Introduction:
Systemic sclerosis is a rare and progressive multisystem autoimmune disorder that is characterized pathologically by vascular abnormalities, connective tissue sclerosis and atrophy of skin and various internal organs (e.g., alimentary tract, lungs, heart, kidney, CNS), and autoantibodies. With an unknown etiology, Scleroderma is a complex polygenetic disease. A recent Genome Wide Association Study (GWAS) confirmed a strong association with the Major Histocompatibility Complex (MHC) and autoimmunity. We provide a case scenario along with a review of the systems involved and challenges physicians can face in dealing with this rare disease.
Case presentation:
Our patient, a known case of systemic sclerosis, was admitted with a history of right femur fracture following a fall. We highlight the medical, anesthetic and surgical challenges faced by our team in the management of this patient. We will explain the stages patient faced in treatment process till her death. We combined the case report with detailed literature review of this rare disease.
Discussion:
Systemic sclerosis is a complex disease process with many different levels of system involvement. Patient needs to be reviewed thoroughly in preoperative period by multidisciplinary team and counseled in detail about the difficulties in procedure, risks and complications.
Conclusion:
Patient with scleroderma presents a challenge to the surgical team and anesthetist and a multidisciplinary approach should be followed with all of these patients to avoid catastrophic results.