Manger Bernhard, Mengel Eugen, Schaefer Roland M
Department of Medicine III, Institute for Clinical Immunology and Rheumatology, Friedrich-Alexander-University Erlangen-Nurnberg, Krankenhausstrasse 12, 91054, Erlangen, Germany.
Clin Rheumatol. 2007 Mar;26(3):335-41. doi: 10.1007/s10067-006-0299-x. Epub 2006 May 6.
Lysosomal storage diseases are rare metabolic disorders, some of which can now be treated using enzyme replacement therapies. Because the time point of treatment initiation significantly influences the outcome in Gaucher disease, Fabry disease, and mucopolysaccharidosis type I, early diagnosis is of utmost importance. All three disorders can present with musculoskeletal symptoms in early stages, therefore, the rheumatologist may be the first to be contacted by these patients. Here, we present three characteristic lysosomal storage disease cases to increase awareness in the rheumatological community of the typical symptom constellations associated with these rare but treatable disorders.
溶酶体贮积症是罕见的代谢紊乱疾病,其中一些现在可以通过酶替代疗法进行治疗。由于治疗开始的时间点对戈谢病、法布里病和I型黏多糖贮积症的治疗结果有显著影响,因此早期诊断至关重要。这三种疾病在早期都可能出现肌肉骨骼症状,因此,风湿病学家可能是这些患者最先联系的对象。在此,我们展示三个典型的溶酶体贮积症病例,以提高风湿病学界对这些罕见但可治疗疾病相关典型症状组合的认识。