POLYMYOSITIS-ASSOCIATED INTERSTITIAL LUNG DISEASE RESPONSIVE TO IV IMMUNOGLOBULIN THERAPY

SESSION TITLE: Critical Diffuse Lung Disease Cases 2 SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Polymyositis (PM) and dermatomyositis (DM) are inflammatory syndromes that have been shown to be associated with interstitial lung disease in up to 64% of cases. Current management strategies for PM/DM-ILD are mostly derived from treatment of PM/DM and rely on the immunomodulatory effects of steroids and steroid sparing medications. Literature supports the use of intravenous immunoglobulins for the management of PM/DM-ILD. However, this stems from isolated case reports. CASE PRESENTATION: Our patient is a 43-year-old female, previously healthy, who was referred to our ILD clinic for evaluation of possible diffuse interstitial lung disease. Her symptoms began May of 2019 with dyspnea, cough, and muscle aches. Her initial workup was significant for reticular opacities in the mid to lower lung fields on x-ray, moderate restriction, and severe diffusion impairment on pulmonary function tests. High resolution CT scan (HRCT) of the lungs showed a radiological pattern suggestive of nonspecific interstitial pneumonitis (NSIP) with mid to lower lung predominant ground glass opacities, mild subpleural reticulation, traction bronchiectasis with no evidence of honeycombing or air trapping. Further workup showed a positive antinuclear antibody (ANA) in a speckled pattern with a titer of 1:320. Her other rheumatological workup was initially largely unremarkable and she denied significant drug/toxin exposure. She was treated with steroids with initial improvement, however had significant clinical deterioration requiring hospitalization and oxygen supplementation and was eventually started on Mycophenolate Mofetil in Aug 2020. Her pulmonary disease remained stable until she was admitted again in Jan 2021 with new hypoxemic respiratory failure requiring 6 L supplemental oxygen. During that admission, she was diagnosed with polymyositis and referred for a lung transplant evaluation, she also started on IVIG for the treatment of active myositis to wean her off steroids prior to transplant listing. She was discharged home and underwent a full transplant workup. She required readmission for worsening hypoxemia now requiring 12 L supplemental oxygen in May 2021, HRCT showed worsening ground glass opacities. She was again treated with IVIG and a steroid course and had near complete resolution of her hypoxia with a 2 L oxygen requirement only on ambulation. She continues to follow up with our interstitial lung disease team and the pulmonary transplant team. Her course has been complicated by multiple admissions with bacterial pneumonia as well as COVID-19 infection. DISCUSSION: Treatment of PM/DM associated ILD relies heavily on the use of steroids as well as steroids sparing agents such as Mycophenolate or Rituximab. IVIG has been implicated in improving underlying lung disease with data derived from case reports. CONCLUSIONS: Our case highlights the importance of further advanced research in this field. Reference #1: Peshbahar, S., & Bendstrup, E. (2020). Remarkable benefits of intravenous immunoglobulin (IVIG) in a patient with polymyositis-associated acute interstitial lung disease. European Clinical Respiratory Journal, 7(1), 1840706. Reference #2: Fathi, M. (2006). Interstitial lung disease in polymyositis and dermatomyositis. Karolinska Institutet (Sweden). DISCLOSURES: No relevant relationships by Hafsa Abdulla No relevant relationships by ALAA ABU SAYF

mSOFA Scores Associated with the Development of Pneumothorax, Pneumomediastinum and Subcutaneous Emphysema in Critically Ill Covid-19 Patient

Introduction: COVID-19 is a well-known cause of severe ARDS (acute respiratory distress syndrome), however recent data suggests that COVID-19 could represent a unique form of lung injury that places patients at increased risk of various uncommon complications such as pneumothorax, pneumomediastinum and subcutaneous emphysema. Studies so far have reported an increased incidence of barotrauma in intubated COVID-19 patients with unclear predictors. Our study aims to identify the different variables associated with development of pneumothorax, pneumomediastinum and subcutaneous emphysema in critically ill COVID-19 patients. Methods: We examined patients admitted to the intensive care unit from March 2020 to Feb 2021 at a large tertiary care center in Detroit, Michigan. We identified a total of 25 patients with COVID-19 ARDS requiring mechanical ventilation who developed pneumothorax, 12 who developed pneumomediastinum and 7 with subcutaneous emphysema. We compared those to 66 patients admitted with COVID-19 ARDS also requiring mechanical ventilation who did not develop any of these complications. The mean age of patients in our subject group was 61.81 years compared to a mean of 69.05 years in the control group. Male patients accounted for 58.33% of the subject group and 60.61% in the control group. Results: we detected a statistically significant difference in the modified Sequential Organ Failure Assessment Score (mSOFA) between the patients who developed these complications compared to those who did not (p<0.0001), with score being surprisingly lower in the group who developed the complication as opposed to those who did not (median mSOFA in subjects 3.5, n=32 vs median mSOFA in controls 11, n=66). Analysis of the subgroups of the mSOFA score revealed no statistically significant difference in the PF ratio (p=0.1995), platelet counts (p=0.065) and total bilirubin (p=0.4403). However, MAP was noted to be significantly lower in the control group than in the subject group accounting for a higher mSOFA score (p=0.0031). Similarly, creatinine was noted to be higher in the control group (p<0.0001) compared to the subject group. Discussion: In viewing our baseline patient characteristics we found a statistically significant difference (p<0.0001) in the rate of baseline chronic kidney disease between our subjects and control patients, with control patients having 100% baseline CKD and subjects having 19.4% baseline CKD. This could account for the higher mSOFA scores in controls. Conclusion: mSOFA did not predict the development of pneumothorax, pneumomediastinum or subcutaneous emphysema in patients admitted with COVID-19 ARDS requiring mechanical ventilation.

