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Abstract Details

Measuring Disease Activity During Maintenance Therapy in Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)
Neuromuscular and Clinical Neurophysiology (EMG)
P7 - Poster Session 7 (8:00 AM-9:00 AM)
9-003

To investigate clinically meaningful outcome measures associated with minimum symptom expression (MSE) in a cohort of treated CIDP patients. 

Clinically derived endpoints for CIDP reflecting patient function are necessary to optimally assess disease activity and treatment efficacy.

We retrospectively analyzed a consecutive Australian cohort fulfilling 2021-EAN/PNS CIDP criteria1 on therapy between April 2015 and January 2017. Comprehensive assessments of clinical impairment, disability and patient-reported outcomes were performed pre- and post-treatment for patients on cyclic therapy. MSE was systematically defined as an I-RODS score ≥44/48, with no meaningful functional limitation (all activities scored as possible).

Forty-two patients were identified (mean age 57 years; 64% male; median disease duration 8.5 years[range 0.33-45]). Phenotypes were typical (48%), multifocal/focal (24%), distal (12%), sensory/sensory-predominant (12%), and motor-predominant (4%). Treatments included intravenous immunoglobulin (IVIG) (90%), plasma exchange (5%), prednisone (14%), mycophenolate (12%) and azathioprine (2%). Average IVIG regimen was 0.7g/kg, 4 weekly. 21% (8/38) at baseline and 27% (9/33) post-treatment (mean interval 12.4 days) fulfilled MSE criteria. MSE status was not significantly different by age, disease duration, muscle strength (MRC-SS), dominant grip strength (p=0.089), sensory function (mISS), fatigue (mFSS) or pain. Outcomes significantly distinguishing MSE were 10MWT (7.19 vs 9.65 sec, median faster walk 2.37 sec, 95%CI 1.25-3.51, p<0.001) and INCAT (2.5 vs 3, median decrease 1-point, 95%CI 0-2, p=0.026). Logistic regression showed MSE associated with faster 10MWT (OR7.7, 95%CI 1.6–37.0, p=0.011; and lower INCAT (OR 2.3, 95%CI 1.06-5, p=0.036). 10MWT showed excellent discrimination of MSE (AUC 0.93, 95% CI 0.82–1.00, p<0.001) with optimal cut-off ≤6.5 s (sensitivity 0.90, specificity 0.80, p<0.001). Discrimination was fair for INCAT (AUC 0.758, 95%CI 0.603–0.914; p = .001); cut-off ≤2 yielding 63% sensitivity and 73% specificity.

MSE was achieved in 21% of CIDP patients on maintenance treatment, best distinguished by measures of gait (10MWT) and disability (INCAT).
Authors/Disclosures
Grace Swart, MD
PRESENTER 		Dr. Swart has received research support from National Health and Medical Research Council. Dr. Swart has received personal compensation in the range of $5,000-$9,999 for serving as a MS Masters Forum Attendee (sponsored education) with Merck.
Nidhi Garg, MBBS (Concord Repatriation General Hospital) Dr. Garg has nothing to disclose.
Con Yiannikas, MBBS Dr. Yiannikas has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Abbvie.
Ostoja (Steve) Vucic, MBBS, FRACP (Concord Hosoital) Prof. Vucic has a non-compensated relationship as a Member-Advisory Board with Biogen Idec Australia that is relevant to AAN interests or activities. Prof. Vucic has a non-compensated relationship as a Honorarium with Merkc Serono Ausralia that is relevant to AAN interests or activities.
John Pollard, MD (University of Sydney) Dr. Pollard has nothing to disclose.
Antonia S. Carroll, MBBS (St Vincent's Hospital) Dr. Carroll has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alnylam and Sanofi. Dr. Carroll has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for CSL.
Matthew K. Silsby, MBBS (Westmead Hospital) Dr. Silsby has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. Dr. Silsby has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx.
Matthew C. Kiernan, MBBS, PhD, FRACP (Neuroscience Research Australia) The institution of Prof. Kiernan has received personal compensation in the range of $50,000-$99,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for British Medical Journal Publishers (UK).
Judith M. Spies, MBBS, PhD (Royal Prince Alfred Hospital) Dr. Spies has nothing to disclose.
Susanna B. Park, PhD Dr. Park has a non-compensated relationship as a Board Member with Toxic Neuropathy Consortium, Peripheral Nerve Society that is relevant to AAN interests or activities.