Peripheral Nervous System Disease in Systemic Lupus Erythematosus: Results from an International, Inception Cohort Study

John G Hanly¹, Qiuju Li ², Li Su ³, Murray Urowitz ⁴, Caroline Gordon ⁵, Sang-Cheol Bae ⁶, Juanita Romero-Diaz ⁷, Jorge Sanchez-Guerrero ⁸, Sasha Bernatsky ⁹, Ann E Clarke ¹⁰, Daniel J Wallace ¹¹, David A Isenberg ¹², Anisur Rahman ¹³, Joan Merrill ¹⁴, Paul Fortin ¹⁵, Dafna Gladman ¹⁶, Ian Bruce ¹⁷, Michelle Petri ¹⁸, Ellen M Ginzler ¹⁹, MA Dooley ²⁰, Kristjan Steinsson ²¹, Rosalind Ramsey-Goldman ²², Asad A Zoma ²³, Susan Manzi ²⁴, Ola Nived ²⁵, Andreas Jönsen ²⁵, Munther A Khamashta ²⁶, Graciela Alarcón ²⁷, Elisabet Svenungsson ²⁸, Ronald F Van Vollenhoven ²⁹, Cynthia Aranow ³⁰, Meggan Mackay ³¹, Guillermo Ruiz-Irastorza ³², Manuel Ramos-Casals ³³, S Sam Lim ³⁴, Murat Inanc ³⁵, Kenneth C Kalunian ³⁶, Soren Jacobsen ³⁷, Christine Peschken ³⁸, Diane Kamen ³⁹, Anca Askanase ⁴⁰, Chris Theriault ¹ and Vernon Farewell ², ¹Dalhousie University, Halifax, NS, Canada, ²University of Cambridge, Cambridge, United Kingdom, ³Universidy of Cambridge, Cambridge, United Kingdom, ⁴University Health Network, University of Toronto, Toronto, ON, Canada, ⁵University of Birmingham, Birmingham, United Kingdom, ⁶Hanyang University Hospital for Rheumatic Diseases, Seoul, Republic of Korea, ⁷Instituto Nacional de Ciencias Medicas y Nutricion Salvador, Zubiran Vasco de Quiroga, Mexico City, Mexico, ⁸Toronto Western Hospital, Toronto, ON, Canada, ⁹Research Institute of the McGill University Health Centre, Montreal, QC, Canada, ¹⁰University of Calgary, Calgary, AB, Canada, ¹¹Cedars-Sinai Medical Centre, Beverly Hills, CA, ¹²Centre for Rheumatology, London, United Kingdom, ¹³University College London, London, United Kingdom, ¹⁴Oklahoma Medical Research Foundation, Oklahoma City, ¹⁵Division de Rhumatologie, Département de Médecine, CHU de Québec – Université Laval, Axe maladies infectieuses et inflammatoires, Centre de recherche du CHU de Québec – Université Laval, Canada, Quebec, QC, Canada, ¹⁶Toronto Western Hospital, Toronto, Canada, Toronto, ON, Canada, ¹⁷University of Manchester, Manchester, United Kingdom, Manchester, England, United Kingdom, ¹⁸Johns Hopkins University School of Medicine, Baltimore, MD, ¹⁹State University of New York Downstate Medical Center, Brooklyn, NY, ²⁰UnC Kidney Centre, Chapel Hill, NC, ²¹Landspitali, University Hospital, Reykjavik, Iceland, ²²Northwestern University, Chicago, IL, ²³University of Glasgow, East Kilbride, United Kingdom, ²⁴Allegheny Health Network, Pittsburg, PA, ²⁵Lund University, Lund, Sweden, ²⁶King's College London School of Medicine, London, United Kingdom, ²⁷University of Alabama at Birmingham, Birmingham, ²⁸Division of Rheumatology, Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden, ²⁹Amsterdam Rheumatology & Immunology Center, Amsterdam, Netherlands, ³⁰Feinstein Institute for Medical Research, Manhasset, NY, ³¹Feinstein Institute for Medical Research, New York, ³²Unidad de Enfermedades Autoinmunes, BioCruces Health Research Institute, Barakaldo, Spain, ³³Department of Autoimmune Diseases, ICMiD. Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX. Department of Medicine, University of Barcelona, Hospital Clínic, Barcelona, Spain., Barcelona, Spain, ³⁴Emory University, Atlanta, GA, ³⁵Istanbul University, Istanbul Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, ³⁶UC San Diego School of Medicine, LaJolla, CA, ³⁷Copenhagen Lupus and Vasculitis Clinic, Copenhagen, Denmark, ³⁸University of Manitoba, Winnipeg, Canada, ³⁹Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, Charleston, South Carolina, USA., Charleston, SC, ⁴⁰Columbia University Medical Center, New York, NY

