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At least 55 records · Page 3Linked to original sources
PubMed1979

Subpopulations of human T lymphocytes. IX. Imbalance of T cell subpopulations in patients with progressive systemic sclerosis.

Peripheral blood lymphocytes from twenty patients with progressive systemic sclerosis (PSS) were analysed for the numbers and proportions of B lymphocytes possessing surface immunoglobulin, cells with Fc receptors, T cella and T cells with receptors for IgM (T mu) or IgG (T gamma). In patients with PSS, B cells and lymphocytes with Fc receptors were comparable in both numbers and proportions to those of the control group. Circulating T lymphocytes were significantly fewer in the patient group. T mu cells were decreased and T gamma cells increased, resulting in lower T mu/T gamma ratios as compared to controls. This study demonstrates a profound imbalance between T mu and T gamma cells (containing a population of helper or suppressor cells, respectively). These results are discussed in relation to immunodeficiencies observed in patients with PSS.

Animals↗
PubMed1979

Lung clearance of soluble radioaerosols of different molecular weights in systemic sclerosis.

Clearance rates of soluble radioaerosols of sodium pertechnetate (99mTcO4; mol wt 163) and diethylenetriaminepenta-acetate (99mTc-DTPA; mol wt 492) were determined in seven normal subjects and ten patients with systemic sclerosis affecting the lungs. Twenty millicuries (mCi) each of 99mTcO4 and 99mTc-DTPA in 5 ml saline were aerosolised and inhaled using a disposable "Blount" nebuliser on two different days. Two regions of interest over each posterior lung field were monitored with scintillation camera, and data were stored on magnetic tape using a Hewlett Packard Data Analyser. Decreasing levels of radioactivity were plotted semilogarithmically and half-time (T 1 1/2) removal rates were calculated. The T 1 1/2) values in normal subjects did not differ significantly from T 142 values of the patients with TcO4. However, the removal rates of the higher molecular weight solute were significantly faster from lower lung zones in patients with systemic sclerosis than in the normal subjects. The faster absorption of DTPA from lower lung zones of the patients could be due to regional abnormalities of alveolar epithelium at the lung bases, presumably as a result of greater retractive forces secondary to fibrosis.

Adult↗
PubMed1979

The 'CREST' syndrome. Comparison with systemic sclerosis (scleroderma).

The CREST syndrome refers to a disorder comprising the manifestations of calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. Thirteen CREST patients (two with CRST) were compared with 26 patients with systemic sclerosis but without the full manifestations of the CRST syndrome. No significant difference was found between the groups in the age of onset of Raynaud's phenomenon, degree of multiphasic digital color changes, ulcerations of fingers, sclerodactyly, or in the frequency of abnormal esophageal peristalsis or dysphagia. Laboratory results were similar, including the frequency of an elevated ESR. However, the CREST patients had a significantly lower frequency of arthralgia (54%) and arthritis (15%) than did those with scleroderma (88% and 65%, respectively). All but one of the CREST patients were women, which was a greater proportion than found among scleroderma cases (69%), and all were white (P less than .05). Most patients with the CREST syndrome had rather severe acrosclerosis. At last evaluation, four patients were chronically ill and three had died. The CREST and CRST syndromes are closely related disorders that seem to be part of the spectrum of systemic sclerosis.

Adult↗
PubMed1978

Atypical esophageal diverticula associated with progressive systemic sclerosis.

Five cases of unusual esophageal diverticula associated with progressive systemic sclerosis (scleroderma) or collagen vascular disease are presented. These wide-mouthed saccular diverticula were infrequently seen in a group of PSS patients with the typical motility disturbance of esophageal involvement and are reminiscent of the diverticula of the colon involved by PSS.

Adult↗
PubMed1979

Absence of oesophageal mucosal folds in systemic sclerosis.

