21/622. A case of systemic sclerosis associated with gastric cancer.We report the case of a 47-year-old woman who began to experience stiffness and Raynaud's phenomenon of her fingers and toes as well as easy fatigability approximately one year before initial evaluation. Histopathologic examination revealed dense fibrosis and perivascular lymphoid cell infiltration in the dermis. The patient's diagnosis was systemic sclerosis (SSc). Esophageal sclerosis was not revealed, but an early type IIb carcinoma of the gastric antrum was noted by endoscopic examination. Other recent reports have discussed the association of SSc with various malignant carcinomas. We also reviewed the literature for associations between SSc and gastric cancer in Japanese patients.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
22/622. in vitro inhibition of hematopoiesis in a patient with systemic sclerosis treated with D-penicillamine.A 66-year-old Japanese woman with severe scleroderma developed anemia and thrombocytopenia due to D-penicillamine (D-Pen) treatment, although the leukopenia was not markedly severe. Cessation of D-Pen and the start of corticosteroid therapy were followed by recovery from bicytopenia. We examined the in vitro inhibition of the clonogenic capacity of bone marrow hematopoietic progenitor cells of this patient by D-Pen, and found strong inhibition in burst-forming unit-erythroid and colony-forming unit-megakaryocyte assays, but not in colony-forming unit-granulocyte/macrophage assays. These findings suggest that bicytopenia in this patient was caused by D-Pen and may be due to different sensitivities in the hematopoietic lineage.- - - - - - - - - - ranking = 0.66666666666667keywords = sclerosis (Clic here for more details about this article) |
23/622. Systemic sclerosis and scleroderma renal crisis in association with carcinoma of the breast.During recent years it has been shown conclusively that the incidence of malignancy in systemic sclerosis (SSc) is significantly increased and that rapidly progressive skin thickening, among other factors, is a strong predictor of scleroderma renal crisis (SRC). We present a case of carcinoma of the breast presenting concurrently with SSc that subsequently progressed to dialysis-dependent renal failure in just 1 month. We discuss the possible relationship between SSc and malignancy at a cellular level. In conclusion, we recommend that patients with rapidly progressive SSc and SRC at an early stage be screened for malignancy. In those patients developing malignancy in SSc, a careful vigil for accelerated renal, cardiac, and pulmonary complications should also be undertaken.- - - - - - - - - - ranking = 0.83333333333333keywords = sclerosis (Clic here for more details about this article) |
24/622. Renal transplantation in scleroderma.Although the outcome of renal transplantation in patients with systemic lupus erythematosus (SLE) has been studied, there are few reports about the outcome of patients with systemic sclerosis who have undergone renal transplantation. We retrospectively collected data from the United Network for Organ Sharing (UNOS) Scientific Renal Transplant Registry from a 10-year period. From 1987 to 1997, 86 patients with systemic sclerosis who had renal transplantation were identified. Of these 86 patients, 70% were women, 86% were Caucasian, and the mean age at transplantation was 50.4 years. The overall mortality was 24% of the patient group; 44% (38/86) of renal grafts failed. First- through fifth-year graft survival rates were 62%, 60%, 57%, 50%, and 47%, respectively. The causes of graft failure could not be ascertained in 24 of 38 patients (63%). Among the known causes, 5 had acute rejection, 4 had chronic rejection, 3 had recurrence of scleroderma, and 1 each had infection and graft thrombosis. Immunosuppressive regimens used in the patients with systemic sclerosis consisted of antilymphocyte globulin in at least 25%. Sixty percent received a combination of steroids, azathioprine, and cyclosporine. The use of cyclosporine was not associated with either improvement of graft survival or an increased rate of graft failure. graft survival at 5 years in patients with systemic sclerosis was comparable to that of patients with SLE who received renal transplantation, according to existent medical literature. Based upon these data, renal transplantation is as effective a treatment for restoring renal function in patients with systemic sclerosis as it is in patients with SLE. Those patients with systemic sclerosis whose renal function did not improve with angiotensin-converting enzyme (ACE)-inhibitor treatments after scleroderma renal crisis should be considered as transplant candidates. Although the data are incomplete, the use of cyclosporine may not confer the advantage of improving graft survival in patients with systemic sclerosis as compared with SLE patients.- - - - - - - - - - ranking = 1.1666666666667keywords = sclerosis (Clic here for more details about this article) |
