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PubMedDate not supplied

[T- and B-lymphocytes in the so-called collagenoses].

Lymphocytes of peripheral blood of patients with systemic lupus erythematosus, scleroderma and dermatomyositis were investigated, and relatively, with regard to T- or B-cell characteristics. T-lymphocytes of most patients with SLE were decreased both absolutely. B-lymphocytes mostly showed only a relative increase. Successful immunosuppressive treatment usually accompanied by normalization of T-cell number and with decrease of B-cells. This differentiation of lymphocytes is an additional aid in the determination of activity of SLE and a useful index of immunosuppressive-cytostatic treatment.

Antibodies, Antinuclear↗
PubMedDate not supplied

The interleukin-1, interleukin-2, interleukin-6 and tumour necrosis factor alpha serological levels in localised and systemic sclerosis.

Serological level of interleukin-1 (IL-1), Interleukin-2 (IL-2), Interleukin-6 (IL-6) and tumour necrosis factor (TNF) alpha was investigated in 26 patients with scleroderma, divided into three lots, by the extension and the progress of the disease. Determinations were performed by ELISA in attack and in remission (after treatment with prednison). Normal values: IL-1 (0-5 pg/ml), IL-2 (0-5 pg/ml), IL-6 (5-15 pg/ml), TNF (0-16 pg/ml). Lot A. Results obtained at the first determination showed that IL-1 is elevated in 4 cases (10-15 pg/ml), IL-2 in 5 cases (10-32 pg/ml), IL-6 in 5 cases (15-42 pg/ml) and TNF in 4 cases (18-34 pg/ml). In the second determination IL-1 was increased in 1 case (8 pg/ml), IL-2 in 1 case (9 pg/ml), IL-6 in 2 cases (12 pg/ml) and TNF was normal. Lot B. In the first determination IL-1 was elevated in 5 cases (8-12 pg/ml), IL-2 in 5 cases (10-15 pg/ml), IL-6 in 7 cases (16-20 pg/ml) and TNF was raised in 3 cases (18-25 pg/ml). At the second determination IL-1 showed normal values in all the cases, IL-2 was raised in 2 cases (10 pg/ml), IL-6 in 2 cases (12.15 pg/ml), TNF in 1 case (20 pg/ml). Lot C. In the first determination there were raised values in 4 cases for IL-1 (6-8 pg/ml), 3 cases for IL-2 (10-18 pg/ml), 5 cases for IL-6 (18-20 pg/ml), 2 cases for TNF (20 pg/ml). At the second determination IL-2 was elevated in 1 case (10 pg/ml), IL-6 in 1 case (15 pg/ml). We consider that in scleroderma there is a disturbance of the investigated cytokines due to the activation and involvement of the secretory cells into the pathogenesis of the disease. The increase of the serological levels of IL-1, IL-2, IL-6 and TNF depends on the extension of the lesions and the clinical and biological activity periods of the disease. The absence of the increase of the serological levels does not exclude their activity at the lesional site.

Adolescent↗
PubMed2000

Brachial plexopathy associated with diffuse edematous scleroderma.

Peripheral nervous system (PNS) involvement is rare in systemic sclerosis (SSc), usually restricted to peripheral nerve entrapment. To our knowledge, only one case of scleroderma with brachial plexus involvement has been reported previously. We report here on a 61-year-old woman with past history of limited cutaneous SSc who developed motor deficiency in the left arm concomitant with diffuse edematous scleroderma without evidence for trauma or compression of the brachial plexus. After six months intravenous pulse cyclophosphamide therapy, dramatic improvement of skin and neurological involvement was observed.

Anti-Inflammatory Agents↗
PubMed1975

[Therapeutic action of coagulation factor XIII in scleroderma. 20 cases].

Clotting factor XIII was used in 13 patients suffering from scleroderma, 17 with generalised scleroderma and 3 with localised scleroderma. The duration of treatment varied between 15 days and 6 months. The effect was marked in 7 cases and more moderate in 5 others. It took the form of increased suppleness of the skin, improved joint mobility and, to a lesser degree, an improvement in vasomotor disturbances of the extremities. Oesophageal abnormalities on X-ray were never altered. The return to normal of alveolar-capillary diffusion seen in two cases requires futher confirmation. The action of factor XIII in scleroderma may be explicable by the formation of transamidation bonds between the alpha chains of the collagen molecule, similar to those obtained on fibrin.

Adolescent↗
PubMedDate not supplied

Euthyroid sick syndrome and inhibitory effect of sera on the activity of thyroid 5'-deiodinase in systemic sclerosis.

