Rationale: Little is known about hospitalization in other types of interstitial lung disease (ILD) besides idiopathic pulmonary fibrosis (IPF). Objectives: To determine the frequency of hospitalizations in various types of ILD and elucidate the association of hospitalization with outcomes. Methods: An analysis of the Pulmonary Fibrosis Foundation Patient Registry data was performed. Inpatient hospitalization rates and survival posthospitalization were compared for various types of ILD. Measurements and Main Results: Hospitalization rates were similar across ILD types: 40.6% of participants with IPF, 42.8% of participants with connective tissue disease-related ILD (CTD-ILD), 44.9% of participants with non-IPF idiopathic interstitial pneumonia (IIP), 46.5% of participants with chronic hypersensitivity pneumonitis (CHP), and 53.3% of participants with "other" ILD. All-cause hospitalization was not associated with decreased transplant-free survival (adjusted hazard ratio [AHR], 1.20; 95% confidence interval [CI] = 0.98, 1.46; P = 0.0759) after adjusting for comorbidities and severity of illness; however, respiratory-related hospitalization was (AHR, 1.53; 95% CI = 1.23, 1.90; P = 0.0001). Participants with CTD-ILD (HR, 0.43; 95% CI = 0.25, 0.75; P = 0.0031) and non-IPF IIP (HR, 0.3; 95% CI = 0.15, 0.58; P = 0.005) had a lower risk of death posthospitalization compared with those with IPF, whereas those with chronic hypersensitivity pneumonitis (HR, 0.67; 95% CI = 0.37, 1.20; P = 0.1747) or other ILD (HR, 0.54; 95% CI = 0.19, 1.54; P = 0.25) had a risk comparable with that for IPF. Conclusions: Rates of hospitalization are similar across ILD subtypes. The risk of death or transplant after posthospitalization is lower in patients with CTD-ILD and non-IPF IIP, compared with patients with IPF. In a mixed population of participants with ILD, all-cause hospitalizations were not associated with decreased transplant-free survival; however respiratory-related hospitalizations were.
Introduction: Induction therapy with basiliximab, a chimeric anti-interleukin-2 monoclonal antibody, was used in over 70% of patients undergoing lung transplantation in 2018 to prevent acute cellular rejection.Rare cases of acute hypersensitivity reaction, cytokine release, and non-cardiogenic pulmonary edema have been reported.We present a case of severe cytokine release syndrome following induction therapy with basiliximab in a lung transplant (LTx) recipient.Case Description: A 49-year old Caucasian male with familial pulmonary fibrosis was admitted for a bilateral LTx.The donor was a 52-year old male with head trauma who donated after cardiac death.Ex Vivo Lung Perfusion was used to facilitate LTx.Cardiac function was normal intraoperatively.In the immediate post-operative period, he required low dose norepinephrine and vasopressin for hypotension and inhaled nitric oxide for hypoxia.Bronchoscopy showed intact anastomoses without secretions.The patient was given basiliximab 20 mg in addition to standard immunosuppression of tacrolimus, mycophenolate and solumedrol.Ten hours post-LTx and 6 hours post basiliximab dosing, the patient developed fever (maximal temperature of 105.3°F (40.7°C)) with worsening vasopressor-refractory shock treated with angiotensin II.Laboratory values were significant for white blood cell count 24 x10 3 /uL, platelet count 68 x10 3 /uL, lactic acid 13.2 mmol/L, and creatinine of 2.5 mg/dL.Veno-venous extracorporeal membrane oxygenation (ECMO) was initiated for worsening primary allograft dysfunction.The patient was treated with dantrolene for possible malignant hypothermia, bivalirudin for possible heparin induced thrombocytopenia (HIT), antibiotics were changed for possible drug fever, and continuous renal replacement therapy was initiated for metabolic acidosis.On post-op day 2, the patient developed mottling of extremities (images attached).Vascular studies suggested severe digital level occlusive or vasospastic disease.Plasmapheresis was initiated for possible thrombotic thrombocytopenic purpura (TTP).Shock resolved by post-op day 3 and thrombocytopenia by day 6.HIT testing was negative and ADAMSTS13 (for TTP) resulted indeterminate.The patient was de-cannulated from ECMO and underwent tracheostomy on post-op day 10 with eventual liberation from mechanical ventilation and renal function recovery.Unfortunately, he required bilateral below-the-knee and thumb amputations for dry gangrene.No other recipients from the same donor reported similar reactions.Discussion: Basiliximab is increasingly used in solid organ transplantation.It is generally well tolerated; however, serious side effects have been rarely reported.We suspect that our patient's presentation was most likely secondary to profound basiliximab related cytokine release.This idiosyncratic reaction is likely extremely rare, but important to recognize.