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Page 5 Bronchiolar
Airway Disorders and Bronchiolitis Obliterans
Miscellaneous Bronchiolitis Obliterans Aspiration bronchiolitis obliterans was first reported in 1908 that occurred in a 2.5 year old child who aspirated a prune pit that initially caused cyanosis, then stabilization but respiratory failure and death at eight 8 weeks (65). Activated charcoal used for management of a medication-related suicide attempt was the cause of bronchiolitis obliterans in a 16 year-old patient (66). Gastroesophageal reflux-related bronchiolitis obliterans was reported in a 51 year-old woman who had undergone a heart-lung transplant for primary pulmonary hypertension (67). She had massive gastroesophageal reflux associated with gastric hypotonia, delayed gastric emptying and pyloric spasm. The patient underwent a successful gastro-jejunostomy 19 months after the heart-lung transplantation that relieved the symptoms and prevented recurrent aspiration with corresponding improvement in the FEV1. Stevens-Johnson-related bronchiolitis obliterans has been described in a 41 year-old woman who developed wheezing and progressive dyspnea 12 days after development of diffuse erythematous lesions associated with blisters after treatment with oral ampicillin and intravenous cephamandole (68). Her respiratory condition deteriorated, hypercapnia developed and mechanical ventilation was begun. Despite intensive management, she died two months later. Autopsy showed obliteration of the bronchi with fibrous scar tissue and bronchiolitis obliterans. Carcinoid-related bronchiolitis obliterans has been described in eight patients among 25 consecutive patients undergoing lung resection for peripheral carcinoid (5). It was concluded that associated bronchiolar fibrosis occurs in a high proportion of these patients, but it is usually asymptomatic. There has been a report of multiple tumorlets and microcarcinoids occurring within the bronchioles resulting in a progressive obliterative bronchiolitis and severe airflow obstruction (69). The report was of a 42 year-old woman with ten years of progressive airflow limitation. She had smoked for five years. The FEV1 was 0.84 liters and the chest radiograph showed hyperinflated lungs with several 5 millimeter opacities in the right lung. She returned 11 years later at age 53 with bilateral inspiratory crackles, a chest radiograph showing hyperinflated lungs and multiple 5 millimeter opacities, and the FEV1 had deteriorated to 0.48 liters (15% of the predicted value). The patient underwent successful single lung transplantation. The resected lung showed parenchymal nodules from two to ten millimeters in diameter consisting of central areas of fibrosis surrounded by islands of uniform cells extending into surrounding lung. The cells stained positive for neuron specific endolase, chromogranin A, bombesin, and calcitonin, confirming their neuroendocrine origin. Many of the nodules appeared to have obliterated the accompanying bronchioles by inducing fibroblast proliferation and connective tissue deposition. The patient remained well two years after transplant and there was no evidence of the nodules developing in the transplanted lung. Primary biliary cirrhosis bronchiolitis obliterans has been reported in a 39 year woman (70). Bronchoalveolar lavage showed 73% neutrophils, 8% lymphocytes and 19% macrophages. There was some improvement with corticosteroid therapy, but later worsening. The patient was admitted for acute respiratory failure and died of septic shock. Lung biopsy showed that in severely affected areas, there was concentric submucosal fibrosis and scarring constricted the lumen of the airway, sometimes leading to bronchiolar destruction with fibrous occlusion of the airway. Immunohistochemical studies suggested the occurrence of an immunological process resembling rejection or graft-versus-host reaction simultaneously affecting both organs, and may be part of a generalized autoimmune process in primary biliary cirrhosis. The authors noted that the clinical examination for sicca syndrome was negative and a Schirmer test for decreased lachrymal secretion was negative, ruling out Sjogrenís syndrome as a cause of the airway disease. Sauropus androgynus constrictive bronchiolitis has been reported among a group of patients who developed progressive dyspnea and cough after ingesting S. androgynus juice in Taiwan (71). S. androgynus is a leafy vegetable cultivated in India, Malaysia, Indonesia, southwest China and Vietnam. The leaf of the vegetable is normally ingested after cooking. It has been blended with pineapples or guavas to make a mixed vegetable-fruit juice and consumed for its alleged effects of body weight reduction and in blood pressure control. For many of these patients, they remained with chronic dyspnea, severe airflow obstruction with FEV1 values less than one liter, and severely decreased diffusing capacity despite corticosteroid therapy. FUTURE CONSIDERATIONS The characterization of acute and chronic bronchiolitis in the adult represents an important need. Epidemiological studies of these disorders would be very helpful in establishing the clinical course and outcome of these disorders. The development of new therapeutic agents for idiopathic and connective tissue bronchiolitis obliterans is needed. Multicenter immunosuppressive therapy studies are needed to determine optimal therapy for lung transplant bronchiolitis obliterans |
