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Results for “Scleroderma, Limited”

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PubMed1995

Circulating interleukin 1 beta and soluble interleukin 2 receptor: evaluation as markers of disease activity in scleroderma.

OBJECTIVES: To investigate whether circulating levels of interleukin 1 beta (IL-1 beta) or soluble interleukin 2 receptor (sIL-2R) reflect clinical disease status and response to therapy in scleroderma. METHODS: Plasma IL-1 beta and serum sIL-2R were measured by ELISA in 19 patients with limited cutaneous scleroderma (9 with extraesophageal internal organ involvement), 5 patients with diffuse cutaneous scleroderma and internal organ involvement, and 11 healthy controls, as well as serially over 12 months in 4 patients with scleroderma treated with cyclosporine. RESULTS: IL-1 beta levels were similar in scleroderma and control subject groups. sIL-2R levels were significantly higher in subjects with scleroderma involving internal organs (elevated in 93%), and correlated with erythrocyte sedimentation rate. sIL-2R levels decreased over 12 months in 2 of 4 patients taking cyclosporine in whom other variables remained unchanged. CONCLUSIONS: Elevated serum sIL-2R is a marker of internal organ involvement in scleroderma and warrants further investigation in assessing disease prognosis and response to therapy.

Adult↗
PubMedDate not supplied

Three cases of osteonecrosis of the lunate bone of the wrist in scleroderma.

This report describes three cases of osteonecrosis of the lunate bone of the wrist in patients with systemic sclerosis presenting with wrist pain. All three patients had limited skin scleroderma but severe Raynaud's phenomenon. Two patients never received corticosteroids and one patient received only low doses for a brief period. None of the patients had other definable risk factors for osteonecrosis. Two patients underwent vascular bone grafting with improvement in symptoms. Osteonecrosis may represent an under-recognized cause of wrist pain in scleroderma patients.

Adult↗
PubMed1991

Peripheral nerve dysfunction in scleroderma.

Peripheral neuropathy in patients with scleroderma is thought to be rare. We have undertaken a quantitative assessment of peripheral nerve function in 29 patients with either limited cutaneous scleroderma or progressive systemic sclerosis. Tactile thresholds were raised in the fingers in 28 per cent of patients and in the foot in 50 per cent. Two-point discrimination was abnormal in 10 patients, thermal thresholds were abnormal in five and vibration thresholds were abnormal in one. Nerve conduction studies showed abnormalities in six patients, five of whom had clinical signs of a mild peripheral neuropathy: the mean duration of disease in these six patients was 10 years longer than that in the remainder of the patients. There was electrophysiological evidence of a subclinical carpal tunnel syndrome in two patients. The sympathetic skin response was recorded in 16 patients who had not been subjected to sympathectomy for Raynaud's phenomenon, and was abnormal in four. These results indicate that peripheral nerve dysfunction in scleroderma, though mild, is not as uncommon as previously thought. The abnormal cutaneous sensory thresholds may be partly due to altered viscoelastic properties of the skin, but abnormal responses in the lower limbs to tests of tactile sensitivity, the clinical findings and the disturbances of nerve conduction argue in favour of an additional neuropathic process in some patients. Low grade distal nerve trunk ischaemia may be responsible.

Adult↗
PubMed1995

Anti-M4 antibodies measured by a sulphite oxidase ELISA in patients with both anti-centromere and anti-M2 antibodies.

In this study the clinical features of patients with a serological overlap between scleroderma and primary biliary cirrhosis (PBC) were analysed. The entity was defined by the presence of both anti-centromere antibody (ACA) and anti-mitochondrial antibodies to the M2 antigen, pyruvate dehydrogenase. In addition the sera were assayed for anti-mitochondrial antibodies to the M4 antigen measured by an ELISA to sulphite oxidase (SO). First, anti-M2 was detected, not only in 58 out of 60 patients with PBC but also in eight out of 61 patients with ACA. These sera, together with sera from 53 normals and 99 from patients with various connective tissue diseases were then evaluated for anti-SO, which has been proposed by Klein and Berg to be a marker of progressive liver disease. Again, a high proportion (62%) of sera from patients with PBC were positive for anti-SO, and three of the eight patients who had ACA and anti-M2 also reacted with SO. We subsequently identified and included for study a further 10 patients positive for ACA and anti-M2, making a total of 18 patients with this profile. Features of limited cutaneous scleroderma were present in 94% and evidence of liver disease in 56%. Eight out of the 18 patients had anti-SO, and of these four had PBC, two had abnormal biochemical liver function tests but two had no evidence of liver disease. These data confirm that detection of anti-SO is limited to an anti-M2 subpopulation, and may be a marker for liver involvement with prognostic significance in scleroderma patients with ACA.

