Cases reported "Scleroderma, Systemic"

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21/26. Scleroderma renal crisis as a presenting feature in the absence of skin involvement.

    A 56-year-old man was admitted to the nephrology unit with a short (6-week) history of severe hypertension that necessitated renal replacement therapy within 7 days after admission. Renal biopsy showed features of thrombotic microangiopathy in arterioles and small arteries with occluding thrombi. The skin was unremarkable at the time of admission. Progressive skin lesions with scleroderma, telangiectasia, sclerodactyly, and generalized cutaneous sclerosis developed within 4 weeks and the specific skin changes were found on skin biopsy. On admission antinuclear antibody titers were high (1:10, 240) with a nucleolar pattern, and PM-Scl antibodies (1:5, 120) were present. In the present case the diagnosis of scleroderma renal crisis was made in vivo by renal biopsy. Renal and skin biopsies documented that renal lesions may precede the clinically manifest skin lesions of progressive systemic sclerosis.
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ranking = 1
keywords = microangiopathy
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22/26. Large vessel occlusive disease associated with crest syndrome and scleroderma.

    OBJECTIVES--To report the cases of three patients with crest syndrome and one patient with diffuse scleroderma who had severe macrovascular disease and only minimal vascular risk factors. methods--The medical histories, physical examinations, and results of clinical investigations were reviewed in four patients. RESULTS--These four patients had severe morbidity from macrovascular disease of the arms and legs in the presence of minimal underlying vascular risk factors. These patients represent 11% of the women with scleroderma seen at our hospital since 1974. This is a greater than threefold increase above the expected proportion of symptomatic vascular disease seen in population studies. In the patients with crest syndrome, large vessel disease was first seen more than 10 years after the onset of Raynaud's phenomenon, which was the first manifestation of the disease. A pathological specimen of the ulnar artery from one patient showed severe luminal narrowing by an acellular material with no evidence of atheroma. CONCLUSIONS--These cases suggest an association of both the crest syndrome and scleroderma with macrovascular disease.
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ranking = 4.6426856023036
keywords = vascular disease
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23/26. Peripheral neuropathy in scleroderma.

    nervous system involvement is rare in progressive systemic sclerosis (PSS). We present a clinical pathological and immunological study of two patients with peripheral sensory motor neuropathy and PSS. In both, the sural nerve biopsies showed axonal degeneration with increased endoneurial connective tissue. There were also clusters of myelinated fibres indicating axonal regeneration. Only mild microangiopathic changes were evident in the endo, peri and epineurial vessels. By Western immunoblots, patients' sera contained a band of reactivity to a protein from peripheral nerve identified as collagen type i. Primary involvement of the peripheral nerves during PSS is very unusual. Abnormal production of collagen tissue and presence of microvascular disease are considered to be two possible causes of neuropathy. We think that our results suggest the important role of the connective tissue proliferation in the pathogenesis of PSS neuropathy.
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ranking = 1.1606714005759
keywords = vascular disease
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24/26. Acute and late reactions to radiation therapy in patients with collagen vascular diseases.

    BACKGROUND. A commonly held belief is that patients with collagen vascular diseases (CVD) have a greater risk of radiation therapy complications than patients without CVD. This impression is based on anecdotal reports, however. methods. A group of 61 patients with CVD were compared with a matched control group of 61 patients without CVD. The CVD group included 39 patients with rheumatoid arthritis (RA), 13 with systemic lupus erythematosus (SLE), 4 with systemic sclerosis (scleroderma) (SSc), 4 with dermatomyositis, and 1 with polymyositis. The control group was matched with respect to age, sex, tumor site and histologic characteristics, treatment aim, general treatment method, radiation therapy technique, site irradiated, radiation dose, date of treatment, and follow-up. RESULTS. overall, there was no significant difference between the CVD and control groups in terms of acute (11% versus 7%, respectively) or late complications (10% versus 7%, respectively). This was also true when only patients who were treated definitively were considered. Furthermore, none of the patients treated palliatively had complications. Three patients in the CVD group had fatal complications, compared with none in the control group. RA was associated with a slight increase in late complications in the definitively treated patients, whereas SLE was associated with a slight increase in acute reactions. No significant acute or late reactions were observed in the patients with SSc, dermatomyositis, or polymyositis. CONCLUSIONS. In general, these differences are less than expected and not statistically significant. Consequently, from these data, the authors could not show a significant increase in radiation therapy complications for patients with CVD.
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ranking = 1.1606714005759
keywords = vascular disease
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25/26. Systemic sclerosis associated with right ventricular cardiomyopathy.

    Systemic sclerosis is an uncommon multisystem disorder of unknown aetiology which predominantly affects the skin. Cardiac involvement, which is far more common than was originally realized, may affect any part of the heart but most frequently affects the left ventricular myocardium. Right ventricular dysfunction is usually associated with pulmonary vascular disease. We report a case of systemic sclerosis associated with right ventricular cardiomyopathy in whom pulmonary artery pressures were normal.
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ranking = 1.1606714005759
keywords = vascular disease
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26/26. Antiphospholipid antibodies and retinal vascular disease.

    BACKGROUND: Antiphospholipid antibody retinopathy is a rare and poorly defined clinical entity in which the proper diagnosis has important ocular and systemic implications. The clinical course of five patients with antiphospholipid antibody retinopathy is described and the role of such antibodies in idiopathic retinal venous occlusive disease is investigated. methods: Five case reports are presented highlighting the presentation, course, therapy, associations and outcomes of patients with antiphospholipid antibody retinopathy. In addition, twenty additional patients presenting with idiopathic retinal venous occlusive disease were tested for the presence of antiphospholipid antibodies. RESULTS: All five patients with antiphospholipid antibody retinopathy had diffuse retinal vascular occlusion. All five patients presented with associated rheumatologic disease, including three with lupus or lupus-like disease. antibodies to antiphospholipid were not detected in any of the twenty patients with idiopathic retinal venous occlusive disease. Prompt panretinal photocoagulation together with varying regimens of corticosteroids, immunosuppressives, or warfarin was partially successful in stabilizing the ocular and systemic disease. CONCLUSION: The diagnosis of antiphospholipid antibody retinopathy should be suspected in patients with diffuse retinal vaso-occlusion, particularly when characterized by involvement of both arteries and veins, neovascularization at presentation, and symptoms of symptoms of systemic rheumatologic disease. Antiphospholipid antibodies do not appear to play an important role in idiopathic retinal vein occlusions.
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ranking = 4.6426856023036
keywords = vascular disease
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