G. R. V. Hughes

Morbidity and mortality in systemic lupus erythematosus during a 5-year period - A multicenter prospective study of 1,000 patients

In the present study we assessed the frequency and characteristics of the main causes of morbidity and mortality in SLE during a 5-year period and analyzed the prognostic significance for morbidity and mortality of the main immunologic parameters used in clinical practice. We started in 1990 a multicenter study of 1,000 patients from 7 European countries. All had medical histories documented and underwent medical interview and routine general physical examination when entered in the study, and all were followed prospectively by the same physicians during the ensuing 5 years (1990-1995). Four hundred thirteen patients (41.3%) presented 1 or more episodes of arthritis, 264 (26.4%) had malar rash, 222 (22.2%) active nephropathy, 139 (13.9%) fever, 136 (13.6%) neurologic involvement, 132 (13.2%) Raynaud phenomenon, 129 (12.9%) serositis (pleuritis and/or pericarditis), 95 (9.5%) thrombocytopenia, and 72 (7.2%) thrombosis. Two hundred seventy patients (27%) presented infections, 113 (11.3%) hypertension, 75 (7.5%) osteoporosis, and 59 (5.9%) cytopenia due to immunosuppressive agents. Sixteen patients (1.6%) developed malignancies, with the most frequent primary localizations the uterus and the breast. Several immunologic parameters (anti-dsDNA or antiphospholipid antibodies) were found to have a predictive value for the development of SLE manifestations during the period of the study. Forty-five patients (4.5%) died; the most frequent causes of death were divided similarly among active SLE (28.9%), infections (28.9%), and thromboses (26.7%). A survival probability of 95% at 5 years was found. A lower survival probability (92%) was detected in those patients who presented at the beginning of the study with nephropathy.

Pregnancy in granulomatous vasculitis.

OBJECTIVE--To study the fetal and maternal outcome of pregnancy in patients with granulomatous vasculitis. METHODS--Four pregnancies in two patients with Wegeners granulomatosis (WG) and one patient with Churg-Strauss syndrome (CSS) were identified and followed in our specialised clinic for pregnancy and connective tissue diseases. RESULTS--Three pregnancies ended with live babies and one with intrauterine death at 25 weeks of gestation. One WG patient remained in remission throughout pregnancy and the other experienced severe activity at 12 weeks. The CSS patient was in remission during her first pregnancy, but the disease flared severely in the second. CONCLUSIONS--Pregnancy in patients with granulomatous vasculitis requires preconceptual planning, careful clinical management, and vigorous treatment of active disease.

Antiphospholipid antibodies in patients with scleroderma: prevalence and clinical significance

Antiphospholipid antibodies (aPL) are detected in a variety of autoimmune disorders, most commonly systemic lupus erythematosus, but also in some infectious diseases, lymphoproliferative disorders, and even in apparently healthy people.nnAlthough a wide prevalence of aPL in systemic sclerosis has been reported (between 0 and 41%), most studies have focused on anticardiolipin antibodies (aCL) and very little is known about other aPL in this disease. We determined the prevalence and clinical significance of aCL, antibodies to β2-glycoprotein I (anti-β2GPI), and antibodies to phosphatidylserine-prothrombin complex (aPS-PT) in 25 patients with scleroderma (18 with limited and 7 with diffuse scleroderma, as defined by LeRoy et al 1) (table 1). Twenty four patients were female (median age 50 years (range 28–70), median disease duration 3 years (range 1–20)). …

Reassessing the status of antiphospholipid syndrome in systemic lupus erythematosus.

The antiphospholipid syndrome was initially described in 1986. To reassess the validity of antiphospholipid antibodies in systemic lupus erythematosus (SLE), 95 patients with SLE were studied. Their antiphospholipid antibody profile was analysed and correlated with clinical findings such as thrombosis, abortions, or thrombocytopenia. A low prevalence of these antibodies was found (13 patients; 14%) with a high specificity for thrombosis (92%) and abortions (92%). The importance of anticardiolipin antibodies as a risk factor for thrombosis or abortions, or both, in patients with SLE is reaffirmed by this work.