Gender and age differences in systemic lupus erythematosus. A study of 489 Greek patients with a review of the literature

We investigated whether gender and age influence the clinical course and outcome in systemic lupus erythematosus (SLE) patients. Thus, we analyzed the clinical and laboratory data of 489 SLE patients at presentation and during follow-up. In addition, disease activity score (using the European Consensus Lupus Activity Measure, ECLAM) and organ damage index (using the Systemic Lupus International Collaboration Clinics/American College of Rheumatology Damage Index, DI) were measured. Furthermore, data from both sexes were analyzed according to the following age groups: < 55 years(younger group) and > 55 years (older group). There were 68 men and 421 women, giving a ratio of 1:7. We found no differences in the mean age, mean age at diagnosis, disease duration as well as duration of follow-up between men and women. Young men presented more frequently with serositis and discoid lesions, while women presented with Raynaud's phenomenon (RP) and malar rash. Regarding the laboratory findings, young women presented more often with anti-Ro(SSA) and anti-La(SSB) antibodies, while increased levels of erythrocyte sedimentation rate (ESR) were found in old women. During follow-up, men had serositis and renal disease more frequently, while the women's group were found to complain of RP, photosensitivity and mucosal ulcers more frequently, especially in young women.Anemia, leukopenia, thrombocytopenia and elevated levels of ESR were also found more frequently in young women during follow-up. However, there were no significant differences concerning ECLAM and DI scores between the two gender groups. Using multiple logistic regression analysis, a statistically significant association of malar rash, discoid lesions, serositis, RP, anti-Ro(SSA)/ La(SSB) and increased ESR with sex was found independently of age, while only malar rash showed a statistically significant association with age independently of sex. Thus, we conclude that gender influences the clinical expresion of the disease independently of age, while both gender and age do not affect the overall damage score.     

Late-onset systemic lupus erythematosus in Northwestern Spain: differences with early-onset systemic lupus erythematosus and literature review

To further investigate into the epidaemiology of systemic lupus erythematosus (SLE) in Southern Europe, we have assessed the incidence, clinical spectrum and survival of patients diagnosed with late-onset SLE (age?≥?50 years) according to the 1982 American College of Rheumatology (ACR) classification criteria at the single hospital for a well-defined population of Lugo, Northwestern (NW) Spain. Between January 1987 and December 2006, 51 (39.3%) of the 150 patients diagnosed as having SLE fulfilled definitions for late-onset SLE. The predominance of women among late-onset SLE (4:1) was reduced when compared with that observed in early-onset SLE (7:1). However, the incidence of late-onset SLE was significantly higher in women (4.2 [95% confidence interval (CI): 3.1-5.6] per 100,000 population) than in men (1.3 [95% CI: 0.6-2.2] per 100,000 population) (p?相似文献   

系统性红斑狼疮合并获得性血友病A一例并文献复习

目的总结系统性红斑狼疮(SLE)合并获得性血友病A的临床表现及治疗,以提高对SLE合并获得性血友病A的认识。方法对1例SLE合并获得性血友病A的临床、实验室资料及治疗进行分析,并进行文献复习。结果患者为25岁女性患者,SLE病史5年,此次以腹痛为主诉,实验室检查活化部分凝血活酶时间(APTT,135．3s)明显延长,因子Ⅷ促凝活性(FⅧ：C,0．9%)减低,因子Ⅷ抑制物滴度26．1U/ml(Bethesda法),B超及核磁共振均发现子宫后血肿,治疗上给予激素、因子Ⅷ制品、大剂量丙种球蛋白等,临床症状及实验室指标明显好转,出院后给予激素及环磷酰胺治疗,随访10个月患者一般情况可。结论SLE合并血友病A在临床上应引起高度的重视,在治疗上目前尚无标准方案。     

Silica-associated systemic lupus erythematosus with lupus nephritis and lupus pneumonitis: A case report and a systematic review of the literature

