SCIENTIFIC Lyme Disease Associated Systemic Vasculitis : Case Report and Review
AUTHORS : Jerome P . Givi , MPH 1
Nathan Williams , MD 1 Erona Reza , MD 1 Md . Shahrier Amin , MD , PhD 1
1
West Virginia University School of Medicine
CASE PRESENTATION
A 72-year-old male from West Virginia ( WV ) presented to the emergency department with worsening cough , intermittent fever , chills , fatigue , and malaise . His medical history was significant for gastroesophageal reflux disease and SARS-CoV-2 infection eight weeks prior , despite being fully vaccinated . At presentation , the patient was febrile ( 37.9 ° C ). Otherwise , the patient was vitally stable , and his physical examination was normal . His urinalysis showed trace leukocytes and large blood . A computerized tomography scan of the abdomen showed perinephric fat stranding . He was started on empiric ceftriaxone for possible pyelonephritis . During his hospital stay , he had an episode of atrioventricular block . An electrocardiogram ( ECG ) showed ST segment changes and episodes of asymptomatic bradycardia . The transthoracic echocardiogram was unremarkable . The patient was given colchicine for potential pericarditis . Renal function was unremarkable at this time . Orders for Lyme antibody titers were placed , which involved performing immunoblots . In this case , the immunoblots returned positive for Lyme IgM and IgG , indicating an active Lyme disease infection .
The patient was started on doxycycline on an outpatient basis , but due to gastrointestinal side effects , he was switched to ceftriaxone two days later . The patient had an outpatient follow-up with cardiology shortly after discharge , where ECG findings included a first-degree atrioventricular block , therefore the patient was diagnosed with Lyme carditis . Following
ABSTRACT
Lyme disease-induced nephritis is commonly seen in canines and has rarely been reported in humans . Anti-neutrophilic cytoplasmic antibody ( ANCA ) formation can be triggered by environmental insults and is a common cause of nephritis and vasculitis . To our knowledge , there have been no reports of Lyme disease-associated pauci-immune glomerulonephritis . Here , we present a case of pauci-immune glomerulonephritis in a 72-year-old male from West Virginia ( WV ) shortly after Lyme infection , raising the possibility of association . The patient initially presented with symptoms
discharge , he returned to the clinic after four days with a fever , headache , progressive left-sided hearing loss , nausea , and vomiting . The patient was hypertensive but otherwise vitally stable . Physical examination revealed mild erythema of the left ear as well as a new 2 / 6 holosystolic murmur . The patient was directly admitted to the hospital from the clinic . At that time , his serum creatinine was 3.99 mg / dL , and urinalysis revealed hematuria and proteinuria . His erythrocyte sedimentation rate was 95 mm / hour and leukocyte count was 17.2x103 / uL . A kidney biopsy was ordered to elucidate the etiology of renal dysfunction . of early-stage Lyme disease and shortly thereafter presented with severely worsening renal function . Biopsy and serum findings showed pauci-immune glomerulonephritis and positive ANCA serology . Despite receiving corticosteroids , he did not regain renal function and remained dependent upon dialysis . Lyme disease triggering ANCA vasculitis may be an underappreciated occurrence . Clinicians should suspect Lyme disease in patients with new onset ANCA vasculitis , especially in endemic areas like the state of WV .
A renal biopsy demonstrated 45 glomeruli , only three of which were globally sclerotic ( Figure 1 ). Cellular crescents and / or fibrinoid necrosis were observed in 32 glomeruli . No mesangial matrix expansion , endocapillary hypercellularity , or double contours were seen . Two glomeruli showed fibrocellular crescents . Many tubules also showed red blood cell ( RBC ) casts and signs of tubular injury with dilated lumina and simplification of the lining epithelium . Changes indicating early interstitial fibrosis were present in 15-20 % of the cortex . A concomitant tubulointerstitial lymphoplasmacytic infiltrate was present in areas with fibrosis and near atrophic tubules . There was no significant intimal fibrosis or hyaline deposition and no features suggestive of vasculitis in the blood vessels . Immunofluorescence studies for IgA , IgG , IgM , C3 , C1q , kappa light chain , and lambda light chain were all negative in the glomeruli and tubulointerstitium . Four of the seven glomeruli present on frozen section showed fibrinogen deposition , indicating crescents or fibrinoid necrosis . Electron microscopy did not show any immune type deposits along the glomerular capillary basement membranes or in the mesangial areas . Serologic studies were positive for proteinase 3 with titers of 1:8 . The features were consistent with pauci-immune , diffuse necrotizing and crescentic glomerulonephritis , as is commonly seen in anti-neutrophilic cytoplasmic antibody ( ANCA ) associated disease .
The patient completed a 28-day course of intravenous ceftriaxone , which improved his Lyme disease manifestations . Following the return of the biopsy results , stress doses of steroids and cyclophosphamide were started . However , the patient ’ s renal function continued to deteriorate with no response to immunosuppressive therapies . The patient developed uremia , causing altered mental status , requiring hemodialysis three times weekly during admission and following discharge .
Repeat immunoblots were positive for Lyme IgG and negative for Lyme IgM approximately three months after this sec-
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