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PubMed1994

The radiographic findings of fibrosing mediastinitis.

We retrospectively reviewed the radiographic findings of fibrosing mediastinitis (FM) in 33 patients. Imaging studies included chest radiographs, computed tomographic scans, magnetic resonance imaging examinations, esophograms, ventilation perfusion scans, angiograms, and venograms. Findings include bronchial narrowing in 11 patients (33 percent), pulmonary artery obstruction/narrowing in 6 patients (18 percent), esophageal narrowing in 3 patients (9 percent), and superior vena cava obstruction/narrowing in 13 patients (39 percent). Two distinctly different radiographic patterns were identified: a localized pattern seen in 27 patients (82 percent) that frequently contained calcification and a diffuse pattern seen in 6 patients (18 percent) that did not contain calcification. The localized pattern is most likely due to histoplasmosis and does not show radiographic evidence of improvement with steroid therapy. The diffuse pattern may more likely be truly idiopathic or of a noninfectious etiology. Several patients with the diffuse pattern showed radiographic evidence of improvement with steroid therapy.

Adolescent↗
PubMed1995

Familial cryptogenic fibrosing pleuritis with Fanconi's syndrome (renal tubular acidosis). A new syndrome.

We describe two siblings with a progressive unrelenting and unique syndrome of bilateral fibrosing pleuritis of unknown cause occurring in association with Fanconi's syndrome (renal tubular acidosis). The parents of the siblings were second cousins. Both siblings had identical pleural histologic characteristics and identical urinary metabolic defects. This condition resulted in the development of severe respiratory failure in both patients and ultimately the death of the older sibling at the age of 21 years.

Acidosis, Renal Tubular↗
PubMed2001

Occupational distribution and geographic clustering of deaths certified to be cryptogenic fibrosing alveolitis in england and wales.

STUDY OBJECTIVES: The etiology of cryptogenic fibrosing alveolitis (CFA) remains largely obscure, although a 1996 report suggested an increased risk from occupational exposure to metal and wood dusts. Using data from death certificates in England and Wales, we sought evidence of any relationship between occupation and CFA and of the extent of any temporospatial clustering of place of birth and place of death as possible evidence of a geographically related environmental factor. DESIGN AND SETTING: Data on occupation and address (postal code) were obtained from certificates of men and women dying as a result of CFA between 1981 and 1990 and were compared with national mortality statistics. Place of birth data were extracted from certificates for deaths between 1993 and 1995, the only available years, and were compared with national birth statistics. MEASUREMENTS AND RESULTS: Standardized mortality ratios (SMRs) were raised (p<0.05) in the following four occupational groups: members of the armed forces (SMR, 217.8); miners and quarrymen (SMR, 142.0); service, sports, and recreation workers (SMR, 118.6); and electrical and electronic workers (SMR, 146.6). Of these four groups, the latter group might be worth testing in a future study. There was statistical evidence of geographic clustering in postal code sectors for the recorded place of death, but the high-rate areas were different in men and women. Deaths were increased for those subjects born in urban areas, although these did not follow a clear geographic pattern. CONCLUSIONS: Overall, these analyses provide little evidence of any important contribution from environmental factors to the etiology of CFA and suggest that more consideration be given to alternative concepts of causation.

Adolescent↗
PubMed1975

Cryptogenic fibrosing alveolitis.

Clinical, radiographic, and histologic data, together with pulmonary function features, in eleven patients with cryptogenic fibrosing alveolitis, are described in the following study. All these patients presented with a history of exertional dyspnea. Eight of the eleven had radiographic evidence of pleural involvement as manifested by blunting of one or both costophrenic angles. A restrictive defect was present in eight patients, all of whom had x-ray changes showing diffuse involvement. One of the patients presented showed marked improvement in all parameters including a repeat lung biopsy. Immunofluorescent studies of the lung biopsies from six patients revealed no evidence of immunologic damage. Based upon the histologic pattern of pulmonary reaction in the biopsies and the intervals between the onset of symptoms and the time of biopsy, a tenative scheme of temporal evolution of this disease is presented. Lung injury may lead either directly to diffuse interstitial pulmonary fibrosis (DIPF) or lungs may react to injury by an initial alveolar, mural or mixed pattern, all of which might ultimately progress to DIPF. An initial alveolar or mural pattern might change to a mixed pattern.

Adult↗
PubMed1976

Diffuse interstitial fibrosing pneumonitis and adenovirus infection.

With electron microscopy, adenovirus particles, which were almost round, variable in density, and 60-80 mmu in diameter, were observed in the nuclei of type II alveolar epithelial cells, infiltrated plasma cells and alveolar macrophages of a man with chronic interstitial fibrosing pneumonitis. The immunofluorescent technique also suggested adenovirus infection.

Adenoviridae Infections↗
PubMed1976

Another presentation of fibrosing alveolitis and alveolar cell carcinoma.

A 73-year-old woman had dyspnea associated with gradually increasing pulmonary collapse. Despite intensive investigation, the diagnosis was only reached at autopsy 18 months later, when histologic examination of the lungs showed changes of both fibrosing alveolitis and alveolar cell carcinoma.

