Unknown ethiology, Challenging differential diagnosis, Unusual or unexpected effect of treatment, Educational Purpose (only if useful for a systematic review or synthesis)
Daniel Arnaud, Zoya Surani, Abhay Vakil, Joseph Varon, Salim Surani
(Department of Research, Dorrington Medical Associates, Houston, USA)
Am J Case Rep 2017; 18:1242-1246
Acute fibrinous organizing pneumonia (AFOP) is a rare condition of the lung that is associated with acute lung injury, and has a poor prognosis. AFOP is characterized histologically by intra-alveolar fibrin. AFOP has been described to be associated with lung infections, connective tissue disorders, drugs, toxic environmental exposure, and in lung transplantation. However, most cases of AFOP remain idiopathic, and because the condition can present with a wide variety of clinical manifestations, open lung biopsy or video-assisted thoracoscopic (VAT) lung biopsy is necessary for the diagnosis. Currently, treatments for AFOP remain under investigation.
CASE REPORT: A 35-year-old woman presented with a cough and dyspnea, and was initially diagnosed to have pneumonia. Due to the progression of her symptoms and increasing respiratory failure she underwent video-assisted thoracoscopic (VAT) biopsy and was diagnosed with AFOP, 19 days following hospital admission. She was treated with mechanical ventilation, intravenous steroids, and cyclophosphamide. She required tracheostomy after 14 days of mechanical ventilation and died two weeks later.
CONCLUSIONS: AFOP is an uncommon clinical condition, with a poor prognosis, which often has a delay in diagnosis. Some patients benefit from steroids and immunosuppressive therapy. Currently, new treatments for AFOP are under investigation.
Keywords: acute lung injury, bronchopneumonia, Thoracoscopy