Kauvery performs rare surgery on patient with auto-immune disease in Chennai
CHENNAI : Kauvery Hospitals Chennai has performed successful bilateral lung transplantation in a woman aged 53, with Interstitial Lung Disease (ILD) related to Scleroderma—a common auto-immune disease, causing inflammation and scarring of various parts of the body. It affects skin with skin tightening; finger tips (Raynaud’s phenomenon) with ulcers of hands and feet; the esophagus with poor food movement and reflux symptoms; lungs with scarring of the air sacs and pulmonary hypertension, and kidneys causing renal failure.
The individual was diagnosed with ILD five years ago, and she was being managed with appropriate medications. Unfortunately, she continued to worsen with breathlessness on performing daily activities and her worsening lung functions detected by pulmonary function testing (PFTs) and CT scans. She was not on oxygen at this stage but had low oxygen levels on minimal exertion. She underwent a thorough evaluation for lung transplantation that revealed a set of complex challenges.
Her disease was optimised with input from the rheumatologist, Dr Sham and gastroenterologist Dr Arvind. She was added to the transplant list and was awaiting a suitable donor for nine months. During this period, she continued to worsen, needing eight liters of oxygen at rest during the day and home ventilator support at night.
Dr Srinivas Rajagopala, senior consultant in Pulmonology, and director of Transplant Pulmonology & Lung Failure Unit said, “The transplant was medically complex and required optimisation and careful planning. Several centres abroad do not accept scleroderma patients because of the concern of aspiration due to poor movement of the esophagus which can cause permanent damage to the lung graft.
Additional concerns in her were that she had severe pulmonary hypertension, and was immunologically sensitised, meaning that she had antibodies (anti-HLA) against potential donor lungs. If the potential donor lungs were to react with these antibodies, it could lead to rejection. Esophageal function was evaluated with 24-hour impedance manometry, pH studies and gastric emptying tests.
Motility was improved through medical management and care was taken to ensure no reflux into the proximal part of her esophagus, to limit the risk of aspiration into the lungs. We obtained virtual and actual cross-match at the time of donor availability to avoid antibody-donor lung reactions. Initially, this led to several donor lungs being unacceptable and she continued to worsen without transplant. Subsequently, we changed our strategy and accepted low-level single antigen reactions that we managed with immunosuppression.”
Bilateral lung transplant with central ECMO support was done by Dr Kumud Kumar Dhital, program director, with support of lead anaesthetist and intensivist Dr Pradeep Kumar. She was weaned off the ventilator support within 24 hours and oxygen support by fourth day post-transplant. She was able to walk normally and use the stairs without difficulty by day-10. She was discharged home on the 12th post-operative day, and has now completed her first follow-up. Her lung function continues to improve and she is doing well with resumption of independent daily activities at home.
ABOUT SCLERODERMA
Scleroderma is a common autoimmune disease that causes inflammation and fibrosis in various organs of the body such as skin, esophagus, lungs, kidneys, and finger tips.
Interstitial Lung Disease (ILD) which causes inflammation and subsequent scarring of the walls of the air sacs leading to permanent reduction in lung capacity and function, is a common feature in Scleroderma, affecting 45-50% of patients.
Pulmonary hypertension can be associated with ILD or exist independently. It worsens breathlessness and can lead to failure of the right side of the heart.