Successful Treatment of Fibrosing Organising Pneumonia Causing Respiratory Failure with Mycophenolic Acid.
Organising pneumonia (OP) is usually promptly responsive to corticosteroid treatment. We describe a series of 3 cases of severe, progressive, biopsy-proven fibrosing OP causing respiratory failure. All cases presented with peribronchial and subpleural consolidations, had a fibro-inflammatory infiltrative component in the alveolar septa, and only had a partial and unsatisfactory response to corticosteroids. However, they responded to mycophenolic acid (MPA) treatment with resolution of respiratory failure as well as clinical and functional improvement. MPA as an additional treatment option for aggressive forms of fibrosing OP and interstitial lung disease needs to be further explored.
Division of Respirology, Western University, London, Ont., Canada.
Christina
C
Paul
Ammy
A
Lin-Shaw
Mariamma
M
Joseph
Keith
K
Kwan
Gianluigi
G
Sergiacomi
Marco
M
Mura
D000328:Adult; D000368:Aged; D018549:Cryptogenic Organizing Pneumonia / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D004791:Enzyme Inhibitors / Q000627:therapeutic use*; D005260:Female; D005355:Fibrosis; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000627:therapeutic use*; D013902:Radiography, Thoracic; D012131:Respiratory Insufficiency / Q000209:etiology; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D004791:Enzyme Inhibitors; D009173:Mycophenolic Acid