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Biomedical subjects

P J Clements

Publications and source records attributed to P J Clements.

At least 55 records · Page 3Linked to original sources
PubMed1991

Cardiac score. A semiquantitative measure of cardiac involvement that improves prediction of prognosis in systemic sclerosis.

Cardiac involvement, assessed yearly by a semiquantitative cardiac scoring technique (the sum of 2 variables, scored 0 or 1 for left axis deviation, and 0 or 2 for moderate-large pericardial effusion) and outcome at 10 years were evaluated in 90 systemic sclerosis (SSc) patients enrolled in a 3-year prospective drug trial. Higher cardiac scores were associated with decreased survival. The inverse relationship of survival with cardiac score was more significant than the relationship between survival and any of the cardiopulmonary variables individually (P less than 0.0000003). Predicted 6-year survival was 79% in patients with a cardiac score of 0, 51% in those with a score of 1, 15% in those with a score of 2, and 0% in those with a score of 3. We conclude that a simple 2-variable weighted cardiac score, derived from routine electrocardiography and echocardiography, is a useful predictor of survival in patients with SSc, and we propose that the application of this cardiac score be tested in other populations of SSc patients.

Adult↗
PubMed1990

Association of L-tryptophan and an illness resembling eosinophilic fasciitis. Clinical and histopathologic findings in four patients with eosinophilia-myalgia syndrome.

The Centers for Disease Control has recently reported an association between the use of oral L-tryptophan preparations and a disease marked by severe myalgia, peripheral eosinophilia, and neuromuscular and cutaneous abnormalities. They have labeled the condition eosinophilia-myalgia syndrome. We report here the clinical and histopathologic findings in 4 patients who developed an illness resembling eosinophilic fasciitis following the initiation of oral L-tryptophan supplementation.

Adult↗
PubMed1990

Skin score. A semiquantitative measure of cutaneous involvement that improves prediction of prognosis in systemic sclerosis.

Cutaneous sclerosis, assessed yearly by a semiquantitative skin scoring technique (sum of 10 body areas, each scored on a scale of 0-3 for tethering, by clinical palpation), and outcome at 10 years were evaluated in 90 systemic sclerosis patients enrolled in a 3-year, prospective drug trial. The inverse relationship of survival to the skin score at study entry (increasing skin score associated with decreasing survival) (P less than 0.003) was more significant than was the relationship found by using classification schemata that rely only on distribution of cutaneous sclerosis. Six-year survival was 40% in patients with entry skin scores greater than or equal to 15 and 73% when skin scores were less than 15 (P less than 0.005). An entry skin score greater than or equal to 15 was associated with a high risk of early fatal renal and cardiac complications.

Adult↗
PubMed1990

Management of systemic sclerosis--a review.

Establishing a correct diagnosis is the first step in the management of this condition. The ARA (American Rheumatism Association) diagnostic criteria is a useful guideline. Next, a multisystem evaluation is needed to define the extent of visceral involvement. Patient education is important to enable them to understand and participate in the management of their disease. Skin care and protection from trauma and cold and physiotherapy to retard contractures are taught. Raynaud's phenomenon may be helped by topical nitrates, ketanserin, nifedipine and vasodilatory prostaglandins. D-penicillamine may be tried for patients with generalised scleroderma of less than 3 years' duration. Colchicine may help but other drugs like N-acetylcysteine and chlorambucil have tested and found ineffective. Cyclosporin seems promising. Finally, all visceral complications should be managed accordingly.

Anti-Bacterial Agents↗
PubMed1990

Elevated serum levels of soluble interleukin 2 receptor, interleukin 2 and neopterin in diffuse and limited scleroderma: effects of chlorambucil.

In a study of 48 patients with systemic sclerosis (SSc). elevated serum levels of soluble interleukin 2 receptor (IL-2R), interleukin 2 (IL-2) and neopterin (indicators of lymphocyte/monocyte activation) were noted in 100, 44 and 40% of early untreated patients with SSc (11 diffuse, 5 Limited). Levels of IL-2R, but not IL-2 or neopterin, were lower in patients with longer duration of disease and possibly with chlorambucil therapy. Pharmacologic alterations of markers of humoral or cell mediated immunity may not be an accurate reflection of clinical efficacy of chlorambucil.

Adult↗
PubMed1989

Home central venous hyperalimentation in fifteen patients with severe scleroderma bowel disease.

Fifteen patients with severe scleroderma bowel disease began receiving home central venous hyperalimentation (HCVH) between 1979 and 1987. The major reasons for instituting HCVH were intestinal pseudo-obstruction, malabsorption, and malnutrition. Eleven patients had an improved quality of life. Serious complications encountered over these 15,700 catheter-use days were 2 episodes of septicemia and 2 episodes of superior vena cava obstruction. Seven patients died, but none directly from their gastrointestinal disease or from the HCVH.

Adult↗
PubMed1989

Immunosuppression with chlorambucil, versus placebo, for scleroderma. Results of a three-year, parallel, randomized, double-blind study.

