系统性红斑狼疮合并妊娠19例分析

目的　探讨系统性红斑狼疮 (SLE)患者合并妊娠后的病情恶化率以及妊高征发生率及妊娠期处理。方法　对1998～ 2 0 0 3年收治的 19例SLE合并妊娠的临床资料进行回顾性分析。结果　SLE合并妊娠后的病情恶化率及妊高征发生率分别为 5 2 6 %及 31 6 % ,与SLE病情活动和缓解有关。结论　SLE合并妊娠后病情容易加重 ,也易并发妊高征 ,应加强妊娠期监测和处理。     

Systemic sclerosis: demographic, clinical and serological features in 100 Iranian patients

To evaluate demographic, clinical and laboratory features associated with scleroderma-specific auto-antibodies. Sera of 100 patients with systemic sclerosis (SSc) were analyzed by an indirect immunofluorescence technique with HEp-2 cells as a substrate. Specific ANA such as anti-centromere antibodies (ACA), anti-topoisomerase (TOPO), anti-RNA polymerase III (Pol 3), anti-U3-RNP (U3-RNP), anti-Th/To (Th/To) and anti-PM/Scl (PM/Scl) were detected by line immunoassay and anti-U1-RNP (U1-RNP) by ELISA. Frequency of clinical features associated with a specific antibody group was reported cumulatively over the follow-up period. Frequency of specific clinical features was compared across the two disease subtype including limited cutaneous (lcSSc) or diffuse cutaneous (dcSSc) as well as the auto-antibody groups. Ninety-four percent of patients were ANA positive with significant higher skin score, Raynauds and digital ulcer/gangrene. Anti-TOPO was detected in 71 % of all patients, in 90.5 % of dcSSC and in 65.8 % of lcSSc. Anti-TOPO was significantly associated with dcSSc, higher skin score, digital ulcer/gangrene, pulmonary fibrosis, DLCO <70 %. U1-RNP antibody was associated with lower fibrosis in lung. ACA was positive in 7 % of patients and exclusively in those with lcSSc. We did not find association between gender and presence of auto-antibodies. Anti-TOPO antibody had a high prevalence in contrast to low prevalence of ACA antibody. There were no differences in clinical subtypes of the disease in patients with positive anti-TOPO and positive ACA. Differences in prevalence of auto-antibodies are suggestive of further genetic study.     

Systemic lupus erythematosus in males: analysis of clinical and laboratory features.

We analysed the clinical and laboratory features of 16 males in comparison with 231 females from a series of 247 unselected patients with systemic lupus erythematosus (SLE). There was no significant difference between male and female patients with regard to age at onset and age at diagnosis. Apart from serositis, which was found to occur at a significantly higher frequency in male patients, the incidence of clinical features at disease onset was similar in both sexes. Analysis of clinical findings during the evolution of the disease showed no significant difference between male and female patients. Similarly, no significant immunological difference was found between the two groups. Thus, except for a higher frequency of serositis as the presenting symptom in males, we could not find any notable differences in clinical and serological parameters of male and female patients with SLE.     

Systemic lupus erythematosus in Saudi patients

Summary Eighty-seven patients with systemic lupus erythematosus (SLE) were retrospectively studied in King Khalid University Hospital, Riyadh. There were 78 females and 9 males (F:M ratio of 91). The mean age (±SD) at onset and at diagnosis were 25.3±10.5 and 28.5±10.9 years, respectively, with peak incidence in the 20–30 year age group.Musculoskeletal (91%), constitutional (76%), cutaneous (72%) and renal (63%) manifestations occurred most frequently, while neuropsychiatric manifestations (26%), photosensitivity (26%) and oral ulcers (16%) were relatively less frequent. The most common laboratory abnormalities included ANA (98%), anti-DNA (93%) LE cells (66%) and lymphopenia (70%). There were seven deaths during the study period and most of them were related to renal failure and complications from infections. Overall, the pattern of SLE observed in the series was comparable to that observed in other series among Caucasians.     

Systemic lupus erythematosus in 24 tunisian males: clinico-biological analysis and clinical course

PURPOSE: The systemic lupus erythematosus (SLE) is rare at the male. The clinic and biological characteristics and the evolution are variable between different studies. From 24 Tunisian observations we try to contribute to the precision of these characteristics on this ground. METHOD: It's a tunisian, multicentric, retrospective study of 24 males lupus, follow-up from 1990 to 1999 within a total lupus cohort of 295 patients. RESULTS: The sex-ratio female/male is 11/29, mean age at diagnosis is 31.75 years (range 10 to 63). The most frequent clinical manifestations are: arthritis (95%), malar rash (71%), photosensitivity (41%), glomerular nephropathy (66%) (classes III and IV = 7 cases/9), the serositis (37.5%) and Raynaud phenomenon (25%). Comparing these characteristics of two patient groups (24 males and 271 females), significant difference was observed : vascular thrombosis in male (16% vs 4% p : 0,027) and alopecia in female (12.5% vs 4% p : 0.03). For the other manifestations as nephropathy and serositis which are frequent in male the difference was not significant. The overall survival rate at 5 years 93% is and it was nearly the same in woman SLE patients. CONCLUSION: SLE in male is rare. In SLE male patients vascular thrombosis and nephropathy are more frequent without survival's influence.     

