Case Presentation: 71-year-old man with a history of atrial fibrillation, presented with four-months of fatigue, 50lb weight loss, night sweats and petechiae spreading from legs to abdomen and arms. On admission, vital signs were unremarkable. On physical exam, he had non-blanching, non-palpable petechiae extending from his lower extremities (LEs) to torso and upper extremities and 2+ LE edema. Lab data showed low hemoglobin (7.6g/L) and platelet count (76k/mL), elevated ferritin (1864ng/mL), LDH (297U/L), and IL-2 receptor level (1268pg/mL), meeting hemophagocytic lymphohistiocytosis (HLH) criteria. Autoimmune panels were positive for ANA (1:80), rheumatoid factor (75U), anti-cyclic citrullinated peptide (21U), anti-histone (1.4U), beta-2 glycoprotein, anticardiolipin IgM, cryoglobulin with cryocrit (5), proteinase 3 (PR3), and C3. Bone marrow biopsy showed hypercellular marrow with trilineage hematopoiesis without evidence of malignancy. Outpatient biopsy of the petechiae showed leukocytoclastic vasculitis. Blood cultures grew Streptococcus mitis and subsequent transesophageal echocardiogram (TEE) showed thickened aortic valve leaflets coated with mobile echogenic material, for which he was started on ceftriaxone. He also had severe aortic and mitral regurgitation. His hospital course was complicated by worsening kidney function requiring hemodialysis. Kidney biopsy demonstrated active immune complex-mediated glomerulonephritis with fibrinoid necrosis and cellular crescents, suggestive of post-infectious glomerulonephritis. He was started on IVIG without much improvement, so was switched to methylprednisolone. During his hospitalization, he also developed symptomatic sinus pauses along with atrial fibrillation with RVR; cardiac catherization showed 60% stenosis of left main coronary artery and thus, he underwent coronary artery bypass graft (CABG) as well as aortic and mitral valve replacements. His vasculitis and renal function significantly improved after valvular repair and prednisone taper; no longer requiring hemodialysis upon discharge.
Discussion: Despite seemingly straightforward diagnostic criteria for infective endocarditis, it could be a challenging diagnosis when patients present with a constellation of non-specific findings. Some of these patients may initially be diagnosed with vasculitis, only later found to have an array of infections including infective endocarditis. Therefore, it is important to rule out infections before the initiation of immunosuppression. Our patient not only had infective endocarditis and vasculitis, but also had glomerulonephritis; the initiation of immunosuppressive treatment in this population is still controversial. Two case reports have demonstrated partial remission and full recovery after the use of steroids, but others have shown multi-organ failure and death after steroids. Hence, the decision to start immunosuppressive therapies including steroids and IVIG should be patient-specific as the risks of worsening infection leading to septic shock and even death may outweigh the benefits.
Conclusions: Infective endocarditis complicated by vasculitis and glomerulonephritis is rare and physicians are usually left without specific recommendations for treatment. There have been some successful cases using antibiotics and valve repair followed by carefully timed steroids. Further review of such cases should be undertaken to delineate guidelines regarding whether and when to start immunosuppression.