School of Medicine and Health Sciences Poster Presentations
Renal failure due to Proteinase-3 antibody positive ANCA-associated glomerulonephritis in a patient with Bartonella quintana endocarditis.
Poster Number
204
Document Type
Poster
Publication Date
3-2016
Abstract
BACKGROUND :
Bartonella species can cause culture-negative bacterial endocarditis (BE). However, their role in pathogenesis of organ-threatening ANCA-associated vasculitis is not well recognized. Our case depicts this unusual association and the importance of accurate diagnosis, which would dramatically alter the treatment strategies and disease outcome.
CASE DESCRIPTION:
A 55 year old African-American male with past medical history of hepatitis-C and alcoholism presented with dyspnea, fever, weight loss, and lower extremity edema to an outside hospital and was noted to be in acute renal failure with creatinine of 5.5, and hypertension. Echocardiography demonstrated severe aortic and mitral valve vegetations. Blood cultures, ANA, HCV PCR, HIV, cryoglobulins, SPEP, C4 were normal. Intensive search for an infectious etiology revealed elevated Bartonella henselae and B. quintana IgG and elevated Bartonella quintana IgM. Patient also had positive C-ANCA/anti-proteinase 3 antibodies and low C3 complement. Drug and toxicology screening were negative except for ethanol. Renal biopsy demonstrated pauci-immune proliferative glomerulonephritis (GN). Subsequently, the patient was treated with IV antibiotics and pulse IV steroids. Treatment with Rituximab was considered but patient refused further therapy and was discharged on Rifampin, Doxycycline, and Prednisone. Ten days later he presented to our hospital with acute respiratory distress and was noted have worsening renal function and pulmonary edema. His echocardiogram showed worsening ejection fraction (65% -> 35%) with aortic valve vegetations. Patient declined aortic valve repair. C-ANCA –associated glomerulonephritis was attributed to Bartonella infection and more aggressive immunosuppressive therapy was withheld. Patient had significant recovery of cardiac and renal function with antibiotics and supportive care.
DISCUSSION:
Positive c-ANCA/ proteinase-3 antibodies (PR3) is one of the diagnostic features of Wegener’s/Granulomatosis with polyangiitis (WG/GPA). Glomerulonephritis in GPA is “pauci-immune”. Therefore, presence of immune-complex and C3 complement deposits in renal biopsy is atypical for GPA but has been reported in ANCA-associated-GN due to cocaine, hydralazine and other drugs. Our patient had consistently tested negative for these drugs. In the two other cases described in the literature, presence of IgG/ IgM/ C3 complexes in renal biopsy of ANCA-associated-GN ,can help distinguish glomerulonephritis seen with WG/GPA from renal involvement caused by Bartonella. Failing to distinguish Bartonella induced infectious glomerulonephritis from Wegener’s/GPA glomerulonephritis, would lead to aggressive immunosuppressive therapy with rituximab or cyclophosphamide, which may potentially lead to catastrophic consequences.
Creative Commons License
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 4.0 License.
Open Access
1
Renal failure due to Proteinase-3 antibody positive ANCA-associated glomerulonephritis in a patient with Bartonella quintana endocarditis.
BACKGROUND :
Bartonella species can cause culture-negative bacterial endocarditis (BE). However, their role in pathogenesis of organ-threatening ANCA-associated vasculitis is not well recognized. Our case depicts this unusual association and the importance of accurate diagnosis, which would dramatically alter the treatment strategies and disease outcome.
CASE DESCRIPTION:
A 55 year old African-American male with past medical history of hepatitis-C and alcoholism presented with dyspnea, fever, weight loss, and lower extremity edema to an outside hospital and was noted to be in acute renal failure with creatinine of 5.5, and hypertension. Echocardiography demonstrated severe aortic and mitral valve vegetations. Blood cultures, ANA, HCV PCR, HIV, cryoglobulins, SPEP, C4 were normal. Intensive search for an infectious etiology revealed elevated Bartonella henselae and B. quintana IgG and elevated Bartonella quintana IgM. Patient also had positive C-ANCA/anti-proteinase 3 antibodies and low C3 complement. Drug and toxicology screening were negative except for ethanol. Renal biopsy demonstrated pauci-immune proliferative glomerulonephritis (GN). Subsequently, the patient was treated with IV antibiotics and pulse IV steroids. Treatment with Rituximab was considered but patient refused further therapy and was discharged on Rifampin, Doxycycline, and Prednisone. Ten days later he presented to our hospital with acute respiratory distress and was noted have worsening renal function and pulmonary edema. His echocardiogram showed worsening ejection fraction (65% -> 35%) with aortic valve vegetations. Patient declined aortic valve repair. C-ANCA –associated glomerulonephritis was attributed to Bartonella infection and more aggressive immunosuppressive therapy was withheld. Patient had significant recovery of cardiac and renal function with antibiotics and supportive care.
DISCUSSION:
Positive c-ANCA/ proteinase-3 antibodies (PR3) is one of the diagnostic features of Wegener’s/Granulomatosis with polyangiitis (WG/GPA). Glomerulonephritis in GPA is “pauci-immune”. Therefore, presence of immune-complex and C3 complement deposits in renal biopsy is atypical for GPA but has been reported in ANCA-associated-GN due to cocaine, hydralazine and other drugs. Our patient had consistently tested negative for these drugs. In the two other cases described in the literature, presence of IgG/ IgM/ C3 complexes in renal biopsy of ANCA-associated-GN ,can help distinguish glomerulonephritis seen with WG/GPA from renal involvement caused by Bartonella. Failing to distinguish Bartonella induced infectious glomerulonephritis from Wegener’s/GPA glomerulonephritis, would lead to aggressive immunosuppressive therapy with rituximab or cyclophosphamide, which may potentially lead to catastrophic consequences.
Comments
Presented at: GW Research Days 2016