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PubMed1997

[A patient with systemic scleroderma showing improvement during long-term hemodialysis after renal crisis].

A 68-year-old man experienced systemic pruritus since he was 63 years old, and systemic sclerosis and skin pigmentation were observed when he was 64. When he developed dyspnea the same year, he was admitted and SSc was diagnosed on the basis of the clinical and skin biopsy findings, lung fibrosis on X-P and TBLB findings. At 65, his dyspnea reappeared along with elevated blood pressure, acute renal failure and lung congestion, and he was diagnosed as having a scleroderma renal crisis (SRC) from the clinical and renal biopsy findings. Hemodialysis was started because he showed mental disturbance, and this and other acute symptoms were subsequently reduced. As he showed no recovery from his renal failure, the patient has been maintained on hemodialysis for over four years now. In the meantime, his sclerosis has improved and antinuclear antibody almost disappeared. Hemodialysis appears to be the most likely reason for his improvement, although spontaneous remission, D-penicillamine and angiotensin converting enzyme (ACE) inhibitor therapy may also have contributed, considering the short period and the small amount of drugs given until improvement.

Aged↗
PubMed1990

[Phospholipids and glycosphingolipids in cultured skin fibroblasts from healthy donors and patients with systemic scleroderma].

A comparative study of phospho- and glycosphingolipids of cultured skin fibroblast from healthy donors and from patients with systemic sclerodermia (SSD) was carried out. It was shown that the total phospholipid content in SSD fibroblasts is elevated. No significant changes in the concentration of neutral glycosphingolipids were observed. The ganglioside composition of SSD cell cultures differs significantly from that of healthy donor cells. The concentration of the gangliosides, GM3 and GM1, is decreased; no ganglioside GD1a was found in SSD fibroblasts. The data obtained are suggestive of changes in the properties of fibroblast surface which can be manifested both in the impaired reception of matrix proteins and in the impairment of basic properties of the membrane. These changes are well correlated with the results of previous studies on the AMP cyclase system.

Adult↗
PubMed1986

Nailfold capillaroscopy: a blinded study of its discriminatory value in scleroderma, systemic lupus erythematosus, and rheumatoid arthritis.

The appearances of the nailfold capillaries can be used to distinguish between various connective tissue diseases. In a study of 30 patients (10 with scleroderma, nine with systemic lupus erythematosus, and 11 with rheumatoid arthritis), photographs were taken of the eight nailfolds of each patient (thumbs excluded) and then coded. Each of the photographs was later analysed by a rheumatology registrar and an attempt was made to predict the patient's diagnosis using only the appearance of the nailfold. The diagnostic specificity and sensitivity were 89% and 80%, respectively. The results indicate that nailfold capillaroscopy, performed by a relatively inexperienced observer, can accurately distinguish between patients with scleroderma and those with systemic lupus erythematosus or rheumatoid arthritis.

Adult↗
PubMed1999

[Influence of age on the clinical and biological characteristics of systemic scleroderma].

PURPOSE: The present study was aimed at assessing the influence of age on clinical and biological features of systemic sclerosis. METHODS: This retrospective study included 151 consecutive patients with systemic sclerosis. The median age at diagnosis was 50.0 years (range: 10-84 years). Patients were divided into two groups according to their age (lower than 50.0 years of age: 73 patients, equal to or above 50 years of age: 78 patients). The following features were compared between the two groups: gender, disease duration, extent of skin sclerosis, Crest syndrome, lung fibrosis, secondary Sjögren's syndrome, antinuclear, anticentromere, and anti-Scl70 antibodies. RESULTS: The disease duration was significantly higher in patients over 50 years of age (7.1 +/- 6.8 years vs 5.5 +/- 5.0 years, P < 0.05). Crest syndrome, secondary Sjögren's syndrome and anticentromere antibodies were significantly more common in patients over 50 years of age (17/73 vs 30/78, P < 10(-2); 9/73 vs 20/78, P < 10(-2), and 19/73 vs 31/78, P < 0.05; respectively). Anti-Scl70 antibodies were significantly more common in patients under 50 years of age (17/73 vs 10/78, P < 10(-2)). No significant difference was found in regard to the other features. CONCLUSION: The clinical and biological patterns of systemic sclerosis are different according to the age at disease onset. Crest syndrome including anticentromere antibodies and Sjögren's syndrome is more common in elderly patients, while anti- Scl-70 antibodies are more common in younger patients. This suggests the involvement of various mechanisms in the pathogenesis of systemic sclerosis, and that these mechanisms may depend on the age.

Adolescent↗
PubMed1990

[Maxillofacial manifestations of systemic scleroderma. Apropos of a case].

