Canine Systemic Lupus Erythematosus

A naturally occurring immunologic disease, characterized by autoimmunehemolytic anemia, idiopathic thrombocytopenic purpura and nephritis, isdescribed in seven dogs. The disease primarily affected formed elements inthe blood, glomeruli and blood vessels. Abnormal serum proteins, includingthe LE factor, rheumatoid factor and antithyroid antibody were found inaffected dogs. Corticosteroids and splenectomy were effective in controllingthe hematologic components of the disease, but apparently had no effect onthe renal lesions. Recurrence was frequent and the prognosis was grave. Additional abnormalities, including malar eruption, intermittent lameness andalopecia were occasionally found as an integral part in the sequential involvement of several tissues during the course of the disease.

Submitted on February 24, 1964 Accepted on May 5, 1964     

系统性红斑狼疮生物疗药物浅谈

近年来,生物靶向治疗越来越多地应用于自身免疫性疾病患者的治疗,并取得了较好的疗效。系统性红斑狼疮(SLE)是一种多系统受累的自身免疫病,患者体内存在多种免疫紊乱。目前治疗SLE的生物制剂包括去除B细胞、抑制T-B细胞间相互作用以及拮抗炎性细胞因子和B细胞活化因子等方面的药物。本文简要介绍已经上市和正在进行临床试验的SLE生物靶向治疗药物。     

Vasculitis in Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a complex heterogeneous autoimmune disease with a wide variety of clinical and serological manifestations that may affect any organ. Vasculitis prevalence in SLE is reported to be between 11 % and 36 %. A diverse clinical spectrum, due to inflammatory involvement of vessels of all sizes, is present. Even though cutaneous lesions, representing small vessel involvement, are the most frequent, medium and large vessel vasculitis may present with visceral affection, with life-threatening manifestations such as mesenteric vasculitis, pulmonary hemorrhage, or mononeuritis multiplex, with detrimental consequences. Early recognition and an appropriate treatment are crucial. Recent studies have shown that vasculitis in patients with SLE may present different clinical forms based on the organ involved and the size of the affected vessel. It is noteworthy that the episodes of vasculitis are not always accompanied by high disease activity. Recent articles on this topic have focused on new treatments for the control of vascular disease, such as biological therapies such as Rituximab and Belimumab, among others.     

Hypercalcaemia in Systemic Lupus Erythematosus

Hypercalcaemia is a common electrolyte abnormality. The vast majority of patients will be shown to have either hyperparathyroidism or malignancy. In less than 10% of patients other, less common causes of hypercalcaemia will be present. Systemic lupus erythematosus is a very rare cause of hypercalcaemia. It may be associated with lymphadenopathy and pleuritis to constitute a distinct clinical entity described as ‘hypercalcaemia–lymphoedema syndrome’. In these cases the pathophysiology of the hypercalcaemia is not completely understood. In some cases it is associated with elevated levels of parathyroid-related peptide (PTHrP). In others the level of PTHrP is normal, and it has been suggested that autoantibodies may cause hypercalcaemia by activating the PTH receptor. We describe a case of a woman who presented with severe hypercalcaemia, developed the hypercalcaemia–lymphodema syndrome and fulfilled the diagnostic criteria of systemic lupus erythematosus. Received: 30 April 2000 / Accepted: 8 November 2000     

Lymphopenia In Systemic Lupus Erythematosus

One hundred fifty-eight patients with active, untreated systemic lupus erythematosus (SLE) were studied from the time of diagnosis. Lymphopenia was present in 75%, and another 18% of these patients developed lymphopenia subsequent to disease reactivation. Lymphopenia of less than 1500 cells/m̈l occurred more frequently than any of the preliminary criteria for the classification of SLE, and it was the most prevalent initial laboratory abnormality. Lymphocyte counts were significantly lower in lupus than in the other connective tissue diseases except mixed connective tissue disease and polymyositis.     

Complement Deficiencies in Systemic Lupus Erythematosus

The complement system is a major, multifunctional part of innate immunity and serves as a bridge between the innate and adaptive immune systems. It consists of more than 30 distinct proteins that interact with one another in a specific sequence. There are three pathways of complement activation: the classical, the lectin, and the alternative pathways. The three pathways are initiated by distinct mechanisms, but they all generate the same core set of effector molecules. Inherited complete deficiencies in complement components are generally very rare and predispose to infections and autoimmune disease. One of the better described associations is between deficiencies in early classical pathway components and the development of systemic lupus erythematosus. The goal of this review will be to discuss the associations between and the causal mechanisms of complement deficiencies and systemic lupus erythematosus.     

Estrogen Therapy in Systemic Lupus Erythematosus

Given the female preponderance of systemic lupus erythematosus (SLE) in humans, the adverse effects of female gender and sex hormones in murine lupus, and numerous reports (retrospective, often anecdotal and uncontrolled) that describe a temporal association between estrogen exposure and development or exacerbation of SLE, it is tempting to accept that estrogens and SLE simply do not mix. While there are valid concerns regarding the use of exogenous estrogens in women with SLE, there are also potential health benefits to be considered. Oral contraceptives (OCs) offer effective birth control and may be bone protective in corticosteroid-treated patients. Recent studies, albeit retrospective, suggest that OCs are well tolerated in patients with SLE. Several salutary effects of postmenopausal estrogens assume particular importance in SLE where the risks of osteoporosis, exaggerated by menopause (natural or cyclophosphamide-induced) and corticosteroids, are substantial. However, the results of the Women's Health Initiative trial significantly limit the use of hormone replacement therapy in the general population, and raise particular concern for SLE patients. Other exogenous hormones (clomifene, gonadotropins, gonadotropin-releasing hormones) may be used to elevate levels of endogenous estrogen and to stimulate ovulation in patients with diminished fertility. Patients with inactive or stable/moderate disease and at low risk for thrombosis may benefit from OCs and other hormonal therapies without a change in lupus activity. Large prospective, double-blind, placebo-controlled studies inclusive of all ethnic groups should provide the basis for more definitive recommendations.