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Biomedical subjects

L T Du

Publications and source records attributed to L T Du.

28 records · Page 2Linked to original sources

Wegener's granulomatosis. Dermatological manifestations in 75 cases with clinicopathologic correlation.

BACKGROUND AND DESIGN: Mucosal and cutaneous manifestations of Wegener's granulomatosis (WG) are usually described separately. Both frequently occur at any time of the illness. The aim of this work was to analyze, retrospectively, dermatologic symptoms of 75 WG cases encountered from 1973 through 1992. All patients fulfilled the American College of Rheumatology criteria for WG. We compared clinical and histologic findings and looked for a relationship between these manifestations, disease activity, and other symptoms of WG. RESULTS: Thirty-five patients had skin or mucosa involvement. Clinical features were palpable purpura (26 cases), oral ulcers (15), skin nodules (six), skin ulcers (five), necrotic papules (five), gingival hyperplasia (three), pustules (two), palpebral xanthoma (two), genital ulcer (one), digital necrosis (one), and livedo reticularis (one). Pathologic findings depended on clinical aspects. Thirty-five involved skin or mucosa biopsy specimens were obtained from 24 patients. Nongranulomatous vasculitis was associated with purpuric lesions. Granulomatous inflammation was associated with nonpurpuric lesions. Dermatologic manifestations were associated with a higher frequency of articular and renal involvement (68% vs 25%; 80% vs 47%, respectively). Except for xanthoma, onset of skin or mucosa lesions indicated active systemic disease. These manifestations responded well to steroids and cyclophosphamide. CONCLUSIONS: Various dermatologic manifestations are frequently observed in WG. They have distinctive pathologic features and usually indicate the presence of active systemic disease, especially with kidney and joint involvement.

Adolescent↗

Behçet's disease.

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Behcet Syndrome↗

[Relapse of Wegener's granulomatosis. Retrospective study of 18 cases].

During a mean therapeutic follow-up of 4 years, 25 out of 61 patients with Wegener's granulomatosis had one or several relapses. As in 7 of them the initial diagnosis had proved erroneous, this study concerns the remaining 18 patients who together totalled 31 relapses after the correct diagnosis was made. Twenty relapses occurred early on, within 4 months of a change in treatment, and they corresponded to recurrent activity of the disease. Ten relapses occurred later on, after treatment had been discontinued or kept at maintenance dose level. The relapses appeared as early as the first year in 41 percent of the cases. The ENT region was affected in 61 percent of the patients. Nine relapses occurred in the absence of treatment, including 6 which took place 1 to 8 years after treatment. Three relapses occurred less than 3 months after a prednisone and oral cyclophosphamide therapy was initiated. Compared with the group of patients without relapse, those in the relapse group were younger, more often treated with azathioprine and cyclophosphamide administered intravenously, and followed up for a longer period; their probability of survival was also higher. In case of early relapse after withdrawal of treatment the mean duration of that treatment had been shorter than in the absence of relapse. None of the patients whose treatment had been stopped more than 20 months after its onset had an early relapse. These data prompted us to make the following proposals: initially, cyclophosphamide should be administered as bolus injections (the time required to obtain results is then shorter than with the oral route), and maintenance therapy should last at least 20 months. Azathioprine should be used only when cyclophosphamide is contra-indicated. The relative indications for intravenous and oral cyclophosphamide remain to be determined. Discontinuing all treatments is a difficult decision to make, but even then the patients should be under close supervision in view of the possibility of late relapses.

Adolescent↗

[Effectiveness of intravenous immunoglobulins in polymyositis and dermatomyositis. An open trial in 15 patients].

Polymyositis (PM) and dermatomyositis (DM) are dysimmune diseases usually treated with corticosteroids and immunosuppressants. Human polyvalent immunoglobulins administered intravenously (IgIV) are known to be effective in some dysimmune diseases. Between August 1987 and September 1989 we conducted an open trial of IgIV in 15 patients (mean age 44 +/- 14 years) with either PM (12 cases) or DM (3 cases) associated with a collagen disease in 2 patients. In 14 of these 15 patients the conventional treatments (corticosteroids, immunosuppressants, plasmapheresis, total body irradiation, lymphopheresis) had failed. One patient was seropositive for picornavirus and received IgIV as initial treatment. IgIV infusions were given 4 +/- 3.9 years on average after the onset of PM or DM. Twelve of the 15 patients received another treatment, starting at least 6 weeks before IgIV and pursued without dosage increase, which consisted of corticosteroids (11 cases), methotrexate (5 cases) or plasmapheresis (1 case). Human polyvalent immunoglobulins for intravenous use were prescribed in doses of 2 g/kg/monthly course. All but two patients (1 course) received 3 to 6 courses on average. The IgIV infusions were well tolerated in 12 patients; 3 patients showed allergic manifestations which regressed. Therapeutic effectiveness was evaluated by muscle testing and by repeated assays of creatine phosphokinase (CPK). Clinical improvement, usually perceptible after the first course, was observed in 13/15 patients; it was associated with a more than 30 percent decrease of the initial CPK level in 13 patients and with a reduction of associated therapies in 9 patients. In the entire patient population a statistically significant lowering of mean CPK value was observed as early as in the first course (P less than 0.001). In view of their effectiveness, rapid action and safety, intravenous Ig infusions may be regarded as an interesting treatment in PM or DM patients.

Adrenal Cortex Hormones↗

[Treatment of lupus erythematosus disseminatus].

Corticosteroids have transformed the prognosis of systemic lupus erythematosus (SLE), but no further advance has been achieved during the last few years. Treatment of SLE rests on the use, as rational as possible, of those therapeutic tools that are available. However, diagnosing SLE does not necessary, they must be given promptly in effective doses whilst preventing their side-effects, notably by the simultaneous management of atheroma factors. Non-steroidal anti-inflammatory agents and synthetic antimalarial drugs may be used to replace corticosteroids or reduce their dosage. Severe forms of SLE which are function- or life-threatening require immunosuppressants, the best one being cyclophosphamide administered monthly as intravenous bolus injections, as this drug has been shown to be effective and to have low morbidity. Plasmapheresis is exceptionally needed. The risk of thrombogenesis due to the frequently associated "antiphospholipid syndrome" can be avoided by anticoagulants or antiplatelets the respective indications of which have not yet been clearly determined. SLE being a chronic disease, patient's compliance with treatment is very important and justifies the efforts made to train and inform these young male or female patients.

Glucocorticoids↗

[Value of "bolus" cyclophosphamide injections in Behçet's disease. Experience of 17 cases].

Seventeen patients with Behçet's disease were treated with intermittent intravenous bolus injections of cyclophosphamide. Twelve patients had severe eye lesions, 7 had central neurological disorders and 2 had both ocular and central nervous system involvement. Cyclophosphamide was given during 4 weeks on average, in mean doses of 922 +/- 127 mg. Each patient received an average of 10 +/- 5 injections. The drug was generally well tolerated; only one patient developed moderate and transient hepatocytolysis. Visual acuity was improved in 12 of the 22 eyes with active lesions, and signs of inflammation disappeared in all cases. Neurological improvement was observed in 5 of the 7 patients affected, with complete regression of the disorders in 2 of them. In 16 patients who were also under corticosteroid therapy, steroid dosage could be significantly reduced by 44 +/- 11 mg per day.

Adrenal Cortex Hormones↗