Volume 36, Issue 10 November / December 2018 SjogrensSyndromeFoundation @SjogrensOrg Peripheral Neuropathy and Neuropathic Pain in Sjögren’s by Constantine Farmakidis, MD efore proceeding into detail about neuropa- thy in Sjögren’s, I would like to provide some Bintroductory information for readers. Sjögren’s is a chronic, autoimmune, inflammatory condition that leads to the classic clinical presentation of dry eyes and dry mouth or “sicca” symptoms, which is a form of the Latin word siccus that means dry. How- ever, beyond “sicca” symptoms, Sjögren’s can manifest outside of the salivary and tear glands. This is known as extraglandular involvement which can be notably variable in Sjögren’s and can affect the lungs, heart, gastro- intestinal system, urogenital systems as well as the central and peripheral nervous systems. The central ner- vous system is comprised of the brain and spinal cord, whereas the peripheral nervous system is comprised of peripheral nerve structures that extend from the spinal cord down to the hands and feet. continued page 2 t Letter From Your CEO, Steven Taylor s another year comes to an end, I start to reminisce about the past year and what the Sjögren’s Syndrome Foundation Ahas accomplished. As you all know, the SSF was founded by a patient for patients, and the SSF remains committed to ensuring that all patients benefit from our work! We also work hard to ensure that the patient voice is in every meeting, every conference and every research decision. We want to make sure the donations we are so thankful to receive, are going to projects and initiatives that patients will find helpful, useful and empowering. This past year, the SSF has been proud to achieve so many advance- Steven Taylor, CEO SSF ments in Sjögren’s – as we march even closer to finally having a ther- continued page 8 t In This Issue 5 SSF Research Winners 9 SSF in Action 11 You Stood Up 13 Clinician’s Corner 17 In Memory/In Honor 2 November / December 2018 / The Moisture Seekers “Peripheral Neuropathy” continued from page 1 t A major component of the peripheral nervous system is pe- ripheral nerves. One can perhaps conceptualize them as two-way Founded by electrical wires that carry information downstream to the limbs and Elaine K. Harris in 1983 upstream to the spinal cord. The core of the nerves is comprised Board of Directors of many thin and elongated nerve cell components that are known Chairman of the Board Janet E. Church as axons. Axons are highly electrically excitable and can conduct Chairman-Elect electrical impulses as signals in the nervous system. To build on Kathy L. Sivils, PhD the wire analogy, axons can be thought of as the copper wire at the Treasurer Monica McGill, EdD center of electrical cable. Myelin is the substance that provides Secretary insulation and support to the axons. It facilitates the transmission Tricia Gooding of electrical impulses along nerves and can be conceived as the Immediate Past Chairman Stephen Cohen, OD familiar plastic covering of electrical wires. Esen K. Akpek, MD Neuropathies are diseases that compromise the structure and Susan Barajas function of peripheral nerves. They have a large number of po- Patricia Hurley, MSc Cathy Ingels tential causes. Diabetes is by far the most common cause of neu- Chadwick Johr, MD Theresa Lawrence Ford, MD ropathy and is an example of a systemic medical illness that can Scott Lieberman, MD cause neuropathy. Notably 20-30% of neuropathies do not have an Cynthia Lopynski Jonathan Morse, MSc identified cause and are termed idiopathic or cryptogenic. Other Jason Nichols, OD Timothy Niewold, MD, FACR causes of neuropathy include hereditary neuropathy that runs in Thomas D. Sutton families, neuropathy Donald E. Thomas, MD Michelle Wallace secondary to exposure Other Diagnosed Health Conditions Ava Wu, DDS to environmental toxins for Sjögren’s Patients Medical & Scientific such as alcohol or medi- There are many known comorbidities or manifestations of Advisory Board cations, neuropathy that Sjögren’s that can occur in conjunction with the disease. Chairman Survey respondents reported having been diagnosed by Theresa Lawrence Ford, MD occurs due to nutrition- health care provider with an average of five other health Esen Akpek, MD al deficiency such as conditions, including Gastroesophageal Reflux Disease Richard Brasington, MD, FACR Michael T. Brennan, DDS, MHS vitamin B12 deficiency, (GERD) (45%), Raynaud’s (38%), Neuropathy (38%), Steven E. Carsons, MD and neuropathies that Sinusitis (33%), Hypertension (31%), and Irritable Bowel Nancy L. Carteron, MD, FACR Syndrome (31%). Troy Daniels, DDS, MS occur secondary to Denise Faustman, MD, PhD H. Kenneth Fisher, MD, FACP, FCCP autoimmune disease RAYNAUD’S NEUROPATHY Gary Foulks, MD, FACS that primarily attack S. Lance Forstot, MD GERD SINUSITIS Philip C. Fox, DDS peripheral nerves. The 38% 38% Robert I. Fox, MD, PhD, FACP classic examples of 45% 33% Tara Mardigan, MS, MPH, RD LOW RED Austin Mircheff, PhD autoimmune illnesses BLOOD CELLS/ 17% 