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Results for “Systemic sclerosis”

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PubMed1989

Determination of an epitope of the diffuse systemic sclerosis marker antigen DNA topoisomerase I: sequence similarity with retroviral p30gag protein suggests a possible cause for autoimmunity in systemic sclerosis.

The possibility that viruses play a role in the etiology of various autoimmune diseases has been proposed. One approach to the search for these agents involves identifying potential crossreactive epitopes in viruses that infect cells of the immune system or of the target tissues. Antibodies to DNA topoisomerase I are the marker autoantibodies for diffuse systemic sclerosis. The major epitope of the antigen was therefore sought through cloning and sequencing of the cDNA for human topoisomerase I and eventually by the synthesis of the smallest possible peptide recognized by sera from patients with the diffuse form of systemic sclerosis. The antigenic 11-amino acid sequence contains 6 sequential amino acids that are identical to a sequence present in the group-specific antigen (p30gag) of some mammalian retroviruses. This sequence is separated by only 1 amino acid from the retroviral epitope sequence that crossreacts with autoantibodies against the marker antigen for mixed connective-tissue disease and systemic lupus erythematosus, the 70-kDa polypeptide of U1 ribonucleoprotein particles. These findings suggest that a retroviral agent may be involved in the pathogenesis of systemic sclerosis and other connective tissue diseases and that antibodies to intracellular antigens are not involved in the pathogenesis of autoimmune disease but may be useful as footprints for tracking the potential etiological agent of autoimmune disease.

Amino Acid Sequence↗

PubMedDate not supplied

Clinical evaluation of systemic sclerosis: a comprehensive panel of diagnostic tests to assess skin, microvascular and visceral involvement. The Genoese Group for the Study of Systemic Sclerosis.

The work proposes a set of diagnostic tests and relative clinical scores for the staging and follow-up of patients with systemic sclerosis. Thirty-one consecutive patients (30 females, 1 male, age range 17-65 years) affected by systemic sclerosis, according to the American College of Rheumatology criteria for classification of this disease, were enrolled in the study at the time of their first admission to the Department of Internal Medicine in Genoa. The following battery of tests was utilized: nailfold capillary videomicroscopy, esophageal manometry and pH-metry, lung vital capacity and diffusing capacity for carbon monoxide, intrarenal duplex Doppler sonography, echocardiography and skin plicometry. Good compliance by the patients was obtained for the tests we used. We found that nailfold capillary videomicroscopy and plicometry scores were statistically associated each other as well as with the scores measuring esophagus and/or lung function. The use of wide range of diagnostic procedures, such as those proposed in this paper, allows appropriate definition of the stage of patients with systemic sclerosis. The use of nailfold capillaroscopy and skin plicometry as the simplest tests for monitoring patients is suggested: the scores derived from these procedures are statistically associated with some internal organ status and could be useful in the evaluation of therapeutical results.

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↗

PubMed2006

Plasma D-dimer concentration in patients with systemic sclerosis.

BACKGROUND: Systemic sclerosis (SSc) is an autoimmune disorder of the connective tissue characterized by widespread vascular lesions and fibrosis. Little is known so far on the activation of the hemostatic and fibrinolytic systems in SSc, and most preliminary evidences are discordant. METHODS: To verify whether SSc patients might display a prothrombotic condition, plasma D-dimer was assessed in 28 consecutive SSc patients and in 33 control subjects, matched for age, sex and environmental habit. RESULTS AND DISCUSSION: When compared to healthy controls, geometric mean and 95% confidence interval (IC95%) of plasma D-dimer were significantly increased in SSc patients (362 ng/mL, IC 95%: 361-363 ng/mL vs 229 ng/mL, IC95%: 228-231 ng/mL, p = 0.005). After stratifying SSc patients according to disease subset, no significant differences were observed between those with limited cutaneous pattern and controls, whereas patients with diffuse cutaneous pattern displayed substantially increased values. No correlation was found between plasma D-dimer concentration and age, sex, autoantibody pattern, serum creatinine, erythrosedimentation rate, nailfold videocapillaroscopic pattern and pulmonary involvement. CONCLUSION: We demonstrated that SSc patients with diffuse subset are characterized by increased plasma D-dimer values, reflecting a potential activation of both the hemostatic and fibrinolytic cascades, which might finally predispose these patients to thrombotic complications.

