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PubMed1998

[Forty years study of systemic scleroderma (data of Institute of Rheumatology, Russian Academy of Medical Sciences)].

The basic stages and the results of 40-year studies of systemic scleroderma (SSD) at the Institute of Rheumatology, Russia Academy of Medical Sciences, are given. The goal-oriented studies of the systemic, peripheral, and visceral manifestations of the disease, which were undertaken in the 1960s basically altered our insight into the disease. Subsequently the diagnostic signs of the disease were developed, which substantially improved the diagnosis of SSD. Long-term studies examined the evolution of SSD and identified three major types of its course: acute, subacute, and chronic, which differ in the rapidity of progression of a pathological process, the pattern of clinical and morphological manifestations and prognosis, as evidenced by survival rates. The identified types of the course (n = 3) and clinical forms (n = 5) formed the basis for the Russia classification of SSD. Studying the pathogenesis of the disease mainly in the context of the mechanisms of formation of fibrosis and impaired microcirculation, as well as its clinical heterogeneity served as the basis for developing pathogenetic therapy regimens by differentially using disease-modifying agents and other therapeutical complexes. At present, the Institute of Rheumatology has accumulated unique experience in studying and following over 1500 patients with SSD and related diseases. There is a rise in the sclerodermal group of diseases, cross-over and combined forms of SSD along with great progress in the diagnosis and treatment, which enhances the quality of life of patients with SSD and prognosis in them.

Academies and Institutes↗
PubMedDate not supplied

[Anti-cardiolipin antibodies and other immunological disorders in patients with systemic scleroderma].

A total of 104 patients with scleroderma were examined. Anticardiolipin antibodies were detected in 37.5 per cent of the patients with systemic scleroderma and in 3 per cent of healthy individuals; they were more often detected in 46.8 per cent of the patients with diffuse affections of the skin, atherosclerosis, Raynaud's syndrome accompanied by ulcero-necrotic affections of the skin as compared to patients with restricted affections of the skin (sclerodactylia and focal scleroderma)--29.8 per cent. No significant changes in the frequency of detecting a rheumatic factor, antibodies to Scl-70 were revealed in subgroups of patients with scleroderma, positive and negative anticardiolipin antibodies. Of the greatest interest is a significant difference in levels of C-reactive protein which were high in half of the patients with anticardiolipin antibodies. Anticentromere antibodies were detected twice as more often in patients without anticardiolipin antibodies that corresponded to systemic sclerodermia with minimum involvement of the skin into the pathological process. It is suggested that ulcero-necrotic affection of the skin in systemic sclerodermia is associated with C-reactive protein but it is not of an immunocomplex nature.

Autoantibodies↗
PubMed1998

[Evaluation of the function of the tubular esophagus in patients with progressive systemic scleroderma based on a standardized symptom score].

BACKGROUND AND OBJECTIVE: The oesophagus is the internal organ most often affected in progressive systemic scleroderma (PSS). Suitable methods for demonstrating oesophageal involvement are available in only a few centres. Aim of this study was to validate a standardized symptom score for assessing oesophageal function in patients with PSS. PATIENTS AND METHODS: Oesophageal symptoms of dysphagia, odynophagia (pain on swallowing), heartburn and regurgitation were assessed in 27 consecutive patients with PSS. Intensity (0 to 3 points) and frequency (1 to 4 points) of these symptoms were quantified using a standardized system (after de Dombal and Hall): points per symptom (0 to 12) and points per patient (0 to 48). Results were compared with long-term manometric recordings as reference. The control group consisted of 20 healthy volunteers matched for age and sex. RESULTS: Healthy controls and patients with PSS had significantly different oesophageal motility: 22 of 27 patients fulfilled the criteria of oesophageal hypomotility. Using a total symptoms score of 8 points (points per patient) as limit of normal, the sensitivity of the scoring for the diagnosis of abnormal oesophageal function was 68%, with a specificity of 100%, positive predictive value of 100% and a negative predictive value of 42%. INTERPRETATION: The reported method of symptom scoring provides a valid means of positive prediction of (abnormal) oesophageal function, making manometric investigation unnecessary. Absence or an only mild degree of oesophageal symptoms does not exclude abnormal motility so that manometry must be performed in such patients.

