UMLS:C0036421 - FACTA Search

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Query: UMLS:C0036421 (PSS)
10,989 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The lungs are frequently affected in systemic sclerosis (SSc), a generalized connective tissue disorder. We evaluated the prevalence of respiratory functional abnormalities and their correlation with symptoms and radiograph features in a group of 34 patients who fulfilled the American Rheumatism Association criteria for the diagnosis of systemic sclerosis. Patients were submitted to a specific respiratory questionnaire and to lung function tests. Measurements were performed according to the European Coal and Steel Community (ECSC) recommendations and results expressed as a SD score, an accurate method that, taking into account the dispersion of the parameters in the reference population, allows precise definition of pathological subjects. Of the patients examined, 38% reported dyspnoea at rest or on exertion. No other respiratory symptoms were reported. Fifty percent had a normal chest radiograph. This study documents the high prevalence of respiratory functional abnormalities in patients with SSc. A restrictive pattern was found in 41% and an isolated diffusion impairment in 18%. No significant relationship was found between the isolated impairment of transfer factor of the lungs for carbon monoxide (TL,CO) and the mean duration of the scleroderma: thus, it does not seem to represent an early sign of severe restrictive disease. No bronchial or bronchiolar obstructive patterns were observed: it can be stated that small airways dysfunction is not a characteristic manifestation of SSc as considered previously. A significant association was found between the group of subjects with chest radiographic abnormalities and that with a restrictive pattern or isolated TL,CO alteration (p = 0.018). Chest radiographic abnormalities were also found in 29% and dyspnoea in 35% of the patients with normal respiratory function. The mean duration of scleroderma was not significantly different between the groups with and without abnormalities on chest radiography, between the groups with and without a restrictive pattern or isolated diffusion impairment, and between the groups of patients with and without dyspnoea. In conclusion, an accurate evaluation of respiratory function is recommended in the assessment of patients with systemic sclerosis, since the functional involvement of the lung cannot be predicted on the basis of the chest radiograph and the respiratory symptoms.
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PMID:Pulmonary function in patients with systemic sclerosis. 915 12

Lone cryptogenic fibrosing alveolitis (CFA) is histologically identical to fibrosing alveolitis associated with systemic sclerosis (FASSc), but it has a much worse prognosis after matching for disease severity at presentation. Thin-section CT scanning (CT) provides a reproducible method of quantifying the morphologic extent of fibrosing alveolitis. The aim of this study was to gain insights into contrasting pathophysiologic mechanisms in the two diseases by comparing patterns of functional impairment after matching for extent of disease on CT, demographic factors, smoking history, and concurrent treatment. Patients with emphysema on CT (n = 16) and patients with FASSc with overt pulmonary hypertension (n = 5) were excluded; 111 patients were studied (CFA, n = 54; FASSc, n = 57). Patients with CFA were distinguished by more severe functional impairment and more extensive disease on CT (40.1 versus 22.1%, p < 0.00005). On multivariate analysis, patients with CFA had greater reduction in arterial P(O2) (p < 0.0005), wider AaP(O2) (p < 0.0005), greater oxygen desaturation on maximal exercise (p < 0.03), and higher dyspnea scores (p < 0.02) than did patients with FASSc after controlling for extent of disease on CT and other covariates. Measures of lung volume and gas transfer did not differ independently between CFA and FASSc. These findings persisted in subanalyses of patients with limited disease, extensive disease, histologic confirmation of fibrosing alveolitis, and with the reinclusion of patients with emphysema and pulmonary hypertension. The patterns of functional impairment were indicative of more severe ventilation-perfusion mismatch or anatomic shunting in CFA after adjustment for disease extent; we speculate that perfusion of poorly ventilated lung parenchyma in CFA occurs through new vessels formed in areas of intense inflammation. This mechanism may contribute to the greater mortality of patients with CFA than of patients with FASSc because of the deleterious effects of hypoxia on concurrent cardiac disease.
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PMID:Functional impairment in lone cryptogenic fibrosing alveolitis and fibrosing alveolitis associated with systemic sclerosis: a comparison. 915 72

A 68-year-old man experienced systemic pruritus since he was 63 years old, and systemic sclerosis and skin pigmentation were observed when he was 64. When he developed dyspnea the same year, he was admitted and SSc was diagnosed on the basis of the clinical and skin biopsy findings, lung fibrosis on X-P and TBLB findings. At 65, his dyspnea reappeared along with elevated blood pressure, acute renal failure and lung congestion, and he was diagnosed as having a scleroderma renal crisis (SRC) from the clinical and renal biopsy findings. Hemodialysis was started because he showed mental disturbance, and this and other acute symptoms were subsequently reduced. As he showed no recovery from his renal failure, the patient has been maintained on hemodialysis for over four years now. In the meantime, his sclerosis has improved and antinuclear antibody almost disappeared. Hemodialysis appears to be the most likely reason for his improvement, although spontaneous remission, D-penicillamine and angiotensin converting enzyme (ACE) inhibitor therapy may also have contributed, considering the short period and the small amount of drugs given until improvement.
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PMID:[A patient with systemic scleroderma showing improvement during long-term hemodialysis after renal crisis]. 931 Dec 84

