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At least 739 records · Page 41Linked to original sources
PubMed1987

Esophageal radiography and manometry: correlation in 172 patients with dysphagia.

Radiographic and manometric examinations of the esophagus were correlated in 172 patients with dysphagia. Esophageal manometry was abnormal in 66 (38%), with diagnoses of nonspecific esophageal motor disorder (26), achalasia (19), nutcracker esophagus (12), diffuse esophageal spasm (seven), and scleroderma (two). Compared with manometry, radiographic sensitivities were 95% (18 of 19) for achalasia, 71% (five of seven) for diffuse esophageal spasm, and 46% (12 of 26) for nonspecific esophageal motor disorder. Nutcracker esophagus was not diagnosed specifically on the radiographic examination. Overall radiographic sensitivity was 56% (37 of 66) but increased to 89% by excluding nutcracker esophagus and nonspecific esophageal motor disorders. In 106 manometrically normal patients, radiographic specificity was 91% with 10 false-positive diagnoses of nonspecific motor disorder. We conclude that radiographic examination of the esophagus is useful in patients with dysphagia for evaluating functional esophageal abnormalities, although detection rates vary widely with the type of motor disorder.

Deglutition Disorders↗
PubMed2001

Phase I/II trial of autologous stem cell transplantation in systemic sclerosis: procedure related mortality and impact on skin disease.

BACKGROUND: Systemic sclerosis (SSc, scleroderma) in either its diffuse or limited skin forms has a high mortality when vital organs are affected. No treatment has been shown to influence the outcome or significantly affect the skin score, though many forms of immunosuppression have been tried. Recent developments in haemopoietic stem cell transplantation (HSCT) have allowed the application of profound immunosuppression followed by HSCT, or rescue, to autoimmune diseases such as SSc. METHODS: Results for 41 patients included in continuing multicentre open phase I/II studies using HSCT in the treatment of poor prognosis SSc are reported. Thirty seven patients had a predominantly diffuse skin form of the disease and four the limited form, with some clinical overlap. Median age was 41 years with a 5:1 female to male ratio. The skin score was >50% of maximum in 20/33 (61%) patients, with some lung disease attributable to SSc in 28/37 (76%), the forced vital capacity being <70% of the predicted value in 18/36 (50%). Pulmonary hypertension was described in 7/37 (19%) patients and renal disease in 5/37 (14%). The Scl-70 antibody was positive in 18/32 (56%) and the anticentromere antibody in 10% of evaluable patients. Peripheral blood stem cell mobilisation was performed with cyclophosphamide or granulocyte colony stimulating factor, alone or in combination. Thirty eight patients had ex vivo CD34 stem cell selection, with additional T cell depletion in seven. Seven conditioning regimens were used, but six of these used haemoimmunoablative doses of cyclophosphamide +/- anti-thymocyte globulin +/- total body irradiation. The median duration of follow up was 12 months (3-55). RESULTS: An improvement in skin score of >25% after transplantation occurred in 20/29 (69%) evaluable patients, and deterioration in 2/29 (7%). Lung function did not change significantly after transplantation. One of five renal cases deteriorated but with no new occurrences of renal disease after HSCT, and the pulmonary hypertension did not progress in the evaluable cases. Disease progression was seen in 7/37 (19%) patients after HSCT with a median period of 67 (range 49-255) days. Eleven (27%) patients had died at census and seven (17%) deaths were considered to be related to the procedure (direct organ toxicity in four, haemorrhage in two, and infection/neutropenic fever in one). The cumulative probability of survival at one year was 73% (95% CI 58 to 88) by Kaplan-Meier analysis. CONCLUSION: Despite a higher procedure related mortality rate from HSCT in SSc compared with patients with breast cancer and non-Hodgkin's lymphoma, the marked impact on skin score, a surrogate marker of mortality, the trend towards stabilisation of lung involvement, and lack of other treatment alternatives justify further carefully designed studies. If future trials incorporate inclusion and exclusion criteria based on this preliminary experience, the predicted procedure related mortality should be around 10%.

Adolescent↗
PubMed1982

Serial pulmonary function in systemic sclerosis.