Placebo and chlorambucil (0.05-0.1 mg/kg/day) were compared as treatments for scleroderma in a 3-year, randomized, double-blind, parallel study of 65 patients. Slopes of change over time, calculated for each organ system of each patient, failed to show significant differences between treatment groups. An immunosuppressive effect in the chlorambucil-treated group was shown by decreases in the white blood cell counts (P = 0.02), platelet counts (P = 0.04), lymphocyte counts (P = 0.001), IgA/IgM concentrations (P less than or equal to 0.05), and wheal size on mumps skin tests (P = 0.02). Three years of immunosuppressive treatment with chlorambucil did not benefit this scleroderma population.

Chlorambucil↗
PubMed1988

Prognostic importance of cardiac arrhythmias in systemic sclerosis.

Ambulatory electrocardiography was performed in 183 patients with systemic sclerosis recruited from five centers who were selected to reflect a balanced population with respect to disease extent and duration. Ventricular ectopy occurred in 67 percent of patients and was strongly correlated by both univariate and multivariate analyses with total mortality and with sudden death. By multivariate analysis, ventricular ectopy was strongly associated with increasing patient age and with other evidence of cardiac and pulmonary involvement but not with clinical and laboratory measures of duration and extent of systemic sclerosis. Evidence of myocardial fibrosis thought to be secondary to microvascular alteration is common in systemic sclerosis, but the clinical implications of myocardial involvement are less well appreciated. The present data suggest the need for ambulatory electrocardiography in the clinical assessment of selected patients with systemic sclerosis, especially those with cardiac or pulmonary involvement, as well as for studies of the effects of antiarrhythmic therapy.

Adolescent↗
PubMed1987

Esophagitis in scleroderma. Prevalence and risk factors.

Of 53 patients with scleroderma (43 women and 10 men) evaluated by esophagoscopy and biopsy, 32 (60%) had erosive esophagitis. Symptoms of heartburn and dysphagia were significantly more frequent in the patients who had erosive esophagitis but often were present in those without this condition. Abnormal motility characterized by loss of peristalsis in the distal esophagus was present in all patients with erosive esophagitis, including the 5 who were asymptomatic. No patient with normal esophageal motility had erosive esophagitis at endoscopy. The patients with erosive esophagitis also had significantly diminished lower esophageal sphincter pressures and increased frequency and duration of gastroesophageal reflux episodes. Stricture was present in 13 of 32 patients with erosive esophagitis and was absent in the other 21 patients. The duration of disease, rate of gastric emptying, and fungal smear and culture were not significantly different in those with or without esophagitis. Treatment of fungal infection for a month had little beneficial effect. The pattern of esophageal motility in scleroderma identifies high and low risk groups for esophagitis and stricture, and can be used to select those who require further investigation, irrespective of symptoms.

Candidiasis↗
PubMed1984

Randomized, double-blind, placebo controlled trial of low-dose pulse methotrexate in psoriatic arthritis.

Thirty-seven patients with psoriatic arthritis were entered into a 12-week prospective, controlled, double-blind multicenter trial comparing placebo and oral pulse methotrexate therapy. Methotrexate was given in a dose of 2.5-5.0 mg every 12 hours in 3 consecutive doses per week. A stable background medication program with nonsteroidal antiinflammatory drugs was allowed. Methotrexate was superior to placebo only in physician assessment of arthritis activity and in improvement of the amount of skin surface area with psoriasis. A small but statistically significant rise of serum total bilirubin occurred in the methotrexate-treated patients. No patients were withdrawn from the study for adverse drug effects.

Adult↗
PubMed1984

Clinical and serological comparison of 17 chronic progressive systemic sclerosis (PSS) and 17 CREST syndrome patients matched for sex, age, and disease duration.

This study compares the clinical and serological differences between 17 PSS and 17 carefully matched CREST patients. Patients were matched for sex, age by decade, and, importantly, disease duration (11.2 +/- 9.2 vs. 12.0 +/- 9.3 years). Muscular and skin involvement were greater for the PSS groups (p less than 0.02) and pulmonary involvement was also greater (p less than 0.05), at least for non-smoking PSS patients. On the other hand no clinically significant differences were found between groups for other visceral involvement--including comparisons of gastrointestinal, cardiovascular, and renal involvement. There were also no laboratory differences except in anti-RNP antibody (p less than 0.04).

Adult↗
PubMed1984

Immunologic abnormalities in hemodialysis patients: improvement after pyridoxine therapy.

8 male patients undergoing maintenance hemodialysis were studied to determine the effect of administering supplements of pyridoxine hydrochloride, 50 mg/day for 3-5 weeks, on tests of immune function. In the 3 patients who initially had abnormal nitroblue tetrazolium reduction tests, the values returned to normal with therapy (p less than 0.05). The generation of chemotactic factors from plasma was defective in all evaluated patients and improved after pyridoxine therapy in 4 of 5 patients (p less than 0.01). The lymphocyte subpopulations changed with a rise in the populations of null cells after supplementation with pyridoxine. In addition, lymphocyte transformation in response to mitogens improved in the 3 patients who initially showed low values in these assays. The improvements occurred with pyridoxine therapy even though some patients who responded had no evidence for vitamin B6 deficiency before therapy, as indicated by a normal erythrocyte glumatic-pyruvic transaminase index. We conclude that several parameters of immune function are improved with pyridoxine supplementation. Studies are necessary to establish the minimum daily intake of pyridoxine which will maintain improved values of these tests of immune function in hemodialysis patients.

