1/172. 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) |
2/172. Atrophie blanche lesions closely resembling malignant atrophic papulosis (Degos' disease) in systemic lupus erythematosus.Two patients with systemic lupus erythematosus are described who in the course of their disease developed small atrophie blanche lesions that closely resembled those found in malignant atrophic papulosis. Preliminary investigation of these two cases indicates that considerable similarities probably exist in the pathogenesis of malignant atrophic papulosis and the atropie blanche lesions of systemic lupus erythematosus. It is concluded that a diagnosis of malignant atrophic papulosis should only be made after systemic lupus erythematosus has been excluded by full investigation.- - - - - - - - - - ranking = 1.2862460666132keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
3/172. The spectrum of reactive hemophagocytic syndrome in systemic lupus erythematosus.We address the relationship between reactive hemophagocytic syndrome (RHS), systemic lupus erythematosus (SLE) activity, and treatment in 4 female patients with SLE. Febrile pancytopenia was related to cytologically proven RHS in all patients. Followup was 45 /-7 months from RHS onset. No causal infection could be identified. Outcome could be classified as: (1) RHS onset during a SLE flare and complete efficacy of high dose steroids; (2) death despite therapy for concomitant severe RHS and active SLE; (3) severe RHS in inactive SLE under immunosuppressants, with remission after steroid tapering and cyclophosphamide withdrawal. Three patients were treated with intravenous IgG. We conclude that (1) when SLE is active, RHS should be considered a specific manifestation and treated with steroids; (2) RHS occurring in otherwise inactive SLE might be related to iatrogenic immunosuppression; (3) intravenous IgG treatment might be indicated in both situations.- - - - - - - - - - ranking = 0.91874719043798keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
4/172. Rowell's syndrome.Rowell's syndrome is the name given to a distinct group of patients with lupus erythematosus who develop erythema multiforme-like lesions and have a characteristic serological picture. We report a case of a 29-year-old woman of Afro-Caribbean origin who presented with an erythema multiforme-like eruption on the hands. Subsequently she developed painful erythematous swellings on the feet and scaly plaques on the forearm and thigh consistent with subacute cutaneous lupus. She developed a positive antinuclear factor and had positive anti-Ro and anti-La antibodies and a positive rheumatoid factor. All of these features are consistent with Rowell's syndrome which we believe is a rare but distinct variant of cutaneous lupus erythematosus.- - - - - - - - - - ranking = 0.32700433573292keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
5/172. 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 = 0.66889047791233keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
6/172. Antiphospholipid antibodies syndrome in 'stroke in young'.Antiphospholipid antibodies syndrome has emerged as an important entity responsible for stroke in young. Seven cases of young stroke (< 40 years of age) with mean age of 30.1 years (age range 25-39 years, 2 males and 5 females), who tested positive for antiphospholipid antibodies are being reported. All subjects had completed strokes. Six had arterial ischaemic and one patient had venous stroke. One patient suffered from four episodes, three ischaemic and one intracerebral haemorrhage. Two patients suffered from foetal loss. Generalised tonic clonic seizures occurred in three patients. Deep vein thrombosis was observed in one case. thrombocytopenia was not observed in any case. All the patients had elevated anticardiolipin antibodies (aCL) IgM or IgG, while Lupus anticoagulant (LA) was elevated in 4 cases. Six cases belonged to primary antiphospholipid antibodies syndrome and one to lupus like illness. Oral anticoagulants were administered to maintain a high intensity international normalized ratio (INR). No recurrences were observed during a follow up period of 6-18 months.- - - - - - - - - - ranking = 0.01075377382052keywords = lupus (Clic here for more details about this article) |
7/172. Insulin receptor antibodies and insulin resistance.The presence of insulin receptor antibodies is a rare cause of insulin resistance. patients usually have a combination of hyperglycemia, insulin resistance, acanthosis nigricans, and autoimmune features. We report a patient with systemic lupus erythematosus and severe insulin resistance due to insulin receptor antibodies. The most striking aspect of the clinical presentation is the resistance to insulin therapy, with our patient unresponsive to doses of up to 154,075 units in a day. While on a low-dose glucocorticoid therapy, the patient had clinical improvement, and glucose levels subsequently became normal even without insulin and glucocorticoid.- - - - - - - - - - ranking = 0.1837494380876keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
8/172. lemierre syndrome in an immunocompromised patient.lemierre syndrome or septic thrombophlebitis of the internal jugular vein is a potentially life-threatening complication. This condition may result from oropharyngeal infection, central venous catheterization, and intravenous drug abuse. Immunocompromised patients and individuals with systemic disease are at higher risk of developing the syndrome. We present here a case of septic jugular vein thrombosis in an adolescent with systemic lupus erythematosus. The etiological factors, pathogenesis, and diagnostic and therapeutic measures are discussed.- - - - - - - - - - ranking = 0.1837494380876keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
9/172. Redefining Rowell's syndrome.Rowell's syndrome is believed to be a distinct and rare clinical entity originally described as lupus erythematosus associated with erythema multiforme-like lesions with immunological findings of speckled antinuclear antibodies, anti-La antibodies and a positive test for rheumatoid factor. We report two additional patients with Rowell's syndrome and review all the diagnostic criteria found in the literature. In view of the inconsistent findings of some of the diagnostic features, we propose that major and minor criteria be used to diagnose Rowell's syndrome.- - - - - - - - - - ranking = 0.1581252809562keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
10/172. Pulmonary renal syndrome in childhood: a report of twenty-one cases and a review of the literature.In adults, the term specific pulmonary renal syndrome describes disorders with pulmonary and glomerular manifestations and includes Wegener's granulomatosis, Goodpasture disease, and systemic lupus erythematosus. Nonspecific pulmonary renal syndrome refers to either pulmonary disease complicating glomerular disease, or glomerular diseases following pulmonary disease. Since little is known regarding pulmonary renal syndrome in childhood, we reviewed the charts of 21 pediatric patients with pulmonary renal syndromes treated by the Department of pediatrics, University of Bern between 1991 and 1998; we also reviewed the pediatric literature that deals with specific pulmonary renal syndromes. Specific pulmonary renal syndrome was noted in 3 children with systemic vasculitis (wegener granulomatosis, N = 2; microscopic polyangiitis, N = 1) and 2 with systemic lupus erythematosus. Nonspecific pulmonary renal syndrome was observed in 12 patients with pulmonary edema (N = 9), pulmonary thromboembolism (N = 2), and pulmonary infection (N = 1) complicating the course of a glomerular disease, and in 4 children with a pulmonary disease followed by a glomerular disease. review of the literature disclosed 52 cases of specific pulmonary renal syndrome other than systemic lupus erythematosus: wegener granulomatosis (N = 28), Goodpasture disease (N = 13), and Henoch-Schonlein purpura (N = 11). In addition, hemolytic uremic syndrome complicated pneumococcal pneumonia in 32 cases. We conclude that pulmonary renal syndromes need to be looked for in childhood. Apart from wegener granulomatosis, Goodpasture disease, and systemic lupus erythematosus, Henoch-Schonlein purpura and hemolytic-uremic syndrome occasionally have both pulmonary and renal features.- - - - - - - - - - ranking = 0.73499775235038keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
| | Next -> |