SESSION TITLE: Tuesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Shrinking lung syndrome (SLS) is a rare pulmonary complication of systemic lupus erythematosus (SLE). It is characterized by progressive dyspnea, elevation of the diaphragm, pleuritic chest pain, low lung volumes on imaging and Pulmonary Function Tests (PFT) findings of a restrictive pattern.(1) SLS prevalence is estimated to be 0.5% to 1.1% among patients with SLE.(2) Its pathophysiology remains largely unclear; however, micro atelectatic changes, diaphragm fibrosis, and phrenic nerve palsy have been sugested.(3) There is no standardized treatment for SLS, though most of these patients are treated with medium to high dose steroids.(1) CASE PRESENTATION: Mrs. PB is a pleasant 51- year old Hispanic female with a past medical history significant for hypothyroidism, obesity, diabetes mellitus and a diagnosis of SLE with multiple flares since 2003; she consistently is lost to follow up and follows up only during her SLE flares. She presents to clinic for a 2 month history of productive cough, shortness of breath, and worsening SLE skin manifestations. History reveals that since her onset of symptoms she has undergone three courses of antibiotic therapy with no improvement of her symptoms. Chest CT and Pulmonary Function testing were ordered. At follow up CT reports found elevation of the right diaphragm with adjacent compressive atelectasis and linear scarring bilateral posterior lung bases. Review of CT imaging did not correlate with the patient’s dyspnea. PFT results demonstrated a total lung capacity (TLC) of 38% percent of predicted (PP), forced expiratory volume in 1 second (FEV1) 26% PP, and a diffusion capacity of the lungs for carbon monoxide (DLCO) of 36% PP, consistent with a restrictive pattern and severe diffusion defect. Results were discussed and consensus was made that findings and history are consistent with SLS. The patient was started on a course of steroids followed by a maintenance therapy of hydroxychloroquine and azathioprine. DISCUSSION: This case illustrates a case of shrinking lung syndrome and highlights the fact that SLS can complicate SLE at any point during the course of the disease. Despite the rare occurrence of SLS in patients with SLE symptoms such as pleuritic chest pain and/or dyspnea in these patients should alert the physicians about this diagnosis. Unfortunately, there are no standard treatment guidelines and medium to high dose steroids, immunosuppressants, theophylline and beta agonists have been studied. Long term prognosis is generally good. CONCLUSIONS: Diagnostic delay due to under recognition of this condition can often lead to delays in appropriate medical therapy and care. Unfortunately, there are no standard treatment guidelines and medium to high dose steroids, immunosuppressants, theophylline and beta agonists have been studied. Long term prognosis is generally good. Reference #1: Borrell, H., Narváez, J., Alegre, J. J., Castellví, I., Mitjavila, F., Aparicio, M., Armengol, E., Molina-Molina, M., … Nolla, J. M. (2016). Shrinking lung syndrome in systemic lupus erythematosus: A case series and review of the literature. Medicine, 95(33), e4626. Reference #2: Bertoli AM, Vila LM, Apte M, et al. LUMINA Study Group. Systemic lupus erythematosus in a multiethnic US Cohort LUMINA XLVIII: factors predictive of pulmonary damage. Lupus 2007; 16:410–417. Reference #3: Selmi, C. Clinic Rev Allerg Immunol (2017) 53: 126. https://doi.org/10.1007/s12016-017-8615-6 DISCLOSURES: No relevant relationships by Mohammed Ali, source=Web Response No relevant relationships by Humayun Anjum, source=Web Response No relevant relationships by Manuel Ramos Ramirez, source=Web Response Speaker/Speaker's Bureau relationship with Astra Zaneca, Sunovian, Paratek Please note: $1001 - $5000 Added 01/13/2019 by Salim Surani, source=Web Response, value=Consulting fee