Discussion

6] We report the first case of ECMO in the setting of DAH secondary to SLE disease flare-up in Korea. DAH caused by SLE is scarce and life-threatening complication and sudden-onset dyspnea is the most consistent symptom. [ 7 ] The incidence of hemoptysis varies between 25 and 100%, and the appearance of new-onset pulmonary infiltrates in both lungs and drops in hemoglobin may indicate DAH when hemoptysis is absent.[ 7 ] Death results from respiratory failure due to massive DAH in most patients despite treatment with pulsed methylprednisolone or other immunosuppressants. However, immediate institution of ECMO could increase oxygenation and it makes it possible for the patient to withstand flare-ups of SLE as a bridge therapy, as seen in our case.[ 5 6 ] In recent years, there are reports of ECMO as a useful salvage therapy for refractory respiratory failure in patients with DAH caused by SLE.[ 5

2 /FiO 2 of < 100 mmHg despite optimal ventilator settings or an alveolar arterial gradient of more than 600 mmHg in the absence of cardiogenic pulmonary edema or hypercapnia with a pH of less than 7.20.[ ECMO is generally used as rescue therapy for cases where conventional ventilation and hemodynamic support measures have failed to improve the patient’s clinical condition. Formal indications for ECMO are severe hypoxemia with a PaO/FiOof < 100 mmHg despite optimal ventilator settings or an alveolar arterial gradient of more than 600 mmHg in the absence of cardiogenic pulmonary edema or hypercapnia with a pH of less than 7.20.[ 8

ECMO is contraindicated in cases of irreversible respiratory or cardiac disease. The case we describe involved acute respiratory failure that did not respond to conventional ventilatory support. Given the patient’s age and her potentially reversible underlying conditions (severe flare-up of SLE and systemic infection), she was a candidate for veno-venous ECMO. Complications of ECMO include thromboembolism, local infection and, less commonly, technical problems related to cannulation.[ 8 ] Complications such as hemorrhage often result from the need for systemic anticoagulation with heparin in order to prevent thrombosis. For this reason, ECMO is often relatively contraindicated in patients at high risk of bleeding.

Patients with DAH also are particularly vulnerable to increased bleeding risk. Despite the increased risk of ECMO circuit thrombosis, heparin for anticoagulation therapy should be withheld initially due to hemorrhage risk until hemostasis is achieved. Advanced ECMO circuit and oxygenation membrane technology can be used for short periods without anticoagulation.[ 9 ] Once hemostasis is achieved, systemic anticoagulation therapy with heparin should be started to reduce the risk of thrombosis in the ECMO circuit and oxygenation membrane. In this case of SLE-induced DAH,[ 6 ] systemic anticoagulation was initiated upon the installation of ECMO. There were no hemorrhagic complications related to ECMO.

In one case series of pediatric patients with DAH secondary to multiple baseline pathologies, patients were anticoagulated, although at slightly lower clotting time levels, and they did not have hemorrhagic complications. [ 10 ] Pediatric patients with severe respiratory failure due to DAH can be successfully treated with ECMO with a continuous heparin drip at the targeted ACT range of 160-180 seconds.[ 10 ] Using anticoagulation with the lower standard clotting time parameters reported in this case should be considered in adult patients with DAH.

ECMO is good choice for rescue therapy in the setting of severe respiratory failure in alveolar hemorrhage caused by SLE. When patients with refractory hypoxemia are not responsive to conventional ventilation strategies, ECMO can improve oxygenation and may improve the clinical outcomes of pulmonary hemorrhage. The use of ECMO may reduce further lung injury related to high airway pressure and oxygen toxicity while allowing more time for aggressive anti-inflammatory therapy. [ 9 ] When inflammation regulation against SLE is started immediately, these interventions should be successful. The treatment strategy for DAH caused by SLE in adults is combination therapy using high-dose glucocorticoids and cytotoxic drug such as IV cyclophosphamide[ 11 ] and plasmapheresis.[ 12 ] As in our case, simultaneous use of ECMO and plasmapheresis has a advantage for sharing vascular access and has been applied for fatal alveolar hemorrhage secondary to other connective tissue diseases.[ 13

Because of the rarity of this fatal condition of DAH caused by SLE, there are no standard treatment guidelines. In addition to, an agreement for the optimal target level of systemic anticoagulation for patients with severe alveolar hemorrhage should be determined. Multi-institutional trials and data collection are needed to establish whether ECMO as a rescue therapy confers better survival outcomes in patients with DAH caused by SLE; in addition, development of safe strategies for anticoagulation in these patients is warranted.