Only a conference abstract.
http://www.abstractsonline.com/pp8/#!/4046/presentation/8338
http://www.abstractsonline.com/pp8/#!/4046/presentation/8338
68th Annual Meeting of the American Academy of Neurology Home Print Page
P5.108 - Autoimmunity & Autonomic Impairment: Preliminary Characterization of a Clinical Syndrome with Sjögren’s Features Associated with Novel Organ Specific Antibodies
Itinerary
April 20, 2016, 8:30 - 7:00 PM
Authors
Mark Gudesblatt,1Karl Wissemann,1Lauren Stiles,1Steven Xian,1Myassar Zarif,1Lori Fafard,1Barbara Bumstead,1Hos Loftus,1Cindy Jadoo,1Marijean Buhse,1Jennifer Jamison-Forgnone,2Lakshmanan Suresh2
OBJECTIVE:
Identification of serological markers of autoimmunity in SS-A/SS-B seronegative patients with clinical presentation suggesting Sjögren’s syndrome (SS).
BACKGROUND:
SS is traditionally associated with dry eyes, dry mouth and positive SS-A/SS-B antibodies or minor salivary gland biopsy. However, SS-A/SS-B seronegativity has been reported in 26-67% of patients and biopsies may be difficult to obtain. Neurological manifestations of SS include central demyelination, ganglionopathies and/or peripheral neuropathies. Novel organ specific autoantibodies, anti-salivary protein-1 (SP-1), anti-parotid secretory protein (PSP) and anti-carbonic anhydrase-6 (CA-6), have recently been described in patients with early SS. Identifying alternative autoantibodies in patients with SS symptoms who are SS-A/SS-B seronegative could provide a basis for treatment.
DESIGN/METHODS:
One-year retrospective single site study evaluating patients who presented with ocular or oral dryness and neurological impairment who remained without a clear diagnosis after extensive diagnostic and serologic testing. Said patients then underwent testing for SP-1, PSP and CA-6.
RESULTS:
96 subjects were identified, 79% female, ages 13-88. Patients reported dry mouth (81%), dry eyes (74%), GI dysmotility (69%), tingling/burning/numbness (68%), cognitive impairment (47%), orthostatic intolerance (44%), weakness (42%), balance problems (27%), and bladder dysfunction (26%). All patients were SS-A/SS-B seronegative. Novel SP-1, PSP and/or CA-6 autoantibodies were detected in 41% of subjects. Within positive subjects we found SP-1 (46%), CA-6 (40%) and PSP (45%), with some patients having multiple autoantibodies. Novel autoantibody positive subjects were more likely to report dry eyes (82%), GI dysmotility (80%), weakness (59%), orthostatic intolerance (49%), and balance problems (36%).
CONCLUSIONS:
Screening SS-A/SS-B seronegative patients with neurological symptoms who report dryness and other symptoms of autonomic impairment for SP-1, PSP and CA-6 can identify patients who may have an autoimmune basis for their symptoms, which may provide a path towards treatment. Further study is needed to understand the significance of these autoantibodies and response to treatment.