Unravelling Anaesthetic Challenges in Patient with Diffuse Systemic Sclerosis: A Case Report

Systemic sclerosis or scleroderma is an uncommon autoimmune condition with a global incidence of 8 to 56 new cases per million per year, which commences from skin and progresses to affect multiple systems in the body. It is marked by abnormalities in blood vessels, sclerosis of connective tissues and atrophy of skin and internal organs. The systemic sclerosis treatment depends on the disease manifestation and are usually treated with vasodilators (for vasculopathy improvement and prevention of Raynaud's phenomenon), prostacyclin analogues (for the prevention of Raynaud's phenomenon refractory to oral vasodilators), immunosuppressants like methotrexate, cyclophosphamide, mycophenolate mofetil, low dose corticosteroids (for the treatment of skin hardening, interstitial lung disease and inflammatory arthritis) and antifibrotics like endothelin receptor antagonists (for the treatment of pulmonary artery hypertension). Avascular necrosis in systemic sclerosis can arise as a result of the macrovascular and microvascular effects of vasculitis and corticosteroid therapy. The multisystem involvement of systemic sclerosis can impact every aspect of anaesthetic care especially airway management. During perioperative management, numerous systemic manifestations like pulmonary artery hypertension, interstitial lung disease and cardiac arrhythmia should be considered. The regional anaesthesia serves as a safe alternative to general anaesthesia and useful adjunct in the management of postoperative pain, but can be technically challenging. This case report described a 61-year-old female patient who had a history of systemic sclerosis for past 25 years and was scheduled for a total hip replacement due to avascular necrosis and secondary osteoarthritis of the left hip. The patient was having difficult cannulation, interstitial lung disease and anticipated difficult airway because of microstomia, limited mouth opening and limited flexion and extension at atlantooccipital joint. But the patient was successfully managed with combined spinal epidural anaesthesia, which provided effective pain control and minimised the perioperative risk associated with general anaesthesia.