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PubMed1996

Lack of association between augmentation mammoplasty and systemic sclerosis (scleroderma).

OBJECTIVE: To examine the possible association between augmentation mammoplasty and systemic sclerosis (SSc; scleroderma). METHODS: Eight hundred thirty-seven women with a clinical diagnosis of SSc, recruited as a volunteer sample from 3 university-based, tertiary care scleroderma clinical research centers, and 2,507 race-matched local control women, recruited by the technique of random-digit-dialing and frequency-matched on age, completed a questionnaire providing data on history of augmentation mammoplasty, including possible complications of the procedure. The odds ratio (OR) and 95 percent confidence interval (95% CI) for the association of augmentation mammoplasty with SSc were estimated by multivariate logistic regression analysis with adjustment for age, race and center, and by conditional logistic regression analysis with adjustment for age. RESULTS: Eleven (1.31%) of the 837 cases reported a history of augmentation mammoplasty prior to diagnosis of SSc, compared with 31 (1.24%) of the 2,507 controls. The adjusted OR from the unmatched analysis was 1.07 (95% CI 0.53-2.13), while that from the matched analysis was 1.11 (95% CI 0.55-2.24). CONCLUSION: These results fail to demonstrate a significant association between augmentation mammoplasty and SSc, and are consistent with those reported from other epidemiologic studies.

Adult↗
PubMed2004

A modified model of graft-versus-host-induced systemic sclerosis (scleroderma) exhibits all major aspects of the human disease.

OBJECTIVE: Diffuse systemic sclerosis (SSc; scleroderma) is a debilitating disease characterized by excessive dermal fibrosis with later progression to internal organs. In addition to the fibrotic component, major aspects of the disease include vascular or circulatory involvement and immune dysregulation evidenced by inflammatory cells in affected tissues and production of autoantibodies. Many animal models resembling this disease have been studied, including genetic models in mice and chickens, challenge with chemicals such as bleomycin or vinyl chloride to induce fibrosis, and models of graft-versus-host (GVH)-induced disease using certain strains of mice with differences in minor histocompatibility loci. The present studies were undertaken to determine if alteration of the induction of GVH-induced scleroderma could result in a model that more fully represented the human condition. METHODS: Disease was induced by injection of spleen cells from B10.D2 mice into BALB/c mice deficient in mature T and B cells (recombination-activating gene 2 targeted). Dermal thickening, collagen deposition, vasoconstriction, and parameters of immunity were analyzed. RESULTS: Similar to the human disease, this modified GVH model of SSc demonstrated evidence of dermal thickening, particularly in the extremities, progressive fibrosis of internal organs, vasoconstriction and altered expression of vascularity markers in skin and internal organs, early immune activation, inflammation in skin and internal organs, and autoantibody generation. CONCLUSION: This modified model of GVH-induced SSc exhibits all major components of human disease and is likely to contribute to better understanding of the disease mechanisms and, ultimately, improved treatments for patients.

Animals↗
PubMed2004

Hepatocyte growth factor both prevents and ameliorates the symptoms of dermal sclerosis in a mouse model of scleroderma.

Systemic sclerosis (SSc) is a connective tissue disorder with an unknown etiology. There are currently no effective therapies for SSc. (In this study, working with a bleomycin(BLM)-induced scleroderma model mice, we performed two transfections of human hepatocyte growth factor (HGF) cDNA into the skeletal muscle and showed that this treatment not only helped to prevent the dermal sclerosis simultaneously injected BLM but also improved the symptoms of dermal sclerosis induced by BLM 4 weeks previously.) RT-PCR, ELISA and an immunohistochemical analysis revealed that both mRNA and protein of human HGF as well as murine HGF were enhanced in the skin, lung, muscle and the serum after two transfections of human HGF cDNA. These analyses also revealed that this treatment significantly reduced both the expression of the TGF-beta1 mRNA and the production of TGF-beta1 on macrophage-like cells that infiltrated the dermis and the fibroblastic cells in BLM-induced scleroderma. Furthermore, HGF-gene transfection both prevented and ameliorated the symptoms of not only dermal sclerosis but also of lung fibrosis induced by a subcutaneous BLM injection. These results indicated that gene therapy by the transfection of the human HGF cDNA may thus be a useful therapy for SSc and lung fibrosis involved with SSc.

Animals↗
PubMed1988

Analysis of the oral manifestations of systemic sclerosis (scleroderma).