25/622. Bilateral scleral pit associated with systemic sclerosis.PURPOSE: To describe a case of a bilateral scleral pit in a patient with systemic sclerosis. methods: Case report. RESULTS: A bilateral scleral pit with surrounding scleral ischemia overlying the pars plana was noted in a 72-year-old woman with known systemic sclerosis. CONCLUSIONS: Scleral pits should be added to the list of ocular findings associated with systemic sclerosis.- - - - - - - - - - ranking = 1.1666666666667keywords = sclerosis (Clic here for more details about this article) |
26/622. Three cases of osteonecrosis of the lunate bone of the wrist in scleroderma.This report describes three cases of osteonecrosis of the lunate bone of the wrist in patients with systemic sclerosis presenting with wrist pain. All three patients had limited skin scleroderma but severe Raynaud's phenomenon. Two patients never received corticosteroids and one patient received only low doses for a brief period. None of the patients had other definable risk factors for osteonecrosis. Two patients underwent vascular bone grafting with improvement in symptoms. osteonecrosis may represent an under-recognized cause of wrist pain in scleroderma patients.- - - - - - - - - - ranking = 0.16666693928348keywords = sclerosis, ms (Clic here for more details about this article) |
27/622. Localized subcutaneous nocardia farcinica abscess in a woman with overlap syndrome between systemic scleroderma and polymyositis.nocardia farcinica is an increasingly common cause of human infection in europe. We observed an isolated nodular subcutaneous abscess due to N. farcinica in the forearm of a young patient with overlap syndrome between systemic sclerosis and polymyositis. Despite in vitro resistance to trimethoprim-sulfamethoxazole, this oral combination completely resolved the lesion within 3 weeks and was maintained for 6 months. The spectrum of cutaneous nocardiosis in the immunocompromised host is discussed.- - - - - - - - - - ranking = 0.16666666666667keywords = sclerosis (Clic here for more details about this article) |
28/622. pemphigus vulgaris induced by D-penicillamine therapy in a patient with systemic sclerosis.D: -penicillamine-induced pemphigus occurs infrequently, typically in patients with rheumatoid arthritis. We describe a patient with systemic sclerosis who experienced this complication 3 months after starting D -penicillamine therapy. Nikolsky's sign, histopathologic findings, and direct immunofluorescence all confirmed the diagnosis. Termination of disease progression required intravenous pulse glucocorticoids, azathioprine, and 3 courses of plasmapheresis. The presentation, treatment, and etiology of D -penicillamine-induced pemphigus are reviewed, and the incidence of this complication in scleroderma patients is examined.- - - - - - - - - - ranking = 0.83333333333333keywords = sclerosis (Clic here for more details about this article) |
29/622. Systemic sclerosis and antineutrophil cytoplasmic autoantibody-associated renal failure.We report three patients who developed antineutrophil cytoplasmic autoantibody (ANCA)-associated crescentic glomerulonephritis, two of whom showed clinical features of limited scleroderma and one whose results of serological tests were suggestive of limited scleroderma without cutaneous features. All had anticentromere antibodies and antimyeloperoxidase antibodies. No patient showed the features of typical scleroderma renal crisis such as accelerated hypertension or microangiopathy. Our patients were normotensive at the time of onset of renal failure, and the clinical picture was characterised by only modest features of limited scleroderma. All three patients had crescentic glomerulonephritis at various stages of chronicity. One patient responded to immunosuppressive therapy with improvement in renal function; the other two patients rapidly developed end-stage renal failure. These patients and others recently described may represent a newly described form of scleroderma renal disease.- - - - - - - - - - ranking = 0.66666666666667keywords = sclerosis (Clic here for more details about this article) |
30/622. Raynaud's phenomenon possibly induced by a compund drug of tegafur and uracil.We describe a 50-year-old woman who noted acral hyperpigmentation, sclerodactyly and Raynaud's phenomenon with 1:160 of antinuclear antibody titer after treatment with a compound drug with tegafur and uracil. Histological findings of the finger and palm included hyperkeratosis, vacuolar degeneration of basal cells, thickened collagen fibers in the dermis, and dilatation of capillary vessels, perivascular mononuclear cell infiltration and melanophages in the upper dermis. IgG, IgA, IgM, C3 and C1q were not deposited in the skin by direct immunofluorescence study. After cessation of the drug, Raynaud's phenomenon and hyperpigmentation disappeared within 1 month and 4 months, respectively, and antinuclear antibody turned negative within 4 months. These observations suggest that tegafur may have caused not only hyperpigmentation in the palms and soles, but also sclerodactyly and Raynaud's phenomenon in the present case.- - - - - - - - - - ranking = 2.726168129895E-7keywords = ms (Clic here for more details about this article) |
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