. Correlations between symptoms, lung function, and roentgenological and histological patterns are discussed. Two circumstances dramatically increased lethality: occurrence of 5 symptoms or more in a period of less than 2 years.

Adult↗