Deglutition Disorders↗
PubMed2002

[Use of pentoxifylline in the treatment of systemic scleroderma].

Analyzes in the paper are chief pharmacological effects of pentoxifylline; the necessity is substantiated of its prescription in a combined therapy of systemic scleroderma (SSD). As many as 220 SSD patients on an in-patient care were examined. Apart from general clinical, biochemical, immunological methods of investigation, the condition of the microcirculation system was studied, the level of the tumor necrosis serumal factor alfa (TNF-alpha) was measured. The data secured suggest a considerable increase in the level of TNF-alpha and pronounced disturbances in microcirculation. The administration of pentoxifylline to SSD patients has been found to improve the microcirculation and reduce the production of the above factor, which fact suggests its immunomodulating properties.

Adult↗
PubMed1976

Pericardial fluid analysis in scleroderma (systemic sclerosis).

A patient with scleroderma who presented with pericarditis and effusion is described. Aspirates from this pericardial effusion had the characteristics of an exudate with no evidence of autoantibodies, immune complexes or complement depletion. These findings suggest that the mechanisms operating in the production of pericardial effusion in scleroderma may be different from those found in rheumatoid arthritis and systemic lupus erythematosus.

Aged↗
PubMedDate not supplied

Systemic lupus erythematosus and systemic scleroderma in patients from the aboriginal people and the newcomers of Yakutia under the extreme conditions of the far north.

There is some information on the course of two main forms of large collagenoses-systemic lupus erythematosus (SLE) and systemic scleroderma (SSD) under the conditions of the North of the Asian part of Russia (Yakutia) in this paper. Seventy-nine cases (59 SLE patients and 20 SSD patients) belonging to different ethnic groups were studied. There were 47 patients of Yakutian nationality, among them SLE 38 patients and SSD nine patients. There were 32 patients (SLE-21, and SSD-11) migrant Europeans. It has been proved that the aboriginal people of the North are more subject to SLE and SSD diseases as compared to the migrants. It has also been proved that the greater spreading of the diseases, with the collagen metabolism disturbance in the first ethnic group, including Marfan's syndrome and rheumatoid arthritis may be explained by genetic peculiarities. Some ethnically stipulated differences in clinical manifestations of two large collagenosis were revealed. Thus, during SLE smaller frequency of the skin impairment in the Yakuts (due to natural hyperpigmentations) is connected with the considerable frequency of large joints impairment and more frequent course of SLE similar to rheumatoid variant with typical wrist deformation. One-third SLE and SSD patients of Yakut nationality reveal "overlap-syndrome", which are typical of other collagenoses, the so-called overlap-syndrome. Some industrial factors (dust of silicon dioxide, vibration, hydrocarbon compounds) are the main reason for the diseases among the newcomers migrants of Russian and Ukranian nationality. One case of silico-lupus was revealed.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Peripheral neuromuscular manifestations in systemic sclerosis (scleroderma).

Systemic sclerosis (scleroderma) is thought to be the least likely of the collagen vascular disorders to cause nervous system damage. We evaluated the peripheral neuromuscular manifestations in 32 patients with scleroderma. A clinically defined peripheral nervous system (PNS) lesion was manifest in 5 of 32 patients (16%), including 2 patients with trigeminal neuropathy and single cases of polyneuropathy, brachial plexopathy, and lumbosacral radiculopathy. Neurophysiological studies suggested subclinical PNS involvement in 6 additional patients (3 with distal axonal polyneuropathy, 1 with probable myopathy and superimposed polyneuropathy, 1 with trigeminal neuropathy, and 1 with focal ulnar neuropathy). Even though subjective muscular complaints were numerous (16 patients, 50%), a defined primary muscular disease could be demonstrated only in 5 patients (16%). Our results indicate that peripheral neuropathy in scleroderma is not as uncommon as previously estimated.

Action Potentials↗
PubMed1984

[Therapeutic trial with fucidin in systemic scleroderma].

The signs and symptoms in systemic sclerosis were improved by systemic therapy with fucidine. In patients with untreated systemic sclerosis, asparaginic acid and glutaminic acid were increased in blood serum and asparagine and glutamine decreased. These compounds with fucidine therapy normalized.

Adolescent↗
PubMed2002

Current treatment options in systemic Sclerosis (Scleroderma).

