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PubMedDate not supplied

Comparison of high frequency (20 MHz) ultrasound and palpation for the assessment of skin involvement in systemic sclerosis (scleroderma).

BACKGROUND AND OBJECTIVES: Systemic sclerosis (SSc) is a connective tissue disease characterized by microvascular changes and fibrosis of the skin and internal organs. Increased skin thickness proximal to the metacarpophalangeal joints is the single major diagnostic criterion. The aim of this study was to evaluate high frequency (20 MHz) ultrasound for the assessment of skin thickness in patients with SSc of different disease durations. METHODS: Skin thickness was measured with high frequency (20 MHz) ultrasound equipment (Dermascan) in 41 patients with SSc (23 women and 18 men) and in 41 controls. Twenty-five patients had limited cutaneous systemic sclerosis (ISSc), 12 had diffuse cutaneous systemic sclerosis (dSSc) and 4 had suspected SSc. RESULTS: Skin thickness of the forearm was inversely correlated to disease duration. Compared to controls, skin thickness was increased over the proximal phalanx of the right second finger and over the forearm in patients with a disease duration of 2 years or less. Assessments of skin thickness in 10 controls by 2 independent investigators showed an inter-observer variability of 1.0% for the proximal phalanx and 0.0016% for the forearm. Patients whose ultrasound showed increased skin thickness on the hand and forearm also had thickened skin by palpation. CONCLUSION: High frequency (20MHz) ultrasound is a feasible method for measuring skin thickness in SSc, and may be useful for diagnosis, long-term follow-up, and assessment in therapeutic studies.

Adult↗
PubMedDate not supplied

[Systemic sclerosis (scleroderma) without scleroderma].

There are two approaches toward dealing with systemic sclerosis (scleroderma) presenting with no skin involvement. According to one of these the condition is to be treated as atypical systemic sclerosis (scleroderma). With the other approach it does not make much difference whether systemic sclerosis presents with skin involvement or not. In the study made a group of systemic sclerosis patients presenting with no skin involvement was examined vs patients with circumscribed skin involvement. No evidence has been found for systemic sclerosis (scleroderma) sine scleroderma to be regarded as running an atypical course.

Adolescent↗
PubMed1996

Detection of scleroderma with capillaroscopic abnormalities of nailfolds.

BACKGROUND: Systemic scleroderma is a problem in Gifu Prefecture, Japan. METHODS: Three hundred and thirty-two men and 731 women over 30 years of age, who attended the residents' health examination in K town of Gifu Prefecture (population 4835 persons over 30 years of age) had a dermatologic examination. Antinuclear antibody was determined in 85 persons (14 men, 71 women; age 30 to 72 years) who had Raynaud's phenomenon or various clinical features related to systemic scleroderma. Of these 85 persons, 19 agreed to a capillaroscopic examination and of these, 16 showed some capillaroscopic abnormalities. Biopsy specimens of three women among these 16 individuals with capillaroscopic abnormalities were examined also histopathologically. RESULTS: All samples taken from the forearm skins showed thick and packed bundles of collagen with hyalinization and thickened small blood vessels in the dermis, similar to histopathologic features of systemic scleroderma, although all these women had only a sausage-like swelling of the fingers and a shortened frenulum of the tongue. These results suggest that the prevalence of systemic scleroderma can be estimated to affect more than 0.38% of the population in this town. CONCLUSIONS: Manual skills and vibration exposure may be associated with systemic scleroderma.

Adult↗
PubMed2005

Targeted therapy comes of age in scleroderma.

Systemic sclerosis (SSc, also known as scleroderma) has the highest case-specific mortality among the rheumatic diseases; however, advances in understanding of pathogenesis and an appreciation of the clinical heterogeneity of this disease, together with therapeutic advances in other areas, have raised the possibility of substantial improvements in its management. Key areas of advance include the development of clinical and laboratory methods for early detection of complications and the integration of vascular, immunomodulatory and antifibrotic therapies. Disease models have facilitated the identification of key mediators or processes that could be targeted therapeutically and have provided a platform for preclinical testing of novel candidate therapies. This review will consider current clinical aspects of SSc and the emergence of targeted therapy that is linked to key pathogenic processes or that targets pivotal mediators.

Age Factors↗
PubMed1982

Cardiovascular function in patients with progressive systemic sclerosis (scleroderma).

