Culture-negative subacute bacterial endocarditis masquerades as granulomatosis with polyangiitis (Wegener’s granulomatosis) involving both the kidney and lung

Abstract

BackgroundSubacute bacterial endocarditis (SBE) occasionally exhibits positive cytoplasmic anti-neutrophil cytoplasmic antibody (c-ANCA) of the anti-proteinase-3 (PR-3) type. Clinically, it mimics ANCA-associated vasculitis, such as Wegener's disease with glomerulonephritis. Lung abscesses are the most common manifestation of lung involvement. We herein report a case of culture-negative SBE strongly c-ANCA/PR3-positive accompanied by pulmonary involvement and glomerulonephritis. In this case, we took biopsies of both the lung and kidney, although renal biopsy is usually preferred over lung biopsy. The lung biopsy showed severe alveolar capillaritis, suggesting vasculitis consistent with polyangiitis. The renal biopsy revealed glomerulonephritis with a membranoproliferative pattern. To our knowledge, this is the first such reported case.Case presentationA 68-year-old Chinese male patient presented to our hospital with a fever, cough, chest pain, and recurrent peripheral edema. He had a past medical history significant for treated schistosomiasis 20 years previously. Physical examination revealed palpable purpura, mild hypertension, hepatosplenomegaly, and a holosystolic cardiac murmur (Levine 2/6). Echocardiography showed tricuspid valve vegetations with moderate to severe regurgitation. Serum c-ANCA/PR3 and cryoglobulin were strongly positive. Renal biopsy results indicated membranoproliferative glomerulonephritis with several crescents. Chest CT revealed multiple intraparenchymal and subpleural nodules, and lung biopsy showed polyangiitis. The patient’s ANCA titers, glomerulonephritis, and pulmonary injury all resolved after antibiotic therapy.ConclusionSBE may present with positive c-ANCA/PR3, multiple pulmonary nodules, pulmonary polyangiitis, and glomerulonephritis clinically mimicking granulomatosis with polyangiitis (Wegener's granulomatosis).

Valvular involvement in granulomatosis with polyangiitis: Case report and systematic review of literature

“…Similar to our study, aortic regurgitation was common in the Lacoste et al study, but none of the patients had tricuspid valve lesion. However, our systematic review comprised three cases including our report with tricuspid valve vegetations and sterile serum; two patients have eventually improved while our patient died.…”

Diagnostic and treatment challenge of unrecognized subacute bacterial endocarditis associated with ANCA-PR3 positive immunocomplex glomerulonephritis: a case report and literature review

“…Nevertheless, recent cohort studies have shown that neurologic involvement [8] or micro bleedings diagnosed by cerebral MRI can be seen not only in AAV patients but also in patients with infectious endocarditis-associated GN (emboli) [7]. In addition, pulmonary inflammatory granulomas, which are more frequently seen in idiopathic AAV patients, have also been reported in ANCA-positive infectious endocarditisassociated GN [10]. On the other hand, fever (a typical sign of infection) can be absent in some patients with ANCA positive infectious endocarditis-associated GN [11,12].…”

“…Literature regarding the appropriate treatment of patients with ANCA positive infectious endocarditis-associated GN is currently not consistent. Some propose treatment with antibiotics alone [10], others propose a combination of antibiotics and steroids [11,[18][19][20] and a third group suggest surgery followed by antibiotics without steroids [21]. Interestingly, in 1998 Haseyama et al [19] proposed that patients with ANCA positive infectious endocarditis-associated GN and low titers of PR3 ANCA (e.g., below 25 IU/ml) can be treated with antibiotics alone, while patients with higher titers of PR3 ANCA antibodies (e.g.…”

Glomerulonefritis rápidamente progresiva asociada a endocarditis afebril y anca anti-proteinasa 3

“…Sin embargo, hasta un 16 % de las endocarditis pueden cursar sin fiebre 2 ni leucocitosis, siendo incluso en algunos casos los hemocultivos negativos 3,4 .…”