Systemic lupus erythematosus. Infrequent clinical aspects

The SLE is a systemic autoimmune disease that presents a tremendous variability regarding its clinical presentation and immunologic expression. This disease can affect any organ, both as clinical onset or during the course of the disease. People in their second and third decade are most frequently affected, but the illness can debut at any age, with some different clinical peculiarities. SLE-associated clinical situations like hemophagocytic syndrome and some recently published clinical aspects like posterior reversible encephalopathy syndrome are reviewed here.     

Systemic lupus erythematosus in men: clinical and immunological characteristics.

Although systemic lupus erythematosus (SLE) has traditionally been considered a disease of women, men may also be affected. Thirty of 261 patients (12%) with SLE seen in this hospital were men. Arthritis was less common as a first symptom in the men, although this group of patients had discoid lesions and serositis more often than the women. During the follow up a lower incidence of arthritis and malar rash and a higher incidence of other skin complications including discoid lesions and subcutaneous lupus erythematosus was found in the men. The incidence of nephropathy, neurological disease, thrombocytopenia, vasculitis, and serositis, was similar in the two groups. No significant immunological differences were found between men and women. These features indicate that several gender associated clinical differences may be present in patients with SLE.     

Systemic lupus erythematosus

Systemic lupus erythematosus is an autoimmune connective-tissue disorder with a wide range of clinical features, which predominantly affects women, especially from certain ethnic groups. Diagnosis is based on clinical assessment supported by investigations, including the finding of autoantibodies. Treatments range from antimalarial agents to corticosteroids and immunosuppressive agents. This Seminar draws attention to advances in the epidemiology, genetics, cardiovascular risks, lupus nephritis, CNS disease, the antiphospholipid syndrome, assessment of disease activity and damage, and pregnancy related and quality of life issues. New therapeutic approaches, such as biological agents and mycophenolate mofetil, will also be discussed.     

Systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a typical autoimmune disease that leads to multiple organ damage. For over half a century, SLE has been treated mainly with nonspecific glucocorticoids and immunosuppressants, and the development of molecular target drugs with few adverse reactions is awaited. The treatment goal is remission without systemic symptoms or organ damage. An anti-B-cell activating factor antibody belimumab and an anti-type I interferon receptor antibody anifrolumab are used for patients with active SLE who respond poorly to standard of cares. Additionally, as many susceptibility genes for SLE are associated with signal transduction of dendritic and B cells, cytokines and signaling molecules that bridge the innate and adaptive immune systems are the current focus of attention. Promising approaches include the development of a Janus kinase inhibitors targeting tyrosine kinase deucravacitinib, plasmacytoid dendritic cell-targeted drugs, proteasome inhibitors (e.g., iberdomide), type II anti-CD20 antibody, and obinutuzumab.     

Systemic lupus erythematosus

Pregnancy occurring in patients with diagnosed and controlled SLE will be associated with a flare of disease in 60 per cent of cases, which is not significantly different from flares in nonpregnant patients. Signs and symptoms of active SLE should be carefully evaluated and treated with steroids according to severity and organ systems involved. When pregnancy occurs with inactive kidney disease there is a 10 per cent rate of reactivation and SLE kidney disease may appear for the first time during pregnancy in 6.8 per cent of patients. These rates are similar in the control group. There will be a significantly increased abortion rate which cannot be improved with maternal treatment. There will also be a high prematurity rate and an increased number of newborns with intrauterine malnutrition that are associated with active maternal disease. The following points are important when caring for a pregnant SLE patient: 1. Maintain maternal disease inactive throughout gestation. 2. Monitor growth and development of fetus. 3. Monitor for fetal distress. 4. Interrupt pregnancy when fetal distress is diagnosed. 5. A neonatal intensive care unit should be available at the time of delivery. The short-term prognosis is good with no maternal mortality and there is no long-term deleterious influence of pregnancy on the evolution of SLE.     

Systemic lupus erythematosus

This review covers major advances in clinical issues related to systemic lupus erythematosus (SLE) published between 1995 and 2000. The classification criteria for both SLE and antiphospholipid syndrome (APS) have been updated, and up to 19 different subsets of neuropsychiatric lupus have been defined. New epidemiological data show that the incidence of new cases and the survival of patients with SLE are both increasing. Several randomised controlled trials have defined the role of cyclophosphamide, methotrexate, antimalarials, and hormonal treatment in the management of SLE. New data are available for drugs such as ciclosporin and thalidomide. Finally, several new treatments for severe refractory cases, such as mycophenolate mofetil and stem-cell transplantation, are being increasingly used. New data also refer to management of thrombosis in APS and high-risk pregnancies in women with SLE or APS.