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

The combination of skin induration with clinical features such as dyspnea, facial telangiectasia, digital infarctions and/or dysphagia supports the diagnosis of systemic sclerosis. The oesophageal dysmotility is associated with symptoms which may delay the diagnosis of gastro-oesophageal cancer. Herein we report a case of long standing systemic sclerosis with heartburn and dysphagia symptoms which were monitored closely. Unfortunately, these symptoms delayed the diagnosis of gastric adenocarcinoma. This case prompted us to review the evidence of the association of cancer and systemic sclerosis and if any oncologic evaluation is required during the follow-up of patients affected with systemic sclerosis.
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PMID:[Systemic sclerosis and cancer]. 1680 8

Respiratory failure from causes exclusive of intrinsic lung disease is rare in systemic sclerosis. We report an unusual case of a young woman with diffuse systemic sclerosis who presented with proximal muscle weakness, dyspnea, weight loss, and nasal regurgitation. On physical examination, she had normal breath sounds but severe limitation of chest wall expansion. Pulmonary function tests (PFTs) were consistent with restrictive lung disease. Pulmonary pressures were normal on right heart catheterization and chest radiography and high-resolution computed tomography showed no evidence of intrinsic lung disease. Quadriceps muscle biopsy was consistent with type II atrophy, without any element of inflammatory myositis. After a meal, the patient aspirated, developed severe persistent hypercapnia, and required ventilatory support. Therapy with corticosteroids and intravenous gamma globulin failed to improve her condition, and the patient subsequently expired. Hypercapnic respiratory failure on the basis of chest wall involvement from systemic sclerosis may occur in the absence of intrinsic lung disease, and the prognosis, as in this case, may be grave.
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PMID:Hypercapnic respiratory failure in systemic sclerosis. 1704 22

A 40-year-old woman with known systemic sclerosis presented with dyspnea. She had been treated for pneumonia, 2 months prior to the present admission and at this time presented with sudden dyspnea and was found to have a spontaneous pneumomediastinum and subcutaneous emphysema. Pneumomediastinum is an extremely rare complication in patients with systemic sclerosis.
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PMID:Spontaneous pneumomediastinum and subcutaneous emphysema in systemic sclerosis. 1756 46

Bilateral vocal fold immobility (BVFI) can be the result of a primary disorder or as an iatrogenic complication of surgery or intubation. Laryngeal involvement can be a rare complication of connective tissue disorders and it usually occurs in association with other symptoms and signs that indicate active disease. We present a case of BVFI in a patient with an overlap syndrome rheumatoid arthritis/systemic sclerosis, referred to our division because of dysphonia and dyspnea. The video-laryngostroboscopy showed the presence of BVFI. Physical examination, blood tests, lung and neck high resolution computed tomography scans did not demonstrate significant abnormalities. She was treated with pulses of intravenous methylprednisolone with slow improvement.
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PMID:Bilateral vocal fold immobility in a patient with overlap syndrome rheumatoid arthritis/systemic sclerosis. 1723 56

Systemic sclerosis (scleroderma) is a chronic debilitating disease that is caused by the occurrence of fibrotic changes and vascular abnormalities at various levels such as: skin, lungs, kidneys or heart. Lung involvement in scleroderma is represented by scleroderma interstitial lung disease and by pulmonary arterial hypertension, and is one of the leading causes of mortality in this disease. Pulmonary arterial hypertension can be successfully treated with specific therapies such as sildenafil, bosentan or epoprostenol, whereas only cyclophosphamide has been shown to be effective for interstitial lung disease. The discussed study has shown that cyclophosphamide improved lung function, functional status and health-related quality of life, and reduced dyspnea in scleroderma patients. Most of these patients had acute alveolitis although higher incidences of treatment-related leukopenia and neutropenia were also detected.
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PMID:Cyclophosphamide for scleroderma interstitial lung disease. Tashkin DP, Elashoff R, Clements PJ et al.: Cyclophosphamide versus placebo in scleroderma lung disease. N. Engl. J. Med. (2006) 354(25):2655-2666. 1679 Jun 98

The term "systemic sclerosis sine scleroderma" (ssSSc) has been used to designate a rare progressive systemic sclerosis of visceral organs without skin manifestations. A variety of visceral organs, including the gastrointestinal tract, lung, heart, and kidney, can be involved. We describe a case of 59-year-old female patient with both Wolff-Parkinson-White (WPW) syndrome and ssSSc. She was diagnosed as having ssSSc with Raynaud's phenomenon, anti-nuclear antibody (ANA) and anti-topoisomerase antibody positivity, interstitial pulmonary infiltrates, suspected pulmonary hypertension, subclinical oesophageal dysmotility but no skin thickening. She had a history of paroxysmal tachycardia together with Raynaud's phenomenon and exercise-induced dyspnoea. Electrophysiological study confirmed WPW syndrome with left posterior bypass tract. This case highlights cardiac arrhythmia caused by WPW syndrome as a clinical manifestation of the heart in ssSSc.
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PMID:Systemic sclerosis sine scleroderma associated with Wolff-Parkinson-White syndrome. 1745 39