Adenocarcinoma, Bronchiolo-Alveolar↗
PubMed1977

Desquamative interstitial pneumonitis. Cellular phase of fibrosing alveolitis.

Since the original description of desquamative interstitial pneumonitis in 1967, several investigators have questioned the specificity of the disease. The concept of desquamative interstitial pneumonitis and usual fibrosing interstitial pneumonitis as two manifestations of a nonspecific disease spectrum has been proposed. Twenty-six patients with desquamative interstitial pneumonitis were evaluated with respect to clinicopathologic correlates. Biopsies were reviewed without knowledge of the patients' clinical course. The absolute histopathologic criteria for the diagnosis were (1) intra-alveolar accumulations of free alveolar cells with PAS-positive diastaseresistant cytoplasmic granules; (2) mononuclear cell interstitial inflammation; and (3) absence of necrosis, hyaline membranes, intra-alveolar fibrosis, asbestos bodies, and birefringent crystalline dust material. Transmission electron microscopic studies revealed the dominant alveolar lining cell to be the granular pneumocyte, with a prominent population of free alveolar macrophages. Patients were segregated into group 1 (cellular phase) and 2 (cicatrized phase). Although favorable short-term responses to corticosteroid therapy were observed in both groups, long-term responses were variable. Apparent complete remissions were noted in both groups.

Adult↗
PubMed1980

Pulmonary infarct: an unusual manifestation of fibrosing mediastinitis.

We have recently seen lung biopsy specimens showing pulmonary infarcts in two patients with fibrosing mediastinitis. The patients were young, and in each, the infarcts were the first manifestation of the underlying mediastinal fibrosis. The infarcts had several distinctive histologic features and may have been caused by constriction of major pulmonary veins by the fibrosis.

Adult↗
PubMed1982

Fibrosing alveolitis, bronchiolitis obliterans, and sulfasalazine therapy.

A patient with ulcerative colitis had extensive upper zone pulmonary disease while taking sulfasalazine. Pulmonary function tests showed progressive restrictive and obstructive disease. Lung biopsy showed bronchiolitis obliterans and chronic interstitial pneumonia or fibrosing alveolitis with a mild eosinophilic infiltrate. The patient improved after receiving steroid therapy. A review of the literature of lung disease related to ulcerative colitis and sulfasalazine is presented.

Aged↗
PubMed1986

Magnetic resonance imaging of spiral vein graft bypass of superior vena cava in fibrosing mediastinitis.

Magnetic resonance imaging (MRI) was used for postoperative evaluation of spiral vein grafts in three patients with fibrosing mediastinitis who had undergone bypass of the superior vena cava (SVC) for SVC syndrome. The MRI images, obtained without ECG triggering, were compared retrospectively with postoperative bilateral arm venograms. Patent grafts were identified by MRI in all patients. Significant stenoses at innominate vein-spiral vein graft anastomoses in two patients were better demonstrated by venography than by MRI. MRI shows promise as a non-invasive technique for postoperative evaluation of spiral vein bypass grafts.

Adult↗
PubMed1989

Changes in phosphatidylglycerol in bronchoalveolar lavage fluids from patients with cryptogenic fibrosing alveolitis.

In view of recent evidence that changes in phospholipid components of pulmonary surfactant occur in bleomycin-induced pulmonary fibrosis, the aim of this study was to examine whether similar changes occur in patients with idiopathic pulmonary fibrosis. We have analyzed phospholipid profiles in bronchoalveolar lavage (BAL) fluids from a group of 32 patients with "lone" cryptogenic fibrosing alveolitis (CFA) compared with 17 control subjects. Prior to treatment, resembling the findings in bleomycin fibrosis, many CFA patients had decreased proportions of phosphatidylglycerol (PG) (percent of total phospholipid; median 10.5, range 0 to 35.1 compared with 18.2, range 9.6 to 24.2 for the control group of similar age range and smoking habits; p less than 0.01). Following the initial lavage, the patients were treated with prednisolone. The initial percentage of PG levels did not predict response to prednisolone, but serial lavage studies conducted in 14 patients suggest that an early and sustained increase in percentage of PG following commencement of prednisolone is associated with clinical improvement. The reasons for the proportional reductions in phosphatidylglycerol, and whether they result in functional deficiency of surfactant in CFA, need to be explored.

Adult↗
PubMed1991

Crackles in patients with fibrosing alveolitis, bronchiectasis, COPD, and heart failure.

We have studied the crackling lung sounds of ten patients with cryptogenic fibrosing alveolitis, ten with bronchiectasis, ten with chronic obstructive pulmonary disease, and ten with heart failure by analyzing frequency, waveform, and timing of crackles. The upper frequency limit of inspiratory sounds was higher in CFA than in COPD or in HF. The period of crackling was shorter in COPD than in CFA or BE. Inspiratory crackling terminated significantly earlier in COPD than in CFA, BE, or HF. The initial deflection width and the two-cycle duration of the expanded waveforms of crackles were smaller in CFA than in BE, COPD, or HF. The largest deflection width was smaller in CFA than in BE, HF, or COPD and smaller in BE than in HF. The results indicate that crackling lung sounds in different diseases have distinctive features and that their analysis can be of diagnostic value.

