Enlarged lymph nodes can be indicative of acute inflammation, and several lines of evidence suggest inflammatory immune processes may be involved in mediating FSHD pathophysiology [9, 15, 16, 61, 62], supporting that an inflammatory immune response was stimulated by low-level DUX4-fl expression in these mice. This evidence concerns the gene DUX4 and facioscapulohumeral muscular dystrophy.