FRI0398 Inflammatory myopathies with camptocormia or dropped head syndrome are associated scleromyositis with late onset and delayed diagnosis
Authors: L Pijnenburg, F Maurier, N Poursac, M Couderc, N Vernier, I Guichard, J Sellam, J Sibilia, A Meyer
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Severe weakness of axial muscle leads to dropped head syndrome or camptocormia. The signification of these symptoms has not been studied in inflammatory myopathies (IM). <h3>Objectives</h3> To assess the signification of dropped head syndrome and/or camptocormia in patients with IM. <h3>Methods</h3> All practitioners of the French Myositis Network and the Club Rhumatisme et Inflammation (&gt;1000 physicians) were invited to report their patients suffering from IM (myopathy with myositis specific autoantibody and/or typical muscle biopsy according to the ENMC criteria) with camptocormia and/or dropped head syndrome. These axial IM cases were included only if no other explanation of axial weakness was found. IM patients without axial involvement (non-axial IM group), were randomly selected from the participative centers, and included as control patients (ratio 1:2). Clinical, serological, muscle pathological features, management and outcomes were studied using a standardized form. <h3>Results</h3> Twenty patients (sex ratio 2.6) with axial involvement (camptocormia: 60%, dropped head syndrome: 40%) were included. Compared with the control group, these axial IM-patients were older (64.05±11.64 y. vs. 48.22±18.28. p&lt;0.005) and diagnosis of IM was delayed (16.4±4.5 months vs. 8.5±2.7, p&lt;0.05). All patient except one had also proximal weakness of the limbs. CK blood level was 2794±870 UI/L, which was similar to the controls (2864±559 UI/L). According to the ENMC classification, non-specific myositis was the most frequent finding on muscle biopsy (n=5/15, 30% vs. n=1/24, 4% in the non-axial group, p&lt;0.05) and dermatomyositis (DM) pattern tended to be less frequent in axial-IM patients (3/12 20% vs. 11/24, 46%, p=0.10). Most of the patients (75%) had also extra muscular involvement including acrosyndrome (45%), interstitial lung disease (35%), sclerodactyly (30%), telangiectasia (25%), digital tip ulcer (10%), sclerodermy (5%). By contrast, no patient had polyarthrithis (vs. 20% in the controls, p&lt;0.05). DM rash was hardly threefold less frequent in axial IM patients (15% vs. 42%, p&lt;0.05). Auto-antibodies associated with scleromyositis were the most frequent in axial-IM patients (30% vs. 10.3%, p=007, manly anti-PM/Scl). One patient (5%) had cancer within the 3 years before or after IM diagnosis (NS vs. controls). Thus, most frequent diagnosis in axial IM-patients were scleromyositis (35% vs. 5% in the controls, p&lt;0.05) and inclusion body myositis (20% vs. 2.6% in the controls, p&lt;0.05). DM was twofold less frequent than in control (10% vs 41%, p&lt;0.05). Other IM subtypes were not statistically different from the control groups. Except patients with diagnosis of sIBM, all axial IM-patients received corticosteroids with another immunomodulatory drugs (median number 2, range 1–5). Half of the axial-IM patients received intravenous immunoglobulin. After a mean follow up of 68.4±3.76 months all patients had improvement, including in axial weakness, except in patients with sIBM. One patient died from ischemic cardiomyopathy. <h3>Conclusions</h3> In IM, camptocormia and dropped head syndrome are associated with late onset scleromyositis and sIBM with delayed diagnosis. <h3>Disclosure of Interest</h3> None declared