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Abstract Number: 1592

“Lupus Headache”: Results From a Prospective, International, Inception Cohort Study

John G. Hanly1, Murray B. Urowitz2, Aidan o'Keeffe3, Caroline Gordon4, Sang-Cheol Bae5, Jorge Sanchez-Guerrero6, Juanita Romero-Diaz7, Ann E. Clarke8, Sasha Bernatsky9, Daniel J. Wallace10, E.M. Ginzler11, David A. Isenberg12, Anisur Rahman13, Joan T. Merrill14, Michelle A. Petri15, Paul R. Fortin16, D. D. Gladman17, Barri J. Fessler18, Graciela S. Alarcon19, Ian N. Bruce20, Mary Anne Dooley21, Kristjan Steinsson22, Munther A. Khamashta23, Rosalind Ramsey-Goldman24, Susan Manzi25, Gunnar K. Sturfelt26, Ola Nived27, Asad A. Zoma28, R. F. van Vollenhoven29, Manuel Ramos-Casals30, Cynthia Aranow31, Meggan Mackay32, Guillermo Ruiz-Irastorza33, Kenneth C. Kalunian34, S. Sam Lim35, Murat Inanc36, Diane L. Kamen37, Christine Peschken38, Søren Jacobsen39, Chris Theriault40, Kara Thompson40 and Vernon Farewell3, 1Division of Rheumatology, Dalhousie University and Capital Health, Halifax, NS, Canada, 2Division of Rheumatology, University of Toronto, Toronto Western Hospital, Toronto, ON, Canada, 3MRC Biostatistics Unit, Institute of Public Health, University Forvie Site, Cambridge, United Kingdom, 4School of Immunity and Infection, College of Medical and Dental Sciences, University of Birmingham, Birmingham, United Kingdom, 5Rheumatology, Hanyang University Hospital for Rheumatic Diseases, Seoul, South Korea, 6UHN Toronto Western Hospital, Toronto, ON, Canada, 7Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, 8Division of Rheumatology, University of Calgary, Alberta, Calgary, AB, Canada, 9Clinical Epidemiology, Research Institute of the McGill University Health Ctre, Montreal, QC, Canada, 10Cedars-Sinai Medical Center, Los Angeles, CA, 11Rheumatology, SUNY-Downstate Medical Center, Brooklyn, NY, 12Centre for Rheumatology Research, Rayne Building, 4th Floor, Centre for Rheumatology, Department of Medicine, University College London, London, United Kingdom, 13Centre for Rheumatology Research,Rayne Institute, 4th Floor, University College London, London, United Kingdom, 14Clinical Pharmacology Research Program, Oklahoma Medical Research Foundation, Oklahoma City, OK, 15Johns Hopkins University School of Medicine, Baltimore, MD, 16Medicine, Centre de Recherche du Chu de Québec et Université Laval, Quebec City, QC, Canada, 17Division of Rheumatology, Toronto Western Hospital and University of Toronto, Toronto, ON, Canada, 18Department of Medicine, University of Alabama at Birmingham, Birmingham, AL, 19Medicine, Department of Medicine, University of Alabama at Birmingham, Birmingham, AL, 20Manchester Academic Health Science Centre, Arthritis Research UK Epidemiology Unit and NIHR Manchester Musculoskeletal Biomedical Research Unit, The University of Manchester, Manchester, United Kingdom, 21University of North Carolina at Chapel Hill, Chapel Hill, NC, 22Rheumatology, Landspital Univ Hospital, Reykjavik, Iceland, 23Lupus Research Unit, The Rayne Institute, St Thomas Hospital, Kings College London School of Medicine, London, United Kingdom, 24Medicine/Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, IL, 25Division of Rheumatology, University of Pittsburgh School of Medicine, Pittsburgh, PA, 26Department of Rheumatology, University Hospital Lund, Lund, Sweden, 27Department of Clinical Science, Lund University, Rheumatology, Lund, Sweden, 28Rheumatology, Hairmyres Hospital, East Kilbride, United Kingdom, 29Clinical Trials Unit Department of Rheumatology, The Karolinska Institute, Stockholm, Sweden, 30Laboratorio de Enfermedades Autoinmunes Josep Font, Hospital Clínic, Barcelona, Spain, 31The Feinstein Institute, Manhasset, NY, 32Autoimmune & Musculoskeletal Disease, Feinstein Institute for Medical Research, Manhasset, NY, 33Autoimmune Diseases Research Unit, Department of Internal Medicine, BioCruces Health Research Institute, Hospital Universitario Cruces, University of the Basque Country, Barakaldo, Spain, 34UCSD School of Medicine, La Jolla, CA, 35Emory University School of Medicine, Division of Rheumatology, Atlanta, GA, 36Department of Internal Medicine, Rheumatology Division, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey, 37Department of Medicine, Medical University of South Carolina, Charleston, SC, 38Medicine & Community Health Sciences, University of Manitoba, Winnipeg, MB, Canada, 39Department of Rheumatology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark, 40Department of Medicine, Queen Elizabeth II Health Sciences Centre and Dalhousie University, Halifax, NS, Canada

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: SLE and neuropsychiatric disorders

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Session Information

Title: Systemic Lupus Erythematosus - Clinical Aspects II: Central Nervous System Manifestations, Therapeutics

Session Type: Abstract Submissions (ACR)

Background/Purpose: “Lupus headache” is controversial and is included in validated measures of global SLE disease activity. We examined the frequency and characteristics of “lupus headache” in a large, prospective, inception cohort of SLE patients and the association with global disease activity and health related quality of life.