IntroductionSeveral epidemiological studies have shown that silica exposure triggers the onset of systemic lupus erythematosus (SLE); however, the clinical characteristics of silica-associated SLE have not been well studied.Patient concernsA 67-year-old man with silicosis visited a primary hospital because of a fever and cough. His respiratory condition worsened, regardless of antibiotic medication, and he was referred to our hospital.DiagnosisThe patient showed leukopenia, lymphopenia, serum creatinine elevation with proteinuria and hematuria, decreased serum C3 level, and was positive for anti-double stranded DNA antibody, anti-nuclear antibody, and direct Coombs test. He was diagnosed with SLE. Renal biopsy was performed, and the patient was diagnosed with lupus nephritis (class IV-G(A/C) + V defined by the International Society of Nephrology/Renal Pathology Society classification). Computed tomography revealed acute interstitial pneumonitis, bronchoalveolar lavage fluid showed elevation of the lymphocyte fraction, and he was diagnosed with lupus pneumonitis.InterventionsPrednisolone (50 mg/day) with intravenous cyclophosphamide (500 mg/body) were initiated.OutcomesThe patient showed a favorable response to these therapies. He was discharged from our hospital and received outpatient care with prednisolone slowly tapered off. He had cytomegalovirus and herpes zoster virus infections during treatment, which healed with antiviral therapy.Review:We searched for the literature on sSLE, and selected 11 case reports and 2 population-based studies. The prevalence of SLE manifestations in sSLE patients were comparative to that of general SLE, particularly that of elderly-onset SLE. Our renal biopsy report and previous reports indicate that lupus nephritis of sSLE patients show as various histological patterns as those of general SLE patients. Among the twenty sSLE patients reported in the case articles, three patients developed lupus pneumonitis and two of them died of it. Moreover, two patients died of bacterial pneumonia, one developed aspergillus abscesses, one got pulmonary tuberculosis, and one developed lung cancer.ConclusionClose attention is needed, particularly for respiratory system events and infectious diseases, when treating patients with silica-associated SLE using immunosuppressive therapies.     

Coronary artery disease in systemic lupus erythematosus: A review of the literature

CONTEXT: Coronary artery occlusive disease is a common though underappreciated complication of systemic lupus erythematosus (SLE), typically a disease of young women. A case of a premenopausal patient with SLE and an acute myocardial infarction is presented, and the etiology and management of coronary artery disease in SLE reviewed. OBJECTIVES: To review the incidence, risk factors, pathology and treatment of coronary artery disease in systemic lupus erythematosus. DATA SOURCES: MEDLINE search of articles in English-language journals from 1980 to 2000. The index words "systemic lupus erythematosus" and the following co-indexing terms were used: "coronary artery disease," "atherosclerosis," "vasculitis," "anticardiolipin antibodies," "antiphospholipid syndrome." SELECTION SYNTHESIS AND ABSTRACTION: Papers identified were reviewed and abstracted by the authors with a presentation of a summary. RESULTS: The prevalence of coronary artery disease among women with SLE between the ages of 35 and 44 years is at least 50-fold greater than among age-matched control subjects. Of these, coronary atherosclerosis accounts for the vast majority of cases; vasculitis of the coronary arteries and other causes generally believed to be more typical of SLE are comparatively rare. CONCLUSIONS: The evidence suggests that SLE is a significant risk factor for coronary atherosclerosis independent of the classic risk factors of hypertension, tobacco use, and hyperlipidemia.     

A study of lymphocytotoxins in families of patients with systemic lupus erythematosus.

Lymphocytotoxic antibodies are a common feature in the sera of patients with systemic lupus erythematosus. This family study, however, has not confirmed the previous findings of an increased incidence in the sera of their relatives. Adsorption studies suggest that when lymphocytotoxins occur in the sera of normal subjects and relatives of patients they have a different specificity and thus significance.     

