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PubMed1984

The anticentromere antibody: disease specificity and clinical significance.

Serum samples from 539 subjects were screened for the presence of the anticentromere antibody on a human laryngeal carcinoma (HEp-2) cell line (Antibodies, Inc.). The antibody was present in 61 patients (11%), most of whom had features of limited scleroderma or the CREST syndrome (calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia), either independently or in association with primary biliary cirrhosis. The antibody was rarely found in patients with rapidly advancing or diffuse scleroderma. The anticentromere antibody is therefore a useful prognostic indicator in patients with early scleroderma, as it may help to predict what pattern of scleroderma will evolve. Screening for this antibody should be conducted in all patients with Raynaud's phenomenon, primary biliary cirrhosis, and scleroderma. Other previous studies have indicated a similar disease specificity and prognostic importance of this antibody.

Antibodies, Antinuclear↗
PubMed2002

The cytoplasmic linker protein CLIP-170 is a human autoantigen.

The purpose of this study was to identify autoantigens that are recognized by human sera and are associated with a speckled cytoplasmic fluorescent staining pattern on tissue culture cells, and to determine clinical features associated with specific autoantibodies. A serum from a patient with systemic lupus erythematosus was used to identify a 3.7-kb cDNA insert from a HeLa cell expression library. The purified cDNA (VLK2.1) encoded a peptide of 1051 amino acids that shared 98.4% similarity with the carboxyl terminal portion of a previously reported 170 kD protein named cytoplasmic linker protein-170 (CLIP-170). Antibodies affinity purified with the recombinant CLIP-170 protein, the prototype human serum and a monoclonal antibody raised against CLIP-170 exhibited identical speckled staining of the cytoplasm in HEp-2 cells. The human autoantibodies reacted with the purified recombinant protein in a Western immunoblot and immunoprecipitated the in vitro translated recombinant protein. Three additional human sera also immunoprecipitated the recombinant CLIP-170 protein. The clinical diagnoses in these patients were limited scleroderma, glioblastoma and idiopathic pleural effusion. This is the first report that identifies CLIP-170 as a human autoantigen.

Autoantigens↗
PubMed2003

Antinuclear antibody testing in a regional immunopathology laboratory.

A systematic review has been undertaken of antinuclear antibody testing over a 6-year period in a regional immunotherapy laboratory servicing a population of 400 000. Twenty-eight per cent of the 20 205 antinuclear antibody tests performed on a hyperexpressing Ro transfected cellular substrate were positive (titre >/= 1 : 80) with the most common immunofluorescent patterns being homogeneous (39%), speckled (20%), mixed (17%), nucleolar (8%), Ro (7%) and centromere (4%). Ro antibody as detected by immunofluorescence was strongly concordant with anti-Ro detected by counter immunoelectrophoresis precipitation; of 261 anti-Ro counter immunoelectrophoresis precipitation positive patients surveyed, only 15 were missed and 20 masked (with homogenous pattern) by immunofluorescence. Ro antibodies were found in patients with a variety of immune disorders, particularly connective tissue disorders, whilst a clinical survey of the anticentromere sera revealed that 67% were derived from patients with limited scleroderma. Extractable nuclear antibodies and their characterization was performed on 10 939 occasions with 12.9% being positive with anti-Ro constituting 30.2%, anti-Ro/La 25.7%, unidentified precipitin line 17.8%, anti-ribo nuclear protein 12.5%, respectively, with anti-Scl70, anti-Jo-1 and anti-Sm and various combinations making up the remainder. Unidentified precipitin lines were particular prominent in patients with connective tissue disorders. DNA quantification was performed on 12 068 occasions with 11% giving elevated values, the majority from patients with systemic lupus erythematosus. Of these positive sera 44% also demonstrated one or more extractable nuclear antibodies and 25% anticardiolipin antibodies. Regular participation in a Quality Assurance Program revealed accurate and consistent performance of antinuclear antibody testing. In conclusion antinuclear antibody detection and characterization for systemic immune disorders can provide the clinician with useful diagnostic and prognostic information; it is important that the laboratory results are relevant, timely, accurate and precise. Systematic reviews as demonstrated in this report, can provide such evidence.

