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At least 829 records · Page 46Linked to original sources
PubMed1982

The effect of cold exposure on diffusing capacity in patients with Raynaud's phenomenon.

Pulmonary hypertension is common in progressive systemic sclerosis (PSS), and the presence of "pulmonary Raynaud's phenomenon" has been postulated from a variety of evidence. In this study, the effect of cold-induced Raynaud's phenomenon on carbon monoxide diffusing capacity (Dco), an indicator of pulmonary capillary blood volume, was determined in patients with Raynaud's phenomenon with an without PSS. Cold exposure caused an increase in Dco in patients without PSS, but no change in patients with PSS. We concluded that patients with systemic sclerosis have an altered pulmonary vascular response to cold exposure compared with other Raynaud's patients, which may be due to either structural or functional abnormalities of the pulmonary vascular bed.

Adolescent↗
PubMed1983

Effect of the cold pressor test on diffusing capacity. Comparison of normal subjects and those with Raynaud's disease and progressive systemic sclerosis.

Single-breath carbon monoxide diffusing capacity (Dsb) was measured before and during immersion of one hand in ice water (cold pressor test) in the following three groups of subjects: (1) normal subjects; (2) patients with isolated Raynaud's disease; and (3) patients with Raynaud's phenomenon and progressive systemic sclerosis. No change in Dsb was found in normal subjects or patients with progressive systemic sclerosis. Patients with isolated Raynaud's disease showed a rise in Dsb during cold pressor testing, the mean increase being 8 percent. These results suggest that a rise in Dsb during exposure to cold is a response unique to patients with isolated Raynaud's disease or Raynaud's phenomenon without progressive systemic sclerosis, and not a normal physiologic response to cold. The lack of change in Dsb in response to cold in progressive systemic sclerosis, interpreted by other authors as an indicator of pulmonary vascular disease, resembles the normal response to a challenge with cold.

Child↗
PubMed1987

Pulmonary involvement in systemic sclerosis. Association with anti-Scl 70 antibody and digital pitting.

The association of clinical and serologic features of 34 patients with systemic sclerosis was examined. Anti-Scl 70 antibody was found to identify patients with abnormal pulmonary function, particularly impaired diffusion (p less than 0.005), as well as patients with digital pitting scars (p less than 0.025). In addition, the presence of digital pitting scars correlated with impaired diffusion (p less than 0.005), suggesting that interstitial pulmonary disease in systemic sclerosis may, like digital pitting, be secondary to vascular pathology. Anticentromere antibody-positive patients were less likely to have abnormalities of pulmonary function (p less than 0.001).

Autoantibodies↗
PubMed1991

Pregnancy complicated by collagen vascular disease.

Pregnancies complicated by collagen vascular diseases present a challenge to the obstetrician, internist, and other consulting physicians caring for the patient. Although most pregnancies complicated by these disorders result in satisfactory outcomes, a significant number develop manifestations sufficiently serious to require admission to an intensive care unit. Such manifestations include lupus nephritis, CNS lupus, ARDS, massive alveolar hemorrhage, scleroderma renal crisis, pulmonary hypertension, severe myositis, and diffuse vasculitis. Of the numerous therapeutic options available, many may be used to optimize maternal and perinatal outcome.

Autoimmune Diseases↗
PubMedDate not supplied

Short-term effect of cold provocation on single-breath carbon monoxide diffusing capacity in subjects with and without Raynaud's phenomenon.

The lung diffusing capacity for carbon monoxide was determined by the single breath method (DLCOsb) before, during, and after immersion of the left hand in cold water in four groups of subjects: (i) normal individuals; (ii) subjects with idiopathic Raynaud's disease; (iii) patients with progressive systemic sclerosis without associated Raynaud's phenomenon; and (iv) patients with connective tissue disease associated with Raynaud's phenomenon. Our results showed significant differences in the evolution of DLCOsb after cold stimulation between the groups. Control subjects (group one) showed a transient but significant reduction in DLCOsb at the end of two minutes of cold stimulation, normalizing ten minutes later. Subjects with primary Raynaud's phenomenon (group two) showed on the contrary a transient but significant rise in DLCOsb after 2 minutes, while subjects with an isolated connective tissue disease without Raynaud's phenomenon (group three) showed a significant decrease in DLCOsb at both observation times. Patients with Raynaud's phenomenon associated with a connective tissue disease (group four) showed a delayed decrease in DLCOsb ten minutes after cold stimulation. This latter result appeared as an addition to the effects of cold stimulation observed in groups two and three.

Adult↗
PubMed1988

Reduced natural killer cell activity in patients with systemic sclerosis. Correlation with clinical disease type.

