SESSION TYPE: Airway Global Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Bronchiolitis obliterans (BO) is disorder of the small airways, pathologically characterized by obliterative bronchiolitis with circumferential narrowing, ulceration, and scarring of the terminal and respiratory bronchioles. BO is characterized clinically by progressive dyspnea accompanied by dry cough. BO was first described in 1977 in 6 patients, 5 of whom had rheumatoid arthritis (RA).
CASE PRESENTATION: Female 77 years old, mild bronchiectasis, that required multiple ATB in last year's. She was stable receiving meprednisone 60 mg day and mycophenolate, that she left 6 months before. Spirometry showed mild obstruction (FEV1 1,2 liters about 60% of predicted). In the last 3 months dyspnea in progression appeared slowly with a severe obstruction with FEV1 0.3 liters No wheezes and only crackles. X ray normal. CT scan showed mosaic pattern of patchy regions of decreased lung attenuation that were accentuated on expiratory images. She had never received D-penicillamine, nor other drugs associated with BO. A diagnosis of steroid-resistant BO was made. Cyclophosphamide was given as adjunctive therapy, and a rapid improvement was seen. Until today she received 6 cycles of cyclophosphamide with a very important clinical and functional improvement. Now FEV1 is 0.8 liters.
DISCUSSION: BO is disorder of the small airways that generally has a poor prognosis, with inexorable progression and poor response to corticosteroids. On examination, inspiratory crackles and squeaks are heard. Pulmonary function tests (PFTs) show the rapid onset and progression of irreversible airflow limitation as demonstrated by a reduction in forced expiratory volume in 1 second (FEV1) and in the ratio of FEV1 to forced vital capacity (FVC). In addition, hyperinflation and air trapping may be present, although in later stages of disease, both restrictive and obstructive physiology due to the severity of the air trapping may be observed. HRCT may be more sensitive than PFTs for detecting small airways disease. The radiographic appearance is that of moderate to severe air trapping, as demonstrated by a mosaic pattern of patchy or segmental regions of decreased lung attenuation that are accentuated on expiratory images. At times, the clinical presentation and radiographic findings may be difficult to distinguish from those in chronic obstructive pulmonary disease (COPD), and may be accompanied by RA-ILD. The diagnosis of BO is usually made indirectly, using clinical, physiologic, and radiographic criteria. Other causes of small airways disease, such as significant tobacco use, should not be present. When surgical biopsy is obtained the pathologic appearance is fibrotic obliteration of the small airways, often accompanied by a lymphoplasmacytic infiltrate. The lesions tend to be patchy. Several reports suggested an association with both D-penicillamine and gold salts. In a recent series of 25 RA patients with BO, most with minimal or no smoking history, 48% had been treated with D-penicillamine, 40% with gold salts, 52% with methotrexate, and nearly all with corticosteroids. Of note, FEV1 was lower in patients who had ever received D-penicillamine than in those who had not. Other risk factors for BO include female sex and long-standing RA. BO generally has a poor prognosis, with inexorable progression and poor response to corticosteroids. However, response to treatment with corticosteroids and other immunosuppressive medications has been reported, so a trial of such therapy is often attempted.
CONCLUSIONS: In this case report of a patient with steroid resistant BO , Cyclophosphamide was given as adjunctive therapy, and a rapid improvement was seen. An early therapeutic trial of cyclophosphamide should be considered in patients with BO who fail to respond to steroids.
1) Purcell I.F. et al. Cyclophosphamide in severe steroid-resistant bronchiolitis obliterans organizing pneumonia. Respiratory Medicine Volume 91, Issue 3 , Pages 175-177, March 1997
DISCLOSURE: The following authors have nothing to disclose: Juan Enghelmayer, Hector Defranchi, Luis Marquez
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