Antibodies, Antinuclear↗
PubMed1996

Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients.

A retrospective chart review was carried out on 344 patients with systemic sclerosis (SSc) followed prospectively for the occurrence of pulmonary hypertension (PHT). Seventeen patients (4.9%) were found to have PHT. Eight patients had isolated PHT, while in nine PHT was associated with restrictive lung disease (RLD). The subset with RLD developed PHT earlier, but had longer survival than patients with isolated PHT. Patients with limited scleroderma tend to develop isolated PHT, while in those with diffuse disease PHT is associated with RLD. Irrespective of disease type, PHT in SSc has an extremely poor prognosis with a median survival of 12 months following diagnosis.

Adult↗
PubMed2004

Single-nucleotide polymorphisms in the SPARC gene are not associated with susceptibility to scleroderma.

OBJECTIVE: SPARC (secreted protein, acidic and rich in cysteine) is a matricellular protein that modulates cell-cell and cell-extracellular matrix interactions. SPARC expression is restricted mainly to sites of tissue remodelling and wound repair, and is prominent in fibrotic disorders. Single-nucleotide polymorphisms (SNPs) in the SPARC gene are reportedly linked to scleroderma in four ethnic groups: Choctaw Indians, Caucasians, African Americans and Mexican Americans. We set out to reproduce and to positionally clone these disease associations in a set of UK Caucasian scleroderma patients and ethnically matched controls. METHODS: One hundred and twenty-one scleroderma subjects and 200 controls were genotyped by polymerase chain reaction with sequence-specific primers differing only in the 3' nucleotide corresponding to each allele of the biallelic SNPs. Scleroderma patients were analysed against controls and on the basis of their fibrosing alveolitis status as judged by high-resolution computed tomography evaluation and the extent of cutaneous involvement. RESULTS: Eight biallelic SNPs were genotyped: three from the last untranslated exon, which had been described previously, and an additional five novel SNPs: two in the promoter region, one in exon three and two in the 3' untranslated region. Six major haplotypes were constructed across all eight SNP positions. No significant differences in genotype, allele or haplotype frequency were observed between scleroderma and controls or within scleroderma subgroups. CONCLUSIONS: SNPs in the SPARC gene are not associated with susceptibility to scleroderma. This research adds to the genetic knowledge of the SPARC gene by identifying five novel SNPs spanning the whole gene and inserting these within the context of clearly defined haplotypes.

Female↗
PubMed2006

Risk factors for lung cancer in patients with scleroderma: a nested case-control study.

OBJECTIVES: To study potential risk factors for the development of lung cancer in patients with scleroderma and explore the chronological relationship between onset of scleroderma symptoms and subtypes of lung cancer. METHOD: Linkage of two population-based registers to identify lung cancer cases and gender-matched controls with scleroderma, followed by retrospective case note review for clinical details. RESULTS: Patients with scleroderma who smoke are seven times more likely to develop lung cancer than non-smokers (p=0.008). Smokers with scleroderma and cancer smoke more than smokers with scleroderma without cancer (p=0.019). Pulmonary fibrosis and anti-topoisomerase antibody do not increase the risk of lung cancer. Peripheral lung tumours occur earlier after the onset of scleroderma symptoms than bronchogenic tumours (p=0.05). CONCLUSIONS: Smokers with scleroderma should be monitored for the presence of lung cancer and counselled to quit smoking. The earlier development of peripheral lung tumours is not consistent, with pulmonary fibrosis being an aetiological factor.

Age of Onset↗
PubMed1995

Myocardial gallium-67 citrate scintigraphy in patients with systemic sclerosis.

OBJECTIVE: To detect myocardial involvement in 16 patients with systemic sclerosis using radiogallium scintigraphy METHODS: Sixteen patients with systemic sclerosis were investigated by myocardial gallium-67 citrate scintigraphy. Echocardiographic studies and (in the positive cases) magnetic resonance imaging were also performed. RESULTS: Increased myocardial gallium uptake was found in five of the 16 patients. None of these positive cases exhibited any 'classical' clinical, electrocardiographic or laboratory signs characteristic of myocarditis. The mean age of the positive cases was 60.8 (SD 5.0) years compared with 44.6 (10.7) years for the negative cases; no difference was detected in the mean disease duration between these two groups. Two of the affected cases belonged to the limited scleroderma subgroup. CONCLUSIONS: In spite of the low number of patients investigated, it is tempting to speculate that elderly patients with a disease onset around 60 years tend to be the candidates in whom a positive gallium-67 scintigraphy test may best indicate a silent myocardial interstitial inflammation.

