Risk factors for osteoporosis, falls and fractures in hereditary myopathies and sporadic inclusion body myositis — A cross sectional survey

摘要: Abstract Background The risk of osteoporosis is known in myopathies requiring long-term steroid treatment and Pompe disease, but not other hereditary or sporadic inclusion body myositis (sIBM). Methods Risk factors osteoporosis, laboratory parameters bone metabolism, frequency falls fractures, walking ability, pain were surveyed using questionnaires 89 patients with sIBM genetically confirmed facioscapulohumeral muscular dystrophy (FSHD), myotonic types 1 2 (DM1, DM2), limb girdle dystrophies (LGMD2A, LGMD2B, LGMD2I), MATR3 myopathy, oculopharyngeal (OPMD). Additionally metabolism determined. Results mean age at examination per disease group ranged from 32 years LGMD2A to 70 sIBM. Myopathies a higher degree impairment had (sIBM, LGMD2A, LGMD2B). At the time 3.4% history osteoporosis. 25-OH D3 level was decreased 20% (and 55% LGMDs), 57% them ambulatory. significantly lower than neurological disorders (p  Conclusion for reduced seems be increased wheelchair-bound myopathy also DM1 autosomal-recessive myopathies.