The September 11, 2001 terrorist attack in New York City (NYC) that caused the collapse of the World Trade Center (WTC) resulted in the release of large amounts of smoke, debris, and particulate matter into the air. Many people involved in the initial rescue and recovery effort were exposed to these respirable materials. There has been an increasing number of pulmonary diseases reported in association with this exposure. We report a case of Swyer-James-Macleod Syndrome (SJMS) in a WTC first responder.
A 38-year-old male nonsmoker presented with 2 years of dyspnea on minimal exertion, cough, and wheezing. He was a first responder at WTC, “Ground Zero,” during which time he was exposed to particulate matter in the air. Prior to this, he was able to run miles and perform various exercises for his police department training. In 2002, he underwent treatment for “pneumonia”. In 2007, during WTC screening at Mt. Sinai, NYC, he reported a negative chest radiograph. Review of systems revealed occasional right-sided chest pressure on exertion. He noted symptomatic relief with albuterol meter-dose-inhaler. Physical examination showed reduced air-entry over the right chest without wheezing or rhonchi. Oxygen saturation on room air decreased to 92% while walking. Pulmonary function testing showed mild restrictive lung disease with minimal response to bronchodilator therapy. Computed tomography (CT) of chest demonstrated a hyperlucent right upper lobe with oligemic lung field consistent with SJMS (Figure 1).
To our knowledge, this is the first case reported of SJMS occuring after exposure to WTC respirable particulate matter. Exposure to materials in the air caused by the collapse of the WTC has been reported to result in bronchial responsiveness, cough, sarcoid-like granulomatous disease as well as bronchiolitis obliterans. SJMS is a rare disorder characterized radiographically by a unilateral hyperlucent lung. It is believed to be a postinfectious manifestation of childhood bronchiolitis obliterans caused by injury of the immature lung. Acute viral respiratory infection during the first 8 years of life is thought to be the main causative factor, however about 40% of cases have documented no history of episodes of childhood respiratory infection. The diagnosis is usually made based on clinical and radiographic findings rather than by pathology. When pathologic specimens have been examined, they have shown bronchiolitis obliterans with various degrees of chronic inflammation, fibrosis, and dilatation of airways and air spaces distal to the obstructed bronchioles. Air trapping results from air entering the spaces via collateral air drift but being unable to exit due to bronchiolar obstruction. Chest CT Scan is valuable because it eliminates other diagnoses that may mimic unilateral hyperlucency, such as central bronchial obstruction, cysts, and vascular disease. The prognosis and severity of SJMS is variable and depends on the development of bronchiectasis and frequency of recurrent pneumonia.
Although pulmonary disease has been reported following WTC exposure, this is the first reported case of SJMS. Our previously healthy male presented with dyspnea on exertion, persistent cough, wheezing, and chest pressure following exposure to WTC debris. We believe the inhalation of toxic material during the intense short-term exposure to WTC led to bronchiolitis obliterans and subsequently to SJMS. Absence of respiratory symptoms with normal functioning capacity prior to 9/11/2001 emphasizes that the WTC exposure was the cause of his disease. This suggests that when presented with a case of SJMS, a history of toxic inhalation should be elicited along with that of recurrent respiratory infections.
Ruby Varghese, No Financial Disclosure Information; No Product/Research Disclosure Information