Sjogren’s syndrome was first described by the Swedish ophthalmologist, Dr. Henrik Sjogren, in 1933. He identified a group of dry eye patients who also had dry mouth and arthritis. Sjogren’s (pronounced “SHOW-grin’s) is an autoimmune disorder where the immune system attacks your body’s own cells and tissues and can affect the entire body. Along with symptoms of extensive dryness, other potential serious complications include profound fatigue, chronic pain, major organ involvement, neuropathies and
lymphomas.Prevalence estimates range from 1.2% to 4.8% of the population, and Sjogren’s affects women almost 10 times more frequently than men and traditionally has been diagnosed in middle age. However, Sjogren’s can develop at any age. Four million Americans are estimated to have Sjogren’s with less than 2.5 million diagnosed. Symptoms may include dry itchy skin, chronic burning mouth syndrome, cough, numbness in the arms and legs, fatigue, muscle and joint pain, vaginal dryness, dry nose and sinus, cognitive issues, and gastrointestinal, pulmonary, kidney and liver problems. Visual symptoms include blurring of vision, intolerance to bright light, and eyes that itch, burn and are prone to infection.
Additional oral symptoms include dry mouth, rampant cavities, loss of teeth, choking upon swallowing, difficulty speaking, dry peeling lips, and problems with taste and smell. Sjogren’s patients have a 6.5%–16% increased risk of developing non-Hodgkin’s lymphoma and a 1000-fold increased risk of developing parotid gland marginal zone lymphoma. The etiology is believed to involve both genetics and the environment, triggered by viral or bacterial infections or a toxic exposure in a genetically susceptible individual. It may be associated with other autoimmune diseases such as rheumatoid arthritis and systemic lupus erythematosus. Sjogren’s is associated with the production of autoantibodies, increased B
cell activity and immune abnormalities affecting exocrine glands and extra-glandular tissue. The histological hallmark is focal lymphocytic infiltration of normal appearing glandular acini. Neurological symptoms can include difficulty concentrating, poor memory, dysautonomia, inattention, reduced perceptual speed, verbal fluency, and a picture similar to subcortical dementia. Parkinson’s disease and other movement disorders such as dystonia have been described. Individuals can develop aseptic meningitis where MRI and spinal fluid may reveal abnormalities. Central nervous system problems can include encephalitis, seizures, stroke, transient ischemic attack, and migraine. Individuals can develop numbness,
coldness, burning, lancinating as if the feet are on fire, weakness, and clumsiness. Autonomic symptoms can affect the heartbeat, respiration, body temperature control and gastric motility causing abdominal distention. Facial pain occurs in the form of trigeminal neuralgia and throat pain with glossopharyngeal neuralgia. The spinal cord can be affected with numbness, weakness, urinary problems, defecating, and sexual disorders. Peripheral nerve findings include fatigue, numbness, burning, and paresthesias. There can be selective involvement of dorsal root ganglions to include ataxia and dysesthesia and difficulty walking which may result in a painful sensory neuropathy. Hypokalemic and respiratory failure associated with renal
tubular acidosis can be the first manifestation of Sjogren’s. Sjogren’s can affect the GI system including biliary, cirrhosis, stomach upset, gastroparesis, pancreatitis, and irritable bowels. Lung involvement can include bronchiectasis, interstitial lung disease, pneumonia. Uro-gynolcological problems can occur, including interstitial cystitis, vaginal dryness, and vulvodynia. Blood vessels can be affected leading to vasculitis and Raynaud’s phenomenon (in which blood flow to fingers and toes is affected leading to digits turning white or blue and feeling numb and cold). Difficulty with blood pressure, cardiac rhythm disturbances, and sweating abnormalities may occur. One can suffer from hearing loss
due to sensorineural hearing loss. Sleep disturbances are not infrequent as well. Diagnosis can be delayed for many reasons. Healthcare providers might not link a wide variety of symptoms as part of one disease. In addition, many symptoms can mimic other disorders or diseases, complicating the diagnosis. Symptoms of Sjogren’s also can be confused with side effects of various drugs including antidepressants, allergy, and blood pressure medications. Proving a diagnosis of Sjogren’s may be difficult as systemic and/or neurological involvement can occur prior to sicca (dryness) symptoms. Diagnostic testing includes looking at blood for Rheumatoid Factor, inflammatory markers, and the autoantibodies SSA and SSB; a lip
biopsy to see if lymphocytic infiltrates are present; ophthalmology tests measuring tear production; and oral tests to measure salivary flow. With peripheral nerve involvement, EMG studies can measure motor and sensory response. Autonomic testing can be performed. Treatment can include disease modifying drugs as well as symptomatic treatment for those with dry eyes and dry mouth. Drugs to be considered when needed include hydroxychloroquine, steroids, biologicals including rituximab, TNF-alpha inhibitors, exercise, and antiepileptics and anticholinergic drugs. For oral dryness, one can use topical fluoride to help preserve teeth, over-the-counter salivary stimulants, and prescription sialogogues are used. For
ocular symptoms, artificial tears and ointments and prescriptions such as Restasis and Xiidra can help. One should suspect Sjogren’s when symptoms are greater than three months without other explanations. Individuals can find education and support locally and nationally through the Sjogren’s Syndrome Foundation (SSF). The SSF offers educational materials and conferences, support groups, and numerous online support venues. The SSF also works hard to advocate for patients, whether for increased medical and scientific research, new and better therapies, and increased insurance coverage, public awareness, and professional education. More information on the SSF can be found at www.sjogrens.org or by calling
1-800-475-6473. Steven Mandel MD FAAN Clinical Professor of Neurology, Donald and Barbara Zucker School of Medicine at Hofstra/ Northwell
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