Systemic Sclerosis (SSc) or Scleroderma is a generalized autoimmune disease with variable involvement of the skin and major organs. Etiology and pathogenesis are still largely unknown, but a variety of humoral and cellular autoimmune phenomena can be observed, and a pivotal role of T lymphocytes in SSc pathogenesis is postulated. The rarity of the disease, the wide spectrum of clinical manifestations and severity as well as a variable course render therapy in SSc a major challenge. In view of the immunopathogenesis of SSc, many (presumed) immunomodulatory agents have been used, but no single agent has been proven to be convincingly effective. Trials with extracorporeal therapies (such as photopheresis, plasmapheresis) or even stem cell transplantation are in progress. In contrast to the hitherto unsuccessful therapeutic approaches for the overall disease course, some life-threatening organ manifestations can often be treated successfully, e.g. interstitial pneumonitis with i.v. cyclophosphamide and scleroderma renal crisis with ACE inhibitors and haemodialysis, respectively. Furthermore, pharmacological and supportive treatment of Raynaud's phenomenon and gastrointestinal involvement can alleviate the burden of the disease. Current therapeutic options as well as hitherto investigated immunomodulators are reviewed in this article.

Antirheumatic Agents↗
PubMed1980

Elevated levels of antibodies to polyuridylic acid detected and quantitated in systemic scleroderma patients by solid phase radioimmunoassay.

A solid support radioimmunoassay has been developed to detect immunoglobulin specific circulating antibodies to polyuridylic acid (Pol U), single-stranded RNA (ss RNA), and single-stranded DNA (ss DNA) in scleroderma and other connective tissue diseases. The assay system uses flex-vinyl microtiter plates on which bovine methyl albumin, the respective polynucleotide, a 1:80 dilution of patient serum, and tritiated high affinity anti-IgG, -IgA, or -IgM are layered. The individual wells containing the sandwich assay are then counted for the presence of labeled immunoglobulins and the results are reported in microgram/ml. Of the 30 scleroderma patients tested, only patients with diffuse systemic scleroderma had antibody levels reactive to Poly U > 4.0 microgram/ml and to ss RNA < 3.0 microgram/ml. Patients with linear scleroderma or morphea had antibody levels to Poly U < 3.0 microgram/ml and very little antibody to ss DNA or ss RNA in their sera. Partial cross reactivity to Poly U was found only in SLE patients with high levels of Ab to ss DNA. Insignificant levels of Poly U antibody were found in patients with other connective tissue diseases and in normal controls. High levels of serum antibody in patients which reacted with Poly U suggest active diffuse systemic scleroderma.

Antibodies↗
PubMed1988

Family studies in scleroderma (systemic sclerosis) demonstrating an HLA-linked increased chromosomal breakage rate in cultured lymphocytes.

An increased chromosomal breakage rate (ICBR) was found in 27 of 28 patients with scleroderma (systemic sclerosis, SS) - 5 with the syndrome including calcinosis cutis, Raynaud phenomenon, esophagus hypomotility, sclerodactyly and telangiectasia (CREST), 4 incomplete CREST, 1 overlapping syndrome, 18 progressive systemic sclerosis (PSS). Not only the patients, but also about half of their first-degree relatives showed an increased chromosomal breakage rate (more than 5 breaks per 100 metaphases). This character segregated as a dominant marker in nine families of scleroderma patients. In the six informative of the nine families, the ICBR trait showed close linkage with the HLA region on chromosome 6 (total lod score 5.5 at theta = 0). In these families, ICBR was predominantly observed in linkage with HLA haplotype A1, Cw7, B8, C4AQ0B1, DR3 which is frequently observed in autoimmune diseases. The nature of the agent inducing chromosomal breakage in cultured lymphocytes of some, but not all family members of scleroderma patients remains to be clarified.

Cells, Cultured↗
PubMed1997

Pancreatitis in systemic scleroderma.

We report the case of a 61-year-old woman, who suffered from abdominal pain, nausea, vomiting and fever. She had a past medical history of acute rheumatism, pyelonephritis and systemic scleroderma. Since 1971 she was hospitalized many times because of recurrent abdominal pain with increased serum amylase and lipase values. On admission, she was in distress and demonstrated clinical signs of acute pancreatitis. The link between systemic lupus erythematosus and acute pancreatitis is discussed in view of the reported cases of the world literature.

