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Hemoptysis and Respiratory Distress in the Postpartum Period: A Rare Presentation of Microscopic Polyangiitis
Abstract Number: F-62
Abstract Type: Case Report/Case Series
Intro: Microscopic polyangiitis during pregnancy is associated with a high risk of fetal and maternal complications. We present a case of microscopic polyangiitis diagnosed postpartum.
Case: A 26 year old primigravida from Burma presented with an episode of hemoptysis on postpartum day three. Her vaginal delivery at 40+ weeks was complicated by chorioamnionitis, postpartum bleeding and urinary retention requiring multiple catheterizations. Her past medical history included a positive PPD followed by 6 months of isoniazid. During this pregnancy, she had persistent hematuria and proteinuria.
The patient appeared anxious on physical examination. Her pulse rate was 116/min, blood pressure was 147/87 and oxygen saturation was 77-83% on room air. She was transferred to the labor floor for closer monitoring. On 7L O2 by face mask, her O2 saturation increased to 93%. CT angiography was planned to evaluate for pulmonary embolus (PE). She had another episode of hemoptysis of approximately 10ml. Infectious disease and pulmonary/ICU consults were performed. Bedside echocardiogram showed normal biventricular function with mild pulmonary hypertension. The ICU team began heparin treatment for presumed PE – a bolus was given and an infusion started. Minutes later, the patient had significant hemoptysis and respiratory distress. The heparin was stopped and she was taken to the operating room for emergent intubation.
Despite 100% oxygen therapy, she had periods of 02 saturations ranging from 70-80%. Tracheal tube suctioning yielded copious frank blood. Bronchoscopy revealed diffuse airway bleeding without any focal source. CXR showed bilateral consolidations. Transabdominal ultrasound and lower extremity dopplers were negative for retained placenta and deep vein thrombosis, respectively. Her hemoglobin was 7.1 g/dL (down from 12.8 g/dL peripartum). Two units of packed RBCs and one of FFP were transfused and she was transferred to the intensive care unit.
She was later diagnosed with p-ANCA vasculitis, specifically microscopic polyangiitis, manifesting as a pulmo-renal syndrome. She endured a prolonged ICU admission, with two rounds of plasmapheresis and multiple blood transfusions.
Discussion: Microscopic polyangiitis is a rare disease. The onset is usually abrupt, with hemoptysis, as well as other pulmonary symptoms, such as cough, occurring in over 80% of patients (1). Renal involvement, specifically rapidly progressive glomerulonephritis, is associated with poorer outcomes (2). The treatment includes steroids and rituximab. These patients are usually managed by multiple subspecialities: nephrology, pulmonary, and rheumatology.
(1) Wilke L, Prince-Fiocco M, Fiocco GP. Microscopic polyangiitis: a large single-center series. J Clin Rheumatol 2014; 20(4):179-182.
(2) Croft AP, Smith SW, Carr S, et al. Successful outcome of pregnancy in patients with anti-neutrophil cytoplasm antibody-associated small vessel vasculitis. Kidney Int 2014 Oct.