Outcome of pregnancy in patients with systemic lupus erythematosus. A prospective study

A prospective study was performed to investigate the outcome and complications of pregnancy in patients with systemic lupus erythematosus. Twenty-nine pregnancies occurred in 22 patients. There were 12 abortions, two spontaneous and 10 induced. Fifteen women had 17 live-born neonates. Neonatal complications included nine premature deliveries, two cases of intrauterine growth retardation, and one of Treacher Collins syndrome. Obstetric complications included threatened abortion (two), placenta previa (two), and preeclampsia (three). Cesarean sections were necessary in five patients. There was no maternal or neonatal mortality. Thirteen episodes of systemic lupus erythematosus relapses were detected by incidents of increasing proteinuria (six), arthritis (four), and vasculitic rash (two). There were no statistical differences in changes in hemoglobin level, erythrocyte sedimentation rate, albumin level, antinuclear antibody titer, or C3 or C4 level between the patients who relapsed and those who did not. Pregnancy could induce a flare of systemic lupus erythematosus in previously normal patients or patients with previously inactive disease. The overall neonatal and maternal survival was good, even in patients who presented during pregnancy. Spontaneous fetal loss was low (2/29 [6.9%]); both cases occurred in mothers with inactive lupus.     

Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus; review of literature

Acquired haemophilia A, secondary to systemic lupus erythematosus (SLE) is a rare bleeding diathesis. Here we report a 37-year-old woman with autoimmune hepatitis who developed SLE and acquired haemophilia caused by factor VIII inhibitors. She presented with spontaneous ecchymosis and haematuria. There were a prolongation of the activated partial thromboplastin time, reduced factor VIII activity and a high titer of FVIII inhibitors. Therapeutic regimen was started with intravenous methylprednisolone pulse, continued with prednisolone, intravenous pulse cyclophosphamide and fresh frozen plasma. After 8 weeks, factor VIII inhibitor assay was negative.     

Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus: review of literature

Acquired haemophilia A, secondary to systemic lupus erythematosus (SLE), is a rare bleeding diathesis. Here we report a 37-year-old woman with autoimmune hepatitis who developed SLE and acquired haemophilia caused by factor VIII (fVIII) inhibitors. She presented with spontaneous ecchymosis and haematuria. There were a prolongation of the activated partial thromboplastin time, reduced fVIII activity and a high titre of fVIII inhibitors. Therapeutic regimen was started with intravenous methylprednisolone pulse, continued with prednisolone, intravenous pulse cyclophosphamide and fresh frozen plasma. After 8 weeks, fVIII inhibitor assay was negative.     

Tuberculosis in patients with systemic lupus erythematosus.

Tuberculosis associated with systemic lupus erythematosus (SLE) was studied in a cohort of 311 patients seen between 1963 to 1979. There were 16 such patients, giving rise to a prevalence rate of 5%. The characteristics of SLE-associated tuberculosis include a high incidence of miliary and far-advanced pulmonary disease, delay in establishing diagnosis, especially the extrapulmonary form, and tendency to attribute symptoms like fever, malaise, and weight loss to the lupus process. Treatment was successful in 9 patients. Of the 7 death 5 were attributed directly to the mycobacterial infection and 2 to complications of SLE.     

Acquired hemophilia in a patient with systemic lupus erythematosus: a case report and literature review

We report the case of a 38-year-old female patient with systemic lupus erythematosus (SLE) who developed acquired hemophilia caused by factor VIII (FVIII) inhibitors. She manifested spontaneous bleeding symptoms such as ecchymoses and hematuria. Laboratory findings showed an isolated prolongation of the activated partial thromboplastin time, reduced FVIII activity, and a high titer of FVIII inhibitors. She was successfully treated with oral predonisolone and cyclosporine in combination with steroid and cyclophosphamide pulse therapy.     

Acquired hemophilia in a patient with systemic lupus erythematosus: a case report and literature review

Abstract

We report the case of a 38-year-old female patient with systemic lupus erythematosus (SLE) who developed acquired hemophilia caused by factor VIII (FVIII) inhibitors. She manifested spontaneous bleeding symptoms such as ecchymoses and hematuria. Laboratory findings showed an isolated prolongation of the activated partial thromboplastin time, reduced FVIII activity, and a high titer of FVIII inhibitors. She was successfully treated with oral predonisolone and cyclosporine in combination with steroid and cyclophosphamide pulse therapy.