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Biomedical subjects

M B Stevens

Publications and source records attributed to M B Stevens.

At least 55 records · Page 3Linked to original sources

Incidence of lung cancer in systemic sclerosis.

During a study of lung function in patients with systemic sclerosis, we followed 71 patients with this diagnosis for a mean of 5 years. During this period, 3 cases of lung cancer were observed in the group, giving a post hoc incidence of lung cancer of 8.6 cases/1000 persons/year compared to an expected incidence of 0.52 cases/1000 persons/year. The relative risk ratio for lung cancer in systemic sclerosis patients is 16.5. There was no definite association of lung cancer with cigarette smoking, but all 3 patients had either radiographic or pulmonary function evidence of interstitial pulmonary fibrosis. Although bronchoalveolar carcinoma is the most prevalent histologic type of lung cancer associated with systemic sclerosis in the reported cases in the literature, this was not present in any of our patients.

Female↗

Sjögren's syndrome. Influence of multiple HLA-D region alloantigens on clinical and serologic expression.

The relationships of HLA-DR and the newer DS (second D locus) B cell alloantigens (MB and MT) to the clinical and serologic expression of primary and secondary forms of Sjögren's syndrome (SS) were examined in 102 patients (86 whites and 16 blacks). Although HLA-DR3 was significantly increased in whites (25 of 50, 50%) and blacks (4 of 5, 80%) with primary SS compared with race-matched normal controls, it was not appreciably elevated in those with systemic lupus erythematosus (SLE)-SS, rheumatoid arthritis (RA)-SS, or connective tissue disease-SS. The MT2 specificity, however, was more strongly associated with primary SS (86% of whites and 100% of blacks) and also with SLE-SS and RA-SS compared with race-matched normal controls. Furthermore, MT2 was significantly increased in SLE-SS and RA-SS when compared with non-sicca SLE and RA controls. Although primary and secondary SS were most strongly associated with this DS specificity (MT2), the anti-Ro (SS-A) and anti-La (SS-B) antibody responses were more closely allied to DR antigens. HLA-DR3 was increased in anti-Ro positive patients, both whites and blacks, with primary SS (74%) and in total anti-Ro positive subjects (54%) compared with their anti-Ro negative counterparts (38% and 31%, respectively). Among DR3 negative patients, HLA-DR2 correlated with anti-Ro in both primary SS (83%) and in the total SS group (58%). Thus, 96% of Ro antibody positive patients with primary SS had DR3 and/or DR2, as did 80% of anti-Ro positive subjects in all categories.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

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↗

Clinical and demographic predictors of loss of pulmonary function in systemic sclerosis.

The course of functional pulmonary involvement in systemic sclerosis remains controversial; and it is not known if specific clinical or demographic features are predictive of subsequent changes in pulmonary function. To address these questions, we conducted a non-concurrent prospective study of serial pulmonary function in 24 patients with systemic sclerosis unselected for pulmonary involvement over a mean follow-up interval of 59.7 months. Initial values for the entire group demonstrated a mild restrictive defect with a mild reduction in gas exchange. Although a restrictive pattern was most common, normal and obstructed pulmonary function were seen. Mean rates of change of FVC, TLC, FEV1/FVC, and Dco for the entire group were not different from normal, but substantial variability in the course of pulmonary functional involvement was seen among individuals. Changes in gas transfer, lung volumes, and airflow can occur independent of each other. Rates of change in pulmonary function were not predicted by initial pulmonary function, race, sex, duration of disease, cardiac involvement, roentgenographic fibrosis, or regression of skin disease. Patients with severe Raynaud' phenomenon exhibited the greatest fall in Dco over time, suggesting an association between peripheral and pulmonary vascular involvement. A correlation between exertional dyspnea and rapid loss of Dco was noted. Former smokers had significantly greater rates of loss of FVC and Dco than either non-smokers or current smokers, suggesting that cessation of smoking was a response to rapidly declining function in a subgroup of susceptible smokers. We detected a wide spectrum of severity of pulmonary prognosis in systemic sclerosis, ranging from normal pulmonary function to rapidly progressive disease leading to death. This study indicates that patients with prolonged survival do not necessarily have a rapidly progressive pulmonary component, but those with severe Raynaud's phenomenon and susceptible smokers are at very high risk for rapid deterioration of pulmonary function.

Adult↗

Antibody to Jo-1 in polymyositis/dermatomyositis: association with interstitial pulmonary disease.

Forty-four patients with polymyositis/dermatomyositis (PM/DM) were studied for precipitating antibody to Jo-1. Ten (23%) had anti-Jo-1 antibodies including 8 (47%) of 17 PM patients and 2 (15%) of 13 PM-overlap patients. None of the 11 DM nor 3 PM/DM-cancer patients had anti-Jo-1 antibody. Interstitial pulmonary disease was present in 5 (50%) of the 10 anti-Jo-1 positive compared to 5 (13%) of the 34 anti-Jo-1 negative patients (odds ratio = 5.8, p = 0.05). No differences in other demographic or clinical features or survivorship were noted between these groups. These data confirm the association of anti-Jo-1 antibody with an increased frequency of interstitial pulmonary disease in PM/DM patients.

Adult↗

Familial occurrence of progressive systemic sclerosis and systemic lupus erythematosus.