Thirty-one female patients with systemic sclerosis and 30 age- and sex-matched control subjects underwent a clinical and radiographic oral examination. The intercommissural distance, the interincisal distance, and the maximum oral aperture were all reduced in patients with systemic sclerosis. Xerostomia was present in 70% of the patients and was associated with an increased frequency of dental caries. Diseased, missing, and filled surfaces, mobile teeth, and periodontal disease were more prevalent in patients with systemic sclerosis than control subjects. Periodontal membrane width was increased in patients with systemic sclerosis and involved all groups of teeth. Mandibular erosions were seen in 9 of 31 patients and involved the mandibular angle, the condylar head, the coronoid process, and the digastric region. These lesions were associated with the presence of periodontal membrane thickening and were seen in patients with more severe systemic sclerosis.

Adult↗
PubMed1994

[Systemic sclerosis (scleroderma): etiology and pathogenesis--where are we today?].

Systemic sclerosis is a rare chronic disease with inflammatory and vascular features leading to increased fibrosis of skin and internal organs. The pathogenesis of systemic sclerosis and scleroderma like diseases appears to be influenced rather by environmental than by genetic factors. Clinical and pathogenetic aspects of systemic sclerosis show similarities to untreated chronic graft-versus-host-disease after bone marrow transplantation. New insights into the pathogenesis of systemic sclerosis give reason to hope that better therapeutic approaches will be developed in the future.

Adult↗
PubMed2000

Development and evaluation of a patient education program for persons with systemic sclerosis (scleroderma).

OBJECTIVE: To develop and evaluate the contents and disposition of a systemic sclerosis (SSc; scleroderma) patient education program. METHODS: Six women with SSc constituted the study group. Contents of the course were decided by the rheumatology team and were based on health assessments of the study participants made through medical examinations, measures of subjective symptoms, disability, physical and psychological function, and perceived self-efficacy, as well as the patients' opinions. The self-efficacy theory was used as the main theoretical basis for the education program. RESULTS: Both self-report and observer-scored measurements of disease impact and function were required to create the contents of the course. The patients reported that the professional medical information met their needs, although skin care and pain are topics that need additional work in the curriculum. The participants were satisfied with the program and the patient contacts, but found goal-setting to be an unfamiliar task. CONCLUSION: This pilot study of a multidisciplinary SSc education program fulfilled the general goals stated concerning the topics included and provided the opportunity for SSc patients to meet others with the same diagnosis. A larger study group is needed in order to analyze the effects of the course and the measurements used.

Aged↗
PubMed2005

Increased expression of p53 and p21 (Waf1/Cip1) in the lesional skin of bleomycin-induced scleroderma.

Systemic sclerosis (SSc) is a connective tissue disorder characterized by excessive deposition of extracellular matrix in the affected skin as well as various internal organs, vascular injury and immune abnormality; however, the etiology of SSc remains still unknown. We previously established an experimental mouse model for scleroderma by repeated local injections of bleomycin, a DNA damaging agent. In this study, we examined the induction of apoptosis and the expression of p53, p21 (Waf1/Cip1), and proliferating cell nuclear antigen (PCNA) in the lesional skin following bleomycin exposure in this model. Dermal sclerosis was induced by alternate day's injections of bleomycin for 4 weeks. TUNEL assay showed that apoptotic cells began to appear at 1 week after bleomycin exposure, and were prominently detected at 3-4 weeks. Immunohistochemical examination showed increased expression of p53 and p21 mainly in the infiltrating mononuclear cells at 2 weeks after bleomycin treatment. Bleomycin treatment markedly enhanced PCNA expression at 1-2 weeks, mainly in mesenchyme, as compared with control phosphate buffered saline treatment. Reverse transcriptase-polymerase chain reaction analysis showed that the expression of p53 and p21 mRNA was concurrently upregulated at 1-2 weeks after bleomycin treatment. Taken together, coordinate increased levels of p53 and p21 preceded the maximal induction of apoptosis and dermal sclerosis. Our findings suggest that apoptotic processes are involved in the pathophysiology of bleomycin-induced scleroderma, which may be mediated, in part, by the upregulation of p53 and p21.

Animals↗
PubMed1983

Immunofluorescence studies in progressive systemic sclerosis (scleroderma) and mixed connective tissue disease.

