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PubMed1981

Three siblings with scleroderma (systemic sclerosis) and two with Raynaud's phenomenon from a single kindred.

A kindred is reported which contains 3 siblings with scleroderma, 2 siblings with Raynaud's phenomenon, and 2 first-degree relatives with histories suggestive of connective tissue syndromes. Studies of microvascular morphology and flow, serum endothelial cytotoxic activity, antinuclear antibodies, and HLA haplotypes in 18 relatives and 6 spouses revealed that 4 of 5 affected siblings expressed the HLA-DRw4 antigen, which was also present in 2 of 3 asymptomatic relatives whose serum contained endothelial cytotoxic activity. The evidence for an inherited susceptibility to scleroderma is reviewed.

Adult↗
PubMed1995

[Pulmonary manifestations of systemic scleroderma: pathophysiologic and clinical significance of the activation of lung fibroblasts].

Fibrosing alveolitis (FA) is a common and often fatal complication of systemic sclerosis (SSC). The purpose of this study was to characterize the fibrotic process within the lungs using bronchoalveolar lavage fluid (BALF). We investigated 25 healthy controls (CON) and 85 SSC patients. In 61 patients (72%) lung function tests, clinical, and radiological findings indicated manifest FA, whereas 24 patients (28%) where free of significant lung disease. Of the latter, 12 had pathologic BAL differential cell counts (= subclinical alveolitis; SUB), 12 had normal BAL cytology (NOR). BAL samples were analysed for chemoattractant activity (CAA) for fibroblasts using Boyden chambers. Procollagen-III-Peptide (P-III-P) and Laminin fragment P1 (Lam-P1) were measured radioimmunologically. CAA (expressed as % of the effect of conditioned medium) was increased in FA and SUB (CON: 17.3 +/- 3.2; FA: 40.8 +/- 5.8, p < 0.01 vs. CON; SUB: 58.6 +/- 11.8, p < 0.01 vs. CON; NOR: 23.7 +/- 6.3; n.s.). Lam-P1 [U/ml ELF] was also elevated in FA and SUB patients (CON: 0.90 +/- 0.17; FA: 2.07 +/- 0.48, p < 0.05 vs. CON; SUB: 2.61 +/- 1.14, p < 0.05 vs. CON; NOR: 1.05 +/- 0.35, n.s. vs. CON). P-III-P [U/ml ELF] was elevated in FA patients (CON: 8.3 +/- 1.1; FA: 26.9 +/- 5.5, p < 0.001 vs. CON) but not in SUB or NOR (SUB: 10.2 +/- 0.7, NOR: 7.9 +/- 2.9; n.s.). There was no significant relationship between P-III-P and LAM-P1 values in ELF and serum, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1984

[Myositis disclosing systemic scleroderma].

A 61 year old patient presented with muscular atrophy and weakness, predominant at the shoulder girdle, as the first manifestation of a progressive systemic sclerosis with the signs of the CREST syndrome: subcutaneous calcinosis, Raynaud phenomenon, esophagus disorders, sclerodactyly, telangiectasia. The histological features were consistent with polymyositis. In spite of corticotherapy, total recovery was not obtained. The nosological relationships with Mixed Connective Tissue Disease is discussed. Myositis is infrequent in progressive systemic sclerosis and corticosteroid therapy is discussed.

Calcinosis↗
PubMed1999

[Pulmonary involvement in systemic scleroderma. Part II. Isolated pulmonary arterial hypertension, bronchopulmonary cancer, alveolar hemorrhage].

INTRODUCTION: Pulmonary interstitial fibrosis is the most frequent cause of lung disease in systemic sclerosis. However, other pulmonary complications exist, including lung cancer, alveolar hemorrhage, and in particular isolated pulmonary arterial hypertension, which is still considered the bête noire as regards this disease. CURRENT KNOWLEDGE AND KEY POINTS: The prevalence of pulmonary arterial hypertension has been reported to range from 5 to 60% in cases of systemic sclerosis; isolated pulmonary arterial hypertension has been principally observed in subjects with a ten-year history of limited forms of the disease. As the patient remains asymptomatic for a long period, with nonspecific respiratory clinical manifestations, the diagnosis is made at a much later stage in the course of the disease. The diagnostic method of choice is echocardiography-doppler, which should be performed during the preliminary investigation, and at follow-up. The prognosis is poor, and patient survival rate at 2 years after onset of symptoms amounts to 40%. To date, no curative therapy for pulmonary arterial hypertension has yet been found. FUTURE PROSPECTS AND PROJECTS: A knowledge of the mechanisms involved in the development of isolated pulmonary arterial hypertension is essential to the determination of new and relevant therapeutic strategies. Vasodilatory treatment, notably calcium channel blockers, prostacyclin and analogs such as iloprost, may be effective at an early stage of the disease before the appearance of permanent vascular damage.

Echocardiography, Doppler↗
PubMed2006

[Endothelial dysfunction in patients with scleroderma systemic].