Methods: An international network of 30 academic medical centers enrolled patients within 15 months of SLE diagnosis. Annual assessments were performed for 19 neuropsychiatric (NP) syndromes as per the ACR case definitions. This included 5 types of headache using the International Headache Society (IHS) criteria. Additional data were demographic and clinical variables, SLE global disease activity (SLEDAI-2K), which includes “lupus headache” as a standalone variable, SLICC/ACR damage index (SDI) and self-report mental (MCS) and physical (PCS) component summary scores of the SF-36. Statistical analysis used linear regression models with generalized estimating equations to account for within patient correlation.

Results: Of the 1732 enrolled patients 89% were female. Race/ethnicity was Caucasian (48%), African (16%), Asian (16%), Hispanic (16%) and other (4%). At enrollment the mean (±SD) age was 34.6±13.4 years, disease duration was 5.6±4.8 months and followup was 3.8±3.1 years. Twenty-six (1.5%) patients had “lupus headache” at 27 (0.36%) of 7523 assessments. Concurrent classification using the IHS headache case definitions were: migraine (13), tension headaches (8), intractable non-specific headaches (5), cluster headaches (1) and intracranial hypertension (1). Two patients had 2 types of headache and in 5 (18.5%) of 27 assessments there were concurrent NP events. “Lupus headache” was reported at both enrollment (n=14) and follow-up (n=13) assessments, in patients from all racial/ethnic groups in 15 of 30 (50%) sites located in 8 of 11 countries (USA, Canada, UK, Spain, South Korea, Mexico, Sweden and Iceland). The estimated mean (±SE) SLEDAI-2K scores, without including “lupus headache” variable, for visits with no headache (n=6019), a non-lupus headache (n=1330) and both a non-lupus and “lupus headache” (n=27) were 3.8±0.08, 3.6±0.18 and 7.2±1.40 respectively (p=0.034). Concurrent SF-36 MCS scores were 47.8±0.28, 42.6±0.56 and 39.4±2.41 (p<0.001) and PCS scores were 42.6±0.30, 38.1±0.53 and 32.4±1.76 (p<0.001). SLEDAI-2K scores, without including “lupus headache” variable, for patients with and without “lupus headache” were 7.2±1.40 vs 3.7±0.08 (p=0.035). In 5/26 (19.2%) patients “lupus headache” was the sole contributor to the SLEDAI-2K score. Concurrent SF-36 MCS and PCS scores for patients with and without “lupus headache” were 39.4±2.41 vs 46.8±0.27 (p=0.002) and 32.4±1.76 vs 41.7±0.28 (p<0.001) respectively.

Conclusion: “Lupus headache”, although infrequent, was associated with higher global disease activity and a lower HRQoL. It was not reproducibly aligned with a uniform IHS classification of headache (e.g. intractable headache). The lack of consistency in diagnosing “lupus headache”, even by experienced clinicians, indicates a need to better define “lupus headache” and to reach consensus on whether it is truly a standalone manifestation of NPSLE.

 


Disclosure:

J. G. Hanly,
None;

M. B. Urowitz,
None;

A. o’Keeffe,
None;

C. Gordon,
None;

S. C. Bae,
None;

J. Sanchez-Guerrero,
None;

J. Romero-Diaz,
None;

A. E. Clarke,
None;

S. Bernatsky,
None;

D. J. Wallace,
None;

E. M. Ginzler,
None;

D. A. Isenberg,
None;

A. Rahman,
None;

J. T. Merrill,
None;

M. A. Petri,
None;

P. R. Fortin,
None;

D. D. Gladman,
None;

B. J. Fessler,
None;

G. S. Alarcon,
None;

I. N. Bruce,
None;

M. A. Dooley,
None;

K. Steinsson,
None;

M. A. Khamashta,
None;

R. Ramsey-Goldman,
None;

S. Manzi,
None;

G. K. Sturfelt,
None;

O. Nived,
None;

A. A. Zoma,
None;

R. F. van Vollenhoven,
None;

M. Ramos-Casals,
None;

C. Aranow,
None;

M. Mackay,
None;

G. Ruiz-Irastorza,
None;

K. C. Kalunian,
None;

S. S. Lim,
None;

M. Inanc,
None;

D. L. Kamen,
None;

C. Peschken,
None;

S. Jacobsen,
None;

C. Theriault,
None;

K. Thompson,
None;

V. Farewell,
None.

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