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Diffuse Alveolar Hemorrhage in ANCA-Negative Pauci-Immune Crescentic Glomerulonephritis: A Rare Case Report
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A3065 - Diffuse Alveolar Hemorrhage in ANCA-Negative Pauci-Immune Crescentic Glomerulonephritis: A Rare Case Report
Author Block: Y. Eslaamizaad, K. Schaphorst, P. Almalouf; Pulmonary and Critical Care, University of South Alabama, Mobile, AL, United States.
Introduction
Pulmonary renal syndrome (PRS) is a combination of diffuse pulmonary hemorrhage and glomerulonephritis (GN). Although frequently seen in anti-neutrophil cytoplasmic autoantibody (ANCA)-associated GN and vasculitis, diffuse pulmonary hemorrhage is rarely reported in ANCA-negative GN. We present a case of an 84-year-old Caucasian female with history of stage IV chronic kidney disease who presented with diffuse alveolar hemorrhage and nephritic syndrome, found to have ANCA-negative crescentic pauci-immune GN.
Case Presentation
An 84-year-old Caucasian female with past medical history of diastolic cardiac dysfunction, hypertension, and stage IV CKD (baseline of 1.5 mg/dL) presented with a three-day history of progressively worsening dyspnea. On examination, she was in respiratory distress with a respiratory rate of 36 breaths/minute, pulse 97 beats/minute and blood pressure 160/92 mmHg, oral temperature was 98°F. She had diffuse bilateral crackles on her lung examination, jugular venous distension and trace peripheral pitting edema. Laboratory findings were significant for a creatinine of 2.4 mg/dL, hemoglobin and hematocrit of 6.3 and 20 respectively and an elevated BNP of 885.
The patient was admitted to the ICU and received diuretics and BIPAP. For progressive respiratory failure she eventually required intubation. Her urine output continued to decline and was resistant to diuretics with furosemide and metolazone. Patient’s hemoglobin and hematocrit continued to decline despite blood transfusion. She also had a negative fecal occult and no other clear source of bleeding. Bronchoscopy and bronchoalveolar lavage (BAL) of right upper and middle lobe revealed bloody lavage with increased bloodiness on subsequent lavages consistent with diffuse alveolar hemorrhage. Immune mediated disease workup revealed negative perinuclear and cytoplasmic ANCA and the remainder of the immunology panel was essentially normal. Considering PRS, the patient was initiated on pulse steroids (methylprednisolone of 1 g for 3 days). Renal biopsy revealed pauci-immune necrotizing and crescentric glomerulonephritis. By the third ICU day patient was extubated, and creatinine normalized to her baseline of 1.4.
Discussion
When left untreated, Crescentic GN can progress to end stage renal disease within a timespan of a few weeks to a few months. Our case illustrates that diffuse alveolar hemorrhage can be a distinct clinical feature even in patients with ANCA-negative pauci-immune crescentic glomerulonephritis. Prompt serologic testing and renal biopsy should be performed in these patients. Patients should be started on high-dose methylprednisolone as soon as possible to ultimately minimize the degree of irreversible kidney injury and to treat respiratory failure to provide better outcome.
Author Block: Y. Eslaamizaad, K. Schaphorst, P. Almalouf; Pulmonary and Critical Care, University of South Alabama, Mobile, AL, United States.
Introduction
Pulmonary renal syndrome (PRS) is a combination of diffuse pulmonary hemorrhage and glomerulonephritis (GN). Although frequently seen in anti-neutrophil cytoplasmic autoantibody (ANCA)-associated GN and vasculitis, diffuse pulmonary hemorrhage is rarely reported in ANCA-negative GN. We present a case of an 84-year-old Caucasian female with history of stage IV chronic kidney disease who presented with diffuse alveolar hemorrhage and nephritic syndrome, found to have ANCA-negative crescentic pauci-immune GN.
Case Presentation
An 84-year-old Caucasian female with past medical history of diastolic cardiac dysfunction, hypertension, and stage IV CKD (baseline of 1.5 mg/dL) presented with a three-day history of progressively worsening dyspnea. On examination, she was in respiratory distress with a respiratory rate of 36 breaths/minute, pulse 97 beats/minute and blood pressure 160/92 mmHg, oral temperature was 98°F. She had diffuse bilateral crackles on her lung examination, jugular venous distension and trace peripheral pitting edema. Laboratory findings were significant for a creatinine of 2.4 mg/dL, hemoglobin and hematocrit of 6.3 and 20 respectively and an elevated BNP of 885.
The patient was admitted to the ICU and received diuretics and BIPAP. For progressive respiratory failure she eventually required intubation. Her urine output continued to decline and was resistant to diuretics with furosemide and metolazone. Patient’s hemoglobin and hematocrit continued to decline despite blood transfusion. She also had a negative fecal occult and no other clear source of bleeding. Bronchoscopy and bronchoalveolar lavage (BAL) of right upper and middle lobe revealed bloody lavage with increased bloodiness on subsequent lavages consistent with diffuse alveolar hemorrhage. Immune mediated disease workup revealed negative perinuclear and cytoplasmic ANCA and the remainder of the immunology panel was essentially normal. Considering PRS, the patient was initiated on pulse steroids (methylprednisolone of 1 g for 3 days). Renal biopsy revealed pauci-immune necrotizing and crescentric glomerulonephritis. By the third ICU day patient was extubated, and creatinine normalized to her baseline of 1.4.
Discussion
When left untreated, Crescentic GN can progress to end stage renal disease within a timespan of a few weeks to a few months. Our case illustrates that diffuse alveolar hemorrhage can be a distinct clinical feature even in patients with ANCA-negative pauci-immune crescentic glomerulonephritis. Prompt serologic testing and renal biopsy should be performed in these patients. Patients should be started on high-dose methylprednisolone as soon as possible to ultimately minimize the degree of irreversible kidney injury and to treat respiratory failure to provide better outcome.
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