The natural history of the pulmonary involvement in systemic sclerosis is not well studied. Reported here are the serial measurements of pulmonary function in a well defined population of patients with systemic sclerosis followed over a mean of 63 months. The mean rate of loss of vital capacity (0.10 liters per year) for the 38 patients serially studied was more than three times the expected rate of loss for a normal population. The mean rate of loss of diffusing capacity (0.33 ml/min/mm Hg per year) for the 27 patients serially studied was similar to that for a normal population. The percent forced vital capacity expired in the first second increased 0.53 percent per year consistent with a progressive restrictive ventilatory defect. Smokers tended to lose vital capacity at a slightly greater rate than nonsmokers (p = 0.069). Individual variability in the course of pulmonary function was observed. Although the overall trend in our population was towards a slowly progressive restrictive ventilatory defect, pulmonary function in the nonsmokers did not change at rates different from those in a nonsmoking reference population.

Adolescent↗
PubMed1984

Carbon monoxide diffusing capacity as predictor of outcome in systemic sclerosis.

In order to determine the predictive value of lung function studies for subsequent prognosis in systemic sclerosis, 71 patients with systemic sclerosis were followed up for a mean of five years after pulmonary function testing. A carbon monoxide diffusing capacity less than or equal to 40 percent of the predicted reference value was associated with only a 9 percent five-year cumulative survival rate compared with a 75 percent cumulative five-year survival in patients with a carbon monoxide diffusing capacity greater than 40 percent of predicted. An obstructive ventilatory defect was also associated with increased mortality, and all six patients with obstruction and a diffusing capacity less than 70 percent of the predicted died during the study period. Male gender, independent of abnormalities of pulmonary function, was also associated with a poor prognosis. Although it is not clear whether a severely impaired diffusing capacity is indicative of interstitial pulmonary fibrosis or pulmonary vasculopathy or is a marker of generalized vascular disease, a severely depressed carbon monoxide diffusing capacity is an important predictor of mortality in patients with systemic sclerosis.

Actuarial Analysis↗
PubMed1990

Cutaneous manifestations of the L-tryptophan-associated eosinophilia-myalgia syndrome: a spectrum of sclerodermatous skin disease.

The natural history of the clinical and pathologic features of skin disease was reviewed prospectively in 30 patients with the L-tryptophan-associated eosinophilia-myalgia syndrome. Overall, cutaneous manifestations developed in 26 patients (87%). Early lesions were nonspecific and characterized predominantly by an erythematous macular eruption on the trunk and extremities. The most characteristic abnormality noted was the spectrum of sclerodermatous disease in 15 patients (50%) often after a subacute stage of peripheral or truncal edema. Clinical and/or biopsy evidence of eosinophilic fasciitis was seen in nine patients (30%). Findings consistent with diffuse, limited, or localized scleroderma were subsequently observed in nine patients (33%). Small mucinous papules, similar to those seen in scleromyxedema, were found in five patients (17%). Alopecia, frequently a late sequela, developed in 11 (37%). Common histologic features included papillary dermal fibrosis, dermal and fascial infiltrates consisting of mononuclear cells and eosinophils, deposition of glycosaminoglycans in the dermis, and, in some patients, numerous mast cells.

Adult↗
PubMed1980

Operation for motor dysfunction of the esophagus.

Modern operative treatment of motor dysfunction of the esophagus began in 1949 with the recognition that anastomotic procedures that bypass or destroy the distal esophageal sphincter are associated with the development of reflux esophagitis and stricture. Thirty years later, reflux esophagitis related to esophagomyotomy or intrinsic esophageal disease remains the dominant concern and challenge. This review examines the current status of operative procedures for the management of three important primary disorders of esophageal motility: achalasia, diffuse esophageal spasm, and scleroderma. Relief of esophageal obstruction by esophagomyotomy or reconstruction is the common surgical goal. The addition of a fundoplication procedure to discourage esophageal reflux remains controversial in each disorder. Esophageal resection may become necessary when stricture persists or esophagomyotomy fails to provide lasting relief of dysphagia.

Esophageal Diseases↗
PubMed1987

Esophageal motor disorders.

Primary esophageal motor disorders are rare, but they respond well to surgery if they are accurately diagnosed and if treatment carefully follows the fundamental principles of management. In the lower esophagus the most important primary disorders are achalasia, diffuse esophageal spasm, and scleroderma. Upper esophageal primary disorders are mostly rare; however, problems of age and neurologic diseases are a significant source of debility, making their management important. Details of investigation and management have been outlined in this article.

Age Factors↗
PubMed2000

Serum levels of tissue inhibitor of metalloproteinases 2 in patients with systemic sclerosis.

