Pentosan polysulfate (PPS), a Food and Drug Adminsitration (FDA)-approved drug with anti-inflammatory and prochondrogenic properties, was shown to improve clinical manifestations of the disease in MPS VI rats, and to reduce urinary GAGs and proinflammatory cytokines (IL-8 and TNF-alpha) in tissues and in the CSF in MPS I dogs [48, 56, 57]. Here, TNF is linked to Scheie syndrome.