Adult↗
PubMed2005

Correlation of endostatin and tissue inhibitor of metalloproteinases 2 (TIMP2) serum levels with cardiovascular involvement in systemic sclerosis patients.

Fibrosis of oesophagus, lungs, heart, and kidney in the course of systemic sclerosis (SSc) may lead to dysfunction of the above organs or even patients death. Recent studies point out the role of angiogenesis and fibrosis disturbances in the pathogenesis of SSc. Heart fibrosis is one of the most important prognostic factors in SSc patients. So, the aim of our study was to examine cardiovascular dysfunction in SSc patients and its correlation with serum levels of vascular endothelial growth factor (VEGF), endostatin, and tissue inhibitor of metalloproteinase 2 (TIMP2). The study group comprised 34 patients (19 with limited scleroderma (lSSc) and 15 with diffuse scleroderma (dSSc)). The control group consisted of 20 healthy persons, age and sex matched. Internal organ involvement was assessed on the basis of specialist procedures. Serum VEGF, endostatin, and TIMP2 levels were evaluated by ELISA. We found cardiovascular changes in 15 patients with SSc (8 with lSSc and 7 with dSSc). The observed symptoms were of different characters and also coexisted with each other. Higher endostatin serum levels in all systemic sclerosis patients in comparison to the control group were demonstrated (P < .05). Also higher serum levels of endostatin and TIMP2 were observed in patients with cardiovascular changes in comparison to the patients without such changes (P < .05). The obtained results support the notion that angiogenesis and fibrosis disturbances may play an important role in SSc. Evaluation of endostatin and TIMP2 serum levels seems to be one of the noninvasive, helpful examinations of heart involvement in the course of systemic sclerosis.

Adolescent↗
PubMed2005

Scleromyxedema: role of high-dose melphalan with autologous stem cell transplantation.

Scleromyxedema, the most severe manifestation of the spectrum of lichen myxedematosus, is characterized by cutaneous mucinosis, extracutaneous manifestations, and a monoclonal gammopathy. Seven of 8 patients evaluated at our center were treated with high-dose melphalan (180 mg/m(2) intravenously) and autologous peripheral blood stem cell transplantation, with marked improvement of gastrointestinal, central nervous system, pulmonary manifestations, and Karnofsky performance status. Five patients obtained a cutaneous complete remission and 2 patients had partial remissions. Three patients with slight progression in the skin at 12, 8, and 4 months after treatment received a second cycle of high-dose melphalan and had further symptomatic improvement. The lichen myxedematosus-scleromyxedema spectrum appears to be a continuum that requires the presence of a serum paraprotein and differs in severity of skin lesions, extracutaneous manifestations, and performance status. High-dose melphalan followed by autologous transplantation appears effective for improving the symptoms and systemic manifestations of scleromyxedema.

Adult↗
PubMed1994

Single lung transplantation in patients with systemic disease.