BACKGROUND: The serum tissue inhibitor of metalloproteinases 1 (TIMP-1) level was reported to be a useful indicator of disease activity, especially of lung fibrosis in patients with systemic sclerosis. TIMP-2 is also an important inhibitor of matrix metalloproteinases (MMPs), such as interstitial collagenase, gelatinase, and stromelysin, which control the metabolism of the extracellular matrix. However, serum levels of TIMP-2 in patients with systemic sclerosis (SSc) have not been investigated. OBJECTIVE: We sought to determine the clinical significance of serum levels of TIMP-2 and MMP-2 in patients with SSc. METHODS: Serum samples were obtained from 128 patients with SSc (68 with limited cutaneous SSc and 60 with diffuse cutaneous SSc). Twenty-seven serum samples from healthy age- and sex-matched individuals were also examined as controls. The TIMP-2 and MMP-2 levels were determined by means of sandwich enzyme-linked immunosorbent assays. RESULTS: The serum TIMP-2 levels were elevated in 29 (22.7%) of the 128 patients with SSc and were significantly higher than those of the healthy control subjects. The serum TIMP-2 levels were significantly correlated with the extent of skin sclerosis in the patients with SSc. The incidence of decreased percentage of the diffusing capacity of lung for carbon monoxide (DLCO ) and that of an elevated erythrocyte sedimentation rate were significantly greater in the patients with elevated TIMP-2 levels compared with the patients with normal TIMP-2 levels (P <.05). When these patients were classified into 2 groups by disease activity, TIMP-2 levels were significantly more elevated in the high active group than in those low active group (P <.001). The serum MMP-2 levels of the patients with SSc were not significantly higher than those of the healthy control subjects. CONCLUSION: These findings suggest that the serum TIMP-2 level is a useful marker of the extent of skin sclerosis and disease activity in patients with SSc. The balance of TIMP-2 and MMP-2 may play an important role in patients with SSc. Furthermore, TIMP-2 may be thought to contribute to the development of disease in patients with SSc.

Adolescent↗
PubMed2002

Long-term administration of beraprost, an oral prostacyclin analogue, improves pulmonary diffusion capacity in patients with systemic sclerosis.

The objective of this study was to assess the effect of beraprost sodium, an oral prostacyclin analogue, on pulmonary function in patients with systemic sclerosis. Seventeen patients, with systemic sclerosis and predicted percent values of carbon monoxide diffusion capacity (%DLCO) of less than 95, received beraprost sodium for at least 12 months. Conventional testing for pulmonary function was performed at 12-month intervals and changes were evaluated with special reference to DLCO. Twelve patients completed the treatment. Nine patients showed improvement in DLCO (12.1 +/- 2.3 to 15.5 +/- 4.4 ml/min/mmHg, P < 0.006) and 10 patients showed an increase in %DLCO (66.6 +/- 11.9 to 87.7 +/- 23.2%, P < 0.004). Total lung capacity, vital capacity and forced expiratory volume remained unchanged. This study showed that DLCO levels in patients with systemic sclerosis improved after the administration of beraprost sodium, probably due to the decrease in pulmonary vascular resistance accompanied by increased cardiac output.

Administration, Oral↗
PubMed2003

Bronchiolitis obliterans with organizing pneumonia associated with scleroderma and scleroderma spectrum diseases.

Systemic sclerosis (SSc) is an autoimmune disorder with a high mortality rate from pulmonary manifestations, such as interstitial lung disease and pulmonary hypertension, seen at autopsy in 90% and 75% of patients, respectively. Bronchiolitis obliterans with organizing pneumonia (BOOP) has numerous causes, but it has only been reported in three patients with SSc. We present three cases of biopsy-proven BOOP in patients with scleroderma spectrum disease, including diffuse cutaneous SSc, mixed connective tissue disease with SSc features, and limited cutaneous SSc that later evolved to dermatomyositis/SSc overlap. BOOP and SSc alveolitis have similar findings on spirometry, radiographs, and BAL; the definitive diagnosis of BOOP was therefore made by open lung biopsy. Cyclophosphamide is the current treatment of alveolitis associated with SSc, whereas high-dose prednisone is used to treat BOOP. As a result of this difference, we emphasize the importance of a thorough investigation of pulmonary disease in SSc patients to exclude BOOP and treat appropriately.

Journal Article↗
PubMed1998

[Change of pulmonary diffusing capacity, membrane diffusing capacity and pulmonary capillary blood volume in patients with COPD and connective tissue disease at rest and post-exercise].

