Limited form of systemic sclerosis improved with enalapril.
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Adult↗
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Results for “Scleroderma, Limited”
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[Clinical value of soluble adhesion molecules in systemic scleroderma].
The aim of the trial was to study clinical significance of estimation of cell adhesion soluble molecules (CASM) in scleroderma systematica (SS). Quantitation of CASM VCAM-1, ICAM-1 and R-selectin was made with enzyme-immunoassay (R&D System kits, USA) in 38 patients with SS (11 with limited SS and 27 with diffuse SS). The levels of VCAM-1, ICAM-1 and R-selectin was elevated in 30 (79%), 17 (45%) and 20 (53%) patients, respectively. Mean values of VCAM-1 and ICAM-1 in patients were significantly higher than in healthy donors. R-selectin was also higher but insignificantly. A mean CASM level and a relative number of patients with elevated count of CASM in patients with diffuse and limited forms of SS did not differ. In 15 patients with active (progressive) course of the disease the level of VCAM-1 was significantly higher than in patients with chronic (non-progressive) course of SS while concentrations of ICAM-1 and R-selectine were almost the same. Thus, SS patients have elevated levels of CASM. CASM VCAM-1 concentration is the most sensitive marker of SS activity compared to other CASM.
Adult↗
Clinical subsets of scleroderma: relevance of fluorescent and precipitating antinuclear antibodies.
Sera from 7 patients with localized and 35 with systemic scleroderma were studied for the presence of fluorescent antinuclear antibodies (FANA) (by indirect immunofluorescence on HEp-2 cells) and antibodies to extractable nuclear antigens (anti-ENA) (by immunodiffusion - ID - and counterimmunoelectrophoresis - CIE). In localized disease, antinuclear autoimmunity was limited to 1 FANA positive serum (14%); in systemic disease, the prevalence of FANA was 94% and that of anti-ENA ranged from 29% to 49% (by ID and CIE, respectively). The commonest ENA system, Scl-70, could be easily detected by CIE, in spite of the reported basic nature of the antigen. The anticentromere antibody occurred only in patients with acrosclerosis (7/26-27%), whereas the association of nucleolar + homogeneous FANA, as well as the anti-Scl-70, were found more frequently in diffuse scleroderma (9/9-100% and 6/9-67%, respectively). The presence of the anticentromere antibody excluded that of any anti-ENA, while a close association was found between nucleolar + homogeneous FANA and the anti-Scl-70. Pulmonary involvement was significantly more frequent in nucleolar + homogeneous FANA positive patients; moreover, in two cases the same pattern proved to predict the development of diffuse scleroderma.
Antibodies, Antinuclear↗
Carbon and nitrogen allocation in ectomycorrhizal and non-mycorrhizal Pinus sylvestris L. seedlings.
We studied carbon and nitrogen allocation in mycorrhizal and non-mycorrhizal Scots pine (Pinus sylvestris L.) seedlings grown in a semi-hydroponic system with nitrogen as the growth limiting factor. Three ectomycorrhizal fungi were compared: one pioneer species (Thelephora terrestris Ehrh.: Fr.) and two late-stage fungi (Suillus bovinus (L.: Fr.) O. Kuntze, and Scleroderma citrinum Pers.). By giving all plants in each treatment the same amount of readily available nitrogen, we ensured that the external mycelium could not increase the total nitrogen content of the plants, thereby guaranteeing that any change in carbon or nitrogen partitioning was a direct effect of the mycorrhizal infection itself. Carbon and nitrogen partitioning were measured at an early and a late stage of mycorrhizal development, and at a low and a high N addition rate. Although mycorrhizal seedlings had a higher net assimilation rate and a higher shoot/root ratio than non-mycorrhizal seedlings, they had a lower rate of shoot growth. The high carbon demand of the mycobionts was consistent with the large biomass of external mycelia and the increased belowground respiration of the mycorrhizal plants. The carbon cost to the host was similar for pioneer and late-stage fungi. Above- and belowground partitioning of nitrogen was also affected by mycorrhizal infection. The external mycelia of Scleroderma citrinum retained 32% of the nitrogen supplied to the plants, thus significantly reducing nitrogen assimilation by the host plants and consequently reducing their growth rate. By contrast, the external mycelia of T. terrestris and Suillus bovinus retained less nitrogen than the mycelia of Scleroderma citrinum, hence we attributed the decreased growth rates of their host plants to a carbon drain rather than a nitrogen deficiency.
