Table 4.

UCLA Lung Transplant Program algorithm in consideration of bilateral lung transplantation (LT) for individuals with underlying systemic sclerosis

General Adherence to established guidelines for referral and active listing for LT of patients with underlying usual interstitial pneumonia/non-specific interstitial pneumonia and/or pulmonary arterial hypertensiona† Age ≤ 60 years for bilateral LT SSc diagnosis for ≥ 5 years defined from the date of first non-Raynaud’s phenomenon (limited or diffuse scleroderma) Baseline 6 minute walk distance (6MW) ≥ 100 meters (with oxygen supplementation) No chronic (≥ 6 months), scheduled narcotic requirement or dependence Oral corticosteroid dose ≤ 10mg prednisone (or equivalent) at time of LT

Extrapulmonary Disease

Gastrointestinalb Absence of symptomatic esophageal stricture, active upper GI ulceration, esophagealaperistalsis, achalasia, or abnormal gastric emptying (<25% clearance at 90 minutes post-ingestion) despite aggressive medical therapyc Mild to moderate GER or esophageal/gastric dysmotility is acceptable if controlled by aggressive medical therapyc based on patient self-report Absence of chronic gastrointestinal bleeding with or without associated anemia

Skin/Musculoskeletal Chest skin induration score of ≤ 2 (by modified Rodnan skin score of 0–3) Absence of rapidly progressive, diffuse skin thickening, and/or active digital ulceration with concern for digital necrosis Absence of uncontrolled active myositis or progressive myopathy/neuropathy

Renal Preserved and stable (≥ 3 months) renal function with creatinine clearance > 50mL/minute A minimum time interval of 5 years between renal crisis and active listing for LT

Cardiacd Normal right and left ventricular systolic function without prior clinical left-sided congestive heart failure (without associated pulmonary vascular disease) Absence of obstructive coronary artery disease requiring concurrent coronary artery bypass grafting during LT Acceptable right ventricular function in the setting of known pulmonary vascular disease, without overt clinical signs of right sided congestive heart failuree or consideration of heart-lung transplantation

^aOrens JB, Estenne M, Arcasoy S, et al. International guidelines for the selection of lung transplant candidates: 2006 update--a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2006;25:745–55.

^bwe recommend the following tests be performed for all potential SSc LT recipients: barium esophagram, nuclear medicine quantitative gastric emptying study, esophageal manometry, and upper endoscopy; consider the following tests: dual pH probe study +/− impedance testing

^ccombination of high-dose proton pump inhibitor +/− H2 blocker, high-dose pro-motility agent, and antireflux measures including lifestyle modification counseling

^dconsider cardiac magnetic resonance imaging

^eabsence of clinical congestive heart failure, and preservation of liver function testing (including synthetic function)

^†the relatively poorer prognosis of SSc PAH compared to idiopathic PAH might warrant earlier referral and active listing for LT