| Author/Editor | Bele, David; Kojc, Nika; Perše, Martina; Černe, Andreja; Lindič, Jelka; Aleš, Andreja; Večerić-Haler, Željka | |
| Title | Diagnostic and treatment challenge of unrecognized subacute bacterial endocarditis associated with ANCA-PR3 positive immunocomplex glomerulonephritis | |
| Type | članek | |
| Vol. and No. | Letnik 21, št. 1 | |
| Publication year | 2020 | |
| Volume | str. 1-7 | |
| ISSN | 1471-2369 - BMC nephrology | |
| Language | eng | |
| Abstract | Background:Diagnosis and treatment of either ANCA disease or silentinfection-related glomerulonephritis is complicatedand is a huge treatment challenge when overlapping clinical manifestations occur. We report a case of ANCA-PR3glomerulonephritis, nervous system involvement, hepatosplenomegaly and clinically silent subacute infectious endocarditis.Case presentation:A 57-year-old man with known mitral valve prolaps was admitted forunexplained renal failure withsigns of nephritic syndrome, hepatosplenomegaly, sudden unilateral hearing loss,vertigo, malaise, new onset hemolyticanemia and thrombocytopenia. Immunoserology revealed positive c-anti-neutrophil cytoplasm antibody (ANCA)/anti-proteinase3(anti-PR3),mixedtypecrioglobulinemiaand lowered complement fraction C3. Head MRI showed manymicroscopic hemorrhages. Common site of infection, as wellas solid malignoma were ruled out. In accordance with clinicaland laboratory findings, systemic vasculitis was assumed, although the etiologyremained uncertain (ANCA-associated,cryoglobulinemic or related to unrecognized infection). After kidney biopsy, clinical signs of sepsis appeared. Blood culturesrevealedStreptococcus 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. Asecond kidney biopsy revealed no residual kidney disease.Four years after treatment, the patient is stable with nosymptoms or signs of vasculitis recurrence.Conclusions:Here we describe the diagnostic and treatment challenge in a patient with unrecognized subacute bacterialendocarditis associated with ANCA-PR3 immunocomplex proliferative and crescentic glomerulonephritis. In patients withANCA-PR3 immunocomplex glomerulonephritis and other overlapping manifestations suggesting systemic disease, it isimportant to recognize and aggressively treat any possible coexisting bacterial endocarditis, This is the most important stepfor a favorable patient outcome, including complete clinical and pathohistological resolution of the glomerulonephritis. | |
| Keywords | endocarditis glomerulonefritis ANCA systemic vasculitis endocarditis glomerulonefritis sistemski vaskulitis ANCA |