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PubMedDate not supplied

[An electro- and vectorcardiographic study of ventricular activation in scleroderma].

Electrical ventricular activation was studied by means of electrocardiogram and vectorcardiogram in 62 patients, 55 women and 7 men, affected by systemic sclerosis of average disease duration of 10.3 years. 45% of the patients showed the "diffused form" of the disease and 55% the "limited form". The vectorcardiogram showed some alterations of the ventricular activation in 55% of the recorded cases. The previous electrocardiogram found only 18%. The alterations, which are more frequent in the diffuse form than in the limited one, are the presence of loss of forces in septal, lateral or inferior or combined areas and intraventricular conduction disorders as complete and incomplete right bundle branch block, left anterior subdivision block, left posterior subdivision block, or combination between incomplete right bundle branch block and left anterior or posterior subdivision block. The vectorcardiogram was more sensitive than the electrocardiogram in showing loss of forces, also in patients with the limited form of systemic sclerosis, when the electrocardiogram was normal. Moreover the vectorcardiogram is helpful to identify left posterior subdivision block. The vectorcardiographic investigation seems to be a useful complement of the electrocardiogram especially for the identification of cases with myocardial involvement that is clinically silent.

Adult↗
PubMed2005

Scleroderma--developing measures of response.

The scleroderma research community continued its focus on the development and proper validation of the outcome measures for clinical trials in scleroderma during the special interest group meeting at OMERACT 7. Deliberations focused on progress in the assessment of gastrointestinal disease, renal physiology, vascular damage, and the unique challenges inherent in studying pediatric patients with scleroderma.

Adolescent↗
PubMed2005

[Pathogenesis of skin scleroderma--literature review].

The pathogenesis of skin scleroderma (LS) is still unknown. Disturbances of vessels system, connective tissue metabolism and humoral and cellular immunological response is observed. Antinuclear antibodies are detected in 30-80% of patients with different types of skin scleroderma. They are present more often in patients with disseminated lesions and linear type of LS compared to morphoea au plaque. In our own analysis 28.5% of patients had also antibodies directed against Borrelia burgdorferi. It is believed that the injury of endothelial cells and proliferation in medial part of small vessels - which both lead to chronic ischemia - are the earliest disturbances observed in histopathological examination of the skin taken from systemic as well as from skin scleroderma patients. During last few years, there were some interesting reports concerning functional changes of endothelial cells which led to disturbances in tension of vessels smooth muscles. Free radicals - in genetically predispose people--can also provoke scleroderma lesions through their injury action on endothelial cells and stimulation of fibroblasts. In morphoea, the process of fibrosis begins around vessels. Deposition of connective tissue matrix is observed, especially collagen type I and III. This stimulation of fibroblasts as well as accumulation of connective tissue matrix are secondary to some stimulatory factors. These are: PDF, bFGF, TGFbeta and some cytokines. In morphoea patients serum levels of IL-1, IL-2, IL-4, IL-6 and IL-8 were elevated. In literature, levels and production of collagenases were decreased, although more authors say that tissue inhibitors of metalloproteinases are the main factor in fibrosis. The analysis of data tends to suspicion that enormous fibrosis observed in different types of scleroderma can be the result of increased production of collagen and other components of connective tissue as well as their incomplete degradation. Presented clinical and laboratory data show how many different factors influence etiopathogenesis of morphoea.

Collagen↗
PubMedDate not supplied

Scleroderma.

Explore the source record for details and available documents.

Humans↗
PubMed2005

[Systemic sclerosis--clinical course and treatment possibilities].