Journal Article↗
Organ manifestations in 100 patients with progressive systemic sclerosis: a comparison between the CREST syndrome and diffuse scleroderma.
Internal organ function, biochemical, and immunological variables were assessed in 100 patients with progressive systemic sclerosis (PSS), 37 with diffuse scleroderma and 63 with the CREST syndrome. The frequency of oesophageal symptoms and dysmotility did not differ between the two subsets, but patients with diffuse scleroderma had more pronounced motility disturbances. Calcinosis, Raynaud's phenomenon, and telangiectasia were equally frequent in the two groups. Vital capacity, total lung capacity, and static lung compliance were lower among patients with diffuse scleroderma (p less than 0.001), who also had a higher frequency of lung fibrosis (p less than 0.001) and cardiomegaly (p less than 0.01). Sixty eight per cent of the patients with diffuse scleroderma and 62% of those with the CREST syndrome had a positive antinuclear antibody test, which was associated with oesophageal hypomotility (p less than 0.001), fat malabsorption (p less than 0.01) and pulmonary dysfunction (p less than 0.05). Thus, the severity of organ manifestations varied not only with the form of PSS, but also with the absence/presence of antinuclear antibody. This study shows that the term 'CREST syndrome' could be replaced by 'limited cutaneous systemic sclerosis'.
Adult↗
Severe gastrointestinal involvement in systemic sclerosis: report of five cases and review of the literature.
OBJECTIVE: To review current literature on the gastrointestinal tract (GIT) manifestations of systemic sclerosis (SSc) and to report on 5 patients with severe gastrointestinal SSc. MATERIALS AND METHODS: The clinical course and histopathology of 5 patients are described. A review of the medical literature registered in MedLine and PreMedLine databases from 1996 through mid-2004 was performed using the keywords systemic sclerosis and scleroderma and combining them with text words such as gastric, gastrointestinal, anorectal, colonic, and hepatic. RESULTS: All 5 patients had severe GIT involvement: 4 with diffuse cutaneous SSc (dcSSc) and 1 with limited cutaneous SSc (lcSSc). Autopsy results of 2 patients who died from severe malnutrition and aspiration pneumonia are presented. Literature review includes involvement from oral cavity to anus with varying degrees of severity. Most GIT manifestations result from dysmotility secondary to infiltration of the gastrointestinal wall with fibrous tissue and can cause life-threatening malabsorption and malnutrition. Diagnostic tests, pathology, and treatments of GIT SSc are reviewed. CONCLUSIONS: GIT involvement in SSc can be severely debilitating and even life-threatening. Although morbidity is inevitable, appropriate supportive treatment can prolong survival. RELEVANCE: GI complications of SSc cause significant morbidity and mortality.
Adult↗
Clinical approach to scleroderma.
Systemic sclerosis (SSc) is a heterogenous disease with a morbidity and mortality that varies widely. Nonetheless, the future clinical course of an individual patient can be estimated based on the severity of skin and internal organ involvement within the first several years of the disease. Patients with limited cutaneous SSc (ISSc) have skin thickening below the elbows or knees and may have face and neck involvement. Patients with this subtype of SSc have Raynaud's phenomenon, digital ulcers, and esophageal dysfunction. Significant morbidity and mortality arises in those patients with ISSc who develop interstitial lung disease or pulmonary artery hypertension. Patients with diffuse cutaneous SSc (dSSc) have skin thickening above the elbows and knees or on the trunk. These patients have a more abrupt onset of disease, often with constitutional symptoms and arthalgias. Severe heart, lung, gut, and renal involvement, if it occurs, tends to develop within the first 5 years of disease, especially within the first several years. Patients with significant internal organ involvement have a poorer prognosis than patients who do not. The goals of the initial history and physical and laboratory examinations are to classify the type of scleroderma as ISSc or dSSc, to estimate disease duration, and to define the extent and severity of organ involvement. Treatment of SSc is organ based. Treatment may reduce morbidity associated with Raynaud's phenomenon, digital ulcers, esophageal dysmotility, esophageal reflux, gut dysmotility, arthralgias, myositis, and pulmonary artery hypertension. Therapy may stabilize lung function in patients with interstitial lung disease with alveolitis and stabilize renal function in patients with renal crisis. The overall prognosis for patients with SSc appears to be improving. Patients with early dSSc should be considered for enrollment onto protocol testing of potential disease-modifying therapies.