Immunofluorescence (IF) investigations of the skin were performed in thirty patients with progressive systemic sclerosis (scleroderma) and eight patients with mixed connective tissue disease (MCTD). The results show that speckled epidermal nuclear immunoglobulin deposition occurs not only in MCTD but also in true scleroderma. Granular IgM deposition at the dermo-epidermal junction of light-exposed skin was detected in both groups of patients, but six of eight MCTD patients also showed a granular IgM band in non-exposed skin. Antinuclear antibodies (ANA) were demonstrated in the sera of 96% and 100% of patients with scleroderma and MCTD respectively. The pattern of nuclear IF staining in scleroderma included dense fine speckles, large coarse speckles, threads, nucleolar and centromere staining. In MCTD, by contrast, the ANA staining pattern consisted of threads. The significance of ANA titres and immunological specificities for the in vivo reaction of serum ANA with epidermal nuclear antigens is discussed.

Adult↗
PubMed2003

Role of monocyte chemoattractant protein-1 and its receptor,CCR-2, in the pathogenesis of bleomycin-induced scleroderma.

Systemic sclerosis is a connective tissue disease characterized by excessive deposition of extracellular matrix in the skin as well as various internal organs. Cellular infiltrates are found in the dermis in early systemic sclerosis, which are suggested to play an important part. Recent studies suggest the involvement of monocyte chemoattractant protein-1, a C-C chemokine, in the fibrotic process. This study examines the role of monocyte chemoattractant protein-1 in the induction of dermal sclerosis in a murine model of bleomycin-induced scleroderma. Immunohistochemical analysis showed that expression of monocyte chemoattractant protein-1 in the infiltrating mononuclear cells was enhanced at 2 to 3 wk following bleomycin treatment, whereas expression of monocyte chemoattractant protein-1 in fibroblasts was detected at later stages in the sclerotic skin. Reverse transcriptase-polymerase chain reaction analysis showed that monocyte chemoattractant protein-1 mRNA expression in the lesional skin peaked at 2 to 3 wk following bleomycin treatment. Expression of CCR-2, a major receptor for monocyte chemo-attractant protein-1, was also upregulated in the lesional skin at both protein and mRNA levels following bleomycin treatment. Administration of anti-monocyte chemoattractant protein-1 neutralizing antibody together with local bleomycin treatment reduced dermal sclerosis, along with a decrease of collagen content in the skin as well as mRNA expression of type I collagen. In vitro analysis showed that stimulation with monocyte chemoattractant protein-1 (10 ng per mL) upregulated alpha1(I) collagen and decorin mRNA expression in normal dermal fibroblasts, whereas mRNA levels of fibronectin and biglycan were not altered. These data suggest that monocyte chemoattractant protein-1 and CCR-2 signaling plays an important part in the pathogenesis of bleomycin-induced scleroderma. Monocyte chemoattractant protein-1 may contribute to the induction of dermal sclerosis via its direct effect of upregulation of mRNA expression of extracellular matrix on fibroblasts, as well as indirect effect mediated by a number of cytokines released from immunocytes recruited into the lesional skin.

Animals↗
PubMed1975

Immunologic reactivity to incompatible blood group substances of the ABO system in scleroderma.

The immunization with incompatible blood groups substances of the ABO system yielded distinct increases in the isohemagglutinin titers and their scores in 16 patients suffering from systemic sclerosis (scleroderma) and in 16 controls. The difference between these two groups was not statistically significant, which suggests an intact humoral immunological reactivity of these antigens. However, investigations with further antigens would be necessary to judge the immunological factors supposed to be involved in the pathogenesis of systemic sclerosis. The method may be recommended for testing the humoral immunological response in similar problems.

ABO Blood-Group System↗
PubMed1996

The nervous system in systemic sclerosis (scleroderma). Clinical features and pathogenetic mechanisms.

The involvement of the nervous system in SSc is well recognized today. Different pathogenetic mechanisms are suggested that may alternatively explain the multiform appearance of the clinical spectrum (mononeuritis, mononeuritis multiplex, carpal tunnel syndrome, and so forth). It is now clear that the ANS is the earliest structure targeted by the disease in the gastrointestinal tract. The importance of this observation has not yet been adequately interpreted but may, together with the increasing evidence of the nervous system involvement in SSc, become a leading factor in understanding of the importance of the nervous system in the onset, development, and maintenance of the disease.

Autonomic Nervous System Diseases↗
PubMed1978

Association of HLA antigen a9 with progressive systemic sclerosis (scleroderma).