Journal Article↗

PubMed2005

Pseudovitiligo in Systemic Sclerosis.

Progressive Systemic Sclerosis is a chronic connective tissue disease characterized by sclerosis of skin with involvement of the internal organs. The cause of PSS is unknown but autoimmune mechanisms and primary damage to blood vessels have been proposed to explain its pathogenesis. We report a case of PSS with widespread depigmentation resembling vitiligo.

Adult↗

PubMed1997

Antiendothelial cell antibodies: useful markers of systemic sclerosis.

BACKGROUND: Systemic sclerosis (SS) encompasses a wide spectrum of clinical presentations. Antiendothelial cell antibodies (AECA) in patients with primary Raynaud's phenomenon (PRP), limited SS (lSSc), or diffuse SS (dSSc) may help to determine the long-term prognosis of the disease. METHODS: Twenty-seven normal controls, 13 patients with PRP, 36 with lSSc, and 31 with dSSc were included in the study. Sera were examined for the presence of AECA, using a cellular enzyme-linked immunosorbent assay (ELISA). Angiotensin-converting enzyme (ACE) activity, plasma von Willebrand factor antigen (vWfAg), and thrombomodulin (Tm) concentrations were also evaluated. The medical records of 50 of the lSSc and dSSc patients were reviewed and the organ system involvement noted. RESULTS: Antiendothelial cell antibodies were present in 3 patients with PRP, 16 patients with lSSc, and 26 patients with dSSc. These autoantibodies were mainly of the IgG isotype. There was no difference in ACE activity between patients and controls. In contrast, vWfAg and Tm concentrations were higher in patients with PRP relative to controls, and higher in patients with lSSc compared with those with PRP. The presence of AECA was associated with digital scars and ulcers (P < 0.004 and P < 0.003, respectively), severe RP (P < 0.01), grade 3 tortuosity of vessels (P < 0.0004), and lung involvement (P < 0.02). CONCLUSION: The significant trend for AECA to increase with disease severity across the three groups of patients studies suggests that the AECA test can identify subsets of SSc with differing prognoses.

Adolescent↗

PubMed2001

Secondary amyloidosis in progressive systemic sclerosis.

Progressive systemic sclerosis (PSS) is a connective tissue disease that may affect many organs, including the kidneys. It is quite rare to see secondary amyloidosis due to PSS. We present a patient with a 9-year history of PSS who developed nephrotic syndrome, and whose renal biopsy was compatible with secondary amyloidosis. He died from massive upper gastrointestinal bleeding caused by oesophageal telangiectasia.

Amyloidosis↗

PubMed1982

Clinical problems: progressive systemic sclerosis.

Progressive systemic sclerosis is a disease of uncertain etiology associated with vascular/collagen tissue changes that may affect only the skin (scleroderma) or any and all internal organs (PSS). A disease primarily of Caucasian females, PSS is usually manifested in the fourth to sixth decade of life. Clinical and laboratory diagnostic testing is often confused by overlap phenomena, but recent research has increased diagnostic specificity. There is no known cure for PSS. Nevertheless, the knowledgeable clinician has an important role to play in the PSS patient's future. Symptom management and function maintenance will require an ongoing and understanding relationship between practitioner and patient.

Adult↗

PubMed1991

Immunogenetic prediction of pulmonary fibrosis in systemic sclerosis.

75 systemic sclerosis patients were independently tested for pulmonary fibrosis, autoantibodies, and MHC class II genes. 24 of 42 (57%) patients with pulmonary fibrosis had either HLA DR3/DRw52a or anti-Scl-70 vs 2 of 33 (6%) patients without pulmonary fibrosis. The presence of DR3/DRw52a or anti-Scl-70 gives a relative risk of 16.7 for the development of pulmonary fibrosis in a patient with scleroderma--a risk substantial enough to require careful monitoring of these patients and treatment at an early stage of disease.

Autoantibodies↗

PubMed1995

Skeletal muscle pathology in systemic sclerosis.

