Home
|
Inbox
|
Search
|
View Abstract
DAH: A Rare Presentation of Anca-Associated Vasculitis
Description
.abstract img { width:300px !important; height:auto; display:block; text-align:center; margin-top:10px } .abstract { overflow-x:scroll } .abstract table { width:100%; display:block; border:hidden; border-collapse: collapse; margin-top:10px } .abstract td, th { border-top: 1px solid #ddd; padding: 4px 8px; } .abstract tbody tr:nth-child(even) td { background-color: #efefef; } .abstract a { overflow-wrap: break-word; word-wrap: break-word; }
A3058 - DAH: A Rare Presentation of Anca-Associated Vasculitis
Author Block: O. Sheriff; Pulmonary Critical Care, Drexel University, Philadelphia, PA, United States.
Diffuse Alveolar hemorrhage due to alveolar capillaritis is a rare life-threatening pulmonary manifestation of Systemic Granulomatous with Polyangitits and occurs in 5-10 percent of patients. Prognosis is poor in DAH with in-hospital mortality ranging from 20 percent to 100 percent.
Patient is a 52 year old Caucasian female without a significant past medical history who presented to the Emergency Department with progressive dyspnea on exertion, nasal congestion, and cough with intermittent hemoptysis over eight weeks. Patient complained of migratory joint pain in her wrists, nasal congestion, and persistent upper respiratory tract infection symptoms despite multiple courses of antibiotics such as Levaquin 750 mg, for seven days, Azithromycin 250 mg for five days, and Augmentin 875-125 mg for ten days over one month prior to admission. Upon presentation she was hemodynamically stable and saturating 97-98 percent on room air. Patient was empirically started on Vancomycin 1000 mg q 12h and Cefepime 1 gram q 6h, for presumed pneumonia. Laboratories revealed a 6 gram hemoglobin drop from her baseline of 13 , 1+ proteinuria, 2+ hematuria with 31-50 RBC, elevated CRP at 21.90 and ESR of 55. Chest x-ray showed a large right lung pneumonic infiltrate concentrated in the right middle and lower lobes. CT scan of the chest showed bilateral diffuse ground glass opacities right greater than left lung with scattered granulomas. Patient was intubated and underwent bronchoscopy which revealed severe diffuse alveolar hemorrhage. Further work up revealed 1:320 titer Cytoplasmic antineutrophil cytoplasmic antibodies , Antiproteinase 3 (PR-3) Ab greater than 100.0 U/mL. The patient underwent right IR Guided kidney biopsy. Pathology showed pauci-immune crescentic glomerulonephritis consistent with Granulomatous with Polyangitits. Patient was started on 1 gram Cyclophosphamide and 1 gram Methylprednisolone. The patient responded well to therapy and was discharged home with 60 mg of Prednisone taper after a twelve day hospital course. Pt Also required supplemental oxygen upon discharge.
DAH is a prominent and life-threatening pulmonary manifestation of ANCA-associated vasculitis. Mortality due to DAH remains very high with an early mortality rate of 60%. Patients with alveolar hemorrhage due to GPA typically present with the rapid onset of dyspnea and may also have cough, hemoptysis, anemia, and/or hypoxic respiratory failure. Untreated, they may progress rapidly to respiratory failure. Immediate immunosuppressive therapy is the cornerstone of treatment. DAH may be the initial manifestation of GPA as seen in this case. Early diagnosis and aggressive immunosuppression is critical in patient outcome
Author Block: O. Sheriff; Pulmonary Critical Care, Drexel University, Philadelphia, PA, United States.
Diffuse Alveolar hemorrhage due to alveolar capillaritis is a rare life-threatening pulmonary manifestation of Systemic Granulomatous with Polyangitits and occurs in 5-10 percent of patients. Prognosis is poor in DAH with in-hospital mortality ranging from 20 percent to 100 percent.
Patient is a 52 year old Caucasian female without a significant past medical history who presented to the Emergency Department with progressive dyspnea on exertion, nasal congestion, and cough with intermittent hemoptysis over eight weeks. Patient complained of migratory joint pain in her wrists, nasal congestion, and persistent upper respiratory tract infection symptoms despite multiple courses of antibiotics such as Levaquin 750 mg, for seven days, Azithromycin 250 mg for five days, and Augmentin 875-125 mg for ten days over one month prior to admission. Upon presentation she was hemodynamically stable and saturating 97-98 percent on room air. Patient was empirically started on Vancomycin 1000 mg q 12h and Cefepime 1 gram q 6h, for presumed pneumonia. Laboratories revealed a 6 gram hemoglobin drop from her baseline of 13 , 1+ proteinuria, 2+ hematuria with 31-50 RBC, elevated CRP at 21.90 and ESR of 55. Chest x-ray showed a large right lung pneumonic infiltrate concentrated in the right middle and lower lobes. CT scan of the chest showed bilateral diffuse ground glass opacities right greater than left lung with scattered granulomas. Patient was intubated and underwent bronchoscopy which revealed severe diffuse alveolar hemorrhage. Further work up revealed 1:320 titer Cytoplasmic antineutrophil cytoplasmic antibodies , Antiproteinase 3 (PR-3) Ab greater than 100.0 U/mL. The patient underwent right IR Guided kidney biopsy. Pathology showed pauci-immune crescentic glomerulonephritis consistent with Granulomatous with Polyangitits. Patient was started on 1 gram Cyclophosphamide and 1 gram Methylprednisolone. The patient responded well to therapy and was discharged home with 60 mg of Prednisone taper after a twelve day hospital course. Pt Also required supplemental oxygen upon discharge.
DAH is a prominent and life-threatening pulmonary manifestation of ANCA-associated vasculitis. Mortality due to DAH remains very high with an early mortality rate of 60%. Patients with alveolar hemorrhage due to GPA typically present with the rapid onset of dyspnea and may also have cough, hemoptysis, anemia, and/or hypoxic respiratory failure. Untreated, they may progress rapidly to respiratory failure. Immediate immunosuppressive therapy is the cornerstone of treatment. DAH may be the initial manifestation of GPA as seen in this case. Early diagnosis and aggressive immunosuppression is critical in patient outcome
|
Home
|
Inbox
|
Search
|