TY - JOUR
T1 - Seronegative patients form a distinctive subgroup of immune-mediated necrotizing myopathy
AU - Lim, Johan
AU - Rietveld, Anke
AU - de Bleecker, Jan L.
AU - Badrising, Umesh A.
AU - Saris, Christiaan G. J.
AU - van der Kooi, Anneke J.
AU - de Visser, Marianne
PY - 2019/1
Y1 - 2019/1
N2 - Objectives To investigate the characteristics of different clinico-serologic subgroups of immune-mediated necrotizing myopathy (IMNM). Methods We retrospectively reviewed data from medical charts of 64 patients diagnosed with IMNM between 2012 and 2017 in 3 neuromuscular referral centers in The Netherlands and 1 in Belgium. Results Seventeen patients had anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) autoantibodies (Abs), of whom 11 had a history of statin use, 15 had anti-signal recognition particle (SRP) Abs, 2 had anti-melanoma differentiation-associated protein 5 (MDA5) Abs, 22 patients were seronegative, and 9 patients did not have a complete Ab assessment. Moderate to severe disability in HMGCR Ab-positive and anti-SRP Ab-positive IMNM was common (71% and 60%, respectively) despite multimodality treatment. Compared with statin-associated anti-HMGCR Ab-positive IMNM, statin-naive anti-HMGCR Ab-positive IMNM patients were more often men (67% vs 45%), had lower rates of dysphagia (17% vs 45%), and more frequently had third-line therapy (50% vs 9%) and poor to fatal outcome (50% vs 0%). Compared with seropositive IMNM, seronegative IMNM was characterized by female predominance (1:3), frequent occurrence of associated connective tissue disorders (22% vs 9%), and significantly higher rates of extramuscular disease activity (50% vs 16%, p 0.014; 2-sided Fisher exact), also after excluding patients with an associated connective tissue disease (35% vs 7%, p 0.038; 2-sided Fisher exact). Conclusions Our findings indicate that seronegative IMNM forms a subgroup with distinctive features from seropositive IMNM.
AB - Objectives To investigate the characteristics of different clinico-serologic subgroups of immune-mediated necrotizing myopathy (IMNM). Methods We retrospectively reviewed data from medical charts of 64 patients diagnosed with IMNM between 2012 and 2017 in 3 neuromuscular referral centers in The Netherlands and 1 in Belgium. Results Seventeen patients had anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) autoantibodies (Abs), of whom 11 had a history of statin use, 15 had anti-signal recognition particle (SRP) Abs, 2 had anti-melanoma differentiation-associated protein 5 (MDA5) Abs, 22 patients were seronegative, and 9 patients did not have a complete Ab assessment. Moderate to severe disability in HMGCR Ab-positive and anti-SRP Ab-positive IMNM was common (71% and 60%, respectively) despite multimodality treatment. Compared with statin-associated anti-HMGCR Ab-positive IMNM, statin-naive anti-HMGCR Ab-positive IMNM patients were more often men (67% vs 45%), had lower rates of dysphagia (17% vs 45%), and more frequently had third-line therapy (50% vs 9%) and poor to fatal outcome (50% vs 0%). Compared with seropositive IMNM, seronegative IMNM was characterized by female predominance (1:3), frequent occurrence of associated connective tissue disorders (22% vs 9%), and significantly higher rates of extramuscular disease activity (50% vs 16%, p 0.014; 2-sided Fisher exact), also after excluding patients with an associated connective tissue disease (35% vs 7%, p 0.038; 2-sided Fisher exact). Conclusions Our findings indicate that seronegative IMNM forms a subgroup with distinctive features from seropositive IMNM.
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85060824243&origin=inward
UR - https://www.ncbi.nlm.nih.gov/pubmed/30345336
U2 - https://doi.org/10.1212/NXI.0000000000000513
DO - https://doi.org/10.1212/NXI.0000000000000513
M3 - Article
C2 - 30345336
SN - 2332-7812
VL - 6
SP - e513
JO - Neurology® neuroimmunology & neuroinflammation
JF - Neurology® neuroimmunology & neuroinflammation
IS - 1
M1 - e513
ER -