- Indian Rheumatology association
Srinivas Rajagopala MD, DM, Fellowship in Lung Transplantation (Toronto)
Clinical Lead, Transplant Pulmonology, Department of Heart & Lung Transplantation, Clinical Lead, Pulmonology & Sleep Medicine, Apollo Speciality Hospital, Teynampet, Chennai, Tamil Nadu.
Lung transplantation (LT) is the only curative treatment for patients with advanced end-stage lung diseases and chronic progressive respiratory failure. LT has been offered as a treatment for over 40 years, primarily in North America, Europe, and Australia. It is an acceptable modality with good survival and quality of life outcomes when selected at the appropriate time in the illness. The proportion of LTs performed for connective tissue disease (CTD) syndromes is small, accounting for 0.9% of the estimated 6,000 LTs in 2019. Rheumatoid arthritis and undifferentiated connective tissue disease (CTD), followed by systemic sclerosis (SSc), account for more than 70% of these transplants. In India, lung transplants were the last solid organ transplants to be successfully performed, and LT programs have only become established since 2017.
Approximately 200-250 LTs are now being offered nationwide, primarily driven by two to three transplant units. About 60-85% of these procedures are for interstitial lung diseases (ILDs) and pulmonary hypertension (PH), influenced by referral bias. The majority of these ILDs are non-idiopathic pulmonary fibrosis ILDs (76%); hypersensitivity pneumonitis (30%), non-specific interstitial pneumonia (likely related to lung-limited CTD, 29.5%), CTDs (17.2%), and sarcoidosis (9%). Certainly, progressive CTD-ILDs and/or CTD-PH are common indications for lung transplantation and have good outcomes at our centre. There are common questions relevant to practicing rheumatologists and pulmonologists when considering lung transplantation as a treatment modality.
Patients should be referred for evaluation for LT when ILD or PH is progressive despite first-line medical management. Patients should be listed for LT when progression is evident, with an expected two-year survival rate of less than 50% and an estimated post-LT survival rate of more than 80%.
The concern regarding LT in CTDs arises from the presence of unfavourable risk factors affecting post-transplant outcomes in patients with CTD-ILDs or PH. These factors include active extra-pulmonary disease, oesophageal disease, bone marrow involvement, cardiac disease, and pleuro-pulmonary disease, which can lead to increased surgical bleeding, anaesthetic challenges, and pulmonary hypertension. Additionally, there are associations with pre-formed anti-HLA antibodies, the difficulty in distinguishing post-transplant rejection from severe flares of CTD, and concerns regarding long-term outcomes for these patients. Careful evaluation and pre-transplant optimization can alleviate most of these concerns. Specifically, in cases of scleroderma, discussions about the anticipated duration of nil per oral post-operatively and the use of post-pyloric feeds should take place pre-operatively before listing. Rarely, LT is performed in life-threatening situations due to lupus pneumonitis or anti-MDA5 syndrome associated with extensive, potentially irreversible lung damage, often requiring extracorporeal oxygenation (ECMO).
A small single-centre series of carefully selected patients with SSc-ILD shows similar outcomes, with no differences in rates of acute rejection or long-term survival. The data on other CTDs and lung-limited CTD syndromes are smaller but promising. Patients can expect a one-year survival rate of 85-90%, a three-year survival rate of 70-75%, a five-year survival rate of 65-70%, and a ten-year survival rate of 45-50%. These figures are substantially higher than the survival of advanced ILDs with progressive respiratory failure, which typically falls below 40-50% at two years. Most patients also enjoy an excellent quality of life after LT, and approximately 70% resume productive work. This has certainly been our experience with carefully selected patients with CTD-ILD in our centre.
Complications following lung transplantation (LT) occur within a timeframe that can be anticipated, often based on the clustering of preoperative risk factors, donor characteristics, and intraoperative considerations. Anticipating these helps mitigate their occurrence, facilitates earlier recognition and appropriate treatment. This also highlights the need for a structured follow-up by a dedicated transplant Pulmonologist to manage these patients.
There are several absolute contraindications to LT, as well as risk factors that significantly increase the risk of complications post-LT. Severe oesophageal dysmotility, kidney disease with GFR <60 ml/min, bone marrow involvement due to disease or therapy, coronary artery disease related to accelerated atherosclerosis, and severe pulmonary hypertension are particularly relevant to patients with CTDs.
