1/151. Systemic lupus erythematosus with optical neuromyelitis (Devic's syndrome). A case with a 35-year follow-up.Optical neuromyelitis or Devic's syndrome is a very uncommon neurological manifestation of systemic lupus erythematosus. It is also associated with antiphospholipid antibodies, limited responsiveness to glucocorticoid treatment and a poor prognosis. We report the case of a female systemic lupus erythematosus patient who developed recurrent flares of optical neuritis and transverse myelitis. These flares consistently responded to glucocorticoid therapy. Despite the absence of overt anticardiolipin antibodies in the course of the disease, long-term anticoagulant therapy has been introduced with positive results. Treatments are usually of limited efficacy in Devic's syndrome. In our patient, however, aggressive glucocorticoid treatment resulted in prolonged survival.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
2/151. Systemic adverse effect of antithyroid drugs.Antithyroid drugs adverse effects are varied and rare. Autoimmune disorders (vasculitis, lupus erythematosus, polyarthritis...) are unusual and serious complications of antithyroid drugs. Since 1945, fewer than 100 cases of systemic manifestations related to antithyroid drugs have been reported in the literature, most frequently with propylthiouracil. The outcome is usually good after drug discontinuation, but some fatal cases have been reported. Because possible cross-sensitivity with other antithyroid drugs, the appropriate treatment for hyperthyroidism relapse if a patient has had an antithyroid drug adverse reaction, should be 131I-iodine or surgery. We report four new cases of systemic manifestations during propylthiouracil therapy.- - - - - - - - - - ranking = 0.15605203835797keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
3/151. Cryosupernatant plasma exchange in the treatment of antiphospholipid antibody syndrome with lupus nephritis.We report a case of a 22-year-old female with antiphospholipid antibody syndrome (APS) associated with systemic lupus erythematosus in whom cryosupernatant plasma exchange was effective and improved both the refractory venous thrombosis in her legs and relapsing thrombocytopenia. A renal biopsy specimen showed not only features of active lupus nephritis but also renal arteriolar thrombosis which is considered to be a type of thrombotic microangiopathy (TMA). Because a pathological role of unusually large von willebrand factor (vWF) multimers has been reported in patients with TMA including thrombotic thrombocytopenic purpura, plasma exchange using replacement with cryosupernatant, which is free of unusually large vWF multimers, is likely to be an option of treatment modality for patients with refractory and chronic relapsing APS manifesting TMA.- - - - - - - - - - ranking = 0.24910210151848keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
4/151. Lupus erythematosus associated with erythema multiforme: does Rowell's syndrome exist?We describe a patient with lupus erythematosus who experienced an unusual erythema multiforme-like eruption suggestive of Rowell's syndrome. We compare our case and 9 other reports of lupus erythematosus associated with erythema multiforme to the 4 cases reported by Rowell. Our findings indicate that Rowell's original criteria are not well preserved. The coexistence of lupus erythematosus with erythema multiforme does not impart any unusual characteristics to either disease, and the immunologic disturbances in such patients are probably coincidental.- - - - - - - - - - ranking = 0.6512023939089keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
5/151. Recurrent thrombo-embolic episodes: the association of cholangiocarcinoma with antiphospholipid syndrome.antiphospholipid syndrome is a disorder of recurrent vascular thrombosis, pregnancy loss and thrombocytopenia associated with persistently elevated levels of antiphospholipid antibodies. It was first described in a group of patients with systemic lupus erythematosus but has since been associated with a wide range of conditions, including other autoimmune disorders and malignancy. It can also occur in isolation, the so-called primary antiphospholipid syndrome. We describe an elderly woman with the antiphospholipid syndrome thought to be associated with a cholangiocarcinoma.- - - - - - - - - - ranking = 0.18789592328406keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
6/151. Recurrent proximal white subungual onychomycosis associated with a defect of the polymorphonuclear chemotaxis.Proximal white subungual onychomycosis (PWSO) is a rare form of nail infection that occurs almost exclusively in immunocompromised patients. Initially, in several reports, PWSO was described in ARC and AIDS patients. Later this pattern of onychomycosis was observed in patients with renal transplants, who received immunosuppressive therapy, and recently in a woman with active systemic lupus erythematosus (SLE) treated with systemic steroid therapy. We report a case of recurrent PWSO in a woman affected by a defect of polymorphonuclear chemotaxis. The association between PWSO and a defect of neutrophil chemotaxis, not yet described in the literature, suggests a point of discussion about the role of polymorphonuclear leucocyte functions in the defense mechanisms of the host affected by dermatophytosis. In this report the close association between PWSO and an immunocompromised condition is once again described. For this reason the authors emphasize the importance of investigating the common and uncommon causes of immunodeficiency in all patients affected by PWSO.- - - - - - - - - - ranking = 0.18789592328406keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
7/151. Development of focal segmental glomerulosclerosis in the renal allograft of a patient with lupus.Nephritis has been a recognized complication of systemic lupus erythematosus since the early 1900s. Almost all lupus patients have some degree of renal involvement related to their condition, but a considerably smaller proportion of these patients actually progress to end-stage renal disease (ESRD). However, lupus patients are also susceptible to other primary renal insults that may significantly contribute to the deterioration in their renal function. We present a case of a patient with clinical and pathological evidence of lupus nephritis that progressed to ESRD and subsequently developed "recurrent" focal segmental glomerulosclerosis in her transplant kidney. Retrospective clinicopathologic correlation suggested the possibility of more than 1 primary renal process that eventually led to her dialysis-dependent state. This case illustrates the importance of meticulously examining both clinical and renal biopsy data in patients with lupus nephritis and considering the presence of co-existing renal pathologies to resolve an otherwise discordant picture of disease progression. These considerations may have important therapeutic and prognostic implications.- - - - - - - - - - ranking = 0.28582580845914keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
8/151. Mitral restenosis in the early postoperative period of a patient with systemic lupus erythematosus.A forty-eight year old woman, who had undergone mitral comissurotomy and subsequently developed early restenosis, presented with major comissural fusion and verrucous lesions on the cuspid edges of the mitral valve, with normal subvalvar apparatus. Patient did well for the first six months after surgery when she began to present dyspnea on light exertion. A clinical diagnosis of restenosis was made, which was confirmed by an echocardiogram and cardiac catheterization. She underwent surgery, and a stenotic mitral valve with verrucous lesions suggesting Libman-Sacks' endocarditis was found. Because the diagnosis of systemic lupus erythematosus (SLE) had not been confirmed at that time, a bovine pericardium bioprosthesis (FISICS-INCOR) was implanted. The patient did well in the late follow-up and is now in NYHA Class I.- - - - - - - - - - ranking = 0.93947961642028keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
9/151. A case of antiphospholipid antibody syndrome diagnosed after thrombosis of an arteriovenous shunt.A 32-year-old male dialysis patient with lupus nephritis was admitted because of shunt obstruction. The arteriovenous fistula was reconstructed, but obstruction recurred twice within several hours after surgery. A high blood level of anticardiolipin beta2-glycoprotein I antibody suggested that shunt obstruction was caused by a thrombotic tendency related to the antiphospholipid antibody syndrome. Accordingly, for the third shunt procedure, antiplatelet therapy (which had been commenced for systemic lupus erythematosus) was combined with dalteparin sodium from before surgery and warfarin was added postoperatively. This regimen prevented shunt obstruction. In conclusion, hemodialysis patients who suffer repeated shunt obstruction should be examined for antiphospholipid antibody syndrome.- - - - - - - - - - ranking = 0.20013715893094keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
10/151. 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 = 0.18789592328406keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
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