We report a very rare case of systemic sclerosis in a 6-year-old girl. She presented with diffuse scleroderma, Raynaud's phenomenon, pulmonary interstitial fibrosis, pulmonary hypertension, and right ventricular failure. The diagnosis was confirmed by skin manifestations, high resolution computed tomography, cardiac catheterization, and anti-nuclear antibodies. Nifedipine, prednisolone, digoxin, and furosemide were given. There was remission of the right ventricular failure and dyspnea, and the skin showed partial improvement. The patient remained asymptomatic for a year. The symptoms of respiratory and right heart failure developed again after an episode of lower respiratory tract infection and she eventually died. We discuss the clinical manifestations, treatment, and outcome.
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PMID:Systemic sclerosis with pulmonary involvement and right ventricular failure in a child. 940 27

It is well known that silica exposure leads in an experimental model to the development of an acute fibrotic process. In human beings two main observations have already been done: (1) silica exposure is frequently associated with the development of connective tissue disease (CTD), especially progressive systemic sclerosis; (2) 10 to 20% patients with CTD developed pulmonary fibrosis. In this context we report 26 cases of coal miners who presented with clinical, radiological, biological and functional characteristics mimicking idiopathic pulmonary fibrosis (IPF), with or without associated coal worker's pneumoconiosis (CWP). All were men; mean age was 68 +/- 9.2 years. Twenty-three were smokers. Duration of exposure was 28.8 +/- 9.1 years. All the patients had dyspnea (stage III, IV in the NHYA classification) and diffuse crackles. Eleven out of 26 had finger clubbing. Computed tomography showed honeycombing (23 cases), and/or ground glass opacities (6 cases) with bronchiectasis (3 cases) predominant in the lower lobes; 19 had radiological signs of CWP, micronodules (n = 16) and nodules (n = 3) predominant in the upper lobes. BAL exhibited an increased % of neutrophils (11.9 +/- 16.1%). Lung function demonstrated a restrictive pattern (TLC = 73 +/- 15.6% and VC = 80 +/- 18% of predicted values) associated with a decreased DLCO (51.8 +/- 23.6% of predicted values) and hypoxemia (at rest = 66.5 +/- 11.2 mmHg, upon effort = 56 +/- 12 mmHg). Lung biopsies were performed in four cases and demonstrated interstitial fibrosis of intraalveolar septum with an accumulation of immune and inflammatory cells similar to the one described in IPF. The association between IPF and silica exposure with or without associated CWP points out the problem of legal recognition of idiopathic-like pulmonary fibrosis as a complication of the occupational exposure of coal workers.
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PMID:["Primary" diffuse interstitial fibrosis in coal miners: a new entity? Study Group on Interstitial Pathology of the Society of Thoracic Pathology of the North]. 941 11

A 51-year-old woman was referred to our hospital with dyspnea. Chest roentgenogram on admission showed dilation of the pulmonary arteries and hyperlucency in the lung fields. An ultrasonic cardiographic examination showed that the right atrium and ventricle were dilated. Pulmonary thromboembolism due to left popliteal vein thrombosis was diagnosed by perfusion scintigram of the lung, which showed multiple wedge-shaped defects, and by digital subtraction angiogram, which showed a filing defect in the left popliteal vein. Antiphospholipid syndrome was diagnosed after IgG anticardiolipin antibody was defected. Scleroderma was subsequently diagnosed because the patient exhibited Raynaud's phenomenon and proximal scleroderma. Although closely associated with lupus erythematosus and other lupus variants, antiphospholipid syndrome has not been recognized as a common complication of scleroderma. This is the first report of a patient with pulmonary thromboembolism associated with antiphospholipid syndrome and scleroderma.
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PMID:[Pulmonary thromboembolism associated with antiphospholipid syndrome in scleroderma]. 956 91

A 47-year-old woman was admitted to our hospital for evaluation of general fatigue and dyspnea. She had been diagnosed with progressive systemic sclerosis (PSS) when she was 39 years of age, on the basis of Raynaud's phenomenon, proximal sclerosis, and pigmentation of the skin. On admission, her blood pressure was 206/128 mmHg. Funduscopy revealed grade III (Keith & Wagener) hypertensive retinopathy. Laboratory data showed positivity for anti-nuclear antibody and anticardiolipin beta 2 glycoprotein I antibody, and the plasma level of renin activity (PRA) was abnormally high. Chest X-ray and UCG revealed massive pericardial effusion. On the second hospital day, she was operated on for pericardiodiaphragmatic fenestration. The volume of pericardial effusion amounted to more than 2000 ml. Post operative malignant hypertension persisted. Laboratory data showed thrombocytopenia, hemolytic anemia, and acute renal failure. We diagnosed scleroderma renal crisis (SRC) associated with antiphospholipid syndrome. Following the initiation of angiotensin converting enzyme inhibitor (ACE-I) combined with calcium antagonist and alpha-one blocker, her blood pressure and PRA decreased. She also had been treated with aspirin 81 mg daily. These therapies were effective in recovering the platelet count and stopped the progression of anemia and renal failure. Although either the finding of large pericardial effusion or SRC is associated with poor prognosis in PSS, this case has had a good clinical course. In this case, the findings suggested that anti-phospholipid antibody may have contributed to the pericarditis and SRC.
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PMID:[A case of scleroderma renal crisis with massive pericardial effusion and positivity on antiphospholipid antibody test]. 965 14

