Philipp Eller1, Holger Flick2, Gernot Schilcher3, Florentine Moazedi-Fürst4, Kathrin Eller5, Emina Talakic6, Josef Hermann4, Yannick Allanore7, Horst Olschewski2. 1. Intensive Care Unit, Department of Internal Medicine, Medical University Graz, Auenbruggerplatz 15, 8036, Graz, Austria. philipp.eller@medunigraz.at. 2. Division of Pulmonology, Department of Internal Medicine, Medical University of Graz, Graz, Austria. 3. Intensive Care Unit, Department of Internal Medicine, Medical University Graz, Auenbruggerplatz 15, 8036, Graz, Austria. 4. Division of Rheumatology, Department of Internal Medicine, Medical University of Graz, Graz, Austria. 5. Division of Nephrology, Department of Internal Medicine, Medical University of Graz, Graz, Austria. 6. Department of Radiology, Medical University of Graz, Graz, Austria. 7. Université Rheumatology A Department, Cochin Hospital, Paris Descartes University, Paris, France.
Abstract
BACKGROUND: There is only limited clinical data on the benefit of intense immunosuppression in patients with severe interstitial pneumonia associated with autoimmune features or new-onset connective tissue disease. CASE PRESENTATION: We here report a series of three consecutive patients suffering from severe interstitial lung disease necessitating endotracheal intubation and mechanical ventilation. The first two patients fulfilled many diagnostic criteria for new-onset antisynthetase syndrome, the third patient for systemic lupus erythematosus. We decided to implement aggressive immunosuppressive strategies in these critically-ill patients including therapeutic plasma exchange, immunoadsorption, cyclophosphamide and rituximab. All three patients improved from respiratory failure, were successfully weaned from the respirator, and eventually dismissed from hospital with ongoing immunosuppressive therapy. CONCLUSION: Patients suffering from severe connective tissue disease-associated interstitial lung disease and respiratory failure may benefit from an aggressive immunosuppressive regimen and extracorporeal blood purification with rapid reduction of circulating autoantibodies. The impressive clinical responses in this small case series warrant a controlled clinical trial.
BACKGROUND: There is only limited clinical data on the benefit of intense immunosuppression in patients with severe interstitial pneumonia associated with autoimmune features or new-onset connective tissue disease. CASE PRESENTATION: We here report a series of three consecutive patients suffering from severe interstitial lung disease necessitating endotracheal intubation and mechanical ventilation. The first two patients fulfilled many diagnostic criteria for new-onset antisynthetase syndrome, the third patient for systemic lupus erythematosus. We decided to implement aggressive immunosuppressive strategies in these critically-illpatients including therapeutic plasma exchange, immunoadsorption, cyclophosphamide and rituximab. All three patients improved from respiratory failure, were successfully weaned from the respirator, and eventually dismissed from hospital with ongoing immunosuppressive therapy. CONCLUSION:Patients suffering from severe connective tissue disease-associated interstitial lung disease and respiratory failure may benefit from an aggressive immunosuppressive regimen and extracorporeal blood purification with rapid reduction of circulating autoantibodies. The impressive clinical responses in this small case series warrant a controlled clinical trial.
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