The lungs are frequently involved in systemic sclerosis ('scleroderma'), a rare, disabling disease of unknown origin, characterised by skin thickening and Raynaud's phenomenon. The pathogenesis of scleroderma is complex, but signs and symptoms of excessive fibrosis, vasculopathy and inflammation are almost universally present. Dyspnoea in scleroderma patients can be due to chest wall tightening from skin thickening, pleural disease, cardiac involvement, myositis of intercostal muscles, or so-called scleroderma lung disease. Scleroderma lung disease encompasses vascular (pulmonary artery hypertension) or interstitial lung disease, or both. A comprehensive work-up is required to delineate the underlying cause of dyspnoea in a scleroderma patient, and to establish the contribution of each component to the symptoms. This should include a 6-minute walk test, pulmonary function testing, high-resolution thoracic CT scanning, ECG, echocardiography and, if pulmonary artery hypertension is suspected, right-heart catheterisation; bronchoalveolar lavage is optional. Lung disease in scleroderma contributes significantly to excess morbidity and early mortality, especially when diffusion capacity drops below 40% and/or forced vital capacity below 50%. However, recent clinical studies have unequivocally demonstrated that scleroderma lung disease is amenable to treatment with new vasodilatory drugs that target specific pathways involved in vasoconstriction, or with cyclophosphamide for interstitial lung disease. Uncontrolled studies have suggested that these therapies also have an impact on survival, but controlled studies with a long follow-up are needed to corroborate this point.
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PMID:Scleroderma lung: pathogenesis, evaluation and current therapy. 1748 44

Interstitial lung disease (ILD) is a leading cause of death in systemic sclerosis (SSc). Two randomized controlled trials recently demonstrated the modest effects of cyclophosphamide on lung physiology (forced vital capacity) and extrapulmonary outcomes (dyspnea, function, quality of life, and skin thickening). Recommendations can now be made about the short-term management for SSc-ILD. However, many questions remain unanswered, including how long to treat with cyclophosphamide; whether patients should take maintenance therapy after the initial or induction phase; whether there are alternative therapies; how to treat patients with ILD and pulmonary hypertension; and how to treat patients with severe ILD.
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PMID:Management of interstitial lung disease in systemic sclerosis: lessons from SLS and FAST. 1750 45

Acral ulcers in patients with progressive systemic sclerosis (PSS) are often recalcitrant to therapy. Sildenafil, an inhibitor of phosphodiesterase-5, dilates small arteries by increasing endothelial cGMP. Oral administration of sildenafil to a 35-year-old white male patient suffering from incapacitating PSS with severe pulmonary arterial hypertension and acral ulcers induced a clinically significant reduction in dyspnea and increase in walking distance within one week as well as complete and long-lasting healing of all ulcers within five weeks. This case demonstrates the efficacy of sildenafil in the treatment of scleroderma-associated refractory acral ulcers.
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PMID:[Fast and efficient healing of scleroderma-associated acral ulcers with sildenafil]. 1761 9

We describe a 73-year-old woman with systemic sclerosis (SSc)-polymyositis (PM) overlap syndrome, primarily SSc followed by PM. She had suffered from SSc and had interstitial pneumonia (IP), which was stable. Eight years after the initial diagnosis of SSc, proximal muscle weakness, myalgia, and dyspnea on effort developed. A chest computed tomography (CT) showed reticular shadows, and serum markers of IP such as KL-6 and surfactant protein-D were elevated at 1,170 U/mL and 176 ng/mL, respectively. Bronchoalveolar lavage fluid showed a remarkably increased number of lymphocytes. Exacerbation of SSc-IP 8 years after the initial diagnosis of SSc is not usual, and a marked increase in the number of lymphocytes in bronchoalveolar lavage fluid is also uncommon in SSc-IP, indicating overlap of another connective tissue disease. The diagnostic criteria for PM were satisfied; thus, SSc-PM overlap syndrome was diagnosed. We emphasize the need to investigate whether another connective tissue disease has developed when symptoms or laboratory findings cannot be explained by the usual clinical course of an existing connective tissue disease.
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PMID:Progression of interstitial lung disease upon overlapping of systemic sclerosis with polymyositis. 1767 76


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