Abstract: PUB224
ANCA Negative, Yet Pulmonary Embolism Positive
Session Information
Category: Trainee Case Report
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Gayle, Latoya N., Englewood Health, Englewood, New Jersey, United States
- Fein, Deborah A., Englewood Health, Englewood, New Jersey, United States
Introduction
Pauci immunue glomerulonephritis(GN) with negative ANCA serology occurs in ~1/3 of pauci immune GN patients.ANCA negative patients are thought to have a lower incidence of extra-renal involvement but poorer renal prognosis than those who are ANCA-positive.We present a case of ANCA-negative Pauci-immune Rapidly Progressive GN(RPGN),returning 4 weeks after diagnosis with pulmonary embolism(PE)
Case Description
A 39-year-old male presented(pre-pandemic) with 2 weeks of fever and chills.For ~3 years,he has had intermittent arthralgia,dyspnea,facial and ankle swelling with gross hematuria and a 12lb weight gain 2 months prior to admission.Baseline creatinine(Cr) was 1.3(0.7-1.3mg/dl) a month prior.On admission Cr was 2.6, Alb 3mg/dl and Urinalysis noted protein 100mg/dl,RBC 20-30 and WBC 10-20.A 24 hour urine protein measured 8g/day.Serologies were positive for dsDNA1:10 and Antistreptolysin O,with negative ANA,ANCA,anti-GBM,SPEP,Hepatitis B,C,HIV and normal C3/C4.Renal sonogram noted normal sized kidneys with increased echogenicity.A renal Biopsy was done and Methylprednisolone pulse therapy commenced.His renal biopsy showed focal necrotizing and crescentic GN with negative IF,consistent with pauci-immune GN.Cr peaked at 3.6 and IV Cyclophosphamide was given.He was discharged on Prednisone with Rituximab given 2 weeks later.4 weeks after discharge,Cr improved to 1.3,however he represented with SOB,pleuritic chest pain and hemoptysis.CT chest revealed acute bilateral PE.Malignancy work-up and lower extremity dopplers were negative.He had normal IgG,A,M,antithrombin III,prothrombin gene analysis,protein C,S activity and factor X with negative Lupus anti-coagulant and antiphospholipid Ab.He was started,then discharged on Enoxaparin for anticoagulation
Discussion
Renal disease manifests as a pauci-immune GN in ANCA-Associated Vasculitis(AAV).~ 1/3 of patients with pauci immune vasculitis are ANCA negative.Whether these patients should be included in the spectrum of AAV or are a separate pathophysiologic mechanism is unknown.Our patient presented with RPGN and a PE after initial treatment despite negative ANCA panel and current evidence suggesting a lower incidence of other organ involvement.The presence of an underlying pulmonary vasculitis could not be excluded.We postulate that all patients with ANCA negative RPGN should be followed for additional organ involvement and further study of this patient population is warranted