Acute Disease↗
PubMed1984

[Effect of isoproterenol on collagen synthesis in cultured skin fibroblasts in patients with systemic scleroderma].

Production of collagen and intracellular content of 3,5'-cAMP were studied after treatment with isoproterenol of fibroblast cultures of II-XI passages obtained from healthy donors and from patients with systemic scleroderma. Incubation of control fibroblasts with isoproterenol within 6 hrs caused an increase in the cell cAMP concentration by 260-385% and inhibited the collagen synthesis by 27-58%. Distinct correlation (r = -0.951) was found in fibroblasts of the patients with sclerodermia between the capacity of the cells to accumulate cAMP in presence of isoproterenol and their ability to produce collagen. The inhibitory effect of isoproterenol on collagen synthesis was not found in the cells with initially increased collagen-producing activity, but the effect was maintained in the strains with the decreased rate of collagen synthesis. The data obtained suggest that impairments in beta-adrenergic functions and the decrease in cAMP concentration in fibroblasts may be responsible for the elevated rate of collagen synthesis in the patients with systemic sclerodermia.

Adult↗
PubMedDate not supplied

[Diagnostic criteria for systemic scleroderma].

Diagnostic informative value was studied of a set of N. G. Guseva's diagnostic criteria (1993) for systemic sclerosis (scleroderma). A total of 104 patients with systemic sclerosis (scleroderma) and 126 patients with other rheumatic diseases were examined. The above set of criteria was found out to be of high sensitivity and specificity--99% and 98.4% respectively. The authors present their own modification of the set of diagnostic criteria by N. G. Guseva (1993) which is not inferior in respect of sensitivity and specificity to the original but is more simple, and with a greater potential for further applications, due to which features it can come into widespread use.

Acute Disease↗
PubMed1990

Autoantibodies to nucleolar antigens in systemic scleroderma: clinical correlations.

Indirect immunofluorescence(IIF) and double immunodiffusion (DID) were performed on the sera of 64 patients who had a nucleolar immunofluorescence pattern on HEp-2 cells. Forty-nine of the sera were from 296 patients with systemic scleroderma (SSc) and 15 sera were from 214 patients with systemic lupus erythematosus (SLE). A homogeneous nucleolar staining pattern was found in 45 of the 64 sera (70.3%), a clumpy fluorescence associated with fibrillarin antibody in 14 (21.8%) and a speckled pattern was found in five of the sera (7.8%). There was a clear correlation between the sera which showed a homogeneous nucleolar staining pattern with symptoms of the polymyositis/scleroderma overlap syndrome that differed from SSc with concomitant myositis. The clumpy pattern was mainly associated with diffuse scleroderma and the speckled pattern with limited scleroderma (previously called acrosclerosis).

Adult↗
PubMed1982

[The kidney in systemic scleroderma. A report of 38 consecutive cases ].

The renal status of 38 patients with progressive systemic sclerosis (scleroderma) has been investigated by the usual clinical tests, urine electrophoresis, glomerular filtration rate (GFR) and renal plasma flow (RPF) determinations and in 4 cases by renal biopsy. Fourteen patients presented with proteinuria and/or a high serum creatinine and/or hypertension with low clearance values in all cases. In 14 other patients, an abnormality was apparent from clearance results (12 cases), renal biopsy (1), urine electrophoresis (1). The earliest sign of renal involvement that could be demonstrated was a reduced RPF and an elevated filtration fraction. Subsequently, a glomerular proteinuria with a electrophoretic pattern was observed as either the only sign (9 cases) or in association with abnormal clearance values (8 cases). The incidence of clinical renal involvement (proteinuria, renal failure, hypertension) ranged from 16 to 60%; 2/3 of patients who presented with proteinuria and hypertension died within 3 years. A mucoid thickening of intima and a fibrosis of adventitia in the proximal part of interlobar and arciform arteries, a fibrinoid necrosis in the distal part of lobular and preglomerular arteries are distinctive although inconstant features. The vascular lesions (seen in 70% of cases) and superimposed but reversible vasoconstriction, account for the decreased RPF. An effective control of blood pressure is mandatory; the therapeutic value of angiotensin converting enzyme inhibition remains to be corroborated.

Adult↗