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: neurologic involvement and patient outcomes, SLE

Session Information

Date: Sunday, November 10, 2019

Title: SLE – Clinical Poster I: Epidemiology & Pathogenesis

Session Type: Poster Session (Sunday)

Session Time: 9:00AM-11:00AM

Background/Purpose: Although there is a large body of work on central nervous system (CNS) disease in SLE patients, involvement of the peripheral nervous system (PNS) is less well established. The objective of our study was to determine the frequency, clinical characteristics, associations and outcomes in different types of PNS disease in a prospective, multi-ethnic/racial, inception cohort study of SLE patients.

Methods: Patients were evaluated annually for 19 neuropsychiatric (NP) events, including seven types of PNS disease using standardized case definitions. Attribution models were used to determine if the PNS events were due to SLE or other causes. SLE disease activity (SLEDAI-2K), organ damage (SLICC/ACR damage index), autoantibodies, patient (SF-36) and physician (Likert score) assessment of outcomes were measured. Statistical analyses included time to event and linear regressions as appropriate.

Results: Of 1,827 SLE patients enrolled, 88.8% were female and 48.8% Caucasian. The mean ± SD age was 35.1 ± 13.3 years, disease duration at enrollment was 5.6 ± 4.2 months and follow-up was 7.6 ± 4.6 years. There were 161 PNS events in 139/1,827 (7.6%) patients. The predominant events were peripheral neuropathy [66/161 (41.0%)], mononeuropathy [44/161 (27.3%)] and cranial neuropathy [39/161 (24.2%)] and the remaining 4 types of PNS disease accounted for only 12/161 (7.5%) events. The majority of PNS events [118/161 (73.3%)] in 104/139 (74.8%) patients were attributed to SLE. Multivariable Cox regression [HR, (95%CI)] indicated a negative association with Asian race/ethnicity [0.42 (0.19, 0.93)] and a positive association with other concurrent NP events attributed to SLE [2.74 (1.49, 5.03)]. By physician assessment, the majority of neuropathies resolved or improved over time. Multivariable analysis indicated that a longer time to resolution was associated with recurrent PNS events [0.38 (0.16, 0.90)], older age at SLE diagnosis [0.76 (0.60, 0.98)], higher SLEDAI-2K scores excluding NP variables [0.71 (0.51, 0.99)] and peripheral neuropathy [0.45 (0.25, 0.82)]. Patients with neuropathy reported significantly lower SF-36 physical and mental component summary scores compared to patients without NP events. Resolution was associated with improvements in SF-36 physical component summary scores that were both clinically and statistically (p< 0.01) significant in patients with peripheral neuropathy (mean change: +8.74) and mononeuropathy (mean change: +9.27).

Conclusion: PNS disease is a manifestation of NPSLE and has a significant negative impact on health related quality of life. The outcome is favourable for most patients, but several factors associated with longer time to resolution were identified.

