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This study aimed to estimate the minimal clinically important difference (MCID) for an improvement in forced vital capacity in the upright seated position (FVC<jats:sub>up<\/jats:sub>) and the 6\u2010min walk test (6MWT) after a year of treatment with enzyme replacement therapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Data were obtained from two prospective follow\u2010up studies. Between\u2010group and within\u2010group MCIDs were estimated using anchor\u2010based methods. Additionally, a distribution\u2010based method was used to generate supportive evidence. As anchors, self\u2010reported change in health and in physical functioning, shortness of breath and a categorization of the Short\u2010Form 36 Physical Component Summary score were used. Anchor appropriateness was assessed using Spearman correlations (absolute values \u22650.29) and a sufficient number of observations in each category.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In all, 102 patients had at least one FVC<jats:sub>up<\/jats:sub> or 6MWT measurement during enzyme replacement therapy. Based on the anchors assessed as appropriate, the between\u2010group MCID for an improvement in FVC<jats:sub>up<\/jats:sub> ranged from 2.47% to 4.83% points. For the 6MWT, it ranged from 0.35% to 7.47% points which is equivalent to a distance of 2.18\u201346.61\u2009m and 1.97\u201342.13\u2009m for, respectively, a man and a woman of age 50, height 1.75\u2009m and weight 80\u2009kg. 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