OBJECTIVE: Studying the changes of pulmonary diffusing capacity (DL), alveolar capillary membrane diffusing capacity (Dm) and pulmonary capillary blood volume (Vc) in patients with COPD and connective tissue disease (CTD) at rest and post-exercise; evaluating the diagnostic values of DL, Dm and Vc in early stage of pulmonary involvement of CTD. METHOD: Spirometry was performed in the sit position. Several measurements of diffusing capacity of the lung for carbon monoxide (DLCO) were measured by single-breath method at increasing alveolar oxygen tension. The estimations of Dm and Vc were done by the method of Roughton and Forster. RESULT: (1) In COPD group, the values of DL were lower than that of the control group at rest. The decreases were due to the reduction of Dm, but Vc were normal. In CTD group, all the value of DL, Dm and Vc were lower than that of its control group at rest. (2) COPD patients had the higher DL, Dm and Vc after the end of exercise than that of at rest. But the changes of DL Dm and Vc in COPD patients were lower compared with the control group. In contrast to the control group, DL, Dm and Vc did not increase or even decreased after the end of exercise in CTD group. (3) There was no significant differences of DL, Dm and Vc between the 7 CTD patients with normal pulmonary volume and DLCO and the control subjects at rest. But after the end of exercise, DL, Dm and Vc of CTD patients kept unchanging or even decreasing. The difference between CTD patients and its control group was found significant. CONCLUSION: The measurements of post-exercise Dm and Vc are sensitive tests for pulmonary involvement in of CTD patients. The results are abmormal much earlier in subject with minimal dyspnea and roentgenologic change. They are useful tests for early stage CTD.

Aged↗
PubMed1979

Esophageal scintigraphy to quantitate esophageal transit (quantitation of esophageal transit).

None of the tests employed currently to investigate esophageal transit is quantitative. The purpose of this study was to evaluate normal subjects and patients with a variety of esophageal disorders using a scintigraphic technique to quantitate esophageal transit. After oral administration of a bolus of water labeled with 99mTc-sulfur colloid, isotopic count rates were measured over the esophagus employing a gamma-camera on line to a digital computer. Esophageal transit was expressed as the percent emptying for each of the first 15-sec after the initial swallow and for 15-sec intervals after serial swallows. Sixty-two subjects were studied, including: normal volunteers; patients with motor disorders of the esophagus such as achalasia, diffuse esophageal spasm, and scleroderma; and patients with symptomatic gastroesophageal reflux both with and without esophageal motor dysfunction on manometic testing. Esophageal transit was decreased significantly after single and multiple swallows in patients with motor disorders of the esophagus. In addition, esophageal transit was abnormal in patients with reflux disease accompanied by abnormal motor function. In contrast, esophageal transit was normal after a single swallow, but incomplete after serial swallows in patients with reflux associated with normal esophageal motor function on manometry. We conclude that esophageal scintigraphy may be used to evaluate esophageal transit.

Adult↗
PubMed1996

Diagnostic imaging in the evaluation of dysphagia.

Evaluation of dysphagia is a challenge commonly encountered by family physicians. Dysphagia may be classified as either the oropharngeal type or the esophageal type and may have a variety of etiologies. Possible causes of oropharyngeal dysphagia include Zenker's diverticulum, pharyngeal carcinoma, pharyngeal webs and strictures, lateral pharyngeal pouches and neuromuscular diseases. Esophageal dysphagia can be caused by esophageal carcinoma, esophageal stricture and webs, achalasia, diffuse esophageal spasm and scleroderma, caustic esophagitis and infectious esophagitis. Studies using different textures of barium allow evaluation of the swallowing mechanism. Static images are obtained to evaluate the integrity of the mucosa.

Algorithms↗
PubMed2004

Gemcitabine-associated scleroderma-like changes of the lower extremities.

Gemcitabine is a nucleosid analog approved for use in the treatment of metastatic urothelial carcinoma of the bladder. We describe an unusual case of scleroderma-like changes of the lower extremities after treatment by gemcitabine for metastatic carcinoma of the bladder. The patient developed initial inflammatory edema (3 kg) restricted to the lower extremities and subsequent scleroderma-like changes after 2 cycles of gemcitabine. Cutaneous biopsy specimen revealed diffuse sclerosis without involvment of the fascia or muscle. Discontinuation of gemcitabine resulted in dramatic removal of the edema, softening of the skin, and partial reversibility of the fibrotic process. This is the first case report of a scleroderma-like reaction associated with gemcitabine. This antineoplastic agent must be added to the very limited number of cytostatic agents capable of giving rise to scleroderma-like features.

Acetaminophen↗