Antifibrotic Risk of Bleeding with Anticoagulation -A Case Report

Introduction: Antifibrotic drugs, including nintedanib and pirfenidone, are approved for the treatment of idiopathic pulmonary fibrosis (IPF). Pirfenidone blocks the synthesis of TGF-beta and does appear to increase the risk of CV or bleeding events. Nintedanib is a Vascular Endothelial Growth Factor Receptors (VEGFR) inhibitor and may increase the risk of bleeding. Bleeding events were reported in 10% of patients in clinical trials (despite excluding patients at risk of bleeding including those on con-current anticoagulation (AC) or antiplatelet (AP) therapy. Consequently, nintedanib is relatively contraindicated for patients with IPF on anticoagulation or antiplatelet therapy. However, results from real-life data demonstrate that 17.8% of our patients with IPF are on anticoagulation or antiplatelet therapy. The overall incident of bleeding events in those patients taking either or with nintedanib is similar to that reported for Nintedanib alone. We describe two patients with antifibrotic and anticoagulation therapy to highlight how to manage these patients. Case 1. A 67-year-old female with history of acute pulmonary embolism secondary to COVID-19 on anticoagulation therapy presents for workup of acute respiratory distress syndrome or suspected exacerbation of underlying IPF-usual interstitial pneumonia. Patient treated as IPF with pirfenidione. Case 2. A 69-year-old man with a history of atrial fibrillation on anticoagulation therapy presents for follow-up of progressive fibrosing interstitial lung disease secondary to hypersensitivity pneumonitis. Nintedanib initiated for progressive fibrosing lung disease. Discussion: Concomitant use of anticoagulation and/or antiplatelet therapy with antifibrotics doesn't increase bleeding risk. Conclusion. Anticoagulation and/or antiplatelet therapy should not be a reason to withhold antifibrotic therapy in patients with IPF.

COVID-19-RELATED CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME: CASE REPORT

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Since the COVID-19 pandemic, numerous reports have described COVID-19 related coagulopathy, including antiphospholipid syndrome (APL). A relationship between COVID-19 and catastrophic antiphospholipid syndrome (CAPS) has been suggested, but to our knowledge, there have been no case reports strongly describing this association. CASE PRESENTATION: A 57-year-old woman with a past medical history significant for asthma presented to our hospital with severe asthma exacerbation. The patient was intubated and admitted to the intensive care unit (ICU). During her ICU admission, she was found to have COVID-19 pneumonia. On day 16 of hospitalization, she was diagnosed with ST-elevation myocardial infarction. The patient also had encephalopathy and underwent brain magnetic resonance imaging on day 24 which revealed innumerable acute embolic infarcts. On day 27, the patient was diagnosed with an acute deep vein thrombosis in her left lower extremity, but she was not started on anticoagulation due to concern of a lower gastrointestinal bleed. Laboratory workup revealed elevated anti-cardiolipin antibodies (aCL), both IgG and IgM, and elevated lupus anticoagulant. She was diagnosed with CAPS and received 4 sessions of plasmapheresis and was also started on methylprednisolone and intravenous heparin. Our patient had no improvement in mental status with initiation of therapy. On day 39, she underwent repeat head computed tomography scans, which showed right frontal intracerebral hemorrhage. Patient was provided comfort care and died on day 41 of hospitalization. DISCUSSION: CAPS is a severe form of APL. IgG aCL is seen in 81% of patients;IgM aCL was identified in 49% and lupus anticoagulant in 83%. [1, 2]. Triggering factors were identified in 65% of the CAPS flares. The most common triggers were infections, (49% of episodes) and the most common site for infections was pulmonary (33%). Infections included viruses, bacteria and parasites. [1, 2]. Other differentials were considered for our patient, such as thrombotic thrombocytopenic purpura, hemolytic uremic syndrome and disseminated intravascular coagulation, but were less likely given the negative hemolysis workup. Although this patient had no known history of previous positive APL antibodies or histopathological evidence of small vessel occlusion, she was diagnosed with CAPS based on evidence of multiple thrombotic events in 3 different organs within <2 weeks, along with positive aPLs. CONCLUSIONS: This case represents a potential link between COVID-19 and CAPS. We hope to raise awareness of the need for continued workup for hypercoagulable states in patients with COVID-19 and suggest that CAPS should be considered as one possible cause. We also hope to raise concern for the possibility of CAPS in critically ill patients with COVID-19 who show evidence of repeated incidence of thromboses in multiple organs, as this is a potentially treatable condition. REFERENCE #1: Cervera R, Bucciarelli S, Plasín MA, et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the "CAPS Registry". J Autoimmun. 2009;32(3-4):240-5. REFERENCE #2: Rodríguez-Pintó I, Moitinho M, et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of 500 patients from the International CAPS Registry. Autoimmun Rev. 2016;15(12):1120-4. DISCLOSURES: No relevant relationships by ALAA ABU SAYF, source=Web Response No relevant relationships by Yahia Al Turk, source=Web Response No relevant relationships by Julia Bachler, source=Web Response No relevant relationships by Sufiya Hussain, source=Web Response No relevant relationships by Vikash Patel, source=Web Response