INTRODUCTION: Bilateral pulmonary infiltrates and fever can be caused by a number of primary lung disorders, however it is also important to look for and exclude extra-pulmonary conditions.
CASE PRESENTATION: A 50 yo Caucasian male presented with a 5 month history of cough and wheezing, mostly in the morning, along with greenish sputum. There were low grade fevers of 100-101 F occurring in the evening, along with night sweats. He had lost 30 lb over the course of the illness. He was diagnosed with pneumonia on the basis of a chest roentgenogram. He was treated with prolonged courses of antibiotics with some improvement in symptoms but persistence of infiltrates. He also had a history of achalasia diagnosed 14 years ago, and had been treated with dilatations and a Botox injection. He reported occasional difficulties swallowing as well as occasional regurgitation of fluids when recumbent. His physical exam was unremarkable, the lungs were clear. His laboratory findings revealed a leukocytosis of 13600 with a left shift. A recent bronchoscopy revealed no endobronchial pathology, and cultures for fungal, bacterial and mycobacterial organisms were negative. His chest roentgenogram showed right upper lobe consolidation and patchy infiltrates in the right and left lower lobes. His chest CT demonstrated similar findings in addition to proximal esophageal dilatation with retained material. The patient underwent repeat esophagogastroduodenoscopy which ruled out masses or pseudoachalasia. Esophageal manometry was suggestive of achalasia. He was referred to thoracic surgery and underwent a laparoscopic Heller myotomy with a partial anterior Dor fundoplication. On return, several weeks later, the swallowing symptoms were much improved, there were no gastroesophageal reflux (GERD) symptoms, the cough disappeared and the CT scan showed improvement in the bilateral infiltrates.
DISCUSSIONS: Achalasia is an idiopathic esophageal dysmotility disorder characterized by loss of peristalsis in the distal two thirds of the esophageus, along with impaired relaxation of the lower esophageal sphincter. It usually presents with gradual dysphagia for solids and liquids, and regurgitation of food. The latter symptom becomes more pronounced as the esophagus progressively dilates. Reflux frequently occurs during sleep, and in the recumbent position. Therefore, aspiration may occur and lead to acute and chronic lung infections. Non tuberculous mycobacterial infections have been described in this setting, although our patient did not have it. Diagnosis of achalasia is confirmed by radiographic, endoscopic and manometric data. It is important to rule out pseudoachalasia that can result from primary or metastatic tumors invading the GE junction. Pulmonary findings associated with achalasia include aspiration pneumonia and diffuse aspiration bronchiolitis (DAB). DAB was initially described in the elderly and later recognized in younger individuals with risk factors like achalasia and GERD. It is an insidious and chronic condition with waxing and waning lung infiltrates. Lung pathology reveals a foreign body reaction in bronchioles. It can be differentiated from pure aspiration pneumonia by the acuity and severity of symptoms present in the latter. We believe our patient falls into the DAB category.
CONCLUSION: Achalasia is associated with increased risks of aspiration, especially later in the course of the disease. Pulmonary infiltrates can develop, either acutely or chronically, as a result of aspiration pneumonia or DAB. Treatment of the achalasia usually results in marked improvement of the pulmonary findings.
DISCLOSURE: Joanne Shirine Allam, None.