Inaugural dropped head syndrome and camptocormia in inflammatory myopathies: a retrospective study.

Inaugural axial muscle involvement, defined as dropped head syndrome (DHS) and/or camptocormia (CC), is poorly described in inflammatory myopathies (IM). This study aimed to further characterize IM patients with inaugural DHS/CC, their outcome and care management. This retrospective study included IM patients diagnosed between 2000 and 2021. The main inclusion criterion was IM revealed by axial muscle deficit (DHS/CC). Twenty-seven patients were included; median (IQR) age at first symptoms was 66.0 years (55.5-75.0); 21 were female (77.8%). There were 9 inclusion-body myositis (IBM, 33.3%), 9 overlap myositis (OM, 33.3%), 5 dermatomyositis (DM, 18.5%), 2 immune checkpoint inhibitor-related myositis (7.4%), 1 focal myositis (3.7%) and 1 myositis with anti-Hu antibodies (3.7%). Age at first symptoms was ≤ 70 years in 16 patients (59.3%), including all DM patients and 8/9 OM patients (88.9%). In this group, partial remission of the disease was obtained in 9/16 (56.3%) and complete remission in 1/16 patient (6.3%); regression of DHS/CC was achieved in 3/16 patients (18.8%). Conversely, in the group of 11 patients aged > 70 years at first symptoms, there were 8 IBM (72.7%). Partial remission was obtained in 5/11 patients (45.5%), the disease was stable in 6/11 patients (54.5%); no complete remission was obtained nor regression of DHS/CC. The analysis of IM patients with inaugural DHS/CC delineates 2 groups of patients according to the age at first symptoms in terms of clinical and outcome specificities, and proposes an adapted diagnostic and care management approach to prevent long-term complications.

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© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology.