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PubMed1996

[Myocardial tomoscintigraphy with 201Tl in the diagnosis of cardiac involvement in systemic scleroderma].

Myocardial microcirculation was assessed in 23 scleroderma systematica patients using 201Tl tomoscintigraphy at rest (23 patients) and upon dipyridamole vasodilation (6 patients). Most patients were found to have impaired blood supply of the myocardium with sites of myocardiofibrosis. In response to dipyridamole introduction improved myocardial perfusion was registered in 4 out of 6 patients, 1 patient showed signs of transient myocardial ischemia, 1 patient had stable defects of myocardial blood supply. It is suggested that some sclerodermic patients may benefit from vasodilators.

Adult↗
PubMed1997

[The evolution of juvenile systemic scleroderma].

60 cases of scleroderma systematica with onset in the childhood or adolescence have been analyzed. Juvenile scleroderma took, as a rule, a favourable course (78%). Slow progression was observed in 43% of patients. Stabilization occurred in 20% of cases. A complete regression was seen in 15%. The disease ran unfavorably in 5% of the cases. Overlap-syndrome occupied an intermediate position between favourable and unfavourable disease. It was registered in 17% of cases. The prognosis is better in early detection of the symptoms and choice of adequate therapy.

Adolescent↗
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↗
PubMed1991

Immunological markers of the subsets of systemic scleroderma and its overlap.

The studies performed repeatedly in 298 cases of systemic scleroderma (SSc) disclosed high prevalence of antinuclear antibody (in about 95% of the cases) with the use of HEp-2 cells as the substrate for indirect immunofluorescence method. Anticentromere antibody (ACA) was found in 25% of acrosclerosis cases, but in 50% of patients with cutaneous involvement limited to the digits, i.e. almost without cutaneous indurations. The visceral involvement was comparable in cases positive and negative for ACA, and a follow-up of these patients showed that the course, although protracted, was not always mild as previously believed. Scl 70 antibody proved to be a most characteristic marker of SSc, appearing in about 87% of diffuse variety and in above 50% of acrosclerosis cases. The most important finding was the detection of the coexistence of ACA and Scl 70 antibody in 10 of 180 cases studied by three methods: indirect immunofluorescence, double immunodiffusion, (for Scl 70) and immunoblotting with recombinant kinetochore antigen for ACA and recombinant Topoisomerase I antigen for Scl 70 antibody. These antibodies, believed to be mutually exclusive, are most often missed due to masking effect on HEp-2 cells by the stronger antibody, usually Scl 70. Its detection proved to be possible by evaluation of different serum dilutions and several cell mitoses, and confirmed by immunoblotting with recombinant antigens. Of practical significance are the clinical correlations between Pm-Scl antibodies, showing homogeneous nucleolar staining on HEp-2 cells, and overlap or atypical SSc cases with concomitant muscle involvement. This antibody was found to have a surprisingly high association with atypical SSc or scleromyositis of children.

Antibodies, Antinuclear↗
PubMed1989

[Analysis of Raynaud's phenomenon in systemic scleroderma: comparative study with that in vibration disease].

Raynaud's phenomenon in systemic scleroderma (SD: 37 cases) was analysed and compared with that of vibration disease (VD: 21 cases) in order to clarify the entity of SD. Raynaud's phenomenon was provoked by cold exposure, high temperature (hot water) exposure, mental stress and vibration stress, which were seen in 37 cases, 22 cases, 7 cases and 3 cases of SD, respectively. However, in VD patients, 21 cases were provoked only by cold exposure other than for vibration stress (1 case). Raynaud's phenomena in SD and VD patients differed in appearance duration, and color changes. These data lead to the conclusion that the vascular responses of SD for extrinsic factors including vibration stress and cold exposure may be different from those of VD.

Adolescent↗
PubMed2003

[Systemic scleroderma: 92 cases in Dakar].

We performed a retrospective study in order to characterize epidemiological data and signs of systemic scleroderma in Sénégal. All patients with the diagnosis according the ACR's criteria of scleroderma were recruited. We included 92 patients, the mean age was 33 years and sex ratio male to female was 0.19 (15 men and 77 women). There were 8 children in our series (8.9%). The initial complaints were cutaneous manifestations (64%) and Raynaud's syndrome (16.3%). Clinical manifestations at admission were muco-cutaneous (100%), osteo-articular and muscular (59%) and Raynaud's syndrome (57%). Focal hypopigmentation was common (70%). Anti-nuclear antibodies were present in 67.4% of patients. Skin biopsy was contributive in all patients in which it was performed (79 cases). Electrocardiogram was abnormal in 55%, non specific inflammatory syndrome was present in 44% and pulmonary function was abnormal in 52%. Our data suggest that systemic scleroderma is more common in younger people in black population. Disorders of pigmentation are common and Raynaud's syndrome is uncommon.

Adolescent↗
PubMed1991

Quantitative nailfold capillary microscopy in cutaneous and systemic lupus erythematosus and localized and systemic scleroderma.

Quantitative television microscopy of nailfold capillaries of the fingers was performed in 12 patients with cutaneous lupus erythematosus (six with discoid type and six with disseminated type), in six patients with localized scleroderma (two with circumscribed type, two with linear types, and two with atrophic type), in 10 patients with systemic lupus erythematosus, and in eight patients with systemic scleroderma. The following features were analyzed and compared with a control group (n = 15) of similar age: venous plexus visibility; density of capillaries; avascular fields; hemorrhages; giant capillaries; diameters of the transitional segment, the arterial, and the venous limbs; loop width; and flow stop caused by local cooling test. The patient groups with cutaneous lesions only showed no essential differences as compared with the controls. Patients with systemic scleroderma differed in almost every finding from the controls and from patients with localized scleroderma. Patients with systemic lupus erythematosus exhibited significant differences in several findings as compared with the controls and the cutaneous lupus erythematosus group, but there was overlap.

Adolescent↗