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A Case of Alveolar Hemorrhage, ANCA-Associated Vasculitis, and Rheumatoid Arthritis
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A3091 - A Case of Alveolar Hemorrhage, ANCA-Associated Vasculitis, and Rheumatoid Arthritis
Author Block: B. E. Henkle, H. Kim; Internal Medicine, Division of Pulmonary, Allergy, Critical Care, and Sleep, University of Minnesota, Minneapolis, MN, United States.
Diffuse alveolar hemorrhage (DAH) is a clinical syndrome characterized by widespread intra-alveolar hemorrhage due to pulmonary microcirculation injury and may be localized to the lung or associated with systemic disorders. It presents with hemoptysis, bilateral pulmonary opacities, and anemia. In rare instances, DAH may present with an ANCA-associated vasculitis (AAV) and rheumatoid arthritis (RA) in an overlap syndrome.
A 65-year-old woman with a history of seropositive RA on chronic prednisone and breast cancer with metastatic disease to bone presented for evaluation of progressive bilateral ground glass opacities (GGO) on computed tomography (CT) imaging of the chest. Bilateral GGOs were incidentally found approximately 10 months prior during routine imaging obtained as part of her oncology evaluation and repeat imaging at 5 and 9 months showed increasing opacities. The patient reported several months of mild but progressive dyspnea on exertion and low energy. There was no evidence of infection, congestive heart failure, or aspiration noted and echo was normal. The patient was scheduled for bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial cryoprobe biopsy. Upon presentation for bronchoscopy the patient was pale and dyspneic and laboratory tests revealed new renal failure, creatinine 2.7 mg/dL, and worsening anemia, hemoglobin 4.7 g/dL. She was admitted for further evaluation. Bronchoscopy with BAL was obtained on hospital day 4 and revealed DAH. Subsequent work up showed a myeloperoxidase-ANCA titer of 1:5120 as well as an elevated rheumatoid factor, anti-citrullinated peptide antibodies, and anti-nuclear antibodies, however, no lung or renal biopsies were obtained. She was treated with pulse-dose intravenous glucocorticoids followed by a prednisone taper and rituximab. Follow up over 2 months later showed a CT chest with almost complete resolution of GGOs and improvement in both renal function and anemia.
RA and AAV are both autoimmune diseases that have significant morbidity and, at times, mortality. DAH is an infrequent pulmonary manifestation of RA but is a more common and severe manifestation of AAV with a mortality up to 30%. In rare instances, patients with rheumatoid arthritis may present with DAH due to a new concomitant AAV. Immunological association between AAV and RA is not clear but may be secondary to genetic predispositions to autoimmunity. Prompt recognition of this uncommon overlap syndrome is required to achieve survival in this patient group.
Author Block: B. E. Henkle, H. Kim; Internal Medicine, Division of Pulmonary, Allergy, Critical Care, and Sleep, University of Minnesota, Minneapolis, MN, United States.
Diffuse alveolar hemorrhage (DAH) is a clinical syndrome characterized by widespread intra-alveolar hemorrhage due to pulmonary microcirculation injury and may be localized to the lung or associated with systemic disorders. It presents with hemoptysis, bilateral pulmonary opacities, and anemia. In rare instances, DAH may present with an ANCA-associated vasculitis (AAV) and rheumatoid arthritis (RA) in an overlap syndrome.
A 65-year-old woman with a history of seropositive RA on chronic prednisone and breast cancer with metastatic disease to bone presented for evaluation of progressive bilateral ground glass opacities (GGO) on computed tomography (CT) imaging of the chest. Bilateral GGOs were incidentally found approximately 10 months prior during routine imaging obtained as part of her oncology evaluation and repeat imaging at 5 and 9 months showed increasing opacities. The patient reported several months of mild but progressive dyspnea on exertion and low energy. There was no evidence of infection, congestive heart failure, or aspiration noted and echo was normal. The patient was scheduled for bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial cryoprobe biopsy. Upon presentation for bronchoscopy the patient was pale and dyspneic and laboratory tests revealed new renal failure, creatinine 2.7 mg/dL, and worsening anemia, hemoglobin 4.7 g/dL. She was admitted for further evaluation. Bronchoscopy with BAL was obtained on hospital day 4 and revealed DAH. Subsequent work up showed a myeloperoxidase-ANCA titer of 1:5120 as well as an elevated rheumatoid factor, anti-citrullinated peptide antibodies, and anti-nuclear antibodies, however, no lung or renal biopsies were obtained. She was treated with pulse-dose intravenous glucocorticoids followed by a prednisone taper and rituximab. Follow up over 2 months later showed a CT chest with almost complete resolution of GGOs and improvement in both renal function and anemia.
RA and AAV are both autoimmune diseases that have significant morbidity and, at times, mortality. DAH is an infrequent pulmonary manifestation of RA but is a more common and severe manifestation of AAV with a mortality up to 30%. In rare instances, patients with rheumatoid arthritis may present with DAH due to a new concomitant AAV. Immunological association between AAV and RA is not clear but may be secondary to genetic predispositions to autoimmunity. Prompt recognition of this uncommon overlap syndrome is required to achieve survival in this patient group.
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