Pulmonary alveolar proteinosis - a crazy presentation of dyspnea
This case report demonstrates 44-year old man, presenting with recurring clinical pneumonias during a period of over 1 year. The patient was clinically affected with, i.a., weight loss, finger clubbing and severely reduced diffusion capacity. Repetitive chest x-rays showed bilateral and consolidatin...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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Taylor & Francis Group
2019-01-01
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| Series: | European Clinical Respiratory Journal |
| Subjects: | |
| Online Access: | http://dx.doi.org/10.1080/20018525.2018.1552065 |
| _version_ | 1818258217497526272 |
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| author | Inge Raadal Skov Elisabeth Bendstrup Jesper Rømhild Davidsen |
| author_facet | Inge Raadal Skov Elisabeth Bendstrup Jesper Rømhild Davidsen |
| author_sort | Inge Raadal Skov |
| collection | DOAJ |
| description | This case report demonstrates 44-year old man, presenting with recurring clinical pneumonias during a period of over 1 year. The patient was clinically affected with, i.a., weight loss, finger clubbing and severely reduced diffusion capacity. Repetitive chest x-rays showed bilateral and consolidating infiltrates, and a high-resolution computed tomography of the thorax (HRCT) exposed ground glass opacities superimposed on a reticular pattern as the so-called ‘crazy paving’ pattern. A bronchoscopic alveolar lavage revealed alveolar proteinaceous material compatible with the diagnosis pulmonary alveolar proteinosis (PAP). PAP is a rare syndrome where surfactant is accumulated in the alveoli, causing respiratory disease in typically young to middle-aged patients with male predominance. Both symptoms and prognosis are variable, and range from spontaneous remission to terminal respiratory failure. The standard treatment is whole lung lavage, where surfactant is mechanically rinsed from the lungs. The lack of specific clinical symptoms makes it easy to overlook the diagnosis, as supported by this case report. It serves as a reminder, that the findings of a crazy paving pattern on HRCT in young adults should alert of this rare disease, and advises on the further examinations required to make the diagnosis. |
| first_indexed | 2024-12-12T17:56:02Z |
| format | Article |
| id | doaj.art-6ffc9bafc5e34f4ca1eb08584cfab172 |
| institution | Directory Open Access Journal |
| issn | 2001-8525 |
| language | English |
| last_indexed | 2024-12-12T17:56:02Z |
| publishDate | 2019-01-01 |
| publisher | Taylor & Francis Group |
| record_format | Article |
| series | European Clinical Respiratory Journal |
| spelling | doaj.art-6ffc9bafc5e34f4ca1eb08584cfab1722022-12-22T00:16:44ZengTaylor & Francis GroupEuropean Clinical Respiratory Journal2001-85252019-01-016110.1080/20018525.2018.15520651552065Pulmonary alveolar proteinosis - a crazy presentation of dyspneaInge Raadal Skov0Elisabeth Bendstrup1Jesper Rømhild Davidsen2Odense University HospitalAarhus University HospitalOdense University HospitalThis case report demonstrates 44-year old man, presenting with recurring clinical pneumonias during a period of over 1 year. The patient was clinically affected with, i.a., weight loss, finger clubbing and severely reduced diffusion capacity. Repetitive chest x-rays showed bilateral and consolidating infiltrates, and a high-resolution computed tomography of the thorax (HRCT) exposed ground glass opacities superimposed on a reticular pattern as the so-called ‘crazy paving’ pattern. A bronchoscopic alveolar lavage revealed alveolar proteinaceous material compatible with the diagnosis pulmonary alveolar proteinosis (PAP). PAP is a rare syndrome where surfactant is accumulated in the alveoli, causing respiratory disease in typically young to middle-aged patients with male predominance. Both symptoms and prognosis are variable, and range from spontaneous remission to terminal respiratory failure. The standard treatment is whole lung lavage, where surfactant is mechanically rinsed from the lungs. The lack of specific clinical symptoms makes it easy to overlook the diagnosis, as supported by this case report. It serves as a reminder, that the findings of a crazy paving pattern on HRCT in young adults should alert of this rare disease, and advises on the further examinations required to make the diagnosis.http://dx.doi.org/10.1080/20018525.2018.1552065pappulmonary alveolar proteinosisgm-csfgranulocyte-macrophage colony-stimulating factorhrctcrazy pavingwllwhole lung lavagerare lung disease |
| spellingShingle | Inge Raadal Skov Elisabeth Bendstrup Jesper Rømhild Davidsen Pulmonary alveolar proteinosis - a crazy presentation of dyspnea European Clinical Respiratory Journal pap pulmonary alveolar proteinosis gm-csf granulocyte-macrophage colony-stimulating factor hrct crazy paving wll whole lung lavage rare lung disease |
| title | Pulmonary alveolar proteinosis - a crazy presentation of dyspnea |
| title_full | Pulmonary alveolar proteinosis - a crazy presentation of dyspnea |
| title_fullStr | Pulmonary alveolar proteinosis - a crazy presentation of dyspnea |
| title_full_unstemmed | Pulmonary alveolar proteinosis - a crazy presentation of dyspnea |
| title_short | Pulmonary alveolar proteinosis - a crazy presentation of dyspnea |
| title_sort | pulmonary alveolar proteinosis a crazy presentation of dyspnea |
| topic | pap pulmonary alveolar proteinosis gm-csf granulocyte-macrophage colony-stimulating factor hrct crazy paving wll whole lung lavage rare lung disease |
| url | http://dx.doi.org/10.1080/20018525.2018.1552065 |
| work_keys_str_mv | AT ingeraadalskov pulmonaryalveolarproteinosisacrazypresentationofdyspnea AT elisabethbendstrup pulmonaryalveolarproteinosisacrazypresentationofdyspnea AT jesperrømhilddavidsen pulmonaryalveolarproteinosisacrazypresentationofdyspnea |