31% HYPERTENSION John Daniel Nelson, MD, FACS that primarily attack the ANEMIA SJÖGREN’S Kelly Nichols, OD 21% 31% Athena Papas, DMD, PhD nerves are Guillain Bar- Ann Parke, MD RHEUMATOID 23% 29% IRRITABLE Andres Pinto, DMD BOWEL ré Syndrome (GBS) and ARTHRITIS SYNDROME Nelson Rhodus, DMD, MPH AUTOIMMUNE Vidya Sankar, DMD, MHS chronic inflammatory THYROID FIBROMYALGIA Daniel Small, MD, FACP demyelinating polyneu- DISEASE Neil Stahl, MD Frederick B. Vivino, MD, FACR ropathy (CIDP). Jeffrey Wilson, MD, FACR continued page 6 t Chief Executive Officer The Moisture Seekers® Newsletter is published by the Sjögren’s Syndrome Foundation Inc., Steven Taylor 10701 Parkridge Boulevard, Suite 170, Reston, VA 20191. Senior Director of Marketing/Editor Copyright ©2018 Sjögren’s Syndrome Foundation Inc. ISSN 0899-637. Elizabeth Trocchio DISCLAIMER: The Sjögren’s Syndrome Foundation Inc. in no way endorses any of the medications, treat- ments, or products mentioned in advertisements or articles. This newsletter is for informational purposes e-mail: [email protected] only. Readers are advised to discuss any research news, drugs, treatments or products mentioned herein www.sjogrens.org with their healthcare providers. Xiidra is the FIIRSTeye drop FDA-approved & ONLYto treat both the signs and symptoms of Dry Eye Indication Xiidra® (lifitegrast ophthalmic solution) 5% is indicated PROVEN TO TREAT THE SIGNS for the treatment of signs and symptoms of dry eye disease (DED). OF INFERIOR CORNEAL STAINING IN 12 WEEKS AND SYMPTOMS Important Safety Information OF EYE DRYNESS IN AS LITTLE AS 2. Xiidra is contraindicated in patients with known hypersensitivity to lifitegrast or to any of the other ingredients. In clinical trials, the most common adverse reactions reported Xiidra helped provide symptom relief from in 5-25% of patients were instillation site irritation, dysgeusia and eye dryness in some patients at week 12, 6, reduced visual acuity. Other adverse reactions reported in 1% to 5% and as little as 2—and a measurable reduction of the patients were blurred vision, conjunctival hyperemia, in signs of inferior corneal staining in just 12 eye irritation, headache, increased lacrimation, eye discharge, weeks. So see how Xiidra may be right for eye discomfort, eye pruritus and sinusitis. your patients. To avoid the potential for eye injury or contamination of the solution, Get to know Xiidra at Xiidra-ECP.com patients should not touch the tip of the single-use container to their eye or to any surface. Four randomized, double-masked, 12-week trials evaluated the efficacy and safety of Xiidra versus Contact lenses should be removed prior to the administration vehicle as assessed by improvement in the signs of Xiidra and may be reinserted 15 minutes following administration. (measured by Inferior Corneal Staining Score) and symptoms (measured by Eye Dryness Score) Safety and efficacy in pediatric patients below the age of 17 years of Dry Eye Disease (N=2133). have not been established. For additional safety information, see accompanying Brief Summary of Safety Information on the adjacent page and Full Prescribing Information on Xiidra-ECP.com. ©2018 Shire US Inc., Lexington, MA 02421. 1-800-828-2088. All rights reserved. SHIRE and the Shire Logo are trademarks or registered trademarks of Shire Pharmaceutical Holdings Ireland Limited or its affiliates. Marks designated ® and ™ are owned by Shire or an affiliated company. S38463 04/18 tested, 3 mg/kg/day (400-fold the human plasma exposure at the recommended human ophthalmic dose [RHOD], based on the area under the curve [AUC] level). Since human systemic exposure to lifitegrast following ocular administration of Xiidra Rx Only at the RHOD is low, the applicability of animal findings to the risk of Xiidra use in humans during pregnancy is unclear. Animal Data BRIEF SUMMARY: Lifitegrast administered daily by intravenous (IV) injection Consult the Full Prescribing Information for complete product to rats, from pre-mating through gestation day 17, caused information. an increase in mean preimplantation loss and an increased INDICATIONS AND USAGE incidence of several minor skeletal anomalies at 30 mg /kg / Xiidra® (lifitegrast ophthalmic solution) 5% is indicated for the day, representing 5,400-fold the human plasma exposure at treatment of the signs and symptoms of dry eye disease (DED). the RHOD of Xiidra, based on AUC. No teratogenicity was observed in the rat at 10 mg /kg /day (460-fold the human DOSAGE AND ADMINISTRATION plasma exposure at the RHOD, based on AUC ). In the rabbit, Instill one drop of Xiidra twice daily (approximately 12 hours an increased incidence of omphalocele was observed at the apart) into each eye using a single-use container. Discard lowest dose tested, 3 mg /kg /day (400-fold the human plasma the single-use container immediately after using in each eye. exposure at the RHOD, based on AUC), when administered by Contact lenses should be removed prior to the administration IV injection daily from gestation days 7 through 19.