Sjogren's syndrome

In summary, anti-Ro and anti-La antibodies are the best available autoantibodies for the diagnosis of SS. In particular anti-La antibodies are both specific and sensitive for the diagnosis of primary SS. The presence of anti-Ro and anti-La antibodies is associated with more severe disease as well as with extraglandular manifestations of SS. Despite our increasing understanding of the pathogenesis of disease in SS, many questions still remain as to the mechanism of autoantibody production and the role of autoantibodies in the pathogenesis of disease. © 2004 Taylor & Francis Ltd

Autoimmune manifestations in common variable immunodeficiency

Common variable immunodeficiency (CVID) is a disorder characterized by decreased serum immunoglobulin concentrations and increased incidence of recurrent infections. Interestingly 20-25% of patients with CVID develop clinical features suggestive of an autoimmune disease. Although this association is well established, the immunodeficiency background of CVID patients manifesting autoimmune disorders is often overlooked. This study describes three CVID patients displaying a variety of autoimmune manifestations. The pathophysiologic mechanisms of autoimmunity in CVID are also reviewed

Interstitial lung disease in a patient with antisynthetase syndrome and no myositis

Interstitial lung disease in patients with antisynthetase syndrome and no evidence of myositis is rare and may precede other disease manifestations. We report a patient who initially presented with symptoms primarily related to lung involvement. The diagnosis of the antisynthetase syndrome without myositis was made many months later when he developed a characteristic hand rash (mechanic’s hands), which was confirmed by positive antibodies to Jo-1. With treatment, both the hand rash and the interstitial lung disease improved. Antisynthetase syndrome should be considered in patients presenting with interstitial lung disease with no evidence of myositis. Appropriate laboratory testing with measurement of specific autoantibodies may help in the early diagnosis and treatment of the syndrome