42 patients were diagnosed as scleroderma and treated at Bach Mai Hospital in three years (1991-1993). 90.47% was female, the age ranged from 18 to 63 years. The control group consisted of 20 healthy normal persons. Results: the rate of anti dsDNA positive with serum 1/10 was 30.95%; the rate of anti ANA positive with serum 1/40 was 71.42% in the patients with scleroderma. In the control group, there is no person who had anti dsDNA positive with serum 1/10. The rate of anti ANA positive with serum 1/40 is 10%. Positive anti dsDNA rate is not high (30.95%). There is no case of scleroderma which had negative anti ANA but positive anti dsDNA in the patients group. The appearance of anti dsDNA and anti ANA with digestive and cardiovascular disorder had a high rate. Anti ANA is more valuable in following the nephroinjuries in the scleroderma than dsDNA
Scleroderma, Systemic ; Diagnosis ; Therapeutics

Scleroderma is the autoimmune diseases. It has clinical characteristics of other autoimmune diseases such as: systemic lupus erythmatosus, juvenile rheumatoid arthritis…Appearance of antinuclear factors, rheumatofactors and increased blood gamma globulin showed that the immunity in this disease is abnormal. Study on 20 patients with scleroderma who were treated at the Allergy Department in two years (1984-1985) showed that: the ratio of leukocyte in the patients was not lower than that in control groups; the percentage of T lymph in peripheral blood was lower; B lymph in peripheral blood increased lightly; T gamma in peripheral blood was lower; the percentage of lymphocyte transformation with Con A and lymphocyte transformation with PHA were lower; lymphocyte transformation with PWM increased
Scleroderma, Systemic ; Diagnosis

The diseases which present with cutaneous sclerodermatous changes are scleroderma, eosinophilic fasciitis, mixed connective tissue disease, sclerederma adultorum, scleromyxedema and cutaneous midline mucinosis. This paper reviews the characteristics and differential diagnosis among of the above mentioned diseases.
Diagnosis, Differential ; Fasciitis/diagnosis ; Human ; Myxedema/diagnosis ; Scleredema Adultorum/diagnosis ; Scleroderma, Circumscribed/diagnosis* ; Scleroderma, Systemic/diagnosis

Diagnostic Errors ; Humans ; Pneumoconiosis ; diagnosis ; Scleroderma, Systemic ; diagnosis

Systemic sclerosis as a connective tissue disease could affect all internal organs of the body and could also manifest as a cutaneous lesion. Cardiac involvement leading to cardiac manifestations in systemic sclerosis patients is not rare. However, cardiac amyloidosis combined with systemic sclerosis is extremely rare. Although there were no definite treatment options in this case, symptomatic treatment is the cornerstone of the management plan. In this case report, we described a correct diagnosis and symptomatic medical care of early reactive cardiac amyloidosis with systemic sclerosis and summarize the current state of the relevant literature.
Amyloidosis* ; Cardiomyopathy, Restrictive ; Connective Tissue Diseases ; Diagnosis* ; Humans ; Scleroderma, Systemic*

Female ; Humans ; Middle Aged ; Sarcoidosis ; diagnosis ; pathology ; Scleroderma, Systemic ; diagnosis ; pathology

Systemic sclerosis (SS) is a connective tissue disease and cardiac involvement is common. Primary cardiac involvement such as conduction system disturbances and arrhythmias can also occur. However, reports of sustained ventricular tachycardia (VT) are rare. We report a case of catheter ablation of sustained ventricular tachycardia in a patient with systemic sclerosis using a conventional mapping system. A 64-yr-old woman with a 10-yr history of SS was referred for management of her ventricular tachycardia. There was no structural abnormality in cardiac chambers. However, electrophysiologic study revealed electrical substrate of ventricular tachycardia which could be ablated with pacemapping and substrate mapping. This case demonstrated successful conventional mapping and catheter ablation in a hemodynamically unstable patient with SS.
*Catheter Ablation ; Electrocardiography ; Female ; Humans ; Middle Aged ; Scleroderma, Systemic/*complications/*diagnosis ; Tachycardia, Ventricular/*etiology/physiopathology/*surgery