Natural killer (NK) cell number and function were determined in 69 systemic sclerosis (SSc) patients (41 with diffuse cutaneous SSc, 24 with limited cutaneous SSc, and 4 with scleroderma in an overlap syndrome). The results were compared with those obtained from 5 patients with Raynaud's disease and from 27 normal controls. Natural and antibody-dependent killing was reduced in the total group of SSc patients compared with controls, but these differences were primarily attributable to patients with the diffuse form of the disease who were seen early in their illness (less than 5 years after onset). NK cell numbers were not significantly reduced in patients compared with controls, although lower numbers were observed in individuals with early diffuse disease. Other clinical parameters, such as treatment with D-penicillamine or the presence of scleroderma-specific autoantibodies, did not exert an independent effect on NK cell function. These findings suggest a possible central role for NK cells in the pathogenesis of SSc.

Adult↗
PubMed1982

Antibodies to collagen in scleroderma.

Using the enzyme-linked immunosorbent assay (ELISA), we detected antibodies to interstitial (type I) and basement membrane (type IV) collagens in the sera of patients with scleroderma (systemic sclerosis). Antibodies against type IV collagen were found in significant levels in these patients and correlated with the presence of abnormal pulmonary diffusion capacity. Levels of antibodies to type I collagens also correlated significantly with pulmonary diffusion capacity. Absorption of sera with type I or type IV collagens before analysis in the ELISA eliminated reactivity in an antigen-specific pattern, indicating that these antibodies reacted with determinants specific for either type I or type IV collagens. The removal of immune complexes by ultracentrifugation had no effect on serum antibody levels. Autoantibodies to basement membrane and interstitial collagens may participate in the pathogenesis of scleroderma.

Adolescent↗
PubMed1980

Diffuse fasciitis with eosinophilia. A clinicopathologic study.

Three cases of diffuse fasciitis with eosinophilia are presented, and their clinical, laboratory and histologic follow-up are outlined. Furthermore histologic comparison with scleroderma is attempted and the literature is reviewed. From our findings, we conclude that diffuse fasciitis with eosinophilia is a distinct clinicopathologic entity.

Adult↗
PubMed1999

[Scleroderma and pregnancy: obstetrical complications and the impact of pregnancy on the course of the disease].

BACKGROUND: To describe the outcome of the pregnancy in patients with scleroderma. PATIENTS AND METHODS: Patients with scleroderma and control group were included in this retrospective study. Two groups were different in pregnant patients with scleroderma: pregnancy before scleroderma (A1) and pregnancy after scleroderma (A2). The presence of clinical problems during pregnancy and the outcome of scleroderma were collected in a questionnaire. Differences in the frequencies of complications were analyzed using the U Mann-Whitney, the chi-square or Fisher's exact test when necessary. RESULTS: The frequency of global fetal complications was increased in patients group, but there was no significantly increased frequency when variables were analyzed independently: number of births, miscarriages, fetal deaths, preterm births and low weight full term babies. There was no increased frequency of renal crisis, hypertension or eclampsia. Differences between diffuse and limited subsets were no observed. Improvement of scleroderma was seen in only 3 patients and worsening of skin thickening was experienced by 2 patients. CONCLUSIONS: The pregnant scleroderma patients are a group with high risk pregnancies and therefore well-supervised pregnancies are necessary.

Adult↗
PubMed1983

Clinical effectiveness of apheresis in the treatment of progressive systemic sclerosis.

Scleroderma is a chronic disease of unknown origin characterized by diffuse sclerosis of the connective tissue of the integument and other organs. As in systemic lupus erythematosus (SLE), rheumatoid arthritis and dermatomyositis, a variety of antibodies may be found in the serum, suggesting that immune mechanisms may be involved. In two uremic patients with scleroderma, the effects were studied of the removal, by pheresis, of antinuclear antibodies, immune complexes, and immunoglobulin factors perpetuating the inflammatory response. We used a membrane plasmapheresis with a hollow-fiber type plasmafilter (Plasmaflo, Asahi) connected online with a hemofilter for conventional dialysis. The aim of this preliminary study was to induce a remission of the edematous inflammatory phase. The correlation of a improvement in clinical response and a decrease in the levels of immune complexes or other factors of inflammation was observed. In our view membrane-plasmapheresis is a process safely carried out by the hemodialysis staff and it may be of clinical effectiveness, especially, in patients with life-threatening complications of progressive systemic sclerosis.

Adult↗
PubMed1997

Systemic scleroderma. Multicenter trial of 1 year of treatment with recombinant interferon gamma.

