Rapidly Progressive Glomerulonephritis Associated with PR3-ANCA Positive Subacute Bacterial Endocarditis

Fukasawa, Hirotaka; Hayashi, Maho; Kinoshita, Naoko; Ishigaki, Sayaka; Isobe, Shinsuke; Sakao, Yukitoshi; Kato, Akihiko; Fujigaki, Yoshihide; Furuya, Ryuichi

doi:10.2169/internalmedicine.51.8081

Cited by 29 publications

(15 citation statements)

References 16 publications

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“…Our literature review, in fact, identified 24 case reports of documented ANCA-positive patients with IE exhibiting renal impairment. 11,24,37,39,40,42–45,47,48,50,51,54,57–59,61 Twenty-one of these latter patients underwent renal biopsy showing: extracapillary GN with immune deposits (N = 5), pauci-immune GN (N = 4), segmental and focal necrotizing GN (N = 4), endocapillary GN with immune deposits (N = 3), interstitial nephritis (N = 1), chronic sclerotic GN (N = 1), both pauci-immune GN and interstitial nephritis (N = 2), and both endocapillary GN and interstitial nephritis (N = 1). 11,24,37,39,40,42–45,47,48,50,51,54,57–59,61 Altogether, our data suggest that whether patients with ANCA-associated IE, develop commonly renal impairment, renal damage are nonspecific in these patients.…”

Section: Discussionmentioning

confidence: 99%

Antineutrophil Cytoplasmic Antibodies Associated With Infective Endocarditis

et al. 2016

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To determine the prevalence of antineutrophil cytoplasmic antibodies (ANCA) in patients with infective endocarditis (IE) in internal medicine; and to compare clinical and biochemical features and outcome between patients exhibiting IE with and without ANCA.Fifty consecutive patients with IE underwent ANCA testing. The medical records of these patients were reviewed.Of the 50 patients with IE, 12 exhibited ANCA (24%). ANCA-positive patients with IE exhibited: longer duration between the onset of first symptoms and IE diagnosis (P = 0.02); and more frequently: weight loss (P = 0.017) and renal impairment (P = 0.08), lower levels of C-reactive protein (P = 0.0009) and serum albumin (P = 0.0032), involvement of both aortic and mitral valves (P = 0.009), and longer hospital stay (P = 0.016). Under multivariate analysis, significant factors for ANCA-associated IE were: longer hospital stay (P = 0.004), lower level of serum albumin (P = 0.02), and multiple valve involvement (P = 0.04). Mortality rate was 25% in ANCA patients; death was because of IE complications in all these patients.Our study identifies a high prevalence of ANCA in unselected patients with IE in internal medicine (24%). Our findings further underscore that ANCA may be associated with a subacute form of IE leading to multiple valve involvement and more frequent renal impairment. Because death was due to IE complications in all patients, our data suggest that aggressive therapy may be required to improve such patients’ outcome.

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Section: Discussionmentioning

confidence: 99%

Antineutrophil Cytoplasmic Antibodies Associated With Infective Endocarditis

et al. 2016

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“…42 Although interpretation of a positive c-ANCA may be difficult, it is likely that hypocomplementemia and the presence of at least one other autoantibody such as RF, ANA, or cryoglobulin are more suggestive of an ANCA-positive bacterial endocarditis rather than an ANCA vasculitis. 46 The patient presented here had hypocomplementemia, a positive RF, and positive c-ANCA titer.…”

Section: Discussionmentioning

confidence: 69%

“…In particular, the presence of c-ANCA is generally thought to be highly specific for GPA. 41 However, c-ANCA directed against PR-3 positivity has also been well documented in infectious endocarditis 42–46 and more specifically in Bartonella endocarditis. 3,5,9–11,26,27,47 The presence of c-ANCA in infectious endocarditis is unclear: it may represent a false-positive or related to the infection or the production of c-ANCA may be induced through B-cell activation after release of PR-3 from neutrophils.…”

