FRI0398 Inflammatory myopathies with camptocormia or dropped head syndrome are associated scleromyositis with late onset and delayed diagnosis. (15th June 2017)

• Record Type: Journal Article
• Authors: Pijnenburg, L; Maurier, F; Poursac, N; Couderc, M; Vernier, N; Guichard, I; Sellam, J; Sibilia, J; Meyer, A
• Is Part Of: Annals of the rheumatic diseases. Volume 76(2017)Supplement 2
• Page Start: 638
• Page End: 638
• Publication Date: 2017-06-15
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2017-eular.6564
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Background: 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). Objectives: To assess the signification of dropped head syndrome and/or camptocormia in patients with IM. Methods: All practitioners of the French Myositis Network and the Club Rhumatisme et Inflammation (>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. Results: 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<0.005) and diagnosis of IM was delayed (16.4±4.5 months vs. 8.5±2.7, p<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<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<0.05). DM rash was hardly threefold less frequent in axial IM patients (15% vs. 42%, p<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<0.05) and inclusion body myositis (20% vs. 2.6% in the controls, p<0.05). DM was twofold less frequent than in control (10% vs 41%, p<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. Conclusions: In IM, camptocormia and dropped head syndrome are associated with late onset scleromyositis and sIBM with delayed diagnosis. Disclosure of Interest: None declared