OBJECTIVE: Our aim was to demonstrate the occurrence of euthyreoid sick syndrome in patients with systemic sclerosis (SSc). Furthermore, the presence of anti-thyroid antibodies and their relationship to thyroid 5'-deiodinase activity was investigated. METHODS: The activity of thyroid 5'-deiodinase was measured by 5' outer ring-deiodination using the sera of patients with SSc (n = 21), undifferentiated connective tissue disease (n = 12), and secondary (n = 19) and primary (n = 11) Raynaud's syndrome (RP). Patients with acute cardiovascular events at the time of the study (n = 16) were investigated as controls. RESULTS: Low FT3 (FT3 < 2.5 pg/ml) was frequently demonstrated in all the patient groups (9/21, 3/12, 10/19 and 8/11, respectively). The high frequency of a FT3/FT4 ratio < 0.2 representing euthyreoid sick syndrome was also often found in SSc (14 cases) and primary (12 cases) and secondary (6 cases) RP patients. Anti-thyroid peroxidase antibody was detected in 17 patients with SSc and in 7, 8 and 3 cases with undifferentiated connective tissue disease, secondary and primary Raynaud's phenomenon, respectively, and in none of the controls. The inhibiting effect of sera on the activity of thyroid 5'-deiodinase was higher in patients with anti-thyroid peroxidase antibodies compared to antibody negative cases (P < 0.01). An inverse correlation was shown between the levels of anti-thyroid peroxidase antibodies and the decreased activity of thyroid 5'-deiodinase (r = -0.6111, P < 0.02) in patients with low FT3. CONCLUSION: The low FT3 or FT3/FT4 ratio observed supports the hypothesis that euthyroid sick syndrome is often present in SSc. Anti-thyroid antibody is also frequently detected in SSc and the positive sera inhibit the activity of thyroid 5'-deiodinase, which can contribute to the low FT3 or FT3/FT4 ratio. Anti-thyroid peroxidase antibodies may play an additive role in the development of low FT3 levels via the inhibiting effect of thyroid 5'-deiodinase. The low FT3 levels may directly influence the already impaired microcirculation in SSc by increasing the systemic vascular resistance.

Acute Disease↗
PubMedDate not supplied

[Morphologic study of the microcirculation in connective tissue diseases].

Capillaroscopy is a non-invasive diagnostic test used to study microvascular abnormalities which are present in many disorders, particularly some rheumatic pathologies, such as connective tissue diseases. In systemic sclerosis, capillaroscopy allows detection of pathognomonic microvascular alterations. In other connective tissue diseases, including systemic lupus erythematosus, dermatopolymyositis, undifferentiated connective tissue diseases, and mixed connective tissue disease, the capillaroscopic patterns, although non-specific, can provide a valid support for the diagnosis. In Raynaud's phenomenon, capillaroscopy enables detection of early microvascular abnormalities that are useful for preclinical diagnosis of secondary Raynaud's phenomenon.

Connective Tissue Diseases↗
PubMed2001

Treatment of Raynaud's phenomenon with intravenous prostaglandin E1alpha-cyclodextrin improves endothelial cell injury in systemic sclerosis.

OBJECTIVE: To evaluate the efficacy and safety of prostaglandin (PG) E1alpha-cyclodextrin for Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc) and its effect on variables of immune activation and endothelial injury in SSc such as tumor necrosis factor-alpha (TNF-alpha), soluble interleukin 2 receptor (sIL-2R), circulating intercellular adhesion molecule-1 (cICAM-1), von Willebrand factor (vWF), and tissue-type plasminogen activator (t-PA). METHODS: We studied 36 women with SSc, 24 of them given three 60 microg intravenous PGE1alpha-cyclodextrin infusions on 5 consecutive days at 6 week intervals during the winter. RP symptoms and healing of digital lesions were evaluated. Twenty age matched healthy women were the controls. TNF-alpha, sIL-2R, cICAM-1, vWF, and t-PA were measured after the first and last infusion of PGEE1alpha-cyclodextrin and correlated with clinical features. RESULTS: RP symptoms improved in 87% of the patients. The benefit of each 5 day cycle lasted 4 or more weeks in 75%. PGE1alpha-cyclodextrin reduced the daily frequency of RP symptoms by 20% (p < 0.05), 41% (p < 0.005), and 53% (p < 0.0005) from baseline after the 1st, 2nd, and 3rd infusions, respectively. The severity of the attacks was reduced to a limited degree. In 12 of the 14 patients with digital lesions, these healed completely. Ten patients had mild side effects during treatment (headache, increased intestinal motility, flushing). TNF-alpha, sIL-2R, cICAM-1, vWF, and t-PA plasma concentrations were significantly higher in patients with SSc than controls (p < 0.05, p < 0.001). TNF-alpha, sIL-2R, and cICAM-1 were higher in diffuse SSc and patients with lung involvement. The plasma levels of cICAM-1 and t-PA were significantly reduced after the 1st infusion of PGE1alpha-cyclodextrin (both p < 0.005) and further reduced after the last (p < 0.0005 and p < 0.005). CONCLUSION: PGE1alpha-cyclodextrin reduces RP symptoms and plasma levels of the markers of endothelial injury in SSc, suggesting that an improvement of endothelial dysfunction contributes to its prolonged therapeutic effect.

Adult↗
PubMed2001

Lichen sclerosus et atrophicus affecting the wrists and left ankle and clinically simulating lichen planus.

Lichen sclerosus et atrophicus (LSA) is a disease of unknown etiology, although hereditary, endocrine, and autoimmune factors are known to be involved. Although the anal and genital regions are predominantly affected, 2.5% of patients only present with extragenital lesions--particularly of the trunk, neck, and upper limbs. The wrists, palmoplantar regions, nipples, and face are less commonly involved. The possible relationship between LSA and both lichen planus and localized scleroderma (morphea) has not been clearly established, although in a number of cases, several of these conditions have been found simultaneously. We report the case of a 61-year-old woman with LSA lesions affecting only the wrists and left ankle. The unusual character of this presentation is pointed out, along with its clinical similarity to lichen planus.

Diagnosis, Differential↗