Anticardiolipin antibodies in patients with retinal vein occlusion and no risk factors: a prospective study

BACKGROUND: Several reports have described the association between antiphospholipid antibodies (APAs) and retinal venous occlusive (RVO) disease. The purpose of this study was to look at the prevalence of these antibodies in patients with RVO disease and no conventional risk factors. We specifically examined how APAs may affect the course of this disease. METHODS: Twenty-four patients with the diagnosis of RVO disease were screened prospectively for APAs. All were free from risk factors for retinal vein thrombosis and other immunologic conditions. Patients were observed for a period of 3 to 12 months. RESULTS: Lupus anticoagulant was negative in all 24 patients. Ten (43%) of 24 patients had anticardiolipin antibodies (ACAs). All patients with ACAs were younger than 45 years of age, with an average age of 33 years. The average age of patients with no ACAs was 66 years. Comparison of the average age of the two groups showed a statistically significant difference. There was no statistical significance between the two groups for development of neovascular disease. Seropositive patients who developed neovascularization had elevated titers for an average of 11.8 weeks versus 3.3 weeks for those who did not have neovascularization. Neovascular complications generally began several weeks after the titers became negative. CONCLUSION: There was a significant prevalence of ACAs in young patients with RVO disease and no associated systemic risk factors. Seropositive patients who developed neovascular disease had elevated titers for more than 6 weeks. However, the role of these transient ACAs in retinal vein occlusion is still not clear and merits further study.     

Family history of lymphoma and risk for solid tumors in patients with Sjogren’s syndrome

It has been recognized that primary Sjogren’s syndrome predisposes to the development of lymphoproliferative disorders. However, it is so far uncertain whether these patients are at an increased risk for solid tumors. Herein we report two cases of primary Sjogren’s syndrome complicated by breast and lung cancer and who have a strong family history for lymphoproliferative diseases.     

Leukotriene antagonists and the Churg-Strauss syndrome

BACKGROUND AND OBJECTIVES: Leukotriene antagonists (LTAs), or antileukotrienes, are a new group of anti-inflammatory drugs used for the treatment of asthma. They might substitute for or allow tapering of corticosteroids in asthmatic patients. These drugs have been associated with the development of Churg-Strauss syndrome (CSS), a rare form of vasculitic angiitis. It is unclear whether the development of CSS is a direct drug effect or an unmasking of a preexisting condition on withdrawal of steroids for asthma. We present a case of CSS in a patient treated with montelukast and review the literature to analyze the association between LTAs and the development of CSS. METHOD: We reviewed the literature using MEDLINE from February 1966 to October 2000. To the cases identified, we present an additional case of a patient who underwent a diagnostic lung biopsy. RESULTS: Twenty-two case reports of patients receiving LTAs who developed CSS were identified. The onset of CSS occurred 2 days to 10 months after starting treatment with LTAs. All patients had received inhaled or oral steroids for asthma. The interval between the last oral corticosteroid treatment and CSS onset ranged from 3 days to 8 months. CONCLUSIONS: To date, there is no compelling evidence that the development of CSS in asthmatic patients receiving LTAs results from a direct drug effect. Rather, it appears that tapering of corticosteroids in these patients unmasks the multiorgan manifestations of the disease. We believe that the use of LTAs should not be influenced by the apparent increase in the incidence of CSS and that these are still safe drugs for asthma.     

Gastrointestinal Beh?et's disease:A review

Beh?et’s disease(BD) is an idiopathic, chronic, relapsing, multi-systemic vasculitis characterized by recurrent oral and genital aphthous ulcers, ocular disease and skin lesions. Prevalence of BD is highest in countries along the ancient silk road from the Mediterranean basin to East Asia. By comparison, the prevalence in North American and Northern European countries is low. Gastrointestinal manifestations of Beh?et’s disease are of particular importance as they are associated with significant morbidity and mortality. Although ileocecal involvement is most commonly described, BD may involve any segment of the intestinal tract as well as the various organs within the gastrointestinal system. Diagnosis is based on clinical criteria- there are no pathognomonic laboratory tests. Methods for monitoring disease activity on therapy are available but imperfect. Evidence-based treatment strategies are lacking. Different classes of medications have been successfully used for the treatment of intestinal BD which include 5-aminosalicylic acid, corticosteroids, immunomodulators, and anti-tumor necrosis factor alpha monoclonal antibody therapy. Like inflammatory bowel disease, surgery is reserved for those who are resistant to medical therapy. A subset of patients have a poor disease course. Accurate methods to detect these patients and the optimal strategy for their treatment are not known at this time.     

Calcitonin in the treatment of transient osteoporosis of the hip

BACKGROUND: Transient osteoporosis of the hip (TOH) is a rare clinical disorder of unknown etiology characterized by hip pain and functional disability that resolve spontaneously in 6-24 months. OBJECTIVES: To report 2 patients with TOH during pregnancy who had rapid resolution of their illness with the use of calcitonin. To review the literature on TOH with special emphasis on its treatment. METHODS: A MEDLINE search of studies published from 1966 to 2002 was performed to review treatment options for TOH and their effect on the natural history of the disease. RESULTS: Our 2 patients developed hip pain during pregnancy with classical changes of TOH on MRI. Both patients received calcitonin, 1 during pregnancy and 1 postpartum with resolution of their symptoms within 6 to 9 weeks. Previous reports in the literature of treatment of TOH showed that antiresorptive agents (bisphosphonates and calcitonin) had shortened the duration of the illness compared with the natural history of the disease. CONCLUSIONS: TOH is an under-recognized entity associated with pain and disability. The use of antiresorptive agents may be of help in reducing the duration of the disease. RELEVANCE: To increase the recognition of TOH and to consider therapeutic interventions to shorten the duration of the disease.     

Successful treatment of cardiac involvement in dermatomyositis with rituximab

Polymyositis and dermatomyositis are autoimmune inflammatory myopathies characterized by muscle weakness and inflammation. Current recommended therapy includes corticosteroids as mainstay treatment in addition to immunosuppressant. We present herein a 25year-old female with dermatomyositis and cardiac involvement resistant to disease modifying anti-rheumatic drugs and anti-tumor necrosis factor-alpha. She was treated with anti-CD20 monoclonal antibody, rituximab. The patient demonstrated a remarkable clinical and laboratory response. B-cell depletion therapy with rituximab may be a viable option in patients with dermatomyositis and heart disease.     

New insights into the pathogenesis and therapy of Behçet's disease

Beh?et's disease is a chronic multisystem inflammatory disease characterized by the predominance of Th1 cytokines. The human leukocyte antigen-B 51 gene is associated with the disease in several ethnic groups, but it is not certain whether it is the susceptibility gene or if it is in linkage disequilibrium with other genes. Recent studies of polymorphisms of neighboring, as well as distant, genes to human leukocyte antigen-B 51 are helpful in elucidating the pathogenesis of the disease and could have therapeutic implications.