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Occupational dust exposure and the aetiology of cryptogenic fibrosing alveolitis.

Cryptogenic fibrosing alveolitis (CFA), synonymous with idiopathic pulmonary fibrosis, is probably a disease entity of increasing frequency. Epidemiological investigations into the aetiology of CFA are at an early age; yet there are several indications that exposures associated with traditionally male manufacturing occupations may be a cause of CFA. Specifically, metal exposure, and to a lesser extent wood dust exposure, was increased in patients with CFA and may explain some cases of CFA. Indeed, the four case-control studies in the literature, to date, have all found an identical occupational exposure to metal or working with metal as a risk factor for CFA and there is now further evidence confirming this association from a case-control study nested within an occupational cohort. There is also evidence from three of the case-control studies that working with wood may be a risk factor for CFA. However, these exposures clearly only explain a minority of cases of cryptogenic fibrosing alveolitis. Further studies amongst different populations with different occupational demographics are required to fully assess the impact of several exposures on the occurrence of cryptogenic fibrosing alveolitis.

Air Pollutants, Occupational↗

Cryptogenic fibrosing alveolitis/idiopathic pulmonary fibrosis.

Cryptogenic fibrosing alveolitis (CFA), synonymous with idiopathic pulmonary fibrosis (IPF), remains a life-threatening disease: 50% of patients die within 5 yrs. Historically, many diseases that are now considered to be quite distinct have been "labelled" as CFA. More recently, high-resolution computed tomography and new appreciation of the histopathological patterns of idiopathic interstitial pneumonias have enabled disease variants to be defined according to their different responses to therapy and survival. CFA is believed to be induced by an external agent, although it is not clear whether CFA represents the final common outcome of numerous pathogenetic mechanisms or has a single cause. In addition, there are currently no prospective double-blind, placebo-controlled trials of treatment showing superiority of one drug regimen over another. This review attempts to dissect the different patterns of cryptogenic fibrosing alveolitis, illustrate the major features of each, and refine the clinico-radiological-pathological descriptors that together define cryptogenic fibrosing alveolitis as it is understood today.

Humans↗

[Postmenopausal frontal fibrosing alopecia: 20 cases].

INTRODUCTION: Postmenopausal frontal fibrosing alopecia is a scarring alopecia that has been recently described by Kossard in 1994 and rarely reported in the literature. OBSERVATIONS: We have conducted a retrospective clinical and histological study of a series of 20 cases of postmenopausal frontal fibrosing alopecia. The results, in agreement with those in the literature, revealed the stereotyped nature of this entity. DISCUSSION: All the clinical and histological data suggest that postmenopausal frontal fibrosing alopecia is a clinical form of lichen planopilaris occurring in elderly women. As with lichen planopilaris its physiopathology is unknown. However, in analogy with other lichen-like dermatoses, a lymphocyte T-mediated autoimmune reaction appears to play a predominant part in its genesis.

Age Factors↗

Endobronchial findings of fibrosing mediastinitis.

Fibrosing mediastinitis is underdiagnosed because of the nonspecific character of the presenting symptoms. The endobronchial findings obtained via flexible bronchoscopy are not defined in the literature. We describe 3 cases of fibrosing mediastinitis, most likely caused by histoplasmosis. All 3 patients presented with hemoptysis and were found to have tracheobronchial concentric narrowing, severe hyperemia, and mucosal edema. The hyperemic blood vessels were treated with neodymium yttrium-aluminum-garnet (Nd:YAG) laser and argon plasma coagulation. We believe that recognition of specific endobronchial findings aids in prompt diagnosis of fibrosing mediastinitis.

Adult↗

A case of nephrogenic fibrosing dermopathy.

INTRODUCTION: Nephrogenic fibrosing dermopathy is a recently recognised fibrosing disorder originally identified as a scleromyxoedema-like cutaneous disease in patients with renal disease. CLINICAL PICTURE: A 45-year-old lady with systemic lupus erythematosus presented with well-defined erythematous, non-tender, indurated plaques on both legs 4 months after haemodialysis for rapidly progressive glomerulonephritis and acute renal failure. Skin biopsy showed dermal fibrosis with increased proliferation of dermal fibroblasts and collagen bundles separated by clefts. There were increased dermal deposits of mucin and an increase in elastic fibres. TREATMENT AND OUTCOME: Haemodialysis was instituted for 2 weeks followed by monthly intravenous cyclophosphamide. Skin lesions remained unchanged 8 months later despite normalisation of renal function. CONCLUSION: Nephrogenic fibrosing dermopathy appears to be a definite new entity in patients with underlying renal insufficiency. Further multi-centre collaborative study is necessary to identify the prevalence, cause, treatment and prognosis of this disorder.

Acute Kidney Injury↗

[TNF blockade in rheumatoid arthritis can cause severe fibrosing alveolitis. Six case reports].