Eight families containing one member with progressive systemic sclerosis and another with systemic lupus erythematosus have been encountered. Two individuals had clinical/serologic/postmortem findings of both diseases and familial pairs were generally concordant for serologic features. A general lack of household contact was encountered, and HLA haplotypes were shared in 5 of 6 pairs studied.

Antibodies, Antinuclear↗

Arthritis and autoimmune pancytopenia.

We report a patient who developed arthritis and pancytopenia without an underlying systemic disease. Autoantibodies to all lines were demonstrated using an 125I-anti human IgG assay. Elution studies documented cross reactivity of an antibody between erythrocytes and platelets, a pathogenetic variation not previously reported.

Adult↗

Absence of positional change in pulmonary diffusing capacity in systemic sclerosis.

Patients with systemic sclerosis frequently have pulmonary function abnormalities, and recent evidence suggests that pulmonary vascular involvement is a common manifestation. To test the hypothesis that patients with systemic sclerosis have impaired ability to recruit or distend the pulmonary vascular bed, the postural change in the coefficient of carbon monoxide diffusing capacity was measured in 11 patients with systemic sclerosis, and the results were compared with results from age-, smoking-, and sex-matched control subjects with rheumatoid arthritis and with results from healthy subjects. In normal subjects and patients with rheumatoid arthritis increased, the coefficient of diffusion by 9.4 percent (p less than 0.005) and 8.4 percent (p less than 0.01), respectively, when they moved from the sitting to the supine position. In contrast, patients with systemic sclerosis did not show a significant increase in coefficient of diffusion, even those who had otherwise normal pulmonary function. Regression analyses showed that the change in coefficient of diffusion decreased with increasing age (r = -0.57) in normal subjects, and that the change in coefficient of diffusion was a function of the percent predicted forced vital capacity, both in patients with systemic sclerosis (r = 0.59) and in those with rheumatoid arthritis (r = 0.70). Thus, these findings indicate that patients with systemic sclerosis have a nondistensible pulmonary capillary bed and that the absence of positional change in the coefficient of diffusion in systemic sclerosis is a subtle indicator of pulmonary involvement.

Adult↗

Sjögren's syndrome: association of anti-Ro(SS-A) antibodies with vasculitis, hematologic abnormalities, and serologic hyperreactivity.

The clinical significance of autoantibodies to Ro(SS-A) and La(SS-B) in Sjögren's syndrome was examined in a retrospective evaluation of 75 patients with symptoms of the sicca complex who had either primary Sjögren's syndrome or Sjögren's syndrome associated with another connective tissue disease. The clinical, hematologic, and serologic features associated with autoantibodies to the small molecular weight ribonucleoproteins Ro(SS-A) and La(SS-B) were ascertained. A striking clinical association of anti-Ro(SS-A) antibodies (found in 33 patients) with extraglandular disease (vasculitis, purpura, and lymphadenopathy) was seen. Hematologic abnormalities (anemia, leukopenia, and thrombocytopenia) were also associated with the presence of anti-Ro(SS-A) antibodies. Furthermore, anti-Ro(SS-A) antibody was associated with hyperglobulinemia, increased serologic reactivity in terms of rheumatoid and antinuclear factors, cryoglobulinemia, and hypocomplementemia. The presence of anti-Ro(SS-A) antibodies defines a subset of patients with Sjögren's syndrome who have systemic clinical manifestations including vasculitis, hematologic abnormalities, and serologic hyperreactivity.

Antibodies, Antinuclear↗

Piroxicam in osteoarthritis: a controlled long-term study.

This six-month double-blind, parallel-design study compared the efficacy and safety of piroxicam 20 mg and 40 mg with that of 12 tablets of aspirin in 29 patients with osteoarthritis and demonstrated that both piroxicam doses were superior to aspirin in the treatment of osteoarthritis with respect to physicians' opinion of improvement and ease of movement. The safety of piroxicam 20 mg and aspirin were comparable, with gastrointestinal side effects being the most prevalent adverse effect. Patients receiving piroxicam are continuing in a five-year efficacy and safety study.

Aged↗

Rheumatoid arthritis in a Chippewa band. II. Field study with clinical serologic and HLA-D correlations.

We present an in-depth study of rheumatoid arthritis (RA) in a Chippewa band. Of the 227 band members, 168 (74%) were evaluated. The unusually high prevalence of RA was confirmed in 7.1% of those studied or, minimally, 5.3% with a 100% completion rate without additional cases found. Seropositivity in those with clinically definite RA was 92% relative to rheumatoid factor and 75% for ANA. Despite the high prevalence (68%) of HLA-DR4 in this closed population, there was a significant correlation of DR4 with RA (100%). The implications of these observations in this population isolate are discussed.

Adolescent↗

Cutaneous vasculitis in adult polymyositis/dermatomyositis.

Seven (9.2%) of 76 patients with adult-onset polymyositis/dermatomyositis (PM/DM) seen over an 11-year period had cutaneous vasculitis. This was manifest by dermal and/or subcutaneous nodules in 4, periungual infarcts in 3 and digital ulceration in 2. When these 7 patients were compared to the remaining 69, a significant association was noted between cutaneous vasculitis and DM (p = .025); only 1 of 31 patients with primary PM and none of 18 with overlap syndromes had vasculitis. Furthermore, 2 (28.6%) of those with vasculitis had an associated malignancy compared to only 4 (5.8%) of those without vasculitis. These data document the occurrence of cutaneous vasculitis in adult-onset PM/DM and suggest that its presence may be a marker of an underlying malignancy.

Adult↗