The purpose of the study was to investigate endothelial lesions and disturbances in the antithrombogenic and vasoregulative vascular wall functions in patients with scleroderma systematica (SS). The subjects of the study were 39 patients with SS aged 46 +/- 1.4 years. Vasomotor endothelial function was studied using reactive hyperemia and nitroglycerin tests. Cuff test was applied to evaluate antithrombogenic (anticoagulative and fibrinolytic activity) properties of the vascular walls. The number of desquamated endotheliocytes in blood was measured by J. Hladovec method (1978). The study found a tendency towards brachial artery narrowing, as well as hypertrophy of its wall in patients with SS. In 28.6% of SS patients flow-dependent dilatation did not exceed 10% (P < 0.01 vs. controls), which suggests the presence of disturbances in endothelium-dependent vasodilatation. SS patients displayed a significant increase in the number of circulating endotheliocytes (P < 0.05), which is evidence of endothelial lesion. The study found a significant decrease in fibrinolytic and anticoagulative activity of the endothelium in SS patients vs. controls. Thus, the study revealed endothelial lesion, as well as disturbances in the vasoregulative and antithrombogenic vascular wall activity in patients with SS.

Blood Flow Velocity↗
PubMed1998

[Primary biliary cirrhosis and systemic scleroderma (Reynolds syndrome): apropos of 8 new cases. The contribution of accessory salivary gland biopsy].

PURPOSE: We report eight new cases of the association primary biliary cirrhosis--systemic sclerosis (Reynolds' syndrome) and study the contribution of labial salivary gland biopsy to the disease diagnosis. METHODS: We retrospectively collected clinical and biological data as well as results of labial salivary gland biopsies in eight patients with Reynolds' syndrome. RESULTS: The eight patients were female. Systemic sclerosis corresponded to a CREST syndrome in three patients, while in two other patients two features of the CREST syndrome were observed. Anticentromere antibodies were detected in these five cases. The last three patients had systemic sclerosis with bilateral pulmonary fibrosis in two cases. All patients presented with Sjögren's syndrome. Labial salivary gland biopsies performed in six patients showed in five cases an inflammatory infiltrate and focal sialadenitis typically associated with Sjögren's syndrome. In four cases, organic microangiopathy and fibrosis were suggestive of systemic sclerosis, and in four other cases, the presence of a pericanalicular infiltrate of lymphocytes and necrosis of the excretory ducts epithelial cells suggested the existence of primary biliary cirrhosis. In three patients, all histological lesions were found in the same labial salivary gland biopsy. CONCLUSION: Coexistence of histological lesions on the same labial salivary gland suggestive of primary biliary cirrhosis, systemic sclerosis and Sjögren's syndrome has never been described previously. The diagnostic value of labial salivary gland biopsy has to be assessed in this context.

Adult↗
PubMedDate not supplied

[The clinical value of determining beta-glucuronidase activity in the cells and blood serum of patients with systemic scleroderma].

The spectrophotometric method was used to determine the activity of beta-glucuronidase (BGU) in the cells (neutrophils, mononuclear cells, thrombocytes) in the blood serum of patients with systemic sclerodermia (SSD). The authors revealed a synchronous change in the activity of BGU in all the cells and in the blood serum, and also a decrease in the activity of the enzyme in the concentration of protein in neutrophils, it being very significant in patients with SSD accompanied by trophic changes. There was a direct relationship between the activity of BGU in the blood serum and the level of C-reactive protein and an inverse one between the activity of BGU and the content of circulating immune complexes in the blood. The level of BGU in the blood serum reflects not only the activity of the inflammatory process but also the markedness of trophic changes in patients with SSD.

Blood Platelets↗
PubMed1976

Radiological features of mixed connective tissue disease and scleroderma--systemic lupus erythematosus overlap.

The radiological, clinical, and laboratory features found in seven patients with scleroderma-SLE overlap, including three with mixed connective tissue disease (MCTD), are described. There were no distinctive roentgenographic features which differentiated those with MCTD from the others with a clinical overlap syndrome of scleroderma and SLE. When features of both coexist in a single patient the radiologist may be the first to suggest the correct diagnosis of overlap syndrome of MCTD.

Adolescent↗
PubMed2003

[Clinical value of soluble adhesion molecules in systemic scleroderma].

The aim of the trial was to study clinical significance of estimation of cell adhesion soluble molecules (CASM) in scleroderma systematica (SS). Quantitation of CASM VCAM-1, ICAM-1 and R-selectin was made with enzyme-immunoassay (R&D System kits, USA) in 38 patients with SS (11 with limited SS and 27 with diffuse SS). The levels of VCAM-1, ICAM-1 and R-selectin was elevated in 30 (79%), 17 (45%) and 20 (53%) patients, respectively. Mean values of VCAM-1 and ICAM-1 in patients were significantly higher than in healthy donors. R-selectin was also higher but insignificantly. A mean CASM level and a relative number of patients with elevated count of CASM in patients with diffuse and limited forms of SS did not differ. In 15 patients with active (progressive) course of the disease the level of VCAM-1 was significantly higher than in patients with chronic (non-progressive) course of SS while concentrations of ICAM-1 and R-selectine were almost the same. Thus, SS patients have elevated levels of CASM. CASM VCAM-1 concentration is the most sensitive marker of SS activity compared to other CASM.

Adult↗