The authors relate the case of one patient 54 years old having a progressive systemic sclerosis. It is a rare progressive disease of unknown etiology. The patient was first seen in 1983, with lesions on the face. The following is the report of this patient who demonstrated resorption of the angle of the mandible, atrophy of the zygomatic arches, characteristics facial muscles and cutaneous lesions.

Facial Muscles↗
PubMed1983

[Arterial hypertension with secondary hyperaldosteronism, reversible by nifedipine, in systemic scleroderma].

A case of severe hypertension with secondary hyperaldosteronism developing in a 71 year old woman affected by "Crest" syndrome is reported. The patient was treated by nifedipine, which led to the correction of blood pressure, plasma renin activity and serum aldosterone. The role of renal arteries vasospasm and renin-angiotension system in the genesis of scleroderma is discussed.

Aged↗
PubMed1978

Nucleic acid metabolism disturbance in progressive systemic sclerosis (systemic scleroderma).

The content of helical forms of RNA and DNA in plasma was determined in 28 patients with progressive systemic sclerosis (PSS). Various disturbances of nucleic acid (NA) metabolism were noted in those patients: the DNA level was increased in comparison with the standard, and the RNA level was decreased. During treatment a normalization of the given indices was observed. The DNA content in plasma of PSS patients correlated with activity of the process, RNA content with the course of the disease.

Acute Disease↗
PubMed1991

Sjögren's syndrome in patients with the CREST variant of progressive systemic scleroderma.

Twenty-three patients with the CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) variant of progressive systemic sclerosis, were clinically, histopathologically and serologically examined for the presence of Sjögren's syndrome (SS). Fourteen were found to be positive. No significant difference could be demonstrated between them and the remaining 9. Characteristics of patients with CREST were compared with those of 29 randomly chosen patients with primary SS. Parotid gland enlargement was more frequently present (p less than 0.01) in the latter than in the former. Virtually no patients with CREST with SS had antibodies to Ro(SSA)/La(SSB).

Autoantibodies↗
PubMed1985

[Treatment of systemic scleroderma with factor XIII in 86 patients, with long-term follow-up].

Eighty-six patients with progressive systemic sclerosis were given coagulation factor XIII intravenously in different dosage regimens. The mean duration of treatment was 19 +/- 18 months and patients were followed up for 22.9 +/- 18.8 months. Improvement or stabilization of the lesions was obtained in 44/86 patients and exclusively concerned skin lesions; there was no improvement in visceral lesions. The drug was well tolerated in short-and long-term treatment. It is concluded that factor XIII demonstrated lasting effectiveness in one-half of the patients treated.

Adult↗
PubMedDate not supplied

[The effect of cyclic nucleotides on the specific binding of estradiol by skin fibroblasts in the normal state and in systemic scleroderma].

Specific binding of estradiol E2 was studied in homogenates of cultivated skin fibroblasts from healthy persons and patients with systemic sclerodermia as well as effect of cAMP and cGMP on the hormone binding was evaluated. Binding of estradiol E2 was decreased 4-fold in systemic sclerodermia. cAMP and cGMP were of importance in activation of the estradiol specific binding. Under conditions of normal state cAMP 10(-6) elevated 2-fold the estradiol specific binding, while the stimulation was not observed in systemic sclerodermia. However, cGMP 10(-6) M did not affect distinctly the estradiol E2 binding by fibroblasts of healthy volunteers but stimulated the hormone binding in systemic sclerodermia as a result of which the binding values were increased practically up to the basal level of normal state.

Adolescent↗
PubMed1999

[Pulmonary involvement in systemic scleroderma. Part I. Chronic fibrosing interstitial lung disease].

INTRODUCTION: Chronic pulmonary interstitial fibrosis is the most frequent respiratory manifestation in systemic sclerosis, occurring in 80% of cases. It remains a severe complication of the disease and is the primary cause of mortality related to respiratory insufficiency in 20 to 60% of cases. CURRENT KNOWLEDGE AND KEY POINTS: The date of onset of interstitial lung disease remains undetermined, and only in rare cases does it reveal the presence of systemic sclerosis. The clinical signs are only observable at a later stage, when at least 50% of the lung parenchyma is affected. The methods of choice adopted for early diagnosis of this disease are high resolution computed tomography and pulmonary functional investigations; they should be carried out during the preliminary investigation and at follow-up once a year. Moreover, high resolution computed tomography also provides prognostic data, for there is a correlation between the type of lesion and its severity as determined by high resolution computed tomography and by histological findings. The value of other methods of investigation, in particular bronchoalveolar lavage, has not yet been clearly established. The association of cyclophosphamide and corticoids is currently being evaluated (indications, administration modalities, duration), and this combination may be the most effective treatment. FUTURE PROSPECTS AND PROJECTS: Interstitial lung disease is one of the major causes of morbidity and mortality in systemic sclerosis. Early diagnosis and management of this disease is therefore of utmost importance.