Sixteen patients with progressive systemic sclerosis (PSS), including 3 with the "CREST" (calcinosis, Raynaud's, esophageal dysfunction, sclerodactyly, and/or telangiectasias) variant, were evaluated with resting M-mode echocardiography and noninvasive measurements of cardiac output at rest and during submaximal exercise to determine the nature and extent of any cardiovascular impairment. No patient had arterial hypertension, significant renal impairment, clinical evidence of large vessel coronary artery disease, or severe pulmonary dysfunction. The duration of disease was 1 to 12 years (9 to 30 for patients with the CREST variant). Echocardiographic abnormalities included increased right ventricular dimension (3 patients), reduced left ventricular ejection fraction (3 patients), and pericardial effusion (3 patients). Cardiac index (CI) and stroke volume index (SVI) at rest were similar for patients and controls. Patients and controls were exercised to similar heart rates (130 +/- 3 vs 124 +/- 4; p, NS). Total peripheral resistance (TPR) was higher for patients (1123 +/- 81 vs 810 +/- 44 dyn X s X cm-5) and their mean SVI failed to increase significantly compared with sitting rest values (30 +/- 2 vs 35 +/- 3 ml/m2). The control subjects had the expected increase in SVI (36 +/- 2 vs 51 +/- 5; p less than 0.01). Ten patients with an abnormal hemodynamic response to exercise had a normal echocardiographic circumferential fiber shortening (VCF) or ejection fraction (EF) at rest. The data indicate that PSS patients have a greater degree of cardiovascular dysfunction than would be predicted from clinical data and laboratory evaluation of cardiovascular and pulmonary function at rest. Multiple mechanisms, including right and left ventricular dysfunction and abnormal vasoconstrictor activity, are likely to contribute to the reduction in exercise capacity seen in patients with PSS.

Adult↗
PubMed1995

Do silicone breast implants cause autoimmune rheumatic diseases?

Current estimates are that up to a million women in the U.S. have breast implants with the predominant type being the silicone gel implant. Concerns have been raised regarding the safety of silicone gel breast implants with focus upon whether escaped gel might cause inflammatory and immune responses that subsequently lead to autoimmune rheumatic diseases such as progressive systemic sclerosis (scleroderma), systemic lupus erythematosus (SLE), Sjögren's syndrome or rheumatoid arthritis. A spectrum of illnesses ranging from local symptoms to systemic disease is seen in some patients with silicone breast implants, however, it remains to be determined whether such illnesses in these patients are coincidentally associated or are secondary to the implants. Our understanding of the relationship between the presence of autoimmune rheumatic diseases and silicone breast implants is limited. The available data indicate that silicone elicits a minimal immunological response as compared to conventional antigens. The histological, immunological and epidemiological experimental data derived from patients with silicone implants, as well as those from animal studies, are reviewed. These data do not convincingly demonstrate that there is a cause and effect relationship between silicone breast implants and autoimmune diseases. Further investigations are needed to clarify the interaction of silicone with the cellular and humoral immune systems, as well as with host and environmental factors.

Antibodies, Antinuclear↗
PubMed1990

Features of systemic sclerosis (scleroderma) in South African goldminers.

Systemic sclerosis is more common among men exposed to silica-containing dust than in the general male population. The clinical features of systemic sclerosis in a group of 24 black goldminers are described. The better-known presenting features of systemic sclerosis, including Raynaud's phenomenon and dysphagia, were rare in this population. Initial presentation was usually with nonspecific features including swelling of the feet or hands, weakness, arthralgia or symptoms of respiratory or cardiac disease. Clinical evidence of pleural or pericardial involvement was more common than is usually described in non-occupational systemic sclerosis. Interstitial lung disease was frequently encountered and renal disease was rare.

Adult↗
PubMed2001

Systemic sclerosis (scleroderma): specific autoantigen genes are selectively overexpressed in scleroderma fibroblasts.

