1/71. Pneumatosis intestinalis in association with connective tissue disease.Pneumatosis intestinalis in association with connective tissue diseases is an unusual combination whose pathogenesis is not yet understood. Furthermore, steroid medication, often used to treat these diseases, may itself cause pneumatosis. Three cases of scleroderma, systemic lupus erythematosus, and amyloidosis in association with pneumatosis and without prior steroid therapy are presented. The small vessel occlusive pathologic processes in these diseases may cause focal areas of mucosal ischemia resulting in small, perhaps transient ulcerations that allow gas to enter the gut wall from the lumen.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
2/71. diagnosis and physiopathology of laryngeal deposits in autoimmune disease.We report the clinical features and pathology of a previously unreported form of vocal fold disease seen in 4 patients, 3 of whom were diagnosed as autoimmune disease. The characteristic features of these lesions were found as bilateral transverse lesions in the mid portion of the vocal folds. The patients had dysphonia and diplophonia. Stroboscopic examinations showed 180-degree phase shifts between the anterior and posterior portion of the vocal folds. Case 1 had systemic lupus erythematosus, case 2 had Hashimoto's thyroiditis, and case 3 had progressive systemic sclerosis. Prior to the onset of hoarseness, autoimmune antibody titers were increased. These cases need differential diagnosis from vocal fold nodules and cysts. Two cases were recurrent after endoscopic surgery, 1 recurring 3 times. Glucocorticoid was effective in preventing the recurrence in the early phase. recurrence may have occurred because the surgical strategy was the same as that used for vocal fold nodules or cysts.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
3/71. 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 = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
4/71. Development of systemic sclerosis in a patient with systemic lupus erythematosus and topoisomerase I antibody.We describe a patient with systemic lupus erythematosus (SLE) associated with topoisomerase I (topo I, Scl-70) antibody, a specific marker for systemic sclerosis (SSc). SSc patients who produce this antibody have severe cutaneous and visceral involvement, and eventually have a poor prognosis. It is rare to find this antibody in patients with other collagen diseases. Only four SLE patients have been reported in the English language literature who were topo I antibody-positive but had no clinical evidence of scleroderma. The serum of our patient with SLE had precipitating topo I antibody from the onset of his disease. Twelve years after the onset of SLE, he developed proximal scleroderma and pulmonary fibrosis. This case reconfirms the prognostic significance of topo I antibody as a predictive marker, and indicates that SLE patients with topo I antibody require careful follow-up for future development of scleroderma.- - - - - - - - - - ranking = 5keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
5/71. A juvenile case of overlap syndrome of systemic lupus erythematosus and polymyositis, later accompanied by systemic sclerosis with the development of anti-Scl 70 and anti-Ku antibodies.We describe a 16-year-old girl with an overlap syndrome consisting of systemic lupus erythematosus (SLE) from the age of 7 and polymyositis (PM) from the age of 10, later accompanied by systemic sclerosis (SSc) from the age of 15. She was diagnosed as having SLE with exudative malar erythema, photosensitivity, and discoid rashes with positive antinuclear antibody (ANA) and anti-dna antibody titers. The diagnosis of PM was also made in accordance with findings of a high titer of muscle enzymes and a muscle biopsy specimen demonstrating marked degeneration of the muscle fibers and perivascular infiltration of mononuclear cells. She developed Raynaud's phenomenon and pitting ulcers on her fingers with positive anti-Scl 70 and anti-Ku antibodies, leading to a diagnosis of SSc. The patient was treated with prednisolone. To our knowledge this is the youngest case of SLE-PM overlap syndrome later accompanied by SSc.- - - - - - - - - - ranking = 5keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
6/71. Progressive appearance of overlap syndrome together with autoantibodies in a patient with fatal thrombotic microangiopathy.We describe an extraordinary patient with overlap syndrome (systemic lupus erythematosus, systemic sclerosis, and rheumatoid arthritis) having positive autoantibodies against Sm, double stranded dna, dna topoisomerase I, and centromere, together with rheumatoid factor. The patient had multiple organ involvement resulting from thrombotic microangiopathy that mimicked so-called normotensive scleroderma renal crisis, and died mainly of massive pulmonary hemorrhage caused by thrombotic thrombocytopenic purpura. The clinical presentations of the case support the concept of strong associations between disease-specific autoantibodies and clinical features.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