Arthralgia↗
[Problems posed by therapeutic trials in vascular acrosyndromes].
Vascular acrosyndromes are mainly subjective clinical phenomena that are difficult to classify, are related to the patient's background, and require a particularly strict protocol for the evaluation of their therapy. An essential criterion is the homogeneity of groups treated, the Raynaud's phenomenon having to be separated into idiopathic Raynaud's disease, digital arteritis and scleroderma. Functional subjective disturbances have to be rated by the patients themselves, while taking into account the conditions of daily life and of climate. Clinical trials must be double-blind against placebo after a pre-inclusion study. They must be conducted entirely during cold spells for Raynaud's phenomena. Static measurements are of limited value amongst functional exploratory techniques, dynamic tests (vascular reactivity to cold) providing more valid data on the action of medication.
Arteritis↗
[Changes in esophageal peristalsis in diverse clinical forms and antibody specificity in scleroderma: a scintigraphic study in 100 cases].
OBJECTIVE: To define the prevalence and severity of esophageal involvement in systemic sclerosis (SSc) and its relationship with the different clinical forms and ANA specificities of the disease. METHODS: A hundred consecutive patients with SSc, 48 with cutaneous limited, 26 with intermediate and 26 with diffuse form of disease, 49 with anti-centromere and 37 with anti-Scl70 ANA pattern, were submitted to scintigraphy using a semisolid orally ingested bolus to detect esophageal hypomotility. RESULTS: An impairment of esophageal function has been observed in 68% of SSc patients. Esophageal dysmotility was significantly more frequent and severe in patients with cutaneous diffuse and intermediate forms of SSc and with anti-Scl70 ANA pattern. CONCLUSIONS: Esophageal involvement is very common in SSc. The scintigraphy confirms to be a useful and non invasive diagnostic method; moreover it permits to quantify the severity of the esophageal dysmotility by analyzing both global and segmental function.
Adult↗
Nailfold capillaroscopy in connective tissue disorders and in Raynaud's phenomenon.
This article focuses on the value and the limitations of nailfold capillaroscopy in the differential diagnosis of certain connective tissue diseases and in the early recognition of secondary Raynaud's phenomenon. The finding of a "scleroderma-pattern" (apical enlargement and drop-out of capillaries) points almost certainly to an underlying progressive systemic sclerosis, mixed connective tissue disease or dermatomyositis.
Arthritis, Rheumatoid↗
The value of the Health Assessment Questionnaire and special patient-generated scales to demonstrate change in systemic sclerosis patients over time.
OBJECTIVE: To determine the validity and usefulness of a modified Health Assessment Questionnaire (HAQ) for measurement of disease status and changes in disease status over time in patients with systemic sclerosis (SSc). METHODS: Since 1985, 1,250 patients attending the University of Pittsburgh Scleroderma Clinic have completed a modified HAQ annually. In addition to the standard HAQ questions about disability, the questionnaire includes visual analog scales (VAS) to evaluate SSc organ system symptoms, Raynaud's phenomenon, gastrointestinal (GI) tract and lung involvement, pain, and overall disease severity. In this study, the disability index (DI) (from the HAQ) and the VAS scores (on a 0-3 scale) were compared with various clinical and laboratory features recorded within 3 months of administration of the HAQ and VAS, using t-tests and Spearman's correlation tests. RESULTS: The HAQ DI correlated directly with skin involvement, scleroderma heart or kidney disease, tendon friction rubs, hand contractures, and proximal muscle strength. Over time, the DI correlated with changes in skin score and was a good predictor of survival. There was a significant improvement in the DI during a 2-year time period in patients treated with D-penicillamine. The VAS for digital ulcers, GI symptoms, and lung symptoms correlated very closely with subjective and objective findings for these organ systems. The presence of new digital ulcers or improvement in digital ulcers showed significant associations with the Vascular scale, new GI symptoms or improvement in GI symptoms and institution of H2-blockers showed appropriate strong correlations with the GI scale, and changes in the forced vital capacity had an excellent correlation with the Lung scale (r = 0.58, P < 0.001). By Cox regression analysis, the HAQ DI was one of the best predictors of survival. CONCLUSION: These data provide convincing evidence that a self-administered questionnaire is an accurate and inexpensive tool to measure disease status changes in SSc. Prospective studies with the HAQ administered at regular intervals, as in controlled trials, should be performed to further assess the benefits and limitations of this instrument.
Disability Evaluation↗
[Systemic sclerosis. A diagnostic and follow-up program].