Autoantibodies↗
PubMed2006

Influence of clinical features on the health status of patients with limited cutaneous systemic sclerosis.

OBJECTIVE: To determine the effect of limited cutaneous systemic sclerosis (lcSSc) on patients' health status, and to identify the contributions to health status of different manifestations of lcSSc. METHODS: The Short Form 36 questionnaire was completed by 213 patients with lcSSc or Raynaud's syndrome and an antinuclear antibody typical of lcSSc as part of the baseline visit of the Quinapril in Scleroderma trial. Results were analyzed after correcting for age and sex using the Welsh Health Survey. Patients' results were related to their clinical characteristics. RESULTS: The mean physical component score (PCS) was 44.0 (95% confidence interval [95% CI] 42.5, 45.5), which was lower than the population norm of 50, and the median mental component score (MCS) was 52.2 (95% CI 48.5, 54.3). Raynaud's disease visual analog scale (VAS) scores, lung function, the number of organ systems affected, and skin score were significantly correlated with PCS. The total score (TDS) of an SSc severity scale showed the highest correlation. The effect of lcSSc on PCS was worse in younger patients. Multiple regression including age demonstrated that Raynaud's disease severity could predict a reduction in PCS beyond that predicted by TDS. Raynaud's disease severity and duration of lcSSc were linked to low MCS. Arthritis reduced PCS and esophageal involvement reduced PCS and MCS. CONCLUSION: Physical health status of patients with lcSSc was reduced, with 30% of the variation predicted by TDS, age, and severity of Raynaud's disease VAS. Mental health status was not reduced in this population.

Health Status↗
PubMed2003

Lack of evidence of foetal microchimerism in female Spanish patients with systemic sclerosis.

Our objective was to study the presence of microchimerism in a series of 47 female Spanish patients with scleroderma (SSc) and to compare with a control group. Polymerase chain reaction was used to identify Y-chromosome sequences in DNA extracted from peripheral blood cells. Y-chromosome sequences were found in DNA from peripheral blood cells in four out of 47 (8.5%) patients with scleroderma (two limited and two diffuse) and in two out of 40 (5%) healthy women (no statistical differences were found). When we compared SSc patients and healthy controls who had had at least one male child, four out of 29 (13.7%) and two out of 26 (7.6%) had microchimerism respectively (no statistically significant differences were found). Patients with both scleroderma and persistent microchimerism had had a male offspring. Foetal microchimerism does not seem to play a major role in most cases of female Spanish patients with SSc.

Adult↗
PubMed1990

Early detection of connective tissue disease in patients with Raynaud's phenomenon.

