SESSION TITLE: Diffuse Lung Disease Student/Resident Case Report Posters
SESSION TYPE: Student/Resident Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Diffuse Alveolar Hemorrhage (DAH) is a rare but potentially catastrophic finding in patients with Systemic Lupus Erythematosus (SLE). Here, we present a case of DAH found in the setting of active SLE as well as biopsy and serology proven microscopic polyangiitis (MPA).
CASE PRESENTATION: A 48 year old female presented to the emergency room with a 2 week history of cough, fever, and malaise. The patient was found to be hypoxic on admission and was promptly intubated and admitted to the intensive care unit. The patient underwent bronchoscopy that showed diffuse alveolar hemorrhage (DAH) and an extensive rheumatologic panel was ordered, which showed positive antinuclear antibody, positive anti-Smith antibody, positive p-ANCA, and positive anti-myeloperoxidase (MPO) antibody. Pulse dose steroids, plasmapheresis, and hemodialysis were initiated and the patient rapidly improved. She was extubated, transferred to a general medicine floor, and a renal biopsy was obtained that was consistent with a mixed SLE and MPA picture. The patient was started on cyclophosphamide which was transitioned to mycophenolate mofetil per the Euro-Lupus protocol, and eventually was discharged to inpatient rehabilitation without an oxygen requirement and at her baseline renal function.
DISCUSSION: p-ANCA positivity has previously been noted in SLE, with up to 10% of patients with SLE testing positive for p-ANCA. However, unlike the case presented, these patients typically do not exhibit signs of disease consistent with vasculitis. In this case, the patient’s mixed serological picture lead us to treat the patient with steroids and cyclophosphamide per the Euro-Lupus protocol but also initiate plasmapheresis, as is commonly done in patients with DAH secondary to vasculitis. A subsequent renal biopsy showed diffuse membranous glomerulonephritis which was noted to be lupus-like in etiology. However, crescentic glomerulonephritis was also noted; due to the absence of endocapillary proliferation and subendothelial deposits, it was believed that this was secondary to active vasculitis - thus confirming our suspicion of a mixed MPA/SLE picture.
CONCLUSIONS: The possibility of concomitant active lupus as well as antibody associated vasculitis such as MPA should be considered in patients with SLE and p-ANCA positivity. Management approaches that are geared towards addressing a possible mixed lupus-vasculitis picture are useful until renal or lung biopsy can be performed.
Reference #1: Isono M, Araki H, Haitani T, et al. Diffuse alveolar hemorrhage in lupus nephritis complicated by microscopic polyangiitis. Clin Exp Nephrol 2011;15(2):294-298.
Reference #2: Hervier B, Hamidou M, Haroche J, Durant C, Mathian A, Amoura Z. Systemic lupus erythematosus associated with ANCA-associated vasculitis: an overlapping syndrome? Rheumatol Int 2012;32(10):3285-3290.
Reference #3: Schnabel A, Csernok E, Isenberg DA, Mrowka C, Gross WL. Antineutrophil cytoplasmic antibodies in systemic lupus erythematosus. Arthritis Rheum 1995;38(5):633-637.
DISCLOSURE: The following authors have nothing to disclose: Ali Syed, Rashmika Potdar, Kshitij Thakur
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