Systemic sclerosis is an autoimmune disease, characterized by fibrosis of the skin and internal organs, such as esophagus, heart, lungs, and kidneys. Three main forms of systemic sclerosis are conspicuous: diffuse systemic sclerosis, limited systemic sclerosis and scleroderma sine sclerosis. Respective variants differ in skin sclerosis exacerbation, progress and prognosis; whereas organ involvement, osseous and articular alterations are comparable in all forms of the disease. Due to the fact that etiology of systemic sclerosis is not known, no specific therapy has been developed. The therapy is based on influencing main pathogenetic factors, such as peripheral circulation disturbances, abnormal immune activation and increase in collagen accumulation. An overview of clinical course of systemic sclerosis and new therapeutic modalities is provided.

Connective Tissue↗
PubMed1990

HLA-antigen frequencies in patients with progressive systemic sclerosis and morphea.

A possible HLA disease association was investigated in 40 patients (38 female, 2 male) with progressive systemic sclerosis (PSS) and 42 patients (32 female, 10 male) with morphea. The HLA A, B, C, DR phenotypes of patients were compared with 193 healthy controls. The following relative risk (RR) values were determined in PSS: A1 (1.38), A2 (1.39), B8 (1.67), B15 (3.22), DRw8 (6.30) and in morphea patients: A3 (1.43), B7 (1.39), B40 (1.81), Bw60 (2.49), DR2 (2.38) and DRw8 (2.55), indication a relatively weak, HLA-linked genetic predisposition for the manifestation of the disease. The HLA "risk" antigens for the two clinically different subtypes of the disease are also different: raised A1/B8 frequencies, like in our PSS group, correlate with a high, pathological immune response (autoimmune disorders). In contrast, A3/B7/DR2 prevalence, like in our morphea group, correlates with a low immune response. HLA typing may contribute to clinical differential diagnosis and possibly also prognosis.

Adult↗
PubMed2006

[Causes of death of patients with systemic sclerosis].

UNLABELLED: Systemic sclerosis (SSc) is a chronic, systemic connective tissue disease, characterized by progressive skin fibrosis, internal organs and disfunction blood vessels. THE AIM: of the study was o analyze death causes in patients with SSc and the assessment of relationship between clinical status, immunologic test results and survival/mortality of patients with SSc. MATERIAL AND METHODS: Case histories of all patients with SSc, hospitalized for six years (since 1'" October 1999 to 1It October 2005) in Department of Rheumatology and Internal Diseases, Medical University of Bialystok, were retrospectively analyzed. Current patients status at the end-point of study was estimated during control examination and phone contact in selected cases. The time and cause of death was based on autopsy results. RESULTS: In study group of 76 patients with SSc, 13 deaths were found (17,1%). Death cause analyses revealed that: interstitial lung disease was the main cause of death (4/13 - 30%). As a secondary cause of death in these patients neoplasms were recognized (3/13 - 23,1%). In all patients with neoplasms exudation in pleura was diagnosed. CONCLUSIONS: Pulmonary complications and neoplasms are predominant causes of death in patients with SSc. Risk factors of death are: pulmonary hypertension and myositis. The presence of exudation in pleura in patients with SSc is a bad prognostic symptom and a possible sign of neoplastic disease.

Adolescent↗
PubMed1991

Facial reconstruction consideration in rheumatic diseases.

In conclusion, the management of facial involvement in JRA, Romberg disease, and scleroderma is dictated by the degree of severity of the disease, age of onset, and length of activity. Functional occlusal abnormalities are best addressed through a team approach consisting of initial orthodontics followed by orthognathic surgery if needed. In all types of scleroderma, surgical facial reconstruction is best delayed until the disease is quiescent for at least a year. The ideal option for facial skeletal and soft-tissue augmentation has not yet been realized. Careful surgical planning and choice of grafts, flaps, or implants are critical to obtain the desired result.

Arthritis, Juvenile↗
PubMed1991

Sjögren's syndrome in patients with the CREST variant of progressive systemic scleroderma.

Twenty-three patients with the CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) variant of progressive systemic sclerosis, were clinically, histopathologically and serologically examined for the presence of Sjögren's syndrome (SS). Fourteen were found to be positive. No significant difference could be demonstrated between them and the remaining 9. Characteristics of patients with CREST were compared with those of 29 randomly chosen patients with primary SS. Parotid gland enlargement was more frequently present (p less than 0.01) in the latter than in the former. Virtually no patients with CREST with SS had antibodies to Ro(SSA)/La(SSB).

