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This is a case of a 24 year old previously healthy African American female who presented with acute hypoxic respiratory failure secondary to a flair up of connective tissue disease related interstitial lung disease CTD-ILD secondary to anti-synthetase syndrome. CASE PRESENTATION: A 24 year old healthy female, with a strong family history of autoimmune diseases and no exposure history, presented to the Emergency Department with a 1 month history of progressive dyspnea and polyarthralgias. She was found to be febrile and in acute hypoxic respiratory failure requiring high flow oxygen. Physical exam revealed bibasilar inspiratory dry crackles, hyperpigmentation of the interphalangeal joints. Plan films demonstrated bilateral lower lobe opacities and she was admitted to the intensive care unit and treated empirically for pneumonia with minimal clinical improvement. Computed tomography demonstrated bilateral ground glass opacities with a positive straight edge sign. Due to concern for underlying autoimmune disease, empiric steroids were administered with improvement in symptoms. Subsequent autoimmune workup was notable for elevated creatinine phosphokinase and aldolase. Pulmonary function demonstrated restriction with severe diffusion impairment. Muscle biopsy demonstrated findings consistent with inflammatory myopathy. She continued to improve on steroids and was discharged to follow up as an outpatient for evaluation of steroid sparing therapy.DISCUSSION: Antisynthetase syndrome is a constellation of clinical findings frequently associated with polymyositis and dermatomyositis and occurs in the setting of other autoimmune diseases with interstitial lung disease, as apparent in our care. A recent paper published in 2018 suggested three radiological patterns of Usual Interstitial Pneumonia which might suggest an underlying connective tissue disease versus idiopathic pulmonary fibrosis. These signs include anterior upper lobe, exuberant honeycombing, and the straight edge sign with the latter manifesting in 25% of patients with CTD vs. 6% in ILD with a 4.22 positive likelihood ratio and a specificity of 94%. The straight-edge sign is a CT finding characterized by a fairly straight interface between fibrosis and normal lung tissue orthogonal to the lateral surface of the lung. Recognizing these signs clues the diagnosis in the undifferentiated patient towards earlier treatment of hypoxemia, further appropriate autoimmune workup, reducing antimicrobial exposure in the setting of positive SIRS criteria, and allows earlier initiation of immunosuppressants.CONCLUSIONS: This case is supportive of detecting radiographic signs of connective tissue disease, as manifested with straight-edge sign to guide earlier diagnosis of CTD-ILD and prompt medical treatment in the acute inpatient and chronic outpatient settings.

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Introduction: There have been major advances in the diagnosis, staging, risk-stratification, and management of multiple myeloma (MM). Novel triplet regimens containing drugs such as Carfilzomib, Elotuzumab, Daratumumab, or Ixazomib have improved the median progression-free survival rates at first relapse to approximately two years. This retrospective study analyzes the responses and survivals of treatment regimens used at first relapse in the real world setting and attempts to evaluate any differences in outcomes based on race. Methods: We reviewed the charts of all patients with relapsed/refractory or progressive multiple myeloma who were treated for first relapse or progression at Henry Ford Hospital in Michigan. Patients who received a combination regimen with any of the following medications: Bortezomib, Lenalidomide, Pomalidomide, Ixazomib, Carfilzomib, Daratumumab, and Elotuzumab between 1/1/2015 and 4/24/2018 were included. Baseline characteristics data using the electronic medical record was retrieved for age, sex, race, performance status, cytogenetics, previous treatment, progression free survival and compared based on race. Patients were excluded if they did not receive treatment for relapse, expired prior to initiating relapse treatment or were lost to follow up. Results: A total of 300 patients were treated with systemic therapy for multiple myeloma, 140 of whom had relapsed/refractory or progressive disease. We excluded 28 patients who did not meet inclusion criteria. The remaining 112 patients were treated for first relapse or progression of myeloma and were included in our study cohort. Patients characteristics are shown in table 1. Out of those 112 patients, 26 (23.2% of the cohort) received a novel triplet regimen for their first relapse, 21 (18.8%) received an older triplet regimen, 14 (12.5%) received a novel doublet regimen, 44 (39.3%) received an older doublet regimen and the remainder 7 patients (6.3%) received other regimens as detailed in table 2. Of the 26 patients who received a novel triplet regimen, 11 were African American and 15 were White. Ten patients had disease progression or death at the time of data analysis and qualified for progression free survival (PFS) calculation. The remaining 16 patients were still receiving their treatment regimen at the time of analysis. Progression free