IT CAN'T BE LUPUS...UNTIL IT IS: A CASE OF DIFFUSE ALVEOLAR HEMORRHAGE IN SYSTEMIC LUPUS ERYTHEMATOSUS

Abstract

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Diffuse Alveolar Hemorrhage (DAH) is a rare, but well-documented phenomenon in systemic lupus erythematosus (SLE). DAH affects only 4% of patients with SLE but has a 50% mortality. CASE PRESENTATION: A 67 year-old female with SLE complicated by lupus nephritis requiring four renal transplants (last in 2015), but currently in remission on maintenance tacrolimus and mycophenolate mofetil with recurrent episodes of hemoptysis and hypoxic respiratory failure. In October 2018 she was admitted with dry cough, scant hemoptysis and developed rapidly progressive respiratory failure requiring intubation. Workup showed normal immunosuppressive levels, elevated white blood count and inflammatory markers. CT revealed diffuse ground glass opacities. Antinuclear antibody (ANA) and anti-double stranded DNA were negative with normal complement levels. Bronchoscopy showed mononuclear predominance with unrevealing microbiology and cytology and no active bleeding despite sequential BAL. She rapidly improved with antibiotics and steroids. In January 2019 she was admitted with similar symptoms of cough, scant hemoptysis, multilobar infiltrates. Again, serologic and bronchoscopic workup were unrevealing, without classical evidence of DAH leading to a broad differential and needing to determine the etiology for the recurrent episodes of hemoptysis. An open lung biopsy was performed and results returned from Mayo Clinic showing acute alveolar hemorrhage, secondary to SLE. Patient was started on high dose steroids. However, 24 hours later she had frank hemoptysis, this time with bronchoalveolar lavage confirming DAH. Unfortunately, despite steroids, dialysis and plasmapheresis, hospital course was complicated by multi-organ dysfunction and she ultimately expired. DISCUSSION: DAH due to SLE is usually in the setting of a flare with thrombocytopenia, hypocomplementemia as well as elevated lupus antibodies. It is often seen within the first 5 years of diagnosis. Our patient had well-controlled disease on immunosuppressive therapy and had consistently negative antibodies and normal complement levels. Her only known risk factor was recurrent lupus nephritis, which is the most associated extrapulmonary manifestation in patients with SLE and DAH. This patient was given high-dose steroids and plasmapheresis; in reviews of DAH in SLE nearly 100% were given steroids, 55% cyclophosphamide and 31% plasmapheresis. Utilizing recombinant factor VII during the massive hemoptysis was considered, but the data to support it was not well-supported. CONCLUSIONS: This may be the first documented patient with well-controlled Lupus, without evidence of a flare, who developed recurrent episodes of DAH from SLE without classical symptoms highlighting need for broad differentials and exact diagnosis. Given the high mortality associated with DAH, it must be recognized that this can occur and recur despite ongoing immunosuppressive therapy. Reference #1: Kusunoki et al. Severe Progressive Diffuse Alveolar Hemorrhage in a Patient with Systemic Lupus Erythematosus. Case Reports in Critical Care. 2018 Reference #2: Kazzaz NM, Coit P, Lewis EE, McCune WJ, Sawalha AH, Knight JS. Systemic lupus erythematosus complicated by diffuse alveolar haemorrhage: risk factors, therapy and survival. Lupus Sci Med. 2015;2(1):e000117. Published 2015 Sep 23. https://doi.org/10.1136/lupus-2015-000117 Reference #3: Ednalino, Christina & Yip, Julie & Carsons, Steven. (2015). Systematic Review of Diffuse Alveolar Hemorrhage in Systemic Lupus Erythematosus: Focus on Outcome and Therapy. Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases. 21. 305-10. 10.1097/RHU.0000000000000291. DISCLOSURES: No relevant relationships by Achal Dhupa, source=Web Response No relevant relationships by Jamie Felzer, source=Web Response No relevant relationships by Shazia Jamil, source=Web Response No relevant relationships by Lakshmi Koripella, source=Web Response