Bronchiectasis↗
PubMed1991

Polysomnography in idiopathic muscle pain syndrome (fibrositis).

Muscle pain occurs in various neuromuscular disorders with characteristic physiological or biochemical abnormalities. There is, however, a group of patients in whom there is no clear physiological or structural basis for their pains. This syndrome has been called fibrositis or fibromyalgia. Sleep abnormalities have been reported in some of these patients, but have not been confirmed by others. We studied 8 patients with this disorder and found sleep abnormalities that were characterized by nocturnal myoclonus, alpha-delta sleep, and abnormalities compatible with depression. Polysomnography was, therefore, instrumental in helping direct the treatment of these patients. Therapeutic approaches aimed to correct the specific disorders were effective in improving the pain symptoms.

Alprazolam↗
PubMed1989

Disorganizing-fibrosing processes in alveolar walls of interstitial pneumonia, "alveolopneumonitis": a morphopathological study.

Disorganizing-fibrosing processes of alveolar walls in 13 autopsied (including 4 previously biopsied) and 3 biopsied cases of acute and chronic interstitial pneumonia were presented. The processes of alveolar walls were characterized initially by histolysis of the alveolar walls and transformation of the tissue into mesh or reticular structure caused by proliferation of fixed cells probably of endothelial cell origin, subsequently by formation of basement membrane-like structures along the proliferated cells, and ultimately by either fibrosis of the entire thickness of the disorganized tissue or axial fibrosis with accumulation of proliferated cells towards axis and peripheral recanalization. These processes tended to occur in the subpleural regions and extend thereafter to the deeper portions of lungs. In some cases recurrent alveolitis which occurred on the basis of axial fibrosis as aforementioned was noted. Problems concerning genesis and nature of these processes were discussed, and a new nomenclature "alveolopneumonitis" was proposed instead of interstitial pneumonia.

Adult↗
PubMed1988

Fibrositis (fibromyalgia). A common non-entity?

The literature on fibrositis (fibromyalgia), which originated in the early years of the last century in the UK and proliferated there in the first half of this century, has since diminished there in the last 30 years or so, but has increased in Canada and the US. Criteria suggested for diagnosis have created a syndrome with no diagnostic tests, serological or radiological signs, and no truly objective physical signs, but with predictable tender spots on pressure. The syndrome is largely, but not completely, confined to females, mostly of middle age; the symptoms include widespread aching of more than 3 months' duration, disturbed sleep, morning fatigue and stiffness, a failure to respond satisfactorily to any one form of therapy and a tendency to persist over long periods, but without permanent tissue changes. Features of psychological disturbance are present in many patients but not in all or even the majority. Definition of the condition as a disorder of pain modulation - a pain amplification syndrome - would seem to fit the facts best. Most would agree that an abnormal response to stress is an important factor in the appearance of the syndrome, as other stress related disorders, such as the irritable bowel syndrome and tension headaches, may coexist. Response to therapy, whether physical or pharmacological, is on the whole unsatisfactory. This type of patient has been well recognised in hospital clinic and general practice for many years.(ABSTRACT TRUNCATED AT 250 WORDS)

Fibromyalgia↗
PubMed2003

Superior vena cava syndrome due to fibrosing mediastinitis histologically identical to xanthogranulomatous pyelonephritis.

We present herein a case of superior vena cava (SVC) syndrome caused by localized fibrosing mediastinitis (FM), which had histological features similar to xanthogranulomatous pyelonephritis (XGP). A 63-year-old woman presented with facial swelling 5 months after undergoing right nephrectomy for XGP. Radiologic investigations of the chest confirmed the presence of SVC obstruction due to an intraluminal tumor. The histological features of the tumor were consistent with those of FM and were very similar to those of XGP. Although the pathogenesis of neither FM nor XGP is known, some pathogenic process of FM and XGP may be the same.

Diagnosis, Differential↗
PubMed1984

Meniere's disease and fibrositis syndrome (psychogenic rheumatism). Relationship in audiometric and nystagmographic results.

A prospective neuro-otological study concerning 30 cases of fibrositis syndrome (psychogenic rheumatism, PR) and 30 age-matched normal controls was made and a retrospective study concerning 33 cases of Meniere's disease (MD) diagnosed and followed-up, examined between 1965 and 1982. Results showed: sensorineural hearing loss at low frequencies in all early stages of MD and in 10/30 of PR; hyperacusis (pain threshold below 100 dBHL bilaterally for all frequencies) without other sign of recruitment in 73.3% of PR and in 3/4 cases of MD where it was measured (discomfort or vertigo due to noise was noted retrospectively in 16/33 of early stages of MD); hyperreactivity of per-rotatory nystagmus in 53.3% of PR without neurological or peripheral vestibular lesions and, with or without vestibular unilateral lesions, in 39.9% of MD. None of the controls showed hyperacusis, hyperreactivity of per-rotatory nystagmus or deafness at low frequencies.

Adult↗