2022
DOI: 10.2169/internalmedicine.8758-21
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Cryptogenic Fibrosing Pleuritis with Rapidly Progressive Restrictive Ventilatory Dysfunction

Abstract: Cryptogenic bilateral fibrosing pleuritis is a rare condition, and its pathogenesis and clinical course are poorly understood, with no established therapy available. A 61-year-old man presented with bilateral pleural thickening and lymphocytic exudative effusions. The patient was diagnosed with fibrosing pleuritis with no evidence of a known etiology on a surgical pleural biopsy. Within 16 months from the onset of respiratory symptoms, restrictive ventilatory impairment progressed rapidly, resulting in hyperca… Show more

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(2 citation statements)
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“…In stage III (dorsal or thoracic) of ankylosing spondylitis (AS), patients may experience chest and thoracoiabdominal pain, along with limited thorax expansion due to inflammation-related damage to the costovertebral and sternocostal joints pleural effusion, 21,22 whether caused by tuberculosis, tumors, connective tissue diseases, or other pleural diseases such as pleural mesothelioma, can also restrict the expansion of thoracic or lung tissue and reduce the compliance of thoracic and/or lung. 23…”
Section: Overview Of Respiratory Diseasesmentioning
confidence: 99%
See 1 more Smart Citation
“…In stage III (dorsal or thoracic) of ankylosing spondylitis (AS), patients may experience chest and thoracoiabdominal pain, along with limited thorax expansion due to inflammation-related damage to the costovertebral and sternocostal joints pleural effusion, 21,22 whether caused by tuberculosis, tumors, connective tissue diseases, or other pleural diseases such as pleural mesothelioma, can also restrict the expansion of thoracic or lung tissue and reduce the compliance of thoracic and/or lung. 23…”
Section: Overview Of Respiratory Diseasesmentioning
confidence: 99%
“…In stage III (dorsal or thoracic) of ankylosing spondylitis (AS), patients may experience chest and thoracoiabdominal pain, along with limited thorax expansion due to inflammation-related damage to the costovertebral and sternocostal joints pleural effusion, 21,22 whether caused by tuberculosis, tumors, connective tissue diseases, or other pleural diseases such as pleural mesothelioma, can also restrict the expansion of thoracic or lung tissue and reduce the compliance of thoracic and/or lung. 23 Furthermore, obesity can contribute to significant restrictive lung physiology and give rise to obesity hypoventilation syndrome (OHS). 24 Individuals with severe obesity may experience severe and potentially life-threatening respiratory complications.…”
Section: Restrictive Pulmonary Disease With Ventilation Dysfunctionmentioning
confidence: 99%