Systemic sclerosis (scleroderma) is a rare chronic progressive multiorgan disease characterized by elevated collagen content in affected organs. The condition is classified in the group connective tissue diseases, and appears in both a systemic and a limited form. The rate of progression and the prognosis differ for the two forms. The heterogeneity of the disease makes systematic examination of the patients necessary in order to provide a correct diagnosis and classification as a basis for therapy and prognosis. Based on the low incidence and prevalence of the disease, the care of these patients should be a specialist task. At our department, a programme has been introduced consisting of clinical examination, immunological and biochemical analysis, histological examination and different imaging techniques, and with an interdisciplinary approach. This programme has made the management of these patients more standardized.
Follow-Up Studies↗
A randomized, double-blind, placebo-controlled trial of photopheresis in systemic sclerosis.
BACKGROUND: Systemic sclerosis is a multisystemic connective tissue disease with marked involvement of the skin and joints for which few effective evidence based therapies are available. To further investigate the efficacy of extracorporeal photochemotherapy on early aggressive cutaneous disease, a randomized, double-blind, placebo-controlled trial was performed. OBJECTIVE: Our aim was to evaluate the efficacy of photopheresis in the treatment of patients with systemic sclerosis (scleroderma). METHODS: This randomized, double-blind, placebo-controlled clinical trial was conducted at 16 investigational sites in the United States, Canada, and Europe. Sixty-four patients with typical clinical and histologic findings of scleroderma, of less than 2 years' duration, were studied. Patients did not receive any other concomitant treatment for scleroderma. Patients were randomized to receive either active or sham photopheresis treatment on two consecutive days monthly for 12 months. Severity of skin (skin scores assessed in 22 body regions) and joint involvement (60 joints examined for contractures) were assessed on a monthly basis. RESULTS: A statistically significant improvement in skin scores as compared with baseline was observed at 6 months (P = .0024) and 12 months (P = .008) among those who received active photopheresis, but not among those who received sham photopheresis. Comparison of skin scores between the two study arms did not achieve statistical significance because of the small sample size of the study arms. Joint involvement was also significantly improved after 6 months (P = .002) and 12 months (P = .001) of active photopheresis when compared with baseline. LIMITATIONS: The study lacks sufficient statistical power to reveal a significant difference in skin and joint manifestations between the active and sham photopheresis arms. CONCLUSION: Photopheresis induced significant improvement of skin and joint involvement in patients with scleroderma of recent onset; however, any effect when compared with sham treatment and a possible placebo effect may be modest.
Adult↗
Immunology of systemic sclerosis.
Systemic sclerosis (scleroderma; SSc) is an autoimmune disorder of unknown etiology characterized by severe and progressive cutaneous and visceral fibrosis, pronounced alterations in the microvasculature, and numerous cellular and humoral immunological abnormalities. Clinically, systemic sclerosis is very heterogeneous, ranging from mild limited forms of skin sclerosis (LcSSc) with minimal internal organ involvement to severe skin to multiple internal organ fibrosis and extensive skin fibrosis (DcSSc). Mortality and morbidity in systemic sclerosis is very high and are directly related to the extent of the fibrotic and microvascular alterations. The interactions between blood vessels, fibroblast activity, and immunological processes play an important role in the pathogenesis of SSc.
Antibody Formation↗
Clinical setting of patients with systemic sclerosis by serum autoantibodies.
Associations of antinuclear (ANA) and anticardiolipin (aCL) antibodies with clinical manifestations were analyzed in patients with systemic sclerosis (SSc). We studied 105 SSc patients: 28 had limited cutaneous SSc (lcSSc) involving fingers; 36 had intermediate cutaneous SSc involving limbs and face; 33 had diffuse cutaneous SSc (dcSSc) involving the trunk; 8 had a sclerosis sine scleroderma. Clinical manifestations and instrumental and laboratory findings were considered to calculate a disease score. Serum anticentromere (ACA), anti-topoisomerase I (anti-topo I) antibodies, and aCL (of IgG/IgA/IgM classes) were investigated by conventional methods. ACA positive patients (n=18), compared to ACA negative, showed higher prevalence of IcSSc (p < 0.001), lower prevalence of restrictive ventilatory defect (p=0.006), and lower disease score (p=0.008). Anti-topo I positive patients (n= 70) showed lower prevalence of lcSSc (p =0.001) compared to anti-topo I negative. In aCL positive patients (n=27) widespread skin and visceral involvement occurred more frequently than in aCL negative. The association with myocardial ischemia or necrosis (p=0.010) was significant. Occurrence of ACA excluded the coexistence of anti-topo I (p < 0.001), and aCL (p=0.037). aCL positive patients showed higher disease score in comparison with ACA positive patients (p=0.003). In conclusion ACA recognize patients with a mild disease. aCL in contrast to ACA are better than anti-topo I in recognizing the most severe pictures of SSc.
