Immunological abnormalities in young patients with dysautonomia

Abstract

Background: Dysautonomia is a poorly understood condition of unknown etiology. Many patients with dysautonomia have symptoms suggestive of immune dysregulation but there is limited information on their immunologic phenotype. Our aim is to characterize the association between dysautonomia and immunity in a group of young patients with this condition. Methods: A retrospective chart review was conducted in a group of patients with dysautonomia, documented by an abnormal head-up tilt test, referred for immune evaluation to the Allergy-Immunology clinic at our institution (2007–2014). Their clinical history and immune laboratory studies (e.g. immunoglobulin levels, specific antibody responses, ANA, anti-SSA/SSO, dysautonomia autoimmune profile, serum cytokines) were reviewed. Results: Twenty patients (3 males, 17 females), mean age of 19.9 years (15–34), were evaluated. Symptoms suggestive of immune dysfunction included arthralgia (7), joint swelling (3), backache (2), dermatitis (5), alopecia (2), sicca (4), Raynaud’s (1), and recurrent infections (5). Five patients (25%) had autoantibodies (i.e. anti-SSA, ANA, anti-GAD65, folate receptor autoantibodies), and 9 of 14 patients (64%), had elevated serum cytokines. Severe dysautonomia was present in one patient with IDDM and 4 patients with B-cell immunodeficiency, all of which had other coexisting autoimmune problems. Conclusions: Young patients with dysautonomia may suffer from immune dysregulation and the severity of their disease is associated with severe dysautonomia. It remains to be determined if this immune dysregulation is a primary or secondary process.