Connective tissue diseases such as scleroderma frequently show an insidious onset. In their early stages a diagnosis of a specific CTD is hard to make, and the disorder may then be designated as "undifferentiated CTD." Raynaud's phenomenon (RP) is the first symptom in many cases and may precede the disease for many years. RP, however, is a common finding in the population, especially in young females. Thus, only a minority of patients with RP will develop a CTD. For reason of prognosis and early diagnosis and to get more insight in the initial pathophysiological processes, it is important to know which patients with RP will develop or are already evolving into a CTD. Patients referred to the clinician because of RP frequently (24-40%) show signs or symptoms of CTD, especially of scleroderma. Pulmonary function disturbances and esophageal hypomotility are asymptomatic in many cases, and should be sought for with sensitive methods. How can we distinguish patients with truly primary RP from those who will evolve into CTD (and thus should be screened for CTD and be followed)? The presence of antinuclear antibodies (ANA) and abnormalities at nailfold capillary microscopy (NCM) have proven to be early indicators of evolution into CTD, especially scleroderma and related disorders. The antigenic specificities of ANA indicate which syndrome the patient will develop, e.g., anti-CENP-B indicates the CREST-syndrome and anti-topoisomerase I diffuse scleroderma. Other factors of prognostic significance in patients with RP are age at onset and severity of RP: older age at onset and a highly severe RP represent risk factors for CTD. What do we know about the evolution of RP into CTD? Follow-up studies on patients referred because of RP have shown that some 15-20% of these patients have an insidious progress to limited cutaneous scleroderma including CREST. Risk factors have already been mentioned. From a pathophysiological point of view, studies in patients with early scleroderma have proved that microvascular changes are crucial in the disease process. These changes, demonstrated morphologically by NCM and functionally, e.g., by pulmonary function studies, are also reflected in increased levels of VIII antigen and in in vivo platelet activation. Both of these phenomena probably result from endothelial damage. There is increasing evidence that T-cells and their products are involved in vascular damage in early scleroderma. Future research should be directed to the elucidation of the target antigens for these T-cells.

Antibodies, Antinuclear↗
PubMed2006

Anti-endothelial cell antibodies from patients with limited cutaneous systemic sclerosis bind to centromeric protein B (CENP-B).

By using a quantitative immunoblotting technique on protein extracts of human macrovascular and microvascular endothelial cells, we have analyzed the self-reactive repertoires of IgG from 20 patients with limited cutaneous SSc, 40 patients with diffuse SSc and 60 age- and sex-matched healthy controls. Serum IgG from 15/20 patients with limited cutaneous SSc and anti-centromere antibodies bound to at least one of the two 75- and 85-kDa protein bands in the different endothelial cell extracts, whereas IgG from healthy controls or patients with diffuse SSc did not. N-terminal sequencing of the 75- and 85-kDa bands identified CENP-B as the sole antigen in both bands. Moreover, IgG from all of the SSc patients who recognized the 75- and/or 85-kDa bands bound to a full-length recombinant CENP-B protein as assessed by ELISA, whereas IgG from other SSc patients did not. The main target of anti-endothelial cell antibodies in patients with limited cutaneous SSc is the nuclear and ubiquitous protein CENP-B.

Antibody Specificity↗
PubMed2002

Antineutrophil cytoplasmic antibodies in scleroderma patients: first report of a case with anti-proteinase 3 antibodies and review of the literature.

OBJECTIVE: To evaluate the incidence, the subspecificities and the clinical correlation of antineutrophil cytoplasmic antibodies in scleroderma patients. METHODS: Sixty-two patients affected by scleroderma in limited or diffuse pattern have been screened for the presence of anti-proteinase 3 and anti-myeloperoxidase antibodies by ELISA method. RESULTS: Two patients affected by diffuse systemic sclerosis were found to be antineutrophil cytoplasmic antibody-positive. One patient presented a weak positivity for anti-myeloperoxidase antibodies in the absence of renal impairment. One patient which clinical course is characterised by rapidly progressive skin and lung involvement presented a high positivity for anti-proteinase 3 antibody. CONCLUSION: Our study confirms the low incidence of antineutrophil cytoplasmic antibodies in scleroderma patients (3.2%); we reportthe first case of isolated scleroderma with positivity of anti-proteinase 3 antibodies.

Adult↗
PubMed2002

Implant-supported, long-span fixed partial denture for a scleroderma patient: a clinical report.

Scleroderma poses many difficulties and challenges for the treating dentist. Until now, dental implant treatment for patients with scleroderma was limited to either complete overdentures or short-span fixed partial dentures. This clinical report describes the use of modified techniques to fabricate a long-span fixed prosthesis delivered after serial extractions and implant placement.

Dental Care for Chronically Ill↗
PubMed2005

Distinct expression of adhesion molecules on skin fibroblasts from patients with diffuse and limited systemic sclerosis. A pilot study.