The pathogenesis of systemic sclerosis (SSc) involves complex interactions between activated fibroblasts eventually leading to fibrosis, and impaired immune tolerance characterized by a variety of circulating SSc-specific autoantibodies. The expression of autoantigens in fibroblasts, a key target tissue in SSc, may play an important role in this process. To obtain a global view of this process, we examined gene expression profiles of SSc dermal fibroblasts using cDNA microarrays. The results show that dermal fibroblasts from SSc patients obtained from either affected or unaffected skin displayed a characteristic pattern of increased SSc autoantigen gene expression compared with that from normal controls. In particular, fibrillarin (p = 0.028), centromeric protein B (p = 0.01), centromeric autoantigen P27 (p = 0.042), and RNA polymerase II (220 kDa; p = 0.02) were significantly overexpressed in SSc fibroblasts. Quantitative RT-PCR confirmed overexpression of these autoantigens and also revealed increased levels of DNA topoisomerase I transcripts in SSc fibroblasts compared with normal control fibroblasts (p = 0.0318). The polymyositis/scleroderma autoantigen gene was overexpressed in some SSc patients (p = 0.09). To examine the specificity of these overexpressed autoantigen genes for SSc and its tissue specificity for fibroblasts, cDNA microarrays of dermal fibroblasts from patients with eosinophilic fasciitis and scleromyxedema were studied as well as PBMC and muscle biopsies from SSc patients. None of these tissues showed significant alterations in gene expression of SSc-specific autoantigens. Therefore, SSc-associated autoantigen genes are selectively overexpressed in SSc dermal fibroblasts, a major tissue involved in disease pathogenesis.

Autoantigens↗
PubMed1990

Autoantibodies against nuclear, nucleolar, and mitochondrial antigens in systemic sclerosis (scleroderma).

One of the most characteristic serologic features of systemic sclerosis (scleroderma) is the occurrence of autoantibodies against nuclear and most notably against nucleolar antigens. This humoral autoimmune response is one of best studied immunologic phenomena in scleroderma. Detailed molecular information on the structure and function, as well as on reactive epitopes of autoantigens targeted by specific serum antibodies, has been revealed by clinical, immunologic, and biochemic studies in several laboratories. Autoantigens such as DNA topoisomerase I (Scl-70), centromere proteins, RNA polymerase I, U3 RNP-associated fibrillarin, PM-Scl, and 7-2 RNP antigens were shown to be specific targets of scleroderma patients and were observed to have clinical correlates within the scleroderma disease spectrum. Therefore, autoantibodies in scleroderma are not only valuable diagnostic tools but also prognosticators of the disease. Although autoantibodies in scleroderma do not appear to play a pathogenetic role in the disease process, the knowledge of the structure and function of their reactive antigens may help in answering questions concerning the etiology of the disease.

Antibodies, Antinuclear↗
PubMed1993

Exaggerated fibrosis in patients with systemic sclerosis (scleroderma) following radiation therapy.

We describe 2 patients with systemic sclerosis (scleroderma) who developed severe localized skin thickening following radiation therapy for cancer. Radiation therapy may result in intense localized areas of fibrosis in patients with scleroderma. The fibrosis may extend beyond the irradiation portals and result in damage to underlying viscera. Radiotherapy may precipitate scleroderma or cause a generalized worsening of the underlying disease.

Adult↗
PubMed1984

Lowered angiogeneic capability of peripheral blood lymphocytes in progressive systemic sclerosis (scleroderma).

Peripheral blood lymphocytes isolated from 19 patients with progressive systemic sclerosis (7 with diffuse scleroderma and 12 with CREST syndrome) and from 19 healthy control individuals were tested in a lymphocyte-induced angiogenesis assay. The cells were injected intradermally into x-ray-immunosuppressed mice and their capability to induce new blood vessel formation was assessed by morphologic criteria. The lymphocytes derived from patients with systemic scleroderma showed a significant decrease in angiogeneic capability compared with controls. No significant difference in this capability was found between patients with diffuse scleroderma and those with CREST syndrome. The decrease in the angiogeneic capability of lymphocytes reflects a depression in cell-mediated immunity and might be relevant to the capillary loss observed in systemic scleroderma.

Adult↗
PubMed1995

Systemic sclerosis (scleroderma). Clinical management of its major complications.

Systemic sclerosis (scleroderma) is a disease of unknown cause whose pathogenesis involves interaction between the vasculature, the immune system, and connective tissue cells. Although specific therapy awaits a better understanding of its pathogenesis, proper management may enhance not only the duration, but the quality of life for most scleroderma patients. Presently, controlled prospective trials of therapeutic agents are lacking. Published reports of drug therapy for skin, kidney, cardiac, pulmonary, and gastrointestinal complications, as well as management of Raynaud's phenomenon, are critically reviewed in this article, and the authors' approach to management of difficult clinical problems is presented.