Upon evaluation of 40 subjects with progressive systemic sclerosis (PSS), a significant association of HLA antigens A9 and Aw24 (a subgroup of A9) was found with diffuse scleroderma. HLA-Aw23 (a second subgroup of A9) was also increased in the patients, however, this was not statistically significant. Diffuse scleroderma appears to be one of the few diseases that shows an association with the HLA-A locus.

Epitopes↗
PubMed1984

Neurological manifestations in systemic sclerosis (scleroderma).

One hundred and twenty-five patients with systemic sclerosis were surveyed for neurological manifestations in a prospective study. Seven (5.6%) were found to have a defined neurologic lesion: 4 with carpal tunnel syndrome and one each with trigeminal neuralgia, mononeuritis multiplex and peripheral neuropathy. Neurological involvement occurs but is uncommon in this connective tissue disorder.

Adult↗
PubMed1988

Correlates between autoantibodies to nucleolar antigens and clinical features in patients with systemic sclerosis (scleroderma).

Immunofluorescence on rat liver sections was used to select high-titer antinucleolar antibodies (ANoA) in the sera of patients with systemic sclerosis (scleroderma). In 646 patients, 53 ANoA sera (8%) were identified, and of these, 46 were available in sufficient quantities for further analysis. The complex of RNA polymerase I was immunoprecipitated by 7 sera (15%), which uniformly produced punctate nucleolar staining. The PM-Scl antigen, a particle consisting of 11 polypeptides, was immunoprecipitated by 8 sera (17%), all of which displayed homogeneous nucleolar staining. A 34-kd nucleolar protein (fibrillarin) of the U3 RNP complex was positive in immunoblotting of 22 sera (48%), which characteristically produced clumpy nucleolar staining. Antibodies against RNA polymerase I were associated with diffuse scleroderma of short duration, which was characterized by a high prevalence of internal organ involvement, including renal crisis. Anti-U3 RNP antibodies had a high prevalence in men with significantly less joint involvement, compared with ANoA-negative patients. Anti-PM-Scl antibodies identified a group of scleroderma patients with a high prevalence of concomitant myositis and renal involvement.

Antibodies, Antinuclear↗
PubMed2002

[Immunological diagnosis of scleroderma].

Systemic sclerosis is characterized by a fibrosis and a microvascular injury. These vascular lesions can affect internal organs causing severe visceral damages. The pathogenesis of this disease is complex but some immunological disorders with a production of antinuclear auto-antibodies can be useful to the clinicians, especially for an early diagnosis and to distinguish the scleroderma/myositis overlap syndromes. This review presents the target antigens and the methods of detection of the different antinuclear auto-antibodies. Sensitivity, specificity and clinical association between the antinuclear auto-antibodies and the different subclasses of scleroderma are also presented in detail.

Autoantibodies↗
PubMed1993

Central nervous system involvement and psychiatric manifestations in systemic sclerosis (scleroderma): clinical and neurophysiological evaluation.

We evaluated central nervous system and psychiatric involvement in a clinical sample of 32 patients with systemic sclerosis (SSc) (scleroderma). All patients underwent clinical neurological examination. Electroencephalography (EEG) and visual evoked potentials (VEPs) were also recorded. Prominent central nervous system (CNS) or psychiatric symptoms were present in 5 patients (16%), including encephalopathy, psychosis, anxiety disorder, grand mal seizures and transient ischemic attack. In addition, abnormal VEPs were recorded from 5/32 patients (16%), suggesting optic neuropathy. EEGs were mainly normal or showed only slight, nonspecific changes. Primary CNS involvement in scleroderma, however, could not be shown in any of the 5 cases with neuropsychiatric symptoms. Our results suggest that neuropsychiatric symptoms in SSc are, if not coincidental, indirectly caused by internal organ involvement of SSc or by possible overlapping connective tissue diseases. On the other hand, optic neuropathy might be a primary complication of SSc.

Adult↗
PubMedDate not supplied

Spinal cord compression by ectopic calcinosis in scleroderma.

Systemic sclerosis (SS) is a chronic, multisystemic disease, characterized by inflammation associated with fibrosis. Calcinosis is one of the manifestations of this disorder, observed in 10 to 20% of cases. It is usually located on the extensor surface of the phalanges, peri-articular tissue and near the bone prominences. There are only a few cases reported of SS with vertebral column involvement and spinal cord compression by calcinosis causing serious neurological complications. We describe a fatal case of SS who showed tetraplegia secondary to ectopic calcinosis in the cervical medulla and present a review of the literature on the subject.

Calcinosis↗