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Comparative Study
. 2025 Mar 1;64(3):1431-1436.
doi: 10.1093/rheumatology/keae129.

Inclusion-body myositis associated with Sjögren's disease: clinical characteristics and comparison with other Sjögren-associated myositis

Quentin Astouati  1   2   3 , Thomas Machet  1   2   3 , Camille Houssais  4 , Jean-Baptiste Noury  5   6 , Yves Allenbach  7   8 , Laure Gallay  9 , Baptiste Quere  4   6 , Florence Assan  10 , Olivier Benveniste  7   8 , Jonathan Broner  11 , Pierre Duffau  12   13 , Alexandra Espitia  14 , Anne Grasland  15 , Gilles Hayem  16 , Véronique Le Guern  17 , Nihal Martis  18   19 , Kuberaka Mariampillai  7   8 , Gaëtane Nocturne  20 , Xavier Mariette  20 , Alain Meyer  21 , Denis Mulleman  22   23 , Valérie Devauchelle-Pensec  4   6 , Aurore Collet  24   25   26 , David Launay  1   2   3   24   25 , Eric Hachulla  1   2   3 , Divi Cornec  4   6 , Dewi Guellec  4   6 , Sébastien Sanges  1   2   3   24   25
Affiliations
Comparative Study

Inclusion-body myositis associated with Sjögren's disease: clinical characteristics and comparison with other Sjögren-associated myositis

Quentin Astouati et al. Rheumatology (Oxford). .

Abstract

Objectives: To describe the characteristics of patients with Sjögren's disease (SjD) and inclusion-body myositis (IBM), and how they compare to SjD patients with other inflammatory myopathies (IM).

Methods: Patients were retrospectively recruited from 13 French centres and included if they met the ACR/EULAR criteria for SjD and for IM. They were categorized as SjD-IBM if sub-criteria for IBM were met, or as SjD-other IM if not.

Results: SjD-IBM patients (n = 22) were mostly females (86%), with a median [Q1; Q3] age of 54 [38.5; 64] years at SjD diagnosis, and 62 [46.5; 70] years at first IBM symptoms. Although most patients displayed glandular and immunological abnormalities, additional extra-glandular manifestations were uncommon, resulting in moderate disease activity at SjD diagnosis (ESSDAI 5.5 [1; 7.8]). Classic IBM features were frequent, such as progressive symptom onset (59%), asymmetrical (27%) and distal (32%) involvements, dysphagia (41%), low CPK (386.5 [221.8; 670.5] UI/l) and CRP (3.0 [3; 8.5] mg/l) levels. Immunosuppressants were reported as efficient in 55% of cases. Compared with SjD-IBM patients, SjD patients with other IM (n = 50) were significantly younger, displayed more frequent additional extra-glandular disease, higher ESSDAI score (11 [3; 30]), shorter delay between SjD diagnosis and myositis onset (0 [-0.5; 26]), more frequent CPK values over 1000 UI/l (36%), and less frequent classic IBM features.

Conclusion: IBM can occur in SjD patients, with muscle features reminiscent of classic sporadic IBM characteristics, but mostly affecting women. In SjD patients with muscle involvement, extra-glandular manifestations, high ESSDAI score, elevated CPK values and shorter delay after SjD diagnosis plead against IBM.

Keywords: ESSDAI; Sjögren’s disease; connective tissue diseases; inclusion body myositis; inflammatory myopathy.

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