DIGITAL ISCHEMIA: IS EPOPROSTENOL EFFECTIVE FOR CONNECTIVE TISSUE DISEASE-ASSOCIATED INTERSTITIAL LUNG DISEASE?

Abstract

SESSION TITLE: Medical Student/Resident Diffuse Lung Disease SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Epoprostenol (EPO), a prostaglandin analog, has been used for connective tissue disease-associated pulmonary arterial hypertension (PAH) with significant improvement in clinical outcomes, however, the efficacy of prostaglandin analogs in treating digital ulcers associated with dermatomyositis and ILD has conflicting results. We present a patient with ischemic digits and cutaneous ulcerations associated with connective tissue disease and interstitial lung disease (CTD-ILD) that was successfully treated with EPO. CASE PRESENTATION: A 52 years old woman with a history of MDA-5 amyopathic dermatomyositis and CTD-ILD presented with worsening cutaneous skin ulcerations and severe pain in bilateral hands along with findings concerning for digital ischemia [Figure 1-3]. Her previous workup included a chest computed tomography revealing bilateral multiple patchy ground-glass opacities along with interstitial thickening and pulmonary function tests with restrictive lung physiology. During this hospital stay, she continued to develop worsening skin ulcerations (negative arterial dopplers) despite treatment with intravenous immunoglobulins, steroids, mycophenolate, sildenafil, topical nitroglycerin, and botulinum toxin injections. She was therefore, transferred to the intensive care unit for continuous EPO infusion as a last measure to prevent digital necrosis. She tolerated this treatment well with improvement in her pain and prevented digital necrosis. DISCUSSION: In this case, EPO was used to treat severe digital ischemia and pain as a last resort to avoid digital amputation. Prostacyclin analogs used commonly in the USA are EPO and treprostinil. EPO in addition to being a potent vasodilator also has anti-platelet effects, inhibits smooth muscle proliferation, and promotes remodeling response (1). Prostacyclin analogs have been shown to be beneficial for critical leg ischemia, with improvement in digital blood flow and preventing gangrene (1). These medications have an effect on both macro and microcirculation by inducing vasodilation, increasing intracellular cyclic AMP, and by preventing smooth muscle cell proliferation (2). There is limited objective data behind increased blood flow in the digits that correlate with wound healing, however, prostacyclin analogs can help with pain relief due to underlying systemic sclerosis. Currently, very limited data are available for the use of prostacyclin analogs in dermatomyositis associated cutaneous skin ulcerations and digital ischemia. CONCLUSIONS: EPO can potentially be used to prevent the worsening of digital ischemia and pain and associated complications in refractory cases of small vessel vasculitis secondary to dermatomyositis and other connective tissue diseases. Further research and clinical trials are needed to analyze the use of EPO and other prostacyclin analogs for the relief of vascular complications in CTD-ILD. Reference #1: Engel G, Rockson SG. Treprostinil for the treatment of severe digital necrosis in systemic sclerosis. Vasc Med. 2005;10(1):29-32. doi:10.1191/1358863x05vm579cr Reference #2: Rhee RL, Gabler NB, Praestgaard A, Merkel PA, Kawut SM. Adverse Events in Connective Tissue Disease-Associated Pulmonary Arterial Hypertension. Arthritis Rheumatol. 2015;67(9):2457-2465. doi:10.1002/art.39220Kawut, MD, MS Reference #3: Shah AA, Schiopu E, Chatterjee S, et al. The Recurrence of Digital Ulcers in Patients with Systemic Sclerosis after Discontinuation of Oral Treprostinil. J Rheumatol. 2016;43(9):1665-1671. doi:10.3899/jrheum.151437 DISCLOSURES: Advisory Committee Member relationship with GSK Please note: $5001 - $20000 Added 05/22/2020 by Antonio Anzueto, source=Web Response, value=Consulting fee Advisory Committee Member relationship with BI Please note: $1001 - $5000 Added 05/22/2020 by Antonio Anzueto, source=Web Response, value=Consulting fee Advisory Committee Member relationship with Mylan Please note: $5001 - $20000 Added 05/22/2020 by Antonio Anzueto, source=Web Response, value=Consulting fee Advisory Committee Member relationship with AstraZeneca Please note: $1001 - $5000 Added 05/22/2020 by Antonio Anzueto, source=Web Response, value=Consulting fee No relevant relationships by Moeezullah Beg, source=Web Response No relevant relationships by Mahnoor Mir, source=Web Response