TNF-blockade has been increasingly used in the treatment of rheumatoid arthritis (RA). However, the safety is unclear and an increased risk of both tuberculosis and other infections has been identified. Recently severe fibrosing alveolitis has also been reported in RA-patients treated with TNF-blockade. We report a further six RA patients, who during treatment with infliximab or etanercept developed fulminant lung fibrosis with alveolitis. For four of the patients, the fibrosing alveolitis was fatal. All patients were RF positive and above 60 years and five had mild fibrosis associated with RA before TNF-blockade treatment. Duration of TNF-blockade treatment was for three patients only two months and for the other three, 20-51 months. Age above 60 years and previous lung fibrosis appear to be risk factors for developing fibrosing alveolitis in RA patients treated with TNF-blockade.

Age Factors↗

A new surgical technique for fibrosed interventricular septum.

We describe a new surgical technique for the treatment of fibrosed interventricular septum, with or without left ventricular aneurysm. It is designed for patients whose ventriculograms revealed important septal dysfunction. Eleven patients ranging from 37 to 66 years of age were operated upon between 1984 and 1988. The left ventricle was opened through the aneurysm or the anterior wall when only anterior septal fibrosis was present. In patients with large aneurysms, two purse-string sutures were placed on the inside surface of the intact ventricle, around its limits, for approximation. The fibrosed septum was thus excluded from the new ventricular cavity. A patch was placed between the fibrosed and the healthy septum, reaching to the ventricular wall, all around the transitional edge. Both, ventricular geometry and function were improved. All patients were asymptomatic after one year follow-up.

Adult↗

Hypothesis of classification of pulmonary fibroses.

A new framework for pulmonary fibroses may be obtained by distinguishing the "true" forms (solely collagen hyperplasia) from the "false" forms or mesenchymal fibril lung diseases (collagen, reticular and elastic hyperplasia with neo-angiogenesis): the "true fibroses" are then divisible into those not causing architectural subversion of the lung and those which bring it about. Pulmonary architectural subversion is common to true granulomatous fibroses and mesenchymal fibril lung diseases: it is, in our opinion, the product of a combination of factors, including the hyperactivity of a fibroblast sub-population normally in the minority, stimulated by T lymphocytes which are activated by an autoimmune response to type I collagen produced in excess.

Autoimmune Diseases↗

[Diffuse fibrosing alveolitis and primary biliary cirrhosis].

Diffuse fibrosing alveolitis (DFA) is a disease with progressive, diffuse fibrosis in the interstitial tissue of the pulmonary parenchyma. As a rule, diffuse fibrosing alveolitis is limited to the lungs alone but it may occur as a partial manifestation of a systemic disease. A case report is presented here where diffuse fibrosing alveolitis was encountered in a patient with primary biliary cirrhosis. The pulmonary symptoms dominated the clinical picture and investigation revealed the autoimmune condition.

Autoimmune Diseases↗

[Evaluation of the functional state of the alveoli and capillary network of the lungs in patients with fibrosing alveolitis].

Regional blood flow and ventilation of the lungs in 48 patients with fibrosing alveolitis, including 19 with idiopathic fibrosing one and 29 with extrinsic allergic one, were investigated. A comprehensive radionuclide evaluation supplemented by a clinicoroentgenologic and functional studies mutually enhances the diagnostic potentialities for fibrosing alveolitis of different genes.

Adolescent↗

The plasma endorphin, prostaglandin and catecholamine profile of patients with fibrositis treated with cyclobenzaprine and placebo: a 5-month study.

During a 5 month, double blind crossover study of the clinical effect of cyclobenzaprine on 7 patients with fibrositis, weekly measurements were done of plasma beta-endorphin (endorphin, prostaglandin E (PGE) and catecholamines). Endorphin levels were normal but varied with tender point tenderness. Mean plasma dopamine and PGE were elevated. Norepinephrine was normal to very high while epinephrine levels were continuously low to normal. We conclude that patients with fibrositis have a neurotransmitter plasma profile like other chronic pain states having stress and increased vasomotor activity with the possible exception of having low circulating epinephrine. This disparity may mark a failure of central nervous system pain modulation in fibrositis.

Adult↗

[Effect of glucocorticosteroid therapy on immunologic indicators in patients with sarcoidosis and idiopathic fibrosing alveolitis].

Immunological findings were recorded in 22 patients with active sarcoidosis and 12 patients with idiopathic fibrosing alveolitis. In addition, the patients' endopulmonary cytograms were considered. Immunological evidence involved the use of bronchoalveolar lavage, bronchoscopy, peripheral blood. Administration of corticosteroid therapy resulted in alveolitis attenuation both in sarcoidosis and fibrosing alveolitis. Common features of immune response of sarcoidosis and idiopathic fibrosing alveolitis patients to glucocorticosteroids whose action proceeds as multicomponent immunomodulating effect implying reconstruction of some links of the immune system while aggravating dysfunction of the others are stated.