OBJECTIVE: To confirm significant improvement of the skin score in systemic sclerosis by treatment with interferon gamma in a larger group of patients and to investigate on a molecular level the influence of interferon gamma on collagen type I messenger RNA expression. DESIGN: Open, noncontrolled multicenter study. SETTING: Five outpatient clinics specializing in the care of systemic scleroderma. PATIENTS: Thirty-two patients suffering from the diffuse or limited form of systemic sclerosis and progressive disease were recruited; 20 patients finished the study. INTERVENTION: Each patient received interferon gamma, 50 micrograms subcutaneously 3 times a week for 1 year. MAIN OUTCOME MEASURE: Skin score, collagen type I messenger RNA in skin biopsy specimens. RESULTS: The patients who completed the study showed an unchanged median skin score after 1 year of therapy. In addition, similar collagen type I messenger RNA levels were detected in skin biopsy specimens taken from involved skin before and after therapy in these patients. CONCLUSIONS: Treatment of systemic scleroderma with interferon gamma is associated with stabilization of the skin score and lack of worsening of visceral involvement.

Female↗
PubMed2003

Effects of oral cyclophosphamide and prednisolone therapy on the endothelial functions and clinical findings in patients with early diffuse systemic sclerosis.

OBJECTIVE: The endothelial damage of microvascular structures in systemic sclerosis (SSc; scleroderma) is associated with increased levels of endothelial adhesion molecules and endothelium-associated cytokines, including E-selectin and thrombomodulin. Although there is still no ideal specific pharmacologic therapy for SSc, cyclophosphamide has resulted in clinical improvement in patients with SSc-related active alveolitis. This study was designed to assess the expression of E-selectin and thrombomodulin in patients with early diffuse SSc, and to investigate the effects of oral cyclophosphamide combined with prednisolone therapy on the levels of these endothelium-associated cytokines and on the patients' clinical outcomes. METHODS: Thirteen patients with early diffuse SSc were treated with oral cyclophosphamide (2-2.5 mg/kg/day) and methylprednisolone (30 mg/every other day) for 1 year. The outcomes were determined as clinical (skin score) and laboratory parameters (including the erythrocyte sedimentation rate, complete blood cell count, levels of C-reactive protein, antinuclear antibody, anti-double-stranded DNA, rate of creatinine clearance, and findings on pulmonary function tests, esophageal manometry, and echocardiography). The concentrations of E-selectin and thrombomodulin were measured in the pretreatment and posttreatment serum samples from the SSc patients and from 12 healthy adults as controls. RESULTS: In the patients with early diffuse SSc, pretreatment and posttreatment mean levels of E-selectin were 51 ng/ml (range 34.2-135.5) and 33.4 ng/ml (range 23-62.5), respectively (P = 0.01), and those of thrombomodulin were 82 ng/ml (range 35.8-120.5) and 74.6 ng/ml (range 23.3-91.3), respectively (P = 0.016). Clinical and laboratory parameters (the skin score and measures of pulmonary function [forced vital capacity and diffusing capacity for carbon monoxide]) were also improved (P < 0.05 for each) at the end of the followup period. CONCLUSION: Combination therapy with cylophosphamide plus prednisolone is effective in the treatment of early diffuse SSc. Circulating levels of E-selectin and thrombomodulin not only demonstrate the extent of endothelial injury and/or activation, but also could be a useful marker to monitor the disease activity in SSc.

Administration, Oral↗
PubMed1997

Racial differences in scleroderma among women in Michigan.

OBJECTIVE: To examine racial differences in disease onset, extent, manifestations, and survival among women with scleroderma. METHODS: A retrospective cohort study of women with scleroderma, diagnosed in Michigan between 1980 and 1991, was conducted. Clinical, laboratory, and demographic data were abstracted from the patients' medical records. RESULTS: A total of 514 women with scleroderma were identified: 117 (23%) were black and 397 (77%) were white. Among black women, the mean age at diagnosis was lower (44.5 years versus 51.5 years; P < 0.001) and diffuse disease was more common (49.6% versus 24.9%; P < 0.001) than among white women. The overall incidence of scleroderma was 14.1 per million per year: 22.5 per million per year in black women versus 12.8 per million per year in white women (P < 0.001). Pericarditis (P = 0.009), pulmonary hypertension (P < 0.001), pleural effusions (P = 0.01), myositis (P = 0.02), and an erythrocyte sedimentation rate >40 mm/hour (P < 0.001) were more frequent among black women, while white women were more likely to have digital infarctions (P < 0.001). Survival at 7 years from diagnosis was 72.5% among black women and 77.6% among white women. Age-adjusted survival was significantly reduced among black women (P = 0.033), most likely because of increased diffuse involvement. Survival among those with renal or pulmonary involvement was also significantly reduced. CONCLUSION: Black women with scleroderma were significantly more likely than white women to develop diffuse disease, be diagnosed at a younger age, have a higher incidence of inflammatory features, and have a worse age-adjusted survival rate.

Adolescent↗