A Case of Sjögren’s Syndrome Associated with Only Seropositive Foraquaporin-4 Antibodies without Clinical Evidences of Neuromyelitisoptica

Case Report

Austin J Clin Neurol 2016; 3(2): 1094.

A Case of Sjögren’s Syndrome Associated with Only Seropositive Foraquaporin-4 Antibodies without Clinical Evidences of Neuromyelitisoptica

Do-Hyung Kim*

Department of Neurology, Eulji University School of Medicine, South Korea

*Corresponding author: Do-Hyung Kim, Department of Neurology, Eulji University Hospital, School of Medicine, Eulji University, DunsanSeo-ro 95, Seo-gu, Daejeon, 35233, South Korea

Received: September 26, 2016; Accepted: November 04, 2016; Published: November 08, 2016


Neuromyelitisoptica (NMO) is characterized by optic neuritis and longitudinally extensive transverse myelitis. With the recent discovery of an antibody to aquaporin-4 (NMO-IgG) which has high sensitivity and specificity for diagnosing NMO, the coexistence of Sjögren’s syndrome (SS) and Neuromyelitisoptica spectrum disorder (NMOSD) has been increasing reported. We report a case of SS with a positive antibody to aquaporin-4 without any evidence of myelitis or clinical features of NMO or NMOSD. To our knowledge, antibody to aquaporin-4 positivity in SS without clinical evidence of NMO was unusual case.

Keywords: Neuromyelitisoptica; Sjogren’s syndrome; Aquaporin 4; Rheumatoid factor


Sjögren’s syndrome is an autoimmune exocrinopathy characterized by exocrine dysfunction of lacrimal and salivary glands and by dysfunction even in the exocrine gland of female genital tract, skin, nose, trachea and gastrointestinal tract. Neuromyelitisoptica is an idiopathic, severe, and inflammatory demyelinating disease of the central nervous system that preferentially affects the optic nerve and spinal cord. NMO-IgG autoantibody as a disease specific marker of NMO arises from B cells, binds to aquaporin 4 expressed on astrocyte foot process. Detecting antibody to aquaporin-4 (anti AQP4-IgG) can be used very helpfully as a serological test in diagnosing NMO and NMOSD.

The role of autoimmunity in NMO was the association with other autoimmune diseases such as thyroiditis, systemic lupus erythematosus (SLE) or SS in 10-40% of patients [1], and those were accepted concept. Although anti AQP4-IgGis frequently found in patients with connective tissue disorders combined with neurological symptoms suggestive of NMO or NMOSD, there have not been reports of discovery of anti AQP4-IgG in any patients of connective tissue disorders without clinical evidences of NMO or NMOSD [2]. Herein, we describe a patient with serologic and radiologicconfirmed SS who showed a serum anti AQP4-IgG without evidences of myelopathy, optic neuropathy and any central nervous system involvement.

Case Presentation

68-year-old Korean woman was consulted to the neurology department because of generalized weakness and bilateral droopy eyelid. From 19 years ago, she has been managed with a diagnosis of Grave’s disease. The patient displayed weight loss of about 4-5 kg during a year without visible muscle atrophy. Although there was reduction in neck muscle power, she denied dysphasia or dysarthria. Symptoms of shoulder and forearm pain, dry mouth and ocular fatigue were accompanied along with reduction in lacrimation and blurred vision.

In neurological examination, except for bilateral droopy eyelids and deterioration of neck muscle power (Grade IV), other abnormalities were not observed. The laboratory test showed positive antinuclear and Ro (SS-A), La (SS-B) antibodies and an increased erythrocyte sedimentation rate of 86 mm/hour. In addition, the fluorescent antinuclear antibody (speckled pattern), anti-RNP, antidsDNA, anti-Smith, anti-TM antibody test, and rheumatoid factor were positive. Thyroid function test revealed mild reduction in free T4 (0.8 ng/dL, normal 0.89-1.76 ng/dL) with normal T3 (70 ng/dL, normal 65-150 ng/dL), thyroid stimulating hormone (0.66 μIU/mL, normal 0.55-4.78 μIU/mL), thyroglobulin antibody (22.2 U/mL, normal 0.0-60.0 U/mL), thyroid microsomal antibody (54 U/mL, normal 0.0-60.0 U/mL) and thyroid stimulation hormone receptor antibody (0.92 IU/L, normal 0.0-1.75 IU/L). Salivary gland scan revealed decreased secretary and excretory function in both parotid and submandibular glands (Figure 1). Schirmer test revealed Sjögren’s dry eye (4 mm/5 mm). The cerebrospinal fluid study and brain magnetic resonance imaging (MRI) were normal. Nerve conduction study, electromyography, visual evoked potential, repetitive nerve stimulation test, and neostigmine test were within normal limits. Chest computed tomography was also normal, and screening for the tumor markers (alpha fetoprotein, carcinoembryonic antigen, cancer antigen-125, 19-9, anti-Hu, Ri, and Yo) and anti-acetylcholine receptor antibody were normal.

Citation: Kim D-H. A Case of Sjögren’s Syndrome Associated with Only Seropositive Foraquaporin-4 Antibodies without Clinical Evidences of Neuromyelitisoptica. Austin J Clin Neurol 2016; 3(2): 1094. ISSN:2381-9154