A repeat TEE demonstrated a vegetation on the mitral valve and thickened mitral valve leaflets, with mild regurgitation at the aortic, mitral and tricuspid valves. A skin biopsy of a periungual lesion demonstrated epidermal necrosis with acute inflammation and thrombi present, but vasculitis was not identified.
A magnetic resonance imaging (MRI) scan of the brain did not reveal any evidence of central nervous system vasculitis. A renal biopsy demonstrated necrotizing glomerulonephritis with crescents and without immune complex deposits, which was consistent with an ANCA-mediated glomerulonephritis (see Figure 2). A repeat bronchoscopy with bronchoalveolar lavage revealed active bleeding from the right upper lobe that was concerning for diffuse alveolar hemorrhage.
We determined the patient’s presentation was most consistent with GPA, and treatment was initiated with 375 mg/m2 of rituximab once per week for four weeks. In addition, plasma exchange was resumed, of which three cycles were completed (a total of five cycles).
Despite receiving extensive immunosuppressive treatments, the patient’s renal function continued to worsen, necessitating placement on regular hemodialysis. Further blood cultures and a lower respiratory culture did not grow any organism. Infectious disease tests for Bartonella henselae IgG and IgM, Coxiella burnetii IgG phases I and II, Brucella antibodies, HIV antibodies and DNA, hepatitis C virus antibodies and quantiferon-gold tuberculosis (TB) were negative.
Twenty-nine days after her initial presentation, the patient was extubated, but maintained a persistent oxygen requirement. The patient’s steroid dose was slowly tapered over her hospital course, eventually switching from methylprednisolone to prednisone, and down to a prednisone dose of 40 mg/day. During her recovery after extubation, it became evident that she was severely deconditioned and unable to leave her bed. She also had difficulty swallowing and suffered from multiple aspiration events, requiring placement of a gastrostomy tube for feeding.
Forty-nine days after her initial presentation, the patient developed respiratory distress thought to be secondary to aspiration, but declined any escalation in care, and she expired shortly thereafter. The patient’s family declined an autopsy.
Discussion
We present the case of a patient with GPA, whose clinical and laboratory findings were consistent with both an infective endocarditis and a small vessel vasculitis. The positive C-ANCA and PR3 antibody found during this patient’s workup did not provide any utility in discerning between these two diseases. The similarity in presentation between patients with ANCA-positive infective endocarditis and ANCA-associated vasculitis with endocardial involvement has been documented in the literature, and these cases are most often associated with a positive C-ANCA or PR3 antibody.7