In a controlled study of barium swallow radiographs in systemic sclerosis, longitudinal oesophageal mucosal folds were absent in eight of 30 patients and in one of 30 control subjects (P less than 0.03). Patients without mucosal folds developed Raynaud's phenomenon at an earlier age than those who retained their mucosal folds (P less than 0.05). A loss of oesophageal mucosal folds did not necessarily signify more severe visceral or cutaneous disease.

Adult↗
PubMed1977

Post partum renal failure due to progressive systemic sclerosis treated with chronic hemodialysis.

An unusual case of progressive systemic sclerosis with rapidly progressing renal failure after a successful delivery is described. The presenting syndrome was malignant hypertension. The blood pressure was refractory to the various therapeutic measures which were instituted, including hemodialysis. The patient, therefore, underwent bilateral nephrectomy, following which the blood pressure returned to normal, and she survived on hemodialysis for 17 months. To the best of our knowledge, the patient presented herein is the only reported case of this kind of fetal and maternal salvage.

Adult↗
PubMed1976

Apparent recurrence of progressive systemic sclerosis in a renal allograft.

A young woman with progressive systemic sclerosis (PSS) and renal failure who received a renal transplant from her mother suffered accelerated loss of allograft function in the absence of hyperacute rejection or severe hypertension. A biopsy specimen and pathologic examination of the transplanted organ showed a fluorescent antibody pattern and vascular changes that were indistinguishable from those in the patient's native kidneys. This clinical sequence is a departure from the relative success of renal transplantation in the few previously reported cases of PSS where it has been used as therapy for renal failure.

Acute Disease↗
PubMed1977

Progressive systemic sclerosis complicated by diffuse pulmonary haemorrhage.

A case is reported of progressive systemic sclerosis with pulmonary fibrosis which was complicated by recurrent haemoptyses due to diffuse pulmonary haemorrhage. We have found no other report of this association. The haemorrhage finally remitted after treatment was started with 40 mg prednisone daily, though previously spontaneous remissions had occurred.

Hemoptysis↗
PubMed1976

Successful renal transplantation in progressive systemic sclerosis.

Hemodialysis and renal transplantation were done in a patient with progressive systemic sclerosis and renal failure. The patient's clinical course following transplantation was uncomplicated, and a biopsy of the renal allograft done 14 months after transplantation did not show recurrence of the original disease. These observations favor the consideration of early renal transplantation in patients with scleroderma and renal failure.

Adult↗
PubMedDate not supplied

Progressive systemic sclerosis: report of a case with colonic involvement.

A case of progressive systemic sclerosis associated with colonic involvement is described. Barium-enema examination revealed segmental involvement. Colonoscopic examination of the whole range of the involved colon revealed wide-mouthed diverticula with interspersed rigid areas and numerous ulcerations. These colonoscopic findings appeared to be distinct from those associated with tuberculosis and Crohn's disease.

Adult↗
PubMed1979

Serial pulmonary function tests in progressive systemic sclerosis.

Serial pulmonary function tests were performed on nine patients with progressive systemic sclerosis over a mean period of ten years. Abnormality was seen to develop both early and late in the course of the disease, and the earliest abnormality observed was impairment of the transfer factor. Deterioration of some aspect of pulmonary function was noted in each case, evidence of restriction or air trapping being seen with equal frequency. Two patients died, both of causes unrelated to their pulmonary involvement, and even pronounced early involvement of the lungs did not necessarily imply a bad prognosis.

Adult↗
PubMed2024

A Strong Dysregulated Myeloid Component in the Epigenetic Landscape of Systemic Sclerosis: An Integrated DNA Methylome and Transcriptome Analysis.

