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- Title
Long-term efficacy of adding intravenous immunoglobulins as treatment of refractory dysphagia related to myositis: a retrospective analysis.
- Authors
Giannini, Margherita; Fiorella, Maria Luisa; Tampoia, Marilina; Girolamo, Francesco; Fornaro, Marco; Amati, Angela; Lia, Anna; Abbracciavento, Letizia; D'Abbicco, Dario; Iannone, Florenzo
- Abstract
Objective Dysphagia is a life-threating manifestation of idiopathic inflammatory myopathies (IIM). However, we lack a univocal protocol for its treatment. The aim of this retrospective analysis was to evaluate the effectiveness of a step-up strategy by adding a 1-day pulse of IVIGs to immunosuppressants in IIM patients with refractory dysphagia diagnosed by Eating Assessment Tool (EAT)-10 and fibreoptic endoscopic evaluation of swallowing (FEES). Methods Dysphagia was defined as a pharyngo-oesophageal disturbance associated with EAT-10 score ≥3 and at least one FEES abnormality among propulsion failure, solid or liquid stasis. Eighteen out of 154 IIM patients had FEES-confirmed dysphagia and underwent 1 day IVIG 2 g/kg repeated 1 month apart for 3 months, because of dysphagia refractory to high-dose glucocorticoids with methotrexate and/or azathioprine. Clinical characteristics along with myositis-specific antibodies and muscle histopathological findings were studied in FEES-dysphagia IIM and IIM control patients. Results After three monthly doses of IVIG, EAT-10 score dropped with complete recover of defective propulsion and progressive decrease in percentage of both solid and liquid stasis. At 52-weeks' follow-up, reached in 12 patients, all these parameters were stable or further improved. An improvement in manual muscle strength test and a steroid-sparing effect of IVIG were also observed. Anti-PM/Scl 75/100 antibodies were much more frequent in the FEES-dysphagia group, while anti-Jo1 antibody was rarely detected. Conclusion Our treatment schedule with 2 g/kg IVIG was effective for IIM-associated refractory dysphagia assessed by the combination of EAT-10 and FEES. These findings need to be prospectively tested in a larger cohort of IIM patients.
- Subjects
THERAPEUTIC use of immunoglobulins; GLUCOCORTICOIDS; INTRAVENOUS therapy; IMMUNOGLOBULINS; AZATHIOPRINE; POLYMYOSITIS; DEGLUTITION disorders; RETROSPECTIVE studies; HEALTH outcome assessment; METHOTREXATE; MUSCLE strength; DESCRIPTIVE statistics; MYOSITIS; ENDOSCOPY
- Publication
Rheumatology, 2021, Vol 60, Issue 3, p1234
- ISSN
1462-0324
- Publication type
Article
- DOI
10.1093/rheumatology/keaa443