Adult↗
[Oncological principles of the treatment of facial skin cancer].
The complete mastery of reconstructive plastic surgery is a basic requirement essential for sufficient radical treatment of basal cell carcinoma in the facial area. Furthermore, the subclinical growth of the basal cell carcinoma must be taken into consideration. One can assume that the growth of the b.c.c. is much greater than the apparent clinical limits, especially in cases of recurrent tumours, tumours of long standing, tumours in frontal and temporal regions, as well as tumours with diameters of more than 2 cm and scleroderma growth. In such cases a safety margin of 8-15 mm is required, whereas in primary and locally well-defined b.c.c. a safety margin of 3-5 mm is regarded as sufficient. Following the examination of the microscopically controlled surgery developed by Mohs, which is suitable for improvement of the five-year cure rate after surgical treatment of b.c.c., the treatment of the squamous cell carcinoma of the bottom lip is dealt with. Taking into account the tendency of these tumours to metastasize, it is advised to carry out an elective neck-dissection confined to the suprahyoidal region in case of large squamous cell carcinoma and also in suspicious metastatic changes in the lymph nodes. The diagnosis of a malignant melanoma is fundamentally histological: The tumour is electrically excised with a clearance safety margin of surrounding skin of 0.5-1 cm if clinically there appears to be a 10% likelihood of the tumour being a malignant melanoma. If the histological frozen section of the excised tumour confirms the suspected diagnosis, in cases of high-risk melanomas an area of not less than 3 cm from the edge of the primary tumour must be reexcised. A free skin graft is preferred to cover the defect rather than a plastic repair by a pedicle-flap graft. The elective lymph node dissection in the case of malignant melanoma stage I is made dependent on the level of invasion and on the thickness of the tumour. Low-risk melanomas are operated on locally only whereas an elective dissection of the regional lymph nodes is generally recommended in cases of high-risk melanomas.
Basal Cell Carcinoma↗
[Small bowel pseudo-obstruction revealing an early scleroderma. Long-term efficacy of octreotide and erythromycin].
Skin abnormalities are the most common manifestations in patients with systemic sclerosis but many other organs can be involved. We report here the case of a 61 year-old-man in whom small bowel involvement was the initial presentation of limited systemic sclerosis. The clinical features consisted of recurrent small bowel obstruction without any organic lesion. The patient responded well to erythromycin (125 mg thrice a day before meals) and octreotide (50 microg subcutaneously at bedtime) with complete symptom relief. This permitted recovery of nutritional autonomy for two years. This observation shows that, in patients with intestinal pseudo-obstruction, systemic sclerosis should be considered. The long-term administration of octreotide and erythromycin can be effective in treating small bowel dysmotility in such patients.
Antibodies, Antinuclear↗
Phototherapy: a promising treatment option for skin sclerosis in scleroderma?
In systemic sclerosis (SSc; scleroderma) fibrosis of the skin can lead to considerable morbidity. No significant improvement has been reported from studies investigating antifibrotic therapies so far. In dermatology, phototherapy with ultraviolet (UV) irradiation is successfully used for treatment of several diseases because of its anti-inflammatory as well as immunosuppressive mechanisms, and its low-risk profile. In addition, the UVA spectrum in particular exerts antifibrotic effects as it leads to reduction of procollagen synthesis and expression of collagenase-1 in vitro. Accordingly, treatment with long-wavelength UVA-1 irradiation or photochemotherapy with UVA plus the photosensitizer psoralen (PUVA) have been successfully used to reduce skin fibrosis in localized scleroderma (morphea). There are only in particular few reports on treatment of skin sclerosis in SSc, but the results are in concordance with the good experience that have been observed at our and other dermatological centres. Phototherapy is able to stop or inhibit the fibrotic processes and to induce softening of sclerotic skin, especially in limited SSc. Phototherapy thus represents a therapeutic alternative for antifibrotic treatment with a low rate of adverse effects, which should be applied before the sclerotic process has proceeded too far.
Fibrosis↗