survivals comparing novel triplet regimens based on patients race are shown in table 2. Of note, median PFS for Carfilzomib, lenalidomide, dexamethasone (KRd) regimen was 26.3 months in the ASPIRE trial and 20.6 months for Ixazomib, lenalidomide, dexamethasone regimen in the TOURMALINE-MM1 trial. The 12-month rate of PFS for Daratumumab, bortezomib, dexamethasone (DVd) regimen was 60.7% in the CASTOR trial, 83.2% for Daratumumab, lenalidomide, dexamethasone (DRd) regimen in the POLLUX trial and 68% for Elotuzumab, lenalidomide, dexamethasone regimen in the ELOQUENT-2 trial. Of the 21 patients who received an older triplet regimen, 9 were African American, 11 were White and 1 was Hispanic. All of them had disease progression or death and qualified for PFS calculation. Of the 44 patients who received an older doublet regimen, 27 were African American, 15 were White and 2 were Hispanic. Thirty two patients had disease progression or death at the time of data analysis. Of the 14 patients who received a novel doublet regimen, 9 were African American and 5 were White. Eight had disease progression or death at the time of data analysis. These results are shown in table 2. Conclusion: Our retrospective cohort study shows a distribution of the treatment regimens used for the management of relapsed multiple myeloma. Progression free survivals for a novel triple drug regimen for myeloma at first relapse seems to be shorter in the real world setting than published data. Specifically, when compared based on race, African American patients had worse outcome overall with shorter progression free survival period. However, data is limited due to the small number of patients. Stratification based on race or focused trials for certain populations such as African American are needed to manage this challenging disease. Disclosures No relevant conflicts of interest to declare.

Introduction: PC is the third leading cause of cancer-related death in the United States. Cigarette smoking is a known risk factor for PC. Furthermore, it has been evaluated as a possible prognostic marker and has been shown that increased number of pack years is associated with worsened overall survival in PC (Yuan et al). However, it has yet to be shown whether patients undergoing active treatment and are current smokers, are facing a worse prognosis. Our study aims to examine the effect of current smoking on survival outcomes of advanced/metastatic PC patients undergoing active treatment. Methods: This is a retrospective study. Patients diagnosed with advanced/metastatic pancreatic cancer at Henry Ford Health between January 2016 and January of 2021 were identified via chart review. Variables collected included age, gender, smoking status (subclassified as never smokers, former smokers, and active smokers), performance status (documented as Eastern Cooperative Oncology Group (ECOG), number of chemotherapy (CTX) lines, and survival status that is most recently documented by May 1st, 2002. Median survival time were calculated to present the survival time for which the proportion surviving reached 50 percent. Kaplan-Meier estimates were used to generate survival curves for each cohort. The log-rank test was performed to compare the survival distributions among smoking status. Cox model was fit to estimate the association between smoking behavior and overall survival, adjusted for demographics and cancer-related variables. CTX was treated as a time-dependent covariate. All statistical tests were 2-sided with an α (significance) level of 0.05. All data was analyzed using R version 4.1.2. Results: A total of 269 PC patients were included in the analysis. The mean age of the cohort was 66 years and 50.9% were males. The mortality rate (from time of diagnosis to May 1st, 2022 was about 89%. Around 8% of patients underwent surgery, 21% have taken radiation therapy, and only one patient did not undergo CTX. For smoking behavior, 46.1% were never smokers, 34.6% were former smokers, and 19.3% were current smokers. Looking at baseline characteristics, the mean age of active smokers were 4 to 5 years less than never/former smokers and the average ECOG score of non-smokers were 0.28 to 0.32 point less than former/active smokers. Median survival time for patients never smoking was 11.7 months, 8.9 months for former smokers, and 11.4 months for active smokers. The log-rank test did not show sufficient evidence of a difference in survival across patients’ smoking status. Conclusion: Active smoking status does not appear to influence overall survival in advanced/metastatic pancreatic cancer patients undergoing various chemotherapy treatments. This study was limited in its retrospective nature and a relatively small sample. Information regarding level of smoking (light smokers vs heavy smokers) was not readily available. The prognostic value of smoking in PC will need to be assessed prospectively. Citation Format: Aula Ramo, Zeinab Nasser, Mohamad Beidoun, Baha' Mustafa Abbad, Nayef Hikmat Abdel-Razeq, Gazala Khan. Is smoking prognostic for advanced metastatic pancreatic cancer (PC) patients undergoing treatment [abstract]. In: Proceedings of the AACR Special Conference on Pancreatic Cancer; 2022 Sep 13-16; Boston, MA. Philadelphia (PA): AACR; Cancer Res 2022;82(22 Suppl):Abstract nr A049.