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Biomedical subjects

B Wechsler

Publications and source records attributed to B Wechsler.

At least 181 records · Page 10Linked to original sources
PubMed1995

Arterial lesions in Behçet's disease. A study in 25 patients.

OBJECTIVE: To identify the prognostic indicators of patients with Behçet's disease complicated with arterial lesions. METHODS: Retrospective chart analysis of 25 consecutive patients with Behçet's disease and angiographically proven arterial lesions. RESULTS: Occlusive lesions were present in 7 patients, aneurysms in 3, and both occlusive and aneurysmal lesions in 15. High dose corticosteroids were not effective in isolated occlusive lesions and probably contributed to one fatal infection. Death was related to aneurysms in 5 patients. Twenty-seven vascular surgical procedures were performed in 15 patients. Arterial lesions recurred in all patients who did not received postoperative corticosteroids. Within a 2 yr period after operation, the rate of therapy failure was lower in the group of patients treated with a postoperative combination of corticosteroids and immunosuppressive drugs, compared to the group treated with corticosteroids alone. In patients treated for lower limb arterial lesions, the rate of relapse was similar whether venous autologous or prosthetic grafts were used. Graft thrombosis occurred in 3/7 patients given anticoagulants and in 3/4 patients with no antiaggregant or anticoagulant therapy. CONCLUSION: Aneurysms have a worse prognosis than occlusive lesions. High dose corticosteroids should not be systematically prescribed for isolated occlusive lesions. Surgery, when feasible, is indicated for aneurysms because they entail a high risk of rupture. Postoperative corticosteroids are necessary to prevent arterial relapse. A combination of corticosteroids and immunosuppressive therapy is more effective than corticosteroids alone. After bypass for lower limb arterial lesions, anticoagulation is warranted to prevent graft thrombosis.

Abdomen↗
PubMed1994

Renal manifestations of the antiphospholipid syndrome.

Although renal manifestations of the antiphospholipid syndrome (APS) have received scarce attention until recently, the kidney probably is a major target organ in APS. Thrombosis may develop at any location within renal vessels, ie, renal artery trunk or branches, intrarenal arteries or arterioles, glomerular capillaries, and renal vein. The clinical consequences consist of highly variable degrees of proteinuria; systemic hypertension ranging from mild to malignant; cortical necrosis; thrombotic microangiopathy, pregnancy-associated or not; and slowly to rapidly progressive renal failure that may require dialysis. These events occur mainly in the course of systemic lupus erythematosus or of "primary" APS. Renal involvement is a frequent feature of the catastrophic APS. A high prevalence of antiphospholipid antibodies has been reported recently in patients with end-stage renal failure, but their clinical significance remains to be determined.

Adrenal Gland Diseases↗
PubMed1994

[Pancreatitis in systemic lupus erythematosus. Review of the literature apropos of 5 cases].

We report five cases of pancreatitis in systemic lupus erythematosus. Three patients died. The cause of death was directly related to pancreatitis in two cases. Several causes of pancreatitis may be suspected: vasculitis, thrombosis when associated to antiphospholipid syndrome, biliary calculi, infection, metabolic abnormalities or adverse effects of therapy. About seventy cases of pancreatitis have been reported in systemic lupus erythematosus in the literature Pancreatitis was the inaugural sign of lupus in six cases. In 12 cases, besides lupus, no other cause of pancreatitis was found. However, pancreatic vasculitis or thrombosis was rarely demonstrated even in post-mortem examinations. The role of corticosteroids in pancreatitis in lupus is controversial since the evolution of pancreatitis in lupus is generally good with corticosteroids. We think that diagnosis of pancreatitis in lupus should not implicate discontinuation or decrease of corticosteroids dosage, unless their responsibility is absolutely demonstrated.

Acute Disease↗
PubMed1994

[French register of lupus in pregnancy: the evaluation. Groupe d'étude sur la grossesse lupique].