OBJECTIVE: To report functional results and survival in patients undergoing single lung transplantation (SLT) for pulmonary involvement associated with systemic disease or prior malignancy, criteria traditionally considered contraindications to SLT. DESIGN: Case series. SETTING: The University of Texas Health Science Center at San Antonio. PATIENTS: Nine patients who have undergone SLT for end-stage lung disease: four patients with sarcoidosis; two patients with limited scleroderma; and three patients with prior malignancies (two with prior lymphoma and bleomycin-induced pulmonary fibrosis and one who received two bone marrow transplants for acute lymphocytic leukemia and subsequently developed chemotherapy-induced pulmonary fibrosis). MEASUREMENTS: Pulmonary function testing, exercise oximetry, quantitative ventilation-perfusion lung scanning. Actuarial survival. RESULTS: All patients had marked improvement in pulmonary function, exercise oximetry, and quantitative ventilation perfusion to the SLT. One patient with scleroderma died 90 days postoperatively from Pseudomonas pneumonia with a sepsis syndrome. One patient with sarcoidosis died 150 days postoperatively from disseminated aspergillosis. At autopsy, there was no evidence of recurrent fibrosis or sarcoidosis in the transplanted lungs in either of these two patients. The seven surviving patients have returned to work or school and are conducting all activities of daily living without pulmonary disability. The 1- and 2-year actuarial survival rates in these nine patients is 68.6 percent as compared with the 1- and 2-year actuarial survival rates of 66.3 percent and 55.8 percent in the remainder of our SLT group as a whole (n = 49). Despite pharmacologic immunosuppression, there is no evidence of recurrent malignancy in the 3 patients with prior malignancies. CONCLUSIONS: We conclude that carefully selected patients with end-stage lung involvement related to systemic disease or chemotherapy-induced fibrosis may benefit from SLT.

Adult↗
PubMed2000

Autonomic nervous system and smooth muscle cell involvement in systemic sclerosis: ultrastructural study of 3 cases.

OBJECTIVE: To investigate morphological abnormalities in nerve and smooth muscle structures of the anorectal wall underlying gastrointestinal dysfunction in patients with systemic sclerosis (SSc). METHODS: We performed deep rectal biopsy in 3 patients with limited scleroderma of relatively recent onset and intestinal symptoms. RESULTS: We found ultrastructural signs of axonal degeneration and cytoskeletal abnormalities in the bundles of unmyelinated fibers. There was also focal degeneration of smooth muscle cells, often in association with the presence of partially degranulated mast cells. Many mast cells were also observed in close relation to nerve fibers and vessels. The enteric vessels often showed basal lamina reduplication and hypertrophied endothelial cells with obliterated lumen. No significant fibrosis was found. CONCLUSION: Our findings indicate early involvement of the autonomic nervous system and to a lesser extent of smooth muscle cells. We confirmed the presence of early vascular lesions and involvement of mast cells in the pathological process.

Adult↗
PubMed1992

[Clinical significance of anti-nucleolar antibodies detected by immunoprecipitation method in patients with systemic sclerosis].

We have characterized a clinical significance of anti-U3RNP, anti-7-2RNP, anti-RNA polymerase I and anti-PM-Scl antibody, autoantibodies to nucleolar proteins detected by immunoprecipitation method in patients with systemic sclerosis (SSc). In 248 patients with SSc, anti-U3RNP antibody was positive in 9 (3.6%), anti-7-2RNP antibody was positive in 7 (2.8%) and anti-RNA polymerase I antibody was positive in 3 (1.2%). But none of 248 patients was positive for anti-PM-Scl antibody. Anti-U3RNP antibody positive SSc patients showed significantly lower frequency of joint and lung involvements, compared with anti-U3RNP antibody negative SSc patients. Anti-7-2RNP antibody was found only in patients with limited scleroderma. The anti-7-2RNP antibody could be detected before appearance of skin thickening, so this indicate the usefulness of detecting anti-7-2RNP antibody in the early stage of SSc. Two of 3 anti-RNA polymerase I antibody positive patients were classified as diffuse scleroderma. All anti-RNA polymerase I antibody positive patients had high incidence of internal organ involvements including lung, heart and kidney, so two of these patients died of heart failure. These data showed the close clinical association of antigenic specificities of anti-nucleolar antibodies analysed by immunoprecipitation method, and indicated the usefulness of detecting these anti-nucleolar antibodies in subgrouping of patients with SSc.

Adult↗
PubMed2005

Relationship between occupational risk factors and severity markers of systemic sclerosis.