BACKGROUND: Anti-topoisomerase I antibodies (anti-topo-I) have been known to be a specific serologic marker for systemic sclerosis (SSc). However, anti-topo-I have also been detected fre-quently in the sera of patients with diagnosis other than SSc since the enzyme linked immunosor-bent assay (ELISA) has been used widely. In order to clarify the clinical significance of anti-topo-I on ELISA, we analyzed the clinical features of the patients positive for anti-topo-I. METHODS: Anti-topo-I and other antinuclear antibodies (ANA) were investigated by conventional ELISA methods. The clinical characteristics were analyzed in 38 patients positive for anti-topo-I and 28 patients with SLE but negative for anti-topo-I. RESULTS: Of 38 patients positive for anti-topo-I, 15 were SLE and eight SSc. The mean level of anti-topo-I in the patients with SSc was higher than that in the patients with SLE (P=0.015). Of 15 anti-topo-I positive patients with SLE, 14 had one or more other ANAs in their sera whereas only one of eight anti-topo-I positive patients with SSc did (P=0.000). There was no significant difference in clinical characteristics between anti-topo-I positive and negative patients with SLE. The preva-lences of restrictive lung disease in both groups with SLE were significantly lower than that in the anti-topo-I positive patients with SSc (P=0.008). CONCLUSIONS: Anti-topo-I is not exclusively specific for SSc and present in a considerable subset of SLE. As well as the level of anti-topo-I, the coexistence of other ANAs is helpful to discriminate SLE from SSc. The Anti-topo-I detected by ELISA does not seem to be a risk factor for restrictive lung disease in the patients with SLE, unlike those with SSc.
Antibodies* ; Antibodies, Antinuclear ; Diagnosis ; Enzyme-Linked Immunosorbent Assay* ; Humans ; Lung Diseases ; Risk Factors ; Scleroderma, Systemic

OBJECTIVE: Raynaud's phenomenon is a vascular disorder characterized by reversible spasm of arteries of fingers. It is the first symptom in 70% of partlents with systemic sclerosis. The more systemic involvment the worse prognosis is expected in patients with systemic sclerosis. A more reliable indication of systemic sclerosis is the microvascular involvement by the disease (characteristic patterns of capillary abnormality in the nail-fold). Our puroses were to evaluate the significance of the degree of nailfold capillary abnormlitry in making the diagnosis fo systemic sclerosis, and in determining organ involvement in patients with systemic sclerosis. METHODS: Twenty-six patients with Raynaud's phenomenon whose diagnosis were systemic sclerosis(10 patients), SLE(10 patient), Raynaud's disease(6 patients) were observed for nailfold capillary abnormalities by widefield microscopy. RESULTS: Capillary abnormalities were seen in 100% of the systemic sclerosis (10 patients), 30% of the SLE (3 patients) and 50% of the Raynaud's disease (3 patients). A significant correlation between degree of finger lesions (r=0.718) or organ involvement (X2=20.4, p=0.015) and capillary abnormality class was found although a significant correlation was not found between the duration of the disease and the degree of capillary abnormality in patients with systemic sclerosis (r=0.32). CONCLUSIONS: Nailfold capillary abnormality can easily be observed and could be used as an assistive tools for the diagnosis and prediction of prognosis and extent of organ involvement in patients with Raynaud's phenomenon especially in patients with systemic sclerosis.
Arteries ; Capillaries* ; Connective Tissue Diseases ; Diagnosis ; Fingers ; Humans ; Microscopy ; Prognosis ; Raynaud Disease ; Scleroderma, Systemic ; Spasm

Lung involvement in systemic sclerosis(SSC) is common but usually occurs late in the course. Skin changes usually occur before the pulmonary findings. In this report, a patient who developed pulmonary interstitial fibrosis without skin changes is presented. A diagnosis of SSC lung involvement was made histologically. The a nti-scl-70 antibody test was positive. Esophageal manometry revealed a lower amplitude in the lower two-third of the esophagus and pressure in the lower esophageal sphincter. Here we report a case of wystemic sclerosis sine scleroderma presenting as pulmonary interstitial fibrosis with a review of the relevant literatures.
Diagnosis ; Esophageal Sphincter, Lower ; Esophagus ; Fibrosis* ; Humans ; Lung ; Manometry ; Scleroderma, Systemic* ; Sclerosis ; Skin