Lung Transplantation in Patient With Scleroderma
A 55-year-old female patient with diffuse scleroderma with involvement of skin, lung (interstitial lung disease), and gastrointestinal (GI) tract, and anti-polymerase I-positive was treated with cyclophosphamide for a year, with improvement. Recently, however, her lung disease deteriorated (she is now New York Heart Association class III), and she developed supraventricular tachycardia (SVT). Diffusing capacity of the lung for carbon monoxide = 17%, forced vital capacity = 54%, pulmonary artery pressure = 40 mm Hg/16 mm Hg, pulmonary capillary wedge pressure = 8 mm Hg, ejection fraction = 60%, and carbon dioxide = 9 mm Hg. A stent was inserted for stenosis of the left anterior descending artery and bosentan was started. Is she a suitable candidate for lung/heart transplantation?

Yolanda Braun, MD

This unfortunate patient can be referred for lung transplantation, but she appears to be high risk. When making the decision with regard to lung transplantation, there are 2 questions that need to be asked on both sides of the equation: first, what is the patient's life expectancy without transplantation; and second, what would her projected life expectancy be with a transplant? The second question invokes the same concept, but pertains to quality of life (QOL) with or without a transplant. In this patient, it does appear that she has significant impairment of her QOL and potentially could benefit from transplantation in this regard. Similarly, patients with scleroderma-associated pulmonary hypertension have an extremely poor prognosis with 1-year survival reported at 55%. In comparison, the most recent lung transplant survival statistics attest to an overall 1-year survival of about 77%. So it appears from both standpoints that this patient could potentially benefit from lung transplantation.

However, when patients are scrutinized as to their candidacy, a focus of the evaluation includes assessment for potential contraindications. In this regard, this patient does have a few "red flags" that raise issues with regard to her overall candidacy. First, she has a systemic disorder and other secondary organ involvement. Although patients with systemic disorders such as scleroderma have been successfully transplanted, they do need to be closely assessed for the extent and impact of other organ involvement. This patient's GI tract involvement would be of concern; if she has significant esophageal dysmotility, she may be at risk of aspiration. If there is any associated gastroesophageal reflux disease, this would be of some concern, especially long-term, since this has an apparent association with chronic allograft rejection. Whether she has any problems with absorption would be relevant for the multiple medications, including immunosuppressive agents, she would require posttransplantation. Her cardiac disease is also of some concern and would likely also place her at higher risk. However, it does not appear that her cardiac issues are significant enough to warrant consideration for heart-lung transplantation. Patients with stable coronary artery disease have been successfully transplanted with acceptable results. Her SVT could potentially be exacerbated but should not preclude her transplant candidacy. Hopefully, her skin involvement is not severe enough in the area of the chest to raise concerns about wound healing.

In summary, this patient could warrant a lung transplant evaluation at an experienced program. None of her issues appear to be absolute contraindications; however, often it is a combination of relative contraindications that can be sufficient to exclude patients as potential transplant candidates.