OBJECTIVE: . Systemic sclerosis (SSc) is characterized by excessive production of collagen and other components of the extracellular matrix (ECM) by fibroblasts. The ECM receptors, integrins and CD44 (hyaluronan receptor), play a key role in the homeostasis of connective tissue and may also have a role in the pathogenesis of fibrosis. We investigated the expression of integrins and CD44 on skin fibroblasts from patients with limited and diffuse SSc. METHODS: We studied 13 patients with SSc, 8 with limited SSc, and 5 with diffuse SSc, and 8 control subjects. Fibroblasts were isolated and cultured from biopsies taken from the lesional skin of the second finger of the left hand. Cell-surface expression of beta1, beta3, alpha1-alpha6, alphav integrins, and CD44 was evaluated by immunofluorescence and flow cytometry analysis. RESULTS: Fibroblasts from limited SSc showed significantly decreased expression of alpha2, alpha3, alpha4 integrins, while diffuse SSc fibroblasts had significantly reduced expression of alpha5, alphav integrins, and CD44. Diffuse SSc also had significantly increased expression of alpha6 integrin on fibroblasts. In controls, the expression of alpha4 and alpha5 correlated positively, while in limited and diffuse SSc it did not. CONCLUSION: This is the first study evaluating separately the expression of adhesion molecules on skin fibroblasts from limited and diffuse subsets of SSc. We detected a distinct pattern of expression with decrease of collagen and fibronectin receptors in limited SSc, and downregulation of fibronectin and hyaluronan receptors in diffuse SSc. These results suggest that changes of fibroblasts/ECM interactions and mechanisms underlying the pathogenesis of fibrosis in SSc may differ in the single subset of the disease.

Cells, Cultured↗
PubMed1997

General and local scleroderma in children and dermatomyositis and associated syndromes.

Scleroderma in children virtually always manifests in the form of localized disease, whereas adult scleroderma usually manifests as systemic disease. The recent literature on childhood scleroderma is limited almost exclusively to localized disease. The treatment of this disease remains a dilemma, especially with regard to which patients should be treated, how they should be treated, and how the treatment response should be monitored. Our current therapeutic approach is reviewed. The recent literature regarding juvenile dermatomyositis is discussed. New understanding of the pathobiology of this disease should lead to improved, less toxic treatments in the future.

Child↗
PubMed1995

Clinical relevance and HLA association of autoantibodies against the nucleolus organizer region (NOR-90).

OBJECTIVE: NOR-90 autoantibodies directed against the nucleolus organizer region (NOR) have been described as rare scleroderma associated antibodies. We studied the clinical features of patients with NOR-90 antibodies as well as their HLA phenotype. METHODS: NOR-90 antibodies were detected by indirect immunofluorescence assay using HEp-2 cells, by chromosome spreads as a substrate and in addition by Western blot analysis with HeLa-S3 nucleolar extract. HLA antigens of the NOR-90 antibody positive patients were typed with the standard NIH complement dependent microcytotoxicity test. RESULTS: Nine sera selected by means of the indirect immunofluorescence revealed a typical double band pattern of about 90 kDa identical with the pattern of 2 NOR-90 reference sera by Western blot analysis. Only one patient positive for NOR-90 antibodies suffered from systemic sclerosis (limited cutaneous scleroderma). The other patients with NOR-90 antibodies showed no signs of systemic sclerosis. All patients with NOR-90 antibodies were women and 8 of 9 patients (89 versus 13% of healthy controls, Pcorr < 0.001) were positive for the HLA-DR1 allele. CONCLUSION: In contrast to the first report on NOR-90 antibodies we demonstrated no association of these antibodies with systemic sclerosis; however, we found strong evidence for an immunogenetic background of NOR-90 antibody formation.

Adult↗
PubMed2006

Proton magnetic resonance spectroscopy reveals central neuroaxonal impairment in systemic sclerosis.