Chronic Disease↗

Gallium-67 scanning in the staging of cryptogenetic fibrosing alveolitis and hypersensitivity pneumonitis.

Gallium-67 citrate is known to localize within inflammatory sites. Gallium-67 scanning is used for the evaluation of lung inflammation (i.e. alveolitis) during interstitial lung diseases. We investigated 27 patients with cryptogenetic fibrosing alveolitis (n = 17) and hypersensitivity pneumonitis (n = 10) using gallium-67 lung scanning and lung function tests (forced vital capacity, diffusing capacity, resting and exercise blood gases). Investigations were performed before and after one year of methylprednisolone treatment. None of eight healthy volunteers had any abnormal gallium-67 uptake. In all patients with cryptogenetic fibrosing alveolitis an initial abnormal gallium-67 uptake was observed (mean fixation index: 163 +/- 18). In addition, analysis of lung function tests a year after initial evaluation showed that unchanged or improving patients presented initially with a lower gallium-67 index than patients with evidence of deterioration (153.9 +/- 23.7 vs 251.0 +/- 23.3.; p less than 0.01). Similarly, among patients with hypersensitivity pneumonitis the index was lower in unchanged or improving patients than in those with deterioration (74.9 +/- 22 vs 226.7 +/- 4.9; p less than 0.05). Thus gallium-67 scanning is useful in the management of cryptogenetic fibrosing alveolitis and hypersensitivity pneumonitis.

Alveolitis, Extrinsic Allergic↗

Increased neurogenic inflammation in fibrositis syndrome.

Mechanically induced vasodilatation or flare on the skin, known as dermatographia, is a common clinical observation in fibrositis syndrome and is thought to be a neurogenically mediated axon reflex response. In our study, mechanically and chemically induced flares were quantitated in 13 patients with fibrositis syndrome and 14 control subjects. There was a reduced threshold for chemically induced flare response and the area of flare was greater in patients compared to controls, although there was a wide range of responses in both groups. There was also a significant positive correlation between mechanically and chemically induced flares, and the number of tender points in all subjects correlated with the size of the chemically induced flare. We suggest that exaggerated neurogenic inflammatory responses in patients with fibrositis syndrome reflect increased activity of polymodal nociceptors of unmyelinated primary afferent nerves. This increased receptor activity may also contribute to the pain and tenderness experienced by these patients.

Adolescent↗

Histocompatability antigens in the fibrositis (fibromyalgia) syndrome.

HLA antigen Class I (A, B, C) and II (DR) were determined in a small group of fibrositis (fibromyalgia) patients and normal controls. Sixty-seven percent of fibrositis patients had DR4 versus 30% of normal controls. There was also an increased relative risk (4.5). No statitical significance of other Class I and II antigens in fibrositis was found.

Adult↗

Fibrositis: misnomer for a common rheumatic disorder.

Fibrositis is a misnomer for a very common form of nonarticular rheumatism. The name implies an inflammatory process in fibroconnective tissue which has never been verified. The symptoms of fibrositis are ill-defined musculoskeletal pain made worse by stress, cold, noise and unaccustomed exercise; there is usually a significant element of depression, nonrestorative sleep, chronic fatigue and early morning stiffness. Results of physical examination are strikingly normal, apart from painful tender spots which are remarkably consistent in location from patient to patient. It is important to realize that fibrositis can complicate diseases such as rheumatoid arthritis and systemic lupus erythematosus, where its prompt recognition is essential in averting inappropriate medication. Drug therapy alone is seldom effective in alleviating symptoms; a carefully planned education program is necessary to readjust both psyche and soma.

Diagnosis, Differential↗

[Fibrosing adenosis (eleven cases reported) (author's transl)].

The authors studied eleven cases of Fibrosing Adenosis of the breast. If the clinical findings and radiologic examination evoke this diagnosis only a histological proof is sure. The difficulties in histological diagnosis, the problems of the histogenesis of fibrosing adenosis are emphasized. The authors recall the excellent prognosis of fibrosing adenosis.

Adult↗

[Cryptogenic fibrosing alveolitis combined with immune haemolytic anaemia or bronchial asthma].

This study deals with the rare occurrence of cryptogenic fibrosing alveolitis together with adventitious immune haemolytic anaemia respectively bronchial asthma. In literature the above mentioned first combination form is the second, and histologically the first case being proved. The author has not met references to the simultaneous incidence of cryptogenic fibrosing alveolitis and endogenic bronchial asthma. In spite of this he believes that the described associations may be more frequent, but can be hardly diagnosed, because the symptoms of connective tissue diseases can merge one into another and seldom develop discernibly. The separation of diseases, having common immunopathological origin and associating one with another, but at the same time being not identified one with another, is the condition of the adequate and up-to-date therapy. In the premised combinations of diseases both simultaneous syntropy and successive syntropy are probable. The histologically confinable distinct forms of cryptogenic fibrosing alveolitis are noticeable when they occur together with other immunopathological diseases.

Adolescent↗