A 54-year-old woman with progressive systemic sclerosis (PSS) was admitted to hospital because of dyspnea and chest pain. Echocardiogram revealed diffuse hypokinesis of the left ventricle (ejection fraction 24%). Methylprednisolone, heparin, and diuretics were administered, without benefit. Anemia, thrombocytopenia, and renal dysfunction rapidly progressed, and she died of heart failure on the 14th hospital day. Immunohistochemical study of the myocardial tissue showed mild to moderate cell infiltration, mainly consisting of natural killer (NK) cells, macrophages, cytotoxic T lymphocytes (CTLs), and T helper cells. Perforin, a cytolytic factor, was expressed in the infiltrating CTLs and NK cells, indicating that these cells were activated killer cells. Furthermore, human leukocyte antigen classes I and II, intercellular adhesion molecule-1, as well as costimulatory molecules B7-1, B7-2, and CD40, all of which are known not to be expressed in cardiac myocytes under normal conditions, were moderately to strongly expressed in cardiac myocytes. There was no detectable level of enterovirus genomes in the polymerase chain reaction products from the myocardial tissue of this patient. These findings strongly suggest that the infiltrating killer cells recognized cardiac myocytes as target cells and directly damaged them by releasing perforin. Enhanced expression of these antigens may have played an important role in the activation and cytotoxicity of the infiltrating killer cells. Absence of enterovirus genomes in the myocardial tissue may suggest that this autoimmune process is primarily induced by PSS.
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PMID:Evidence of cell-mediated cardiac myocyte injury involved in the heart failure of a patient with progressive systemic sclerosis. 1008 93

Octreotide has proven to be effective for the treatment of intestinal dysmotility in patients with scleroderma in short-term administration. We report a global improvement of scleroderma manifestations under long-term administration of octreotide. A 53-year-old black woman was diagnosed with a four-year history of progressive and severe systemic scleroderma, with diffuse skin sclerosis, myositic involvement, impaired carbon monoxide transfer factor (57% of the predicted normal value and severe digestive involvement with pseudo-obstruction and bacterial overgrowth into the intestinal lumen). After one month of octreotide (75 mug/d), oral feeding was restarted and weight gain of 6.5 kg was achieved. After 8 months of treatment, normal weight was obtained and skin induration was spectacularly reduced and pigmentation returned to a normal state. Dyspnea disappeared and physical activity was quite normal. Octreotide effects on intestinal transit are unclear and may be secondary to immunomodulation or neurotransmission effects. Extradigestive effects of octreotide in scleroderma have not been studied. This report suggests that long-term administration of octreotide may be beneficial in the treatment of patients with systemic scleroderma. Long-term trials are required to confirm these preliminary results.
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PMID:Global improvement of systemic scleroderma under long-term administration of octreotide. 1049 99

A 46-year-old female was admitted to our hospital due to general fatigue, systemic edema and dyspnea with history of systemic sclerosis (SSc). The patient was diagnosed as mixed connective tissue disease (MCTD) based on Raynaud phenomenon, a high anti-RNP antibody level and clinical symptoms and laboratory findings suggesting SSc, dermatomyositis (DM) and systemic lupus erythematosus (SLE). After the admission, both alveolar hemorrhage and a rapidly progressive glomerulonephritis (RPGN) also developed and laboratory findings showed a positive remark of myeloperoxydase-antineutrophil cytoplasmic antibody (MPO-ANCA) and anti-glomerular basement membrane (GBM) antibody. She was therefore re-diagnosed as microscopic polyarteritis nodosa (microscopic PAN) combined with MCTD and treatment with high dose prednisolone and steroid pulse therapy dramatically improved general conditions and lung symptoms, but maintenance dialysis was persistent because of irreversible renal failure. However, 3 months after the admission, she died of acute exacerbation of interstitial pneumonitis that was unresponsive to steroid pulse therapy. Autopsy revealed interstitial pneumonitis with alveolar hemorrhage and crescentic glomerulonephritis (CrGN), in which immunofluorescent microscopy showed no deposition in agreement with pauciimmune type. The histological findings supported the diagnosis; primary microscopic PAN combined with MCTD, which is a quite rare case, to our knowledge. Furthermore, co-existence of MPO ANCA and anti-GBM antibody, clinical and histological findings of the case also lead us to reconsider the relevance of these antibodies to pathogenesis and/or categories of microscopic PAN and Goodpasture's syndrome.
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PMID:[A case of mixed connective tissue disease with microscopic polyarteritis nodosa associated with perinuclear-antineutrophil cytoplasmic antibody and anti-glomerular basement membrane]. 1061 88

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