A register of systemic lupus erythematosus has been open between 1st January 1987 and 31 December 1992 in France. One hundred and seventeen cases of pregnancy from more than 40 centers origin have been prospectively collected. One hundred and three were analyzed. Pregnancy outcome was as following: full term birth (n = 28), premature birth (n = 48), fetal wastage (n = 18 i.e. 13 early spontaneous abortions, two late spontaneous abortions and three stillbirths), therapeutic abortion (n = 5), elective abortion for unwanted pregnancy (n = 4). Four preterm babies died in neo-natal period. Lupus activity was present at pregnancy diagnosis in 28 cases (27%). Of 75 patients with inactive lupus at pregnancy beginning, 27 relapsed during pregnancy and seven in post-partum period. Two patients with nephrotic syndrome died of opportunistic infection. Fetal prognosis is mostly related to proteinuria and absence of anti-SSA antibodies. History of fetal losses, lupus activity at pregnancy beginning, hypertension, use of 20 mg/d or more prednisone dosage during pregnancy influence prematurity. The fetal hypotrophy factors are short duration of pregnancy, lupus activity at beginning of pregnancy, low serum levels of C3 or C4, hypertension, absence of anti-SSA antibodies. Three out of 22 newborns from mother with anti-SSA antibodies developed neonatal lupus: two with cutaneous lupus and one with complete congenital auriculo-ventricular block.

Acute Disease↗
PubMed1994

[Treatment of systemic vasculitis].

Progress in the treatment of systemic vasculitis have permitted a decrease of mortality but with an increase in iatrogenic morbidity. Steroids remain the cornerstone of the treatment but precise modalities and other concomitant treatments are depending upon the type of vasculitis. In most cases, systemic vasculitis are primary and the treatment, although important, is symptomatic. However, in some cases such as hepatitis B virus-induced polyarteritis nodosa or hepatitis C virus-induced cryoglobulinemia, the treatment can be etiologic and is directed against the antigen responsible for the systemic vasculitis. In the future, a better understanding of pathological mechanisms, particularly of etiologic factors, and new treatment such as monoclonal antibodies should increase the prognosis of systemic vasculitis.

Adrenal Cortex Hormones↗
PubMed1994

[Localized or systemic amyloidosis? Value and limitations of Iodine-123 labelled amyloid P component scintigraphy, role of biopsy of minor salivary glands].

We evaluated the value of serum amyloid P component scintigraphy (123I-SAP) and labial salivary gland biopsy in four patients with apparently localized amyloidosis. One patient had pharyngeal amyloidosis, the second laryngeal amyloidosis, the third retro-ocular amyloidosis and the fourth had carpal tunnel syndrome. Three patients (no 2, no 3, no 4) have abnormal whole body retention of 123I-SAP and positive labial salivary gland biopsy for amyloidosis. Only the first patient had effectively localized amyloidosis and local treatment was sufficient. Melphalan was given to the patients no 2 and no 3, colchicine was given to the patient no 4. Labial salivary gland biopsy and 123I-SAP scintigraphy may give positive argument for the diagnosis of systemic amyloidosis in patients with apparently localized amyloidosis.

Adult↗
PubMed1994

Pregnancy and its outcome in systemic lupus erythematosus.

In a multi-centre prospective study of systemic lupus erythematosus and pregnancy in France, 117 cases were identified from 1987 to 1992. We report significant morbidity and mortality for mother and fetus from an analysis of 103 cases. Pregnancy outcome was as follows: 28 full-term births, 48 premature births, 18 fetal losses (13 early and two late spontaneous abortions, three stillbirths), five therapeutic abortions and four elective abortions (for unwanted pregnancy). Four preterm neonates died. Lupus was active at pregnancy onset in 28 patients. Of 75 patients with inactive lupus at conception, 27 relapsed during pregnancy, and seven postpartum. Two patients with nephrotic syndrome treated with high-dose corticosteroids died from opportunistic infections. Fetal loss correlated with a history of proteinuria and absence of anti-SSA antibodies. Prematurity was related to a history of fetal loss, active lupus at pregnancy onset, hypertension and > or = 20 mg/day prednisone during pregnancy. Intrauterine growth retardation correlated with pregnancy of short duration, low serum C3 or C4, hypertension, and absence of SSA antibodies. Three of 22 newborns whose mothers had anti-SSA antibodies developed neonatal lupus: two with cutaneous involvement and one with complete atrioventricular block.

