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 hypercapnic respiratory failure requiring home oxygen and non-invasive positive pressure ventilation therapies.

• Authors: Ayane Torii, Yoshitaka Ono, Shohei Obayashi, Asako Kitahara, Kana Oshinden, Yukihiro Horio, Kyoko Niimi, Naoki Hayama, Tsuyoshi Oguma, Kazuhito Hatanaka, Koichiro Asano, Yoko Ito
• Journal: Internal medicine (Tokyo, Japan) (Intern Med) Vol. 61 Issue 17 Pg. 2649-2653 (Sep 01 2022) ISSN: 1349-7235 [Electronic] Japan
• PMID: 35135921 (Publication Type: Case Reports, Journal Article)

Topics

• Biopsy (adverse effects)
• Fibrosis
• Humans
• Male
• Middle Aged
• Pleura (pathology)
• Pleural Effusion (etiology)
• Pleurisy (complications, diagnosis)
• Respiratory Insufficiency (complications, therapy)