OBJECTIVE: Systemic sclerosis (SSc) is characterized by abnormal deposition of collagen in the skin and by visceral involvement. Muscle weakness is a relatively frequent complication of SSc, although severity varies. We studied muscle pathology in patients with SSc with progressive muscle involvement. METHODS: We performed histochemical and immunohistochemical investigations to detect neural cell adhesion molecule (NCAM). RESULTS: Five of the 6 cases of SSc expressed NCAM in atrophic angulated fibers (some fibers stained heavily with oxidative enzymes). CONCLUSION: Neurogenic involvement in SSc is more frequent than reported.

Adolescent↗

PubMed2002

Effects of cisapride on colonic transit in patients with progressive systemic sclerosis.

Progressive systemic sclerosis (PSS) may involve any portion of the gastrointestinal tract, including the colon. Constipation is common in patients with PSS. Cisapride, a benzamide derivative, is a potentially useful agent in the treatment of chronic idiopathic constipation. The effect of cisapride on colonic transit was evaluated in 16 PSS patients by a radionuclide colonic transit method. Each patient received cisapride orally three times a day for a week. The results showed acceleration in colonic transit in response to cisapride. We conclude that cisapride is effective in the treatment of constipation in patients with PSS.

Adult↗

PubMed2005

Quantitative echographic analysis of photochemotherapy on systemic sclerosis skin.

Systemic sclerosis (SSc) is a connective tissue disease of unknown etiology, in which development of fibrosis, vascular insufficiency and inflammatory processes are prominent in the skin as well as in other organs. We studied the effect of photochemotherapy with quantitative echography in SSc. Dermal echo intensity and dermal thickness was measured using high-frequency dermal echography before and after therapy. The dermal echo intensity after photochemotherapy (33.51+/-9.34) significantly increased than that before therapy (21.23+/-6.00) (P < 0.01), while dermal thickness (1.20+/-0.20) significantly decreased than that before therapy (1.38+/-0.18) (P < 0.05). Photochemotherapy was more likely to improve dermal edema, not fibrosis, because echo intensity after treatment was significantly elevated with that before treatment in patients with edema. Quantitative echographic analysis was concluded to be a reliable method in evaluating the change of skin edema in SSc.

Adult↗

PubMed1984

Periodontal ligament changes in patients with progressive systemic sclerosis.

Progressive systemic sclerosis (PSS) is a generalized collagen disease which has oral manifestations, including widening of the periodontal ligament (PDL) space. The purpose of this study was to measure the extent of thickening of the PDL space in a group of 26 patients with PSS and in a similar group of age and sex-matched control patients. The width of the PDL was measured from periapical radiographs at four sites around seven selected teeth for every patient. The mean PDL width was found to be larger in all the patients with PSS than in the control patients, around both anterior and posterior teeth. One third of the teeth of patients with PSS had PDL widths thicker than the range observed in the control patients. When average thickness of PDL widths for each patient is compared, at least 65% of the patients with PSS have mean PDL widths which are greater than the range for control patients. The increased thickness of the PDL space associated with PSS is more pronounced in posterior teeth than in anterior teeth.

Adult↗

PubMed2004

[Nuclear auto-antibodies: a useful tool for the diagnosis, the classification and the prognosis of systemic sclerosis].

PURPOSE: Systemic sclerosis (SSc) like other connective tissue diseases is characterized by the occurrence of antinuclear auto-antibodies (ANA). We reviewed the diagnostic value and prognostic value of ANA in SSc. CURRENT KNOWLEDGE AND KEY POINTS: LeRoy proposed new criteria of limited forms of SSc which take into account the immunological profile of the patients. Anticentromere, antitopoisomerase I, antifibrillarin, anti-PM-Scl, antifibrillin or anti-RNA-polymerase I or III are considered as specific anti-autobodies. Whereas anticentromere antibodies are more associated with limited SSc and antitopoisomerase I with diffuse SSc, other auto-antibodies were associated with other organ involvement : anti-RNA-polymerase I or III and renal crisis, anti-U1-RNP and pulmonary hypertension and pulmonary fibrosis, etc. Patient with persistent antitopoisomerase I antibodies have a poor survival, those with antitopoisomerase antibodies that disappear with time have a better prognosis. FUTURE PROSPECTS AND PROJECTS: A better identification of the patients with SSc, an individual biological profile and organ involvement may help better management of the disease and better access to clinical trials. The development of new immunological tests and their clinical application should help us in that way.

Antibodies, Antinuclear↗

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↗

PubMed2003

[Neurological involvement in systemic sclerosis].