OBJECTIVE: To investigate a potential association between occupational risk factors and severity markers of systemic sclerosis (SSc) defined by diffuse cutaneous extent, pulmonary involvement, and immunologic profile, i.e., presence of antitopoisomerase I antibody (anti-topo I). METHODS: Occupational exposures were assessed in 105 patients with SSc from 1998 to 2002. Exposures to silica dust, welding fumes, solvents, and epoxy resins were investigated. A group of 39 exposed SSc patients and a group of 66 unexposed ones were identified and compared according to severity markers of SSc. The stage of cutaneous extent was defined according to the classification of Leroy, as limited scleroderma (lSSc) or diffuse scleroderma (dSSc). Respiratory status was defined by pulmonary function tests and high resolution computed tomography. Immunological profile was determined by the presence of anti-topo I or anticentromere antibodies (ACA). Statistical relationships between occupational exposures and severity markers of SSc were evaluated using a multiple correspondence analysis and Fisher's exact test. RESULTS: Diffuse scleroderma affected mainly patients exposed during their occupational life to toxic agents. There were significant or close to significant associations between toxic exposure and dSSc (p = 0.06), pulmonary involvement (p = 0.10), and negative ACA (p = 0.03). The most incriminated products seemed to be epoxy resins (p = 0.06), white spirit (p = 0.07), aromatic solvents (p = 0.07), and silica coupled to welding fumes (p = 0.10). CONCLUSION: Our results indicate that occupational toxic factors have an influence on the severity of SSc.

Adult↗
PubMed1987

Primary biliary cirrhosis and the CREST syndrome: a report of 22 cases.

The syndrome of primary biliary cirrhosis and limited scleroderma (the CREST syndrome) is described in 22 patients. The syndrome is expanded to include a serologic marker--the anticentromere antibody--and keratoconjunctivitis sicca. The acronym 'PACK' (primary biliary cirrhosis, anticentromere antibody, CREST syndrome, and keratoconjunctivitis sicca) with 'syndrome' is suggested to facilitate recall of the various components of this entity.

Adult↗
PubMedDate not supplied

Increased levels of circulating intercellular adhesion molecule-1 in patients with systemic sclerosis.

OBJECTIVE: To determine the value of the circulating intercellular adhesion molecule (cICAM-1) as a marker for the inflammatory and fibrotic processes in systemic sclerosis (SSc). METHODS: We determined serum levels of cICAM-1 and of the soluble interleukin-2 receptor (sIL-2R) by an enzyme-linked immunosorbent assay in 33 patients with SSc. These values were compared to the concentrations of acute phase reactants and to the extent of skin involvement in diffuse and limited scleroderma. RESULTS: cICAM-1 was elevated in patients with diffuse SSc (498 +/- 134 ng/ml) as compared with 82 healthy controls (312 +/- 71 ng/ml) (mean +/- SD, p < 0.0001). The elevation of cICAM-1 did not correlate with the duration of disease, the pattern of organ manifestations or the type of treatment. While the concentrations of acute phase proteins were not elevated in SSc, a significant correlation between increased serum sIL-2R and cICAM-1 was observed. CONCLUSION: Increased levels of cICAM-1 indicate an activation of immune processes in SSc. The clinical value of the cICAM-1 determination in SSc can only be judged in longitudinal studies.

Adult↗
PubMed1995

Mortality in systemic sclerosis: a comparison with the general population.

OBJECTIVE: To compare the mortality rate in patients with systemic sclerosis (SSc) with that of the general population. METHODS: Standardized mortality ratios (SMR) were calculated for 237 patients with SSc followed prospectively, using age and sex specific mortality rates in Ontario for the period 1976-1990. RESULTS: The overall SMR for the SSc cohort was 4.69. The mortality rate was greater with diffuse than with limited scleroderma (SMR 6.18 and 3.80, respectively), but not different between men and women. CONCLUSION: The mortality rate in SSc is increased compared to that of the general population, especially in the subset with diffuse disease.

Adult↗
PubMed1996

Cystic lung disease in systemic sclerosis: a case report with high resolution computed tomography findings.

A 31-year old black female presented with limited scleroderma. Although she had no chest symptoms, chest radiographs showed a basal reticular pattern with large cysts in both lung fields. High resolution computed tomography revealed almost complete effacement of normal lung architecture by multiple large cysts, honeycombing and interstitial fibrosis. Cystic lung disease is an uncommon manifestation of interstitial lung disease in systemic sclerosis, and its natural history needs to be better defined.

Adult↗