Adrenal Cortex Hormones↗
PubMed1986

[Effect of a medium with a low serum content on protein synthesis and secretion and RNA and DNA synthesis in a skin fibroblast culture from patients with rheumatoid arthritis and systemic scleroderma].

Synthesis and secretion of protein, as well as synthesis of RNA and DNA by skin fibroblasts of patients with systemic sclerodermia (SSD) and rheumatoid arthritis (RA) upon prolonged culturing of fibroblasts in the medium with low (0.5-1%) serum content differ markedly in their direction and intensity from protein, RNA and DNA synthesis by skin fibroblasts of healthy donors (HD) and by fetal fibroblasts. It has been found that skin fibroblasts of patients with RA and SSD, as well as those of HD, secrete 75-80% of protein synthesized by fibroblasts de novo upon their culturing in DMEM medium with 1% human serum. Under the same conditions, on days 2-5 of culturing, RNA synthesis in the fibroblasts of patients with RA and SSD was increased 3-4-fold, while DNA synthesis was increased 2-3-fold. Collagenolytic and caseinolytic activity in the culture medium of skin fibroblasts from HD and patients with RA reached maximal levels on days 3-5. High protein secretion was observed in DMEM serum-free medium in the presence of vitamin mixture upon culturing skin fibroblasts of patients with SSD. The results obtained show that skin fibroblasts from HD differ in their functional activity from those of patients with rheumatic disorders. It might be suggested, therefore, that the mechanism of protein secretion plays an important role in the maintenance of constant intracellular protein levels in resting cells.

Arthritis, Rheumatoid↗
PubMed2004

[Pulmonary hypertension in patients with systemic scleroderma with CREST-syndrome and without it].

AIM: To study incidence rate and characteristics of pulmonary hypertension development in patients with systemic sclerosis (SS). MATERIAL AND METHODS: The study included 31 SS patients (30 females, 1 male, age 33-75 years, mean age 47.7 +/- 1.7 years). RESULTS: Pulmonary hypertension occurred more frequently in SS patients with CREST-syndrome than in SS patients free of this syndrome. SS patients with CREST-syndrome had also more severe ventricular hypertrophy than ventricular dilation. CONCLUSION: Echocardiography proved to be a highly informative method for detection of pulmonary hypertension in patients with SS. The necessity of hemodynamical study in SS patients is emphasized.

Adult↗
PubMed1990

Capillary microscopy during eosinophilic fasciitis in 15 patients: distinction from systemic scleroderma.

PURPOSE: Eosinophilic fasciitis (EF) is a newly recognized syndrome that bears much discussion in regard to its distinction from progressive systemic sclerosis (PSS). In vivo microscopic examination of the nailbed capillaries has elicited the description of a characteristic vascular pattern seen in PSS dermatomyositis, and mixed connective tissue disease. To clarify the capillaroscopic aspects of this syndrome and to seek criteria distinguishing it from PSS, we performed nailbed capillary microscopy in 15 patients with EF and compared the results of this examination with those in 98 patients with PSS and those in 75 normal control subjects. PATIENTS AND METHODS: The diagnosis of EF was made in 15 patients aged 25 to 69 years (average 43 years) who had an acute course, with painful edema and subcutaneous sclerotic induration sparing the extremities. There was a peripheral hypereosinophilia in all 15 patients. Twelve underwent muscle or deep cutaneous biopsy, including the fascia. Nine of these had fascial thickening, and an inflammatory cell infiltrate was observed in eight. The diagnosis of PSS was made in 98 patients, according to the usual criteria. Seventy-five normal control subjects were examined. All the capillaroscopic examinations were performed by one observer. RESULTS: None of the patients in the EF group had a scleroderma-like capillaroscopic pattern. Thirteen had normal results of capillary microscopy. Two had a nonspecific organic microangiopathic picture. In the group of 98 patients with PSS, 89 had numerous megacapillaries (p less than 0.001), seven had a nonspecific organic microangiopathic pattern, and two had normal findings (p less than 0.001). In the whole group of 75 control subjects, the features were normal. CONCLUSION: Our results show a clear distinction between the results of capillary microscopy in cases of EF and PSS. The normal pattern in EF seems to be another argument for its differentiation from PSS.

Adult↗