Gastrointestinal Diseases↗
PubMed1980

Human respiratory nasal mucosa in progressive systemic sclerosis (scleroderma). An electron microscopic study.

The fine structure of the respiratory nasal mucosa in cases of systemic scleroderma was studied in 5 patients. The cilia were absent in all the sections examined, and the epithelium showed characteristic morphological details. Absence of the mucous elements in the sero-mucinous glands was noticed with evidence of increased activity of the serous units. The capillaries and small arterioles showed severe changes. Electron-dense deposits were seen within the various constituents of the nasal mucosa. It was assumed that the characteristics of the disease are dependent on individual reactivity of a genetically determined immune system stimulated by an undetermined factor.

Adult↗
PubMed1985

[Hemosorption in the complex treatment of rheumatic diseases].

The authors describe the results of the use of hemosorption in combined treatment of 42 patients with rheumatic diseases. The main group comprised patients with thromboangitis obliterans (23), nonspecific aortoarteritis was recognized in 6 cases, systemic scleroderma and systemic lupus erythematosus in 4, nodular periarteritis in 2. The functional skin tests were made with the aid of the KDU-3 unit, the level of the peripheral blood flow was measured with tetrapolar rheography and impedometry. The degree of redox processes and oxygen tension in the tissues was studied by the polarographic technique, the character of microcirculatory processes with the aid of biomicroscopy of the eyeball conjunctiva, the intensity of immunologic shifts by means of precipitation with polyethylene glycol at different dilutions. In addition, alterations in cellular and serum enzymes were also examined. The data obtained suggest that hemosorption is one of the effective additional methods of the treatment of patients with rheumatic diseases.

Adult↗
PubMed1992

In situ localization of interleukin-6 in normal skin and atrophic cutaneous disease.

Interleukin-6 (IL-6) is a multifunctional cytokine that participates in the inflammatory and immune responses. In human skin, keratinocytes produces IL-6, although the in vivo role of this cytokine is unknown. In the present study we investigated the in situ localization of IL-6 in normal epidermis (n = 10) and in a group of skin diseases characterized by epidermal atrophy. Formalin-fixed paraffin-embedded skin biopsies from patients with clinical and histopathological features consistent with localized scleroderma (n = 10), systemic scleroderma (n = 5), lichen sclerosus et atrophicus (n = 9) and balanitis xerotica obliterans (n = 7) were tested using polyclonal antibodies and avidin-biotin-peroxidase immunostaining. We demonstrated the presence of IL-6 in normal epidermis and in atrophic skin diseases. In normal skin there was moderate intercellular and intracellular reactivity detected using a high antibody concentration. In specimens with epidermal atrophy we detected intense cytoplasmic and intercellular immunostaining using a lower antibody concentration. The immunoreactivity was independent of the epidermal thickness. Plasma IL-6, measured by radioimmunoassay, was not elevated in plasma from patients with localized or systemic scleroderma. Increased IL-6 in the epidermis of selected skin diseases suggests that IL-6 may be related to the pathophysiology of dermatologic diseases characterized by epidermal atrophy.

Antibody Specificity↗
PubMed1993

Cyclophosphamide and low-dose prednisone therapy in patients with systemic sclerosis (scleroderma) with interstitial lung disease.

Fourteen patients with systemic sclerosis (SSc, scleroderma) and interstitial lung disease were treated with oral cyclophosphamide (1-2 mg/kg/day) and low dose prednisone (< 10 mg/day). There was a significant improvement in FVC after 6 months compared to entry values (2.21 +/- 0.19 l vs. 2.03 +/- 0.15 l, p < 0.02). Improvement was maintained at 12 months (2.27 +/- 0.27 l, p < 0.05) and 18-24 months (2.60 +/- 0.28 l, p < 0.001). In 12 cases followed for 18-24 months, FVC was stable or improved. No significant improvement or decline was noted for the DLCO. Side effects included cytopenia (2), infection (1), and hemorrhagic cystitis (2), and one possible related malignancy. A controlled prospective trial of cyclophosphamide is warranted in patients with SSc and active interstitial lung disease.

Adult↗