To Clot or to Bleed: Thrombosis as a First Sign of Microscopic Polyangiitis
Mishra K, Naffouj S, and Gorgis S. To Clot or to Bleed: Thrombosis as a First Sign of Microscopic Polyangiitis. J Gen Intern Med 2019; 34(2):S667.
J Gen Intern Med
Learning Objective #1: Recognize ANCA-associated vasculitis in a patient with thrombosis Learning Objective #2: Management of diffuse alveolar hemorrhage in the setting of acute thrombosis CASE: A 59-year-old female with a history of stroke with residual right-sided weakness presented with dyspnea and lower extremity swelling. She was febrile, tachycardic, and required supplemental oxygen (O2). Laboratory studies showed an acute kidney injury (AKI). Lower extremity dopplers revealed bilateral deep vein thrombosis (DVT), and she was started on heparin for DVT and presumed pulmonary embolism (PE). Computed tomography (CT) PE was not performed due to AKI. Chest xray showed bilateral airspace opacities, so antibiotics were started. The patient clinically worsened and developed hemoptysis with increasing O2 requirements. CT chest showed concern for alveolar hemorrhage. Anticoagulation was stopped and the patient was intubated. Bronchoscopy confirmed diffuse alveolar hemorrhage. Rheumatologic workup showed ESR 111, ANA 1: 320 (speckled), and positive MPO (p-ANCA) of 115. Left kidney biopsy revealed focal segmental necrotizing and crescentic glomerulonephritis consistent with ANCA-associated pauci-immune glomerulonephritis, microscopic polyangi-itis (MPA). Plasmapheresis and hemodialysis was started due to worsening kidney function. She clinically improved, was extubated, and eventually weaned off oxygen. Induction with Cyclophosphamide and high-dose steroids was begun. Her AKI resolved, no longer requiring dialysis. After a risk-benefit discussion, warfarin was started, and the patient returned home. She was transitioned to maintenance therapy on Azathioprine and remained clinically stable at her three year follow up. IMPACT/DISCUSSION: We present a case of thrombosis as a first sign of severe microscopic polyangiitis. The most common clinical symptoms of MPA include fatigue, cough, dyspnea, and arthralgias. Although it is a pulmonary-renal syndrome, renal insufficiency is only present in 18% of patients on presentation. In our patient, a DVT (originally thought to be due to immobilization from stroke) was the first sign of extensive MPA. The accompanying respiratory symptoms were presumed to be due to PE, and therefore a CT scan was not initially performed. The etiology of the hyper-coagulable state in such patients is unclear, but circulating antiplasminogen antibodies have been demonstrated in those who develop clots. This case was further complicated by diffuse alveolar hemorrhage, a rare but life-threatening complication of MPA. Immunosuppressive therapy is required in almost all patients with active disease. Conclusion: Patients with ANCA-associated vasculitis have a high risk of thrombosis, but it is rarely the presenting sign. Suspicion in patients with concomitant renal insufficiency and pulmonary symptoms can lead to early diagnosis. Despite being hypercoagulable, anticoagulation may not be the appropriate initial treatment as pulmonary hemorrhage can worsen outcomes.