OBJECTIVE: . Involvement of the central nervous system (CNS) in systemic sclerosis (SSc) is rare. Proton magnetic resonance spectroscopy (1H-MRS) assesses in vivo cerebral metabolites. We investigated the biochemical modifications of the CNS in SSc. METHODS: N-acetylaspartate/creatine ratio (NAA/Cr) and choline/creatine ratio (Cho/Cr) at right centrum semiovale (RCS) and at right basal ganglia (RBG) were evaluated by 1H-MRS in 12 patients with limited (lSSc) and 8 patients with diffuse SSc (dSSc) and 20 control subjects. RESULTS: With 1H-MRS, a significant reduction of NAA/Cr ratio at RBG (p < 0.02) and at RCS (p < 0.002) was detected in SSc patients. Cho/Cr ratio was increased (p < 0.02) in the RCS, but not in RBG. In patients with lSSc, a significant reduction of NAA/Cr was detected in RCS but not in RBG. CONCLUSION: Evidence of neuroaxonal damage strongly suggests the existence of CNS involvement in SSc.

Adult↗
PubMed2006

The efficacy of self-administered stretching for finger joint motion in Japanese patients with systemic sclerosis.

OBJECTIVE: To determine the efficacy of self-administered stretching of each finger in Japanese patients with systemic sclerosis (SSc). METHODS: Forty-five patients with SSc (32 with diffuse cutaneous SSc and 13 with limited cutaneous SSc) were given instructions on self-administered stretching and were directed to perform it every day. Individual fingers were maintained in a stretched position using the opposite hand for 10 seconds and this was repeated 3-10 times. To evaluate the effect of the stretching program, finger passive range of motion (ROM) was assessed using a goniometer on the first visit and after 1 month and 1 year of the stretching program. The Health Assessment Questionnaire (HAQ) was also assessed on the first visit and 1 year afterward. RESULTS: The total passive ROM was significantly improved in each finger after 1 month of finger stretching. The total passive ROM was further improved or maintained within 1 year after the first visit. Although ROM was less in patients with diffuse cutaneous SSc than in those with limited cutaneous SSc at the first visit, ROM increased significantly irrespective of disease duration or severity of skin sclerosis. Finger stretching may improve the finger function, since the HAQ score for hand functions such as eating and gripping was significantly decreased. CONCLUSION: Our original self-administered stretching program may be useful for improving finger joint motion in patients with SSc; future studies in various ethnic populations will be needed to determine the universal efficacy of this method.

Female↗
PubMed2006

A novel autoantigen to differentiate limited cutaneous systemic sclerosis from diffuse cutaneous systemic sclerosis: the interferon-inducible gene IFI16.

OBJECTIVE: To investigate the presence and clinical significance of autoantibodies against the interferon-inducible gene IFI16 in systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and other autoimmune diseases. METHODS: Immunohistochemical analysis was used to evaluate the expression of IFI16 in skin biopsy specimens obtained from patients with SSc and patients with SLE. Levels of antibodies against IFI16 in sera from 82 patients with SSc and 100 patients with SLE were determined by enzyme-linked immunosorbent assay. Other autoimmune diseases such as primary Sjögren's syndrome (SS), rheumatoid arthritis (RA), chronic urticaria, and hepatitis C virus (HCV) infection were also examined. RESULTS: Expression of IFI16 was greatly increased and was ubiquitous in all layers of the epidermis and in the dermal inflammatory infiltrates of lesional skin from both patients with SLE and patients with SSc. Patients with SLE, those with primary SS, and those with SSc exhibited significantly higher anti-IFI16 IgG antibody levels compared with normal controls (for SLE, P < 0.002; for primary SS, P < 0.001; for SSc, P < 0.0005). Anti-IFI16 titers above the ninety-fifth percentile for control subjects were observed in 26% of the patients with SLE, 50% of those with primary SS, and 21% of those with SSc (28% of patients with limited cutaneous SSc [lcSSc] versus 4% of patients with diffuse cutaneous SSc [dcSSc]). In contrast, the prevalence of anti-IFI16 was 4% in patients with RA, 5% in those with chronic urticaria, and 13% in those with HCV infection. CONCLUSION: The results of this study provide evidence that an IFN-inducible gene, IFI16, may be involved in the pathophysiologic mechanisms of connective tissue disorders such as SSc. Moreover, a strict correlation with lcSSc was also demonstrated, thus providing a novel tool in the differential diagnosis of lcSSc from dcSSc.

Adult↗