IMMUNE SYSTEM AND IMMUNE RESPONSES TO THE THERAPEUTICS IN LYSOSOMAL DISORDERS WORKSHOP

The immune system involvement poses one of the most challenging issues in LDSs with vast ramifications on different disease pathology, organ system involvement and therapeutic aspects.  Health care providers are often not able to discern different aspects of immunological involvement and clinical management in different LSDs. They are often unaware of the emerging immunologic associations such as autoimmunity, skeletal involvement and response to therapy.   Early diagnosis of immune system involvement either presenting as Immune Hypersensitivity reactions or Antidrug antibody formation including, autoimmunity requires a high degree of suspicion, as the presentation is often non-specific.  Delay of diagnosis and subsequent treatment management can result in life threatening complications, and/or treatment failure.  Health care providers are not usually familiar with consequences of Immune system involvement and treatment of complications.