7/71. Scleroderma cerebritis, an unusual manifestation of progressive systemic sclerosis.A 42-year-old female with scleroderma experienced two exacerbations in which behavioral changes were the main clinical features. On both occasions she presented with paranoid delusions, perceptual aberrations, and disorientation. After treatment with corticosteroids, the patient's mental status returned to normal, and her electroencephalogram showed an increase in alpha wave frequency, which is consistent with a resolving delirium. Unlike systemic lupus erythematosus, scleroderma rarely involves the central nervous system. This case illustrates an unusual manifestation of progressive systemic sclerosis, primary cerebral involvement which presented as an acute organic brain syndrome. connective tissue diseases, notably systemic lupus erythematosus, often present neuropsychiatric symptoms. Despite the fact that there appears to be a clinical and pathological continuum among the connective tissue diseases, an organic psychosis rarely occurs in progressive systemic sclerosis (scleroderma. Described here is a patient with scleroderma in whom behavioral abnormalities were the main features of two exacerbations of the disease.- - - - - - - - - - ranking = 2keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
8/71. Renal crisis in asclerodermic scleroderma--lupus overlap syndrome.A 24-year-old woman with overlapping features of sclerodermia sine scleroderma and systemic lupus erythematosus (SLE) presented with rapidly accelerating hypertension accompanied by neuropsychological deficits and tonic-clonic seizures. kidney biopsy showed severe intimal hyperplasia of small renal arteries but no glomerulonephritis. Following treatment with ACE inhibitor, prednisolone and cyclophosphamide complete remission was achieved with minimal brain damage and normal kidney function. Anti-rna polymerase i, II and III antibodies have remained positive during follow-up for 2 years, suggesting a linkage with the underlying pathogenetic pathway.- - - - - - - - - - ranking = 1.1797926150066keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
9/71. Papular and nodular mucinosis as a presenting sign of progressive systemic sclerosis.Papular and nodular mucinosis is a distinct form of cutaneous mucinosis associated with systemic lupus erythematosis. We report a case in which papular and nodular mucinosis predated early findings of progressive systemic sclerosis.- - - - - - - - - - ranking = 0.077106944194805keywords = lupus, systemic lupus (Clic here for more details about this article) |
10/71. pityriasis versicolor rubra.We report six typical cases with pityriasis versicolor (PV) rubra, with a background of collagen diseases in five cases and none in one case. Two cases of systemic lupus erythematosus (SLE) and one case of systemic scleroderma (SSc) had both PV rubra and nigra on the trunk. diagnosis of superficial infections of PV was made by microscopic examination of skin scrapings following KOH, and many small whitish colonies were obtained in Sabouraud's slant agar medium culture containing cycloheximide with olive oil in all cases. malassezia sympodialis was isolated from the scales of two different lesions at a 6 week-interval in a same person (a 32 year-old male without SLE nor SSc) by the method of Makimura et al. [5], although the other five cases were not examined for the isolation. Histopathological features of the lesion on the dorsum of the trunk showed no epidermal hyperplasia without elongation of rete ridges and no inflammatory cell infiltration in the dermis, however there was only dilatation of small blood vessels in the dermis, which was reconfirmed capillaroscopically. In the horny layers, several yeastlike and fine filamentous structures were seen which were positive with PAS and Grocott stains. Both clinical and histological features led us to speculate PV rubra. All the patients were treated with anti-fungal ointment, and the lesions diminished in less than 2 weeks. No recurrence has been seen in any of the cases. This PV rubra may be independent from PV nigra, although Horiuchi [2] suggested the earlier lesion occurs in advance of PV alba or nigra.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
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