Adult↗
PubMed1984

Case control study of antibodies to ENA in progressive systemic sclerosis patients.

Sixteen antibody to extractable nuclear antigen (anti-ENA) (+) and 16 anti-ENA(-) progressive systemic sclerosis (scleroderma, PS) patients were matched for disease duration, age, and sex in a case control study of antibody to ENA (anti-ENA) in scleroderma. Anti-ENA (+) PSS patients more frequently fulfill only minor criteria for PSS than anti-ENA (-) PSS controls (31% vs 0%). Anti-ENA(+) patients had less skin and muscle involvement (p less than 0.05) than their matched controls and tended to overlap more with other diseases (3 vs 0 patients). Besides antinuclear antibody and ENA, no significant serological differences were found between the 2 groups.

Antibodies, Antinuclear↗
PubMed1983

Esophageal abnormalities and dysphagia in polymyositis and dermatomyositis.

Structural or functional abnormalities of the distal esophagus were demonstrated by cineradiography in 14 of 16 patients with polymyositis or dermatomyositis who had been incompletely responsive to high dose corticosteroids and, in some cases, immunosuppressive therapy, or who had adverse effects associated with these therapies. These distal abnormalities occurred in the absence of proximal esophageal skeletal muscle dysfunction in 70% of patients, usually were more frequent with increasing disease duration, and were functionally similar to abnormalities reported in scleroderma. Symptomatic improvement occurred to some patients with administration of antacids and measures designed to decrease esophageal reflux, but improved esophageal symptoms did not correlate with improvement in the myositis. In 18 autopsy subjects with similar histories, distal esophageal smooth muscle atrophy and fibrosis occurred in only 1 and 2 cases, respectively. Of 5 in whom distal esophageal dysmotility had been documented ante-mortem, only 1 had fibrosis and none had smooth muscle atrophy. These findings suggest that factors other than fibrosis and muscle atrophy may contribute to distal esophageal dysmotility in polymyositis and dermatomyositis.

Adult↗
PubMed1983

Prevalence and clinical correlates of pulmonary arterial hypertension in progressive systemic sclerosis.

Forty-nine patients with progressive systemic sclerosis who had undergone extensive studies including pulmonary artery catheterization as part of an ongoing prospective study of the natural course of progressive systemic sclerosis were evaluated. The overall prevalence of pulmonary arterial hypertension in this population of patients with progressive systemic sclerosis was 33 percent, and among 10 subjects with the CREST syndrome the prevalence of pulmonary hypertension was 50 percent. The relation between pulmonary arterial hypertension documented at catheterization and abnormal results of noninvasive studies suggesting pulmonary hypertension, including physical examination, chest x-ray, electrocardiography, echocardiography, single-breath diffusing capacity, and vital capacity, was studied. Diffusing capacity was significantly lower in those patients with definite pulmonary hypertension (mean pulmonary artery pressure of 22 mg Hg or more) compared with those with a normal mean pulmonary artery pressure, and a diffusing capacity below 43 percent of predicted showed the greatest sensitivity (67 percent) of any single diagnostic test in detecting definite pulmonary hypertension. Chest x-ray suggesting pulmonary hypertension was the least sensitive of the tests evaluated, but showed the greatest specificity (100 percent) in identifying patients with pulmonary hypertension. A classification matrix based on discriminant function analysis utilizing the combination of diffusing capacity below 43 percent of predicted and chest x-ray and electrocardiographic findings correctly identified 75 percent of patients with definite pulmonary hypertension and 97 percent of patients with a normal pulmonary artery pressure, but failed to identify correctly patients with mild pulmonary hypertension (mean pulmonary artery pressure of 20 mm Hg). These findings indicate that specific noninvasive studies are helpful in assessing the likelihood of normal or definitely elevated pulmonary artery pressures in patients with progressive systemic sclerosis, but patients with mild pulmonary hypertension are not likely to be identified by these noninvasive studies.

Adult↗
PubMed1981

Abnormalities of pulmonary vascular dynamics and inflammation in early progressive systemic sclerosis.

Abnormalities of pulmonary function were studied in 10 patients with progressive systemic sclerosis (PSS) and 3 control subjects. All underwent 81M krypton lung scanning and total body gallium scanning. Immune complexes were measured by Raji cell radioimmunoassay and polyethylene glycol (PEG) assay. Perfusion scans were abnormal in 7 of 9 patients, and 5 of 9 showed a decrease in pulmonary perfusion after cold challenge. Increased gallium uptake was noted in the lungs of 6 of 9 patients. Krypton scans were normal in the control group. Elevated immune complexes were noted in 8 of 10 patients by the Raji assay and in 5 of 10 with the PEG assay. Efforts to separate patients with PSS into subgroups may lead to a better understanding of and advances in therapy for PSS.

Adult↗