INTRODUCTION: Acute fibrinous and organizing pneumonia (AFOP) has been recently identified as an unusual variant of acute lung injury. The typical features of AFOP are an acute or subacute onset of illness, bilateral basilar infiltrate and histologic findings of intra-alveolar fibrin in the form of fibrin “balls”. All the previously reported seventeen patients with AFOP were identified by a retrospective review of case material at the Armed Forces Institute of Pathology. We report a patient who had received a heart transplant and developed AFOP while on corticosteroids and other immunosuppressants. To the best of our knowledge, this is the first prospectively diagnosed patient with AFOP.
CASE PRESENTATION: A 70 year old white male was referred to the pulmonary clinic for evaluation of a recent increase in shortness of breath on exertion with wheezing but no other Sympotms. He had received a cardiac transplant eight years ago for ischemic cardiomyopathy and had been receiving cyclosporine and prednisone until two months ago when prednisone was discontinued. In addition, he had twenty pack-year history of smoking (with abstinence since cardiac transplant), mild COPD, and a prior positive PPD skin test for which he received INH for six months.On physical exam, the patient had bilateral wheezing and tachycardia. Pulmonary function testing revealed mild worsening of his baseline airway obstruction with significant improvement post bronchodilator. The chest x-ray was normal, but CT scan of the chest showed central bronchiectasis. The patient's airway obstruction responded both clinically and spirometrically to a tapering dose of prednisone for six weeks. First relapse: when the prednisone dose was decreased to 10 mg po qd, the patient developed productive cough, fever, chills and a left lower lung infiltrate on chest X-ray. Bronchoscopy was performed, but the bronchalveolar lavage, the microbiology and cytology brushes, and transbronchial biopsy were non-diagnostic. He was treated with a two-week course of Levofloxacin and prednisone was again increased to 40 mg po qd. The patient improved clinically and radiographically within one month. Second relapse: two months later, he again developed severe dyspnea, fever, and productive cough. Chest x-ray showed a new infiltrate in the left lower lung. The patient was admitted to the hospital and treated with intravenous methylprednisolone and broad spectrum antibiotics. Bronchoscopy with bronchial wash and microbiology and cytology brushes was non-diagnostic once more. The patient's respiratory status deteriorated with worsening dyspnea, hypoxemia, and progressive bilateral interstitial markings. Multiple surgical biopsies from the left upper and the left lower lobes showed acute fibrinous and organizing pneumonia with prominent intra-alveolar fibrin balls.The patient showed significant improvement with a maintenance dose of prednisone 15 mg po qd. Follow up: six months after the hospital discharge he was stable and clinical and physiological functions had returned to baseline.
DISCUSSIONS: Our patient is the first reported patient with complete clinical description of his pulmonary status before developing the disease as well as during follow-up and recovery. He presented at age 70, which was within the range reported by Travis (33-78, average 62 years). When added to Travis' data, race continues to be non-contributory with 4 hispanics, 1 black, and now 11 whites affected (2 cases had unknown race)(1).Our patient is interesting in that he has history of obstructive airway disease, which may represent a risk factor for future development of AFOP under certain triggering factors.
CONCLUSION: Acute fibrinous and organizing pneumonia is likely underreported and under diagnosed. Identification of precipitating factors, clinical course, and factors affecting prognosis and treatment still need to be established.
DISCLOSURE: Mohammad Jarbou, None.