OBJECTIVE: Nongenetic factors influence systemic sclerosis (SSc) pathogenesis, underscoring epigenetics as a relevant contributor to the disease. We aimed to unravel DNA methylation abnormalities associated with SSc through an epigenome-wide association study. METHODS: We analyzed DNA methylation data from whole-blood samples in 179 patients with SSc and 241 unaffected individuals to identify differentially methylated positions (DMPs) with a false discovery rate (FDR) <0.05. These results were further integrated with RNA sequencing data from the same patients to assess their functional consequence. Additionally, we examined the impact of DNA methylation changes on transcription factors and analyzed the relationship between alterations of the methylation and gene expression profile and serum proteins levels. RESULTS: This analysis yielded 525 DMPs enriched in immune-related pathways, with leukocyte cell-cell adhesion being the most significant (FDR = 4.91 &#xd7; 10-9), prioritizing integrins as they were exposed by integrating methylome and transcriptome data. Furthermore, through this integrative approach, we observed an enrichment of neutrophil-related pathways, highlighting this myeloid cell type as a relevant contributor in SSc pathogenesis. In addition, we uncovered novel profibrotic and proinflammatory mechanisms involved in the disease. Finally, the altered epigenetic and transcriptomic signature revealed an increased activity of CCAAT/enhancer-binding protein transcription factor family in SSc, which is crucial in the myeloid lineage development. CONCLUSION: Our findings uncover the impaired epigenetic regulation of the disease and its impact on gene expression, identifying new molecules for potential clinical applications and improving our understanding of SSc pathogenesis.

Humans↗
PubMed1976

Progressive systemic sclerosis (scleroderma). First case report in a Nigerian.

The first case of progressive systemic sclerosis in a Nigerian is described. In addition to the typical features of the disease, the case shows affection of the peripheral nerves, a very rare complication. The latter led to a mistaken diagnosis of leprosy. The reasons for the rarity of this disorder in the indigenous Africans and its differentiating features from leprosy are discussed.

Adult↗
PubMed1977

Oral radiographic changes in patients with progressive systemic sclerosis (scleroderma).

Intraoral and panoramic radiographs were made of 35 patients with progressive systemic sclerosis. Of those patients, 13 (37%) were found to have abnormally thickened periodontal ligament spaces. Six of the 35 demonstrated mild to significant resorption of the posterior mandibular angle. Three patients, including two showing angle resorption also had some degree of coronoid process destruction. All patients with this disease should receive initial and follow-up panoramic radiographic examinations to determine any osseous changes.

Adult↗
PubMed1976

Loss of colonic haustration in progressive systemic sclerosis.

Complete loss of colonic haustration was observed in six cases of progressive systemic sclerosis. Varying degrees of haustral loss or sacculation were noted in twelve others. An increase in colonic length was characteristic and, occasionally, there was slight to moderate generalized dilatation. Correlation of these findings with the microscopic appearance of the colon showed that muscular atrophy and fibrous replacement were consistent findings, varying in degree. Although the data are insufficient to permit definite conclusions, it is suggested that these changes may be responsible for colonic lengthening and loss of haustration. Attention is drawn to the importance of not confusing this appearance with other conditions, particularly chronic ulcerative colitis.

Adult↗
PubMed1976

Skin capillary abnormalities as indicators of organ involvement in scleroderma (systemic sclerosis), Raynaud's syndrome and dermatomyositis.

Forty-four study patients with scleroderma (systemic sclerosis) (28 patients), Raynaud's syndrome (13 patients) or dermatomyositis (three patients) were observed for skin capillary abnormalities by widefield microscopy and compared with three control groups of 20 subjects each: (1) patients with other rheumatic disease, (2) hospitalized patients with nonrheumatic conditions, and (3) healthy volunteers. The distinctive microvascular pattern (dilated and distorted capillary loops alternating with avascular areas) previously reported in scleroderma and dermatomyositis was observed almost exclusively in the study patients. The severity of capillary abnormalities varied among the diagnostic subgroups, and a positive correlation was found between the degree and extent of abnormal microvascular patterns and multisystem involvement. On this basis, widefield nailfold capillary observations are proposed as a simple, inexpensive, reproducible technic for making an improved early diagnosis and predicting multisystem involvement in scleroderma, Raynaud's syndrome and dermatomyositis, presently a group of loosely associated and overlapping connective tissue disorders which often defy early and precise diagnosis.

Adult↗