Diaphragmatic Amyloidosis Causing Respiratory Failure: A Case Report and Review of Literature
Neuromuscular respiratory failure is a rare complication of systemic immunoglobulin light chain amyloidosis. We describe a case of a 70-year-old Caucasian man with multiple myeloma who presented with worsening dyspnea. The patient was diagnosed with and treated for congestive heart failure but conti...
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| Format: | Article |
| Language: | English |
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Wiley
2015-01-01
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| Series: | Case Reports in Oncological Medicine |
| Online Access: | http://dx.doi.org/10.1155/2015/917157 |
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| author | Aleksey Novikov Horatio Holzer Robert A. DeSimone Ghaith Abu-Zeinah David J. Pisapia Tomer M. Mark Raymond D. Pastore |
| author_facet | Aleksey Novikov Horatio Holzer Robert A. DeSimone Ghaith Abu-Zeinah David J. Pisapia Tomer M. Mark Raymond D. Pastore |
| author_sort | Aleksey Novikov |
| collection | DOAJ |
| description | Neuromuscular respiratory failure is a rare complication of systemic immunoglobulin light chain amyloidosis. We describe a case of a 70-year-old Caucasian man with multiple myeloma who presented with worsening dyspnea. The patient was diagnosed with and treated for congestive heart failure but continued to suffer from hypercapnic respiratory insufficiency. He had restrictive physiology on pulmonary function tests and abnormal phrenic nerve conduction studies, consistent with neuromuscular respiratory failure. The diagnosis of systemic immunoglobulin light chain amyloidosis was made based on the clinical context and a cardiac biopsy. Despite treatment attempts, the patient passed away in the intensive care unit from hypercapnic respiratory failure. Autopsy revealed dense diaphragmatic amyloid deposits without phrenic nerve infiltration or demyelination or lung parenchymal involvement. Only 5 cases of neuromuscular respiratory failure due to amyloid infiltration of the diaphragm have been described. All cases, including this, were characterized by rapid progression and high mortality. Therefore, diaphragmatic amyloidosis should be on the differential for progressive neuromuscular respiratory failure in patients with multiple myeloma or any other monoclonal gammopathy. Given its poor prognosis, early recognition of this condition is essential in order to address goals of care and encourage pursuit of palliative measures. |
| format | Article |
| id | doaj-art-8c159e78238a4b45aea0856904cb4976 |
| institution | Kabale University |
| issn | 2090-6706 2090-6714 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Oncological Medicine |
| spelling | doaj-art-8c159e78238a4b45aea0856904cb49762025-02-03T01:11:13ZengWileyCase Reports in Oncological Medicine2090-67062090-67142015-01-01201510.1155/2015/917157917157Diaphragmatic Amyloidosis Causing Respiratory Failure: A Case Report and Review of LiteratureAleksey Novikov0Horatio Holzer1Robert A. DeSimone2Ghaith Abu-Zeinah3David J. Pisapia4Tomer M. Mark5Raymond D. Pastore6Department of Internal Medicine, New York Presbyterian Hospital, Weill Cornell Medical College, New York, NY 10065-4897, USADepartment of Internal Medicine, New York Presbyterian Hospital, Weill Cornell Medical College, New York, NY 10065-4897, USADepartment of Pathology and Laboratory Medicine, New York Presbyterian Hospital, Weill Cornell Medical College, New York, NY 10065-4897, USADepartment of Internal Medicine, New York Presbyterian Hospital, Weill Cornell Medical College, New York, NY 10065-4897, USADepartment of Pathology and Laboratory Medicine, New York Presbyterian Hospital, Weill Cornell Medical College, New York, NY 10065-4897, USADepartment of Internal Medicine, New York Presbyterian Hospital, Weill Cornell Medical College, New York, NY 10065-4897, USADepartment of Internal Medicine, New York Presbyterian Hospital, Weill Cornell Medical College, New York, NY 10065-4897, USANeuromuscular respiratory failure is a rare complication of systemic immunoglobulin light chain amyloidosis. We describe a case of a 70-year-old Caucasian man with multiple myeloma who presented with worsening dyspnea. The patient was diagnosed with and treated for congestive heart failure but continued to suffer from hypercapnic respiratory insufficiency. He had restrictive physiology on pulmonary function tests and abnormal phrenic nerve conduction studies, consistent with neuromuscular respiratory failure. The diagnosis of systemic immunoglobulin light chain amyloidosis was made based on the clinical context and a cardiac biopsy. Despite treatment attempts, the patient passed away in the intensive care unit from hypercapnic respiratory failure. Autopsy revealed dense diaphragmatic amyloid deposits without phrenic nerve infiltration or demyelination or lung parenchymal involvement. Only 5 cases of neuromuscular respiratory failure due to amyloid infiltration of the diaphragm have been described. All cases, including this, were characterized by rapid progression and high mortality. Therefore, diaphragmatic amyloidosis should be on the differential for progressive neuromuscular respiratory failure in patients with multiple myeloma or any other monoclonal gammopathy. Given its poor prognosis, early recognition of this condition is essential in order to address goals of care and encourage pursuit of palliative measures.http://dx.doi.org/10.1155/2015/917157 |
| spellingShingle | Aleksey Novikov Horatio Holzer Robert A. DeSimone Ghaith Abu-Zeinah David J. Pisapia Tomer M. Mark Raymond D. Pastore Diaphragmatic Amyloidosis Causing Respiratory Failure: A Case Report and Review of Literature Case Reports in Oncological Medicine |
| title | Diaphragmatic Amyloidosis Causing Respiratory Failure: A Case Report and Review of Literature |
| title_full | Diaphragmatic Amyloidosis Causing Respiratory Failure: A Case Report and Review of Literature |
| title_fullStr | Diaphragmatic Amyloidosis Causing Respiratory Failure: A Case Report and Review of Literature |
| title_full_unstemmed | Diaphragmatic Amyloidosis Causing Respiratory Failure: A Case Report and Review of Literature |
| title_short | Diaphragmatic Amyloidosis Causing Respiratory Failure: A Case Report and Review of Literature |
| title_sort | diaphragmatic amyloidosis causing respiratory failure a case report and review of literature |
| url | http://dx.doi.org/10.1155/2015/917157 |
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