Disclosure: J. Hanly, None; Q. Li, None; L. Su, None; M. Urowitz, Janssen Research & Development, LLC, 2, UCB Pharma, 9; C. Gordon, Bristol-Myers Squibb, 5, 8, Centers for Disease Control and Prevention, 5, Eli Lilly, 5, 8, EMD Serono, 5, EMD Serono, UCB, 5, GlaxoSmithKline, 5, 8, Merck Serono, 5, 8, UCB, 2, 5, 8, Versus Arthritis/GSK, 2; S. Bae, None; J. Romero-Diaz, None; J. Sanchez-Guerrero, None; S. Bernatsky, None; A. Clarke, AstraZeneca/MedImmune, 5, Bristol-Myers Squibb, 5, Exagen Diagnostics, 5; D. Wallace, None; D. Isenberg, None; A. Rahman, None; J. Merrill, Xencor, 2; P. Fortin, None; D. Gladman, AbbVie, 2, 5, Amgen, 2, 5, BMS, 5, Celgene, 2, 5, Eli Lilly, 2, 5, Galapagos, 5, Galapagos NV, 5, Gilead, 5, GSI, 5, Janssen, 5, Janssen Research & Development, LLC, 2, Novartis, 2, 5, Pfizer, 2, 5, UCB, 2, 5; I. Bruce, Astra Zenica, 5, AstraZeneca, 5, Eli Lilly, 5, 8, Genzyme Sanofi, 2, GlaxoSmithKline, 2, 5, 8, GSK, 2, 5, 8, ILTOO, 5, Iltoo, 5, MedImmune, 5, 8, Medimmune, 5, Merck Serono, 5, 8, Merk Serono, 5, Roche, 5, 8, Sanofi Genzyme, 2, UCB, 2, 5, 8, UCB Pharma, 5, 8; M. Petri, Eli Lilly and Company, 5, Exagen, 2, 5; E. Ginzler, Ablynx, 5, Aurinia, 2, Genentech, 2, GlaxoSmithKline, 2, Guidepoint Global Gerson Lerman Group, 5, Janssen, 5; M. Dooley, None; K. Steinsson, None; R. Ramsey-Goldman, Exagen, 2; A. Zoma, None; S. Manzi, Allegheny Health Network, 3, AstraZeneca, 2, 5; O. Nived, None; A. Jönsen, None; M. Khamashta, None; G. Alarcón, None; E. Svenungsson, None; R. Van Vollenhoven, Abbvie, 5, 8, Astra-Zeneca, 5, 8, Biotest, 5, 8, BMS, 2, 5, 8, Celgene, 5, 8, GSK, 2, 5, 8, Janssen, 5, 8, Lilly, 2, 5, 8, Novartis, 5, 8, Pfizer, 5, 8, Roche, 5, 8, UCB, 2, 5, 8; C. Aranow, EMD Serrono, 2, GlaxoSmithKline, 2, Janssen, 2, Takeda, 2, UCB, Inc, 2, Xencor, 2; M. Mackay, None; G. Ruiz-Irastorza, None; M. Ramos-Casals, None; S. Lim, None; M. Inanc, None; K. Kalunian, Ablynx, 2, Anthera, 5, Bristol-Myers Squibb, 2, 5, Eli Lilly, 5, Equillium, 5, Exagen Diagnostics, 5, Genentech, 5, Human Genome Sciences/GlaxoSmithKline, 2, Kyowa Hakko Kirin, 2, Pfizer, 2, Takeda, 2, UCB, 2; S. Jacobsen, None; C. Peschken, Astra Zeneca, 2, Celgene, 2, Janssen, 2; D. Kamen, None; A. Askanase, None; C. Theriault, None; V. Farewell, None.

To cite this abstract in AMA style:

Hanly J, Li Q, Su L, Urowitz M, Gordon C, Bae S, Romero-Diaz J, Sanchez-Guerrero J, Bernatsky S, Clarke A, Wallace D, Isenberg D, Rahman A, Merrill J, Fortin P, Gladman D, Bruce I, Petri M, Ginzler E, Dooley M, Steinsson K, Ramsey-Goldman R, Zoma A, Manzi S, Nived O, Jönsen A, Khamashta M, Alarcón G, Svenungsson E, Van Vollenhoven R, Aranow C, Mackay M, Ruiz-Irastorza G, Ramos-Casals M, Lim S, Inanc M, Kalunian K, Jacobsen S, Peschken C, Kamen D, Askanase A, Theriault C, Farewell V. Peripheral Nervous System Disease in Systemic Lupus Erythematosus: Results from an International, Inception Cohort Study [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/peripheral-nervous-system-disease-in-systemic-lupus-erythematosus-results-from-an-international-inception-cohort-study/. Accessed .

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