Abortion, Therapeutic↗
PubMed1994

Multiple subungual splinter hemorrhages in the antiphospholipid syndrome: a report of five cases and review of the literature.

Multiple subungual splinter hemorrhages have been initially described as an important sign of subacute endocarditis. Secondly, they were reported in various other conditions, especially in isolated cases of primary antiphospholipid syndrome. We report five patients with multiple fingernail subungual splinter hemorrhages occurring in the course of antiphospholipid syndrome. Antiphospholipid syndrome was secondary to systemic lupus erythematosus in two, to Fasciola hepatica infection in one and was considered as primary in two. In all patients multiple subungual splinter hemorrhages occurred concomitantly with thrombotic events of diverse arterial sites. The mechanism of subungual splinter hemorrhages is most probably thrombotic.

Adult↗
PubMed1994

Intravenous gamma globulin as first line therapy in polymyositis and dermatomyositis: an open study in 11 adult patients.

OBJECTIVE: Polymyositis (PM) and dermatomyositis (DM) are inflammatory muscle diseases of presumed autoimmune origin. Many possible interventions are available to treat these patients: corticosteroids, immunosuppressive drugs, plasmapheresis, and total body irradiation. But these therapies are not always effective and may be responsible for certain serious side effects. Polyvalent intravenous immunoglobulin (IVIG) has been tried with success in inflammatory myopathies after failure of traditional treatment. An attempt was made to evaluate the efficacy of IVIG as first line therapy in patients with PM or DM. METHODS: Eleven Caucasian patients [6 women, 5 men, mean age 55.6 (SD 10.1) years], with active recent inflammatory myopathy, were treated by high doses of IVIG as first choice. The average duration of inflammatory myopathy before IVIG was 9.6 months (SD 10.2 months, with a range of 1 month to 3 years). Five patients had PM and 6 had DM. None had myositis associated with connective tissue disease. Two patients had a history of malignant disease: 1 lymphoma and 1 breast tumor with relapse of the malignancy during the study. One patient had a probable lung carcinoma and in another patient, ovarian carcinoma was diagnosed a few months after the onset of IVIG. We used preparations of polyvalent human i.v. gamma globulins with intact IgG. All patients received 1 g/kg daily for 2 days each month. The mean course of treatment was 4 months. RESULTS: Clinical assessment, evaluated by proximal muscle power and biochemical tests, was carried out before each treatment period. Significant clinical improvement was noted in only 3 of the 11 patients (one with acute coxsackie virus B infection, and one with possible drug induced myopathy). Mean muscle power estimated for the 11 patients before and after IVIG therapy was not significantly improved. Eight patients showed significant biochemical improvement (more than 50%). Mean CK levels for the 11 patients showed a statistically significant decrease during IVIG therapy (p < 0.01). Minor IVIG side effects were noted in one patient. CONCLUSION: IVIG therapy seems effective rarely as first therapy in patients with inflammatory myopathy but may be considered especially in viral or drug induced myopathy. IVIG therapy as the first treatment may also be tried in patients with contraindication for steroids, and in mild myopathy, especially in the elderly, to avoid steroid induced side effects.

Adult↗
PubMed1994

[Treatment of pulmonary vasculitis].

Pulmonary vasculitides require major therapeutic regimens which, though symptomatic, are often effective. Corticosteroids have greatly changed the prognosis of most of these vasculitides, particularly polyarteritis nodosa and the Churg and Strauss syndrome. Cyclophosphamide must be added for Wegener's granulomatosis although the route of administration is still under discussion. Plasma exchanges are rarely indicated. Other treatments for Wegener's granulomatosis including combined trimethoprim-sulfamethoxazole in limited forms and methotrexate in sub-acute systemic forms are currently under study. A better understanding of the underlying mechanisms, including the role of antineutrophil cytoplasmic antibodies, and especially the identification of the causative factors will lead to an aetiologic treatment still unknown today.

Adrenal Cortex Hormones↗
PubMed1994

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↗