INTRODUCTION: Systemic sclerosis (SS) is recognized as the connective tissue disease which less frequently presents neurological complications; in recent studies it is demonstrated, however, that neurological involvement in SS is more frequent of what it had been assumed. PATIENTS AND METHODS: Clinical neurological exploration was done in 26 patients with definitive SS; an electroneurogram was carried out in 23 cases in order to determine the prevalence of central neurological pathology and of peripheral neuropathy, to define its characteristics, and to investigate possible associations with clinical parameters and with autoimmunity. RESULTS: 23 cases (88%) were females and 3 cases (12%) males; the median age was 57.5 12.0 (SD) years, while the median age to the diagnosis was 51.3 12.3 (SD) years and the median period of natural history of disease was 6.2 3.1 years. Seven patients (26.9%) showed involvement of the CNS, being the headache and the neuropsychiatric manifestations the most common conditions (11.5%). Peripheral neuropathy prevalence was 39.1% (9 cases); according to the distribution of the injury, the polyneuropathy prevailed in 30.4% of cases. With regard to the functional selectivity, the sensitive-motor forms were most frequent (55.6%); according to the most involved structure, the axonal neuropathy was most common (44.4%).Discussion. The possible pathogenic mechanisms of the neurological pathology in this disease are discussed.

Adult↗

PubMed1993

[Computed tomography in the follow-up of interstitial lung disease in progressive systemic sclerosis].

Progressive systemic sclerosis (PSS) is a chronic multisystemic disease characterized by vascular changes, lung fibrosis and skin conditions. The lung is one of the most commonly involved organs in the patients suffering from this disease. Lung changes, along with heart involvement, represent one of the major causes of death in PSS patients. CT and high-resolution computed tomography (HRCT) scans and chest radiographs were obtained in 26 patients with PSS. The evaluation of chest films included the assessment of an average profusion score. The HRCT evaluation included pattern, extent and distribution of the disease. HRCT scans showed thickened septal lines in all patients and parallel subpleural lines in 6 patients (23%), while ground-glass opacification was seen in 2 cases (7.6%). Honeycomb pattern was observed in 8 patients (31%). Chest films showed definite interstitial opacification patterns in 8 cases only; 6 radiographs were equivocal and 12 (46%) normal. Five patients were followed 6 months to 3 years: 2 of them exhibited parallel worsening of skin conditions and of CT patterns, while X-ray findings in the chest were unchanged. In 2 cases skin and lung involvement were different (skin worsening and unchanged lung patterns, or viceversa). Finally, in 1 patient, the presence of patchy areas of air-space consolidation without air cysts on second-look was more consistent with bronchiolitis obliterans organizing pneumonia.

Adult↗

PubMed2000

Endothelial injury in internal organs of University of California at Davis line 200 (UCD 200) chickens, an animal model for systemic sclerosis (Scleroderma).

Systemic sclerosis (SSc) is a multisystem disorder characterized by mononuclear cell infiltration and fibrosis. Using skin samples from human SSc and UCD 200 chickens, which spontaneously develop a hereditary disease closely resembling human SSc, we have shown previously that endothelial cell apoptosis is a primary event in the pathogenesis of SSc. The aim of the present study was to investigate the initial disease stage in visceral organs of UCD 200 chickens with special emphasis on endothelial apoptosis, mononuclear cell infiltration and collagen deposition using tissue samples from oesophagus, lung, heart, kidney and liver. Apoptotic endothelial cells were detected by terminal deoxynucleotidyl transferase-mediated FITC-dUTP nick end labeling (TUNEL), mononuclear cell infiltrates were stained with hematoxylin and eosin, and increased collagen deposition was demonstrated by Goldner staining. Apoptotic endothelial cells were detected in oesophagus, lung and kidney of UCD 200 chickens at the initial stage of the disease. No apoptotic endothelial cells were found in heart or liver of UCD 200 or in visceral organs of healthy normal UCD 058 control chickens. Oesophagus of UCD 200 chickens, which was the most affected internal organ, showed mononuclear cell infiltrations and increased deposition of collagen. Perivascular inflammatory infiltrates and collagen deposition appeared later than endothelial cell apoptosis. These data support the hypothesis that endothelial cell apoptosis initiates the disease process, followed by mononuclear cell infiltration and fibrosis.

Animals↗