Autoantibodies↗
PubMed1991

Cutaneous and serologic subsets of systemic sclerosis.

The relevance of the extent of skin sclerosis and of other clinicoserological features in diagnosis, severity and prognosis of disease was studied in a large number of unselected patients with systemic sclerosis (SSc). One hundred and fifty-one patients with SSc (126 F and 25 M, mean age 48 +/- 14 SD) followed for 5.3 +/- 3.2 years were included. Patients were divided into 3 cutaneous subsets: limited (68), intermediate (46) and diffuse SSc (37). Serological markers were detected in 288 patients with Raynaud's phenomenon and other connective tissue diseases (CTD). Limited and intermediate SSC prevailed in female patients while the diffuse subset was more frequent in males (p less than 0.0001). Duration of Raynaud's phenomenon before disease onset was shorter in the diffuse variant (p less than 0.0001). A wider cutaneous involvement was associated with more severe forms of SSc. Diffuse subset showed the poorest prognosis at 10 years of followup compared with intermediate (p less than 0.05) and limited variant (p less than 0.001). Intermediate SSc seems a distinct variant of SSc on the basis of clinical manifestations and survival. Among serological markers, anticentromere, anti-Scl-70 and antinucleolar antibodies were found in 21, 40 and 27% of the cases, respectively; these were statistically less frequent (p less than 0.0001) in other CTD. In 83.5% of patients with SSc at least one of these specific markers was recorded. Anticentromere antibodies were correlated to sex (female), limited SSc, calcinosis and telangiectasia. On the contrary anti-Scl-70 was associated with diffuse and intermediate subsets and with more severe SSc manifestations.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1991

Radionuclide esophageal transit studies in progressive systemic sclerosis: an analysis of longitudinal data.

Nineteen patients with progressive systemic sclerosis (PSS) were studied by radionuclide esophageal transit (ET) and followed longitudinally for 3 to 5 years. Results were expressed as percent retention at 20 s and 10 min. There was gradual deterioration of ET at both 20 s and 10 min. When results were grouped into quartiles, deterioration occurred in 58.5% of followup studies in patients who initially had potential to deteriorate regarding 20 s retention and in 48% of similar patients regarding 10 min retention.

Adult↗
PubMed1991

[Pre-auricular sclerodermiform basocellular carcinoma extending to the temporomandibular joint. Presentation of a case].

Morphea form basal-cell epitheliomas of the external ear are rare often poorly delineated clinically, present a high rate of recurrence caused by inadequate treatment, deeply invasive and destructive lesions, development over embryonic fusion plane and other anatomical particularity of the external ear. For tumor superior to 2 cm or recurrent lesion conventional excisions are very multilating on this part of face with a 10 to 15% of recurrent rates. The micrographic surgery with total microscopic control of excision described by Mohs which was not disponible for this case seems to be the treatment of choice for morphea form basal-cell carcinoma.

Adult↗
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↗
PubMed1990

[Classification of circumscribed scleroderma. A multicenter study of 286 patients. The Scleroderma Study Group of the Society of Dermatologic Research].

In a multicentre study performed by the German Working Group for Dermatological Research a total of 286 female and male patients with circumscribed scleroderma (morphoea) were examined with reference to form and extent of their skin manifestations (e.g. progressive idiopathic atrophodermia, Pasini-Pierini Type) and systemic involvement. On the basis of the literature and our observation of circumscribed scleroderma classification into three types appears to be appropriate: a plaque-type (with generalized circumscribed scleroderma as the most severe variant), a linear type (with pansclerotic disabling morphoea as the most severe variant) and a profound type (with eosinophilic fasciitis as the most severe variant).

Adolescent↗