Section: Discussionmentioning

confidence: 99%

Bartonella Endocarditis and Pauci-Immune Glomerulonephritis

Raybould¹,

Raybould²,

Morales³

et al. 2016

Infect Dis Clin Pract

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Among culture-negative endocarditis in the United States, Bartonella species are the most common cause, with Bartonella henselae and Bartonella quintana comprising the majority of cases. Kidney manifestations, particularly glomerulonephritis, are common sequelae of infectious endocarditis, with nearly half of all Bartonella patients demonstrating renal involvement. Although a pauci-immune pattern is a frequent finding in infectious endocarditis–associated glomerulonephritis, it is rarely reported in Bartonella endocarditis. Anti–neutrophil cytoplasmic antibody (ANCA) positivity can be seen with many pathogens causing endocarditis and has been previously reported with Bartonella species. In addition, ANCA-associated vasculitis can also present with renal and cardiac involvement, including noninfectious valvular vegetations and pauci-immune glomerulonephritis. Given the overlap in their clinical presentation, it is difficult to differentiate between Bartonella endocarditis and ANCA-associated vasculitis but imperative to do so to guide management decisions. We present a case of ANCA-positive Bartonella endocarditis with associated pauci-immune glomerulonephritis that was successfully treated with medical management alone.

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“…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.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2020

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Background: Diagnosis and treatment of either ANCA disease or silent infection-related glomerulonephritis is complicated and is a huge treatment challenge when overlapping clinical manifestations occur. We report a case of ANCA-PR3 glomerulonephritis, nervous system involvement, hepatosplenomegaly and clinically silent subacute infectious endocarditis. Case presentation: A 57-year-old man with known mitral valve prolaps was admitted for unexplained renal failure with signs of nephritic syndrome, hepatosplenomegaly, sudden unilateral hearing loss, vertigo, malaise, new onset hemolytic anemia and thrombocytopenia. Immunoserology revealed positive c-anti-neutrophil cytoplasm antibody (ANCA)/antiproteinase 3 (anti-PR3), mixed type crioglobulinemia and lowered complement fraction C3. Head MRI showed many microscopic hemorrhages. Common site of infection, as well as solid malignoma were ruled out. In accordance with clinical and laboratory findings, systemic vasculitis was assumed, although the etiology remained uncertain (ANCA-associated, cryoglobulinemic or related to unrecognized infection). After kidney biopsy, clinical signs of sepsis appeared. Blood cultures revealed Streptococcus cristatus. Echocardiography showed mitral valve endocarditis. Kidney biopsy revealed proliferative, necrotizing immunocomplex glomerulonephritis. Half a year later, following intravenous immunoglobulins, glucocorticoids, antibiotic therapy and surgical valve repair, the creatinine level decreased and c-ANCA and cryoglobulins disappeared. A second kidney biopsy revealed no residual kidney disease. Four years after treatment, the patient is stable with no symptoms or signs of vasculitis recurrence. Conclusions: Here we describe the diagnostic and treatment challenge in a patient with unrecognized subacute bacterial endocarditis associated with ANCA-PR3 immunocomplex proliferative and crescentic glomerulonephritis. In patients with ANCA-PR3 immunocomplex glomerulonephritis and other overlapping manifestations suggesting systemic disease, it is important to recognize and aggressively treat any possible coexisting bacterial endocarditis, This is the most important step for a favorable patient outcome, including complete clinical and pathohistological resolution of the glomerulonephritis.

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Rapidly Progressive Glomerulonephritis Associated with PR3-ANCA Positive Subacute Bacterial Endocarditis

Cited by 29 publications

References 16 publications

Antineutrophil Cytoplasmic Antibodies Associated With Infective Endocarditis

Antineutrophil Cytoplasmic Antibodies Associated With Infective Endocarditis

Bartonella Endocarditis and Pauci-Immune Glomerulonephritis

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

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