Fortuitous Vasculitis

Abstract

A 43-year-old man with a cardiac device for dilated cardiomyopathy presented with fever, night sweats, and weight loss. Investigations revealed pancytopenia, acute renal failure, abnormal lung function, and raised inflammatory markers. A renal biopsy demonstrated pauci-immune necrotizing crescentic glomerulonephritis. He was diagnosed with pulmonary-renal antineutrophil cytoplasmic antibody-negative systemic small vessel vasculitis. He commenced immunosuppression with prednisolone and cyclophosphamide with recovery from pancytopenia and improvement in renal function 3 months later. Subsequently, a bone marrow culture grew Mycobacterium fortuitum. Isolation on repeat peripheral mycobacterial blood cultures prompted treatment with ciprofloxacin and clarithromycin. Four months later, he presented with neutropenic sepsis, influenza A/H1N1, and Aspergillus flavus pneumonia. Despite treatment he deteriorated. A transthoracic echocardiogram revealed a vegetation on the right ventricular pacing wire. The device was removed. The vegetation revealed acid and alcohol fast bacilli on Ziehl-Neelsen staining and grew M. fortuitum on culture, sensitive to ciprofloxacin and clarithromycin. Despite device removal and antimicrobial therapy, the patient succumbed to treatment-related complications. The association between glomerulonephritis and endocarditis is well known; however, this is the first case to our knowledge describing pauci-immune necrotizing crescentic glomerulonephritis in the context of M. fortuitum endocarditis. Clinicians should maintain a high index of suspicion for endocarditis in patients with a cardiac device who present with fever and pauci-immune necrotizing crescentic glomerulonephritis. Patients should be investigated with mycobacterial blood cultures, at least three sets of standard blood cultures and transthoracic and transesophageal echocardiography. Clinicians should beware the perils of immunosuppression in the face of an occult sepsis.

Mycobacterial endocarditis: a comprehensive review

“…Of the febrile patients, fever grade was not indicated in 22 [ 11 , 14 , 18 , 23 , 24 , 26 , 28 , 29 , 33 , 35 , 36 , 38 ] . In the remaining 13 patients, 5 (38.5%) had a high fever [ 12 , 17 , 19 , 20 , 22 , 29 ] , 5 (38.5%) had a moderate fever [ 13 , 15 , 21 , 25 , 34 ] and 3 (23.1%) had a low-grade fever [ 9 , 27 , 37 ] (χ 2 =0.9, P =0.630). Of them, 4 were prolonged fever [ 24 , 35 , 36 ] and 2 were fever of unknown origin [ 14 , 16 ] .…”

“…The cardiac murmur was mentioned in 15 patients: a cardiac murmur in 12 (80%) (a pansystolic murmur at the apex in 5 [ 14 , 21 , 27 , 30 , 38 ] , a diastolic murmur in the aortic region in 2 [ 13 , 18 ] , a systolic murmur in the aortic region [ 36 ] , a pansystolic murmur at the apex + a diastolic murmur in the aortic region [ 25 ] , a pansystolic murmur at the apex + a systolic murmur in the aortic region [ 15 ] , a systolic murmur at the lower left sternal boarder [ 37 ] and an unspecified cardiac murmur [ 32 ] in 1 patient each) and an absence of a cardiac murmur in 3 (20%) patients [ 20 , 22 , 24 ] (χ 2 =10.8, P =0.001).…”

“…Laboratory examinations revealed their hemoglobin was 9.9±1.8 (range, 5.9-12.5; median, 10) g/L (n=10) [ 9 , 10 , 12 , 13 , 15 , 18 , 20 , 27 , 30 , 36 , 38 ] , white blood cell count was 8.9±4.4 (range, 3.3-16.8; median, 8.2) ×10 9 /L (n=13) [ 9 , 10 , 13 , 15 , 20 - 22 , 24 , 27 , 28 , 30 , 33 , 38 ] , platelet count was 165.6±128.7 (range, 67-450; median, 124)×10 9 /L (n=7) [ 10 , 13 , 15 , 18 , 20 , 27 , 38 ] , C-reaction protein was 6.8±4.7 (range, 0.14-11; median, 8) mg/dL (n=4) [ 10 , 20 , 27 , 28 ] , and CD4 was 240.5 ± 206.3 (range, 44-587; median 196)/mm 3 (n=6) [ 9 , 10 , 13 , 14 , 19 , 37 ] . CD4 was normal (>500/mm 3 ) in 1 (16.7%) patient, and was abnormal (<500/mm 3 ) in 5 (83.3%) patients (χ 2 =5.3, P =0.021).…”

“…The latency from the presence of the predisposing risk factors to symptom onset was 21.9±25.9 (range, 0.067-96; median, 12) months (n=31) [ 7 , 9 , 11 , 12 , 14 - 18 , 20 , 21 , 23 , 26 , 27 , 31 - 33 , 37 ] . There were 9 (29.0%) early onsets (latency <8 weeks), 2 (6.5%) intermediate onsets (latency was between 8 weeks and 8 months) and 20 (64.5%) late onsets (latency >8 months) (χ 2 =23.9, P =0.000).…”

“…Of them, 12 (27.3%) patients did not have a visualized vegetation [ 7 , 9 , 11 , 14 , 17 , 28 , 31 ] , but one of them with an abscess along the inferior and septal walls, instead [ 31 ] and 32 (72.7%) patients had (χ 2 =18.2, P =0.000). The vegetation locations of the remaining 32 patients were mitral valve in 10 (31.3%) [ 11 , 20 , 21 , 30 , 36 , 37 ] (two of them were in the prosthetic mitral valve) [ 20 , 1 ] , aortic valve in 7 (21.9%) [ 11 , 13 , 18 , 32 , 33 , 38 ] (one was in the prosthetic aortic valve) [ 33 ] , pacemaker lead in 3 (12.5%) [ 16 , 26 , 27 ] , tricuspid valve in 3 (9.4%) [ 10 , 19 , 35 ] , both mitral valve and right subclavian catheter in 1 (3.1%) [ 36 ] , both mitral and aortic valves in 2 (6.3%) [ 12 , 15 ] , both aortic and tricuspid valves in 2 (6.3%) [ 22 , 24 ] , mitral, aortic and tricuspid valves in 1 (3.1%) [ 25 ] , tricuspid valve and ventricular septal defect patch in 2 (6.3%) [ 35 ] and atrial septal patch in 1 (3.1%) patient [ 23 ] , respectively. The detection time for a positive vegetation was described in 3 patients, which was 5 [ 13 ] , 21 [ 20 ] and 105 days [ 15 ] after admission, respectively.…”