Idiopathic Pulmonary Fibrosis and Esophageal Cancer, Association or Incidental?

Abstract

INTRODUCTION: Interstitial Lung Disease (ILD) revised and termed as Diffuse Parenchymal Lung Disease (DPLD) is a slowly progressive inflammatory disease of diverse etiology, primarily involving the interstitium and not uncommonly the alveoli and lower airways. Of them, Idiopathic Pulmonary Fibrosis (IPF) is a distinct clinical entity rather than a diagnosis of exclusion, diagnosed with High Resolution Computerised Tomography (HRCT) duly supported by suggestive clinical profile. It is well known for considerable morbidity, progression with reduced survival after daignosis. The course is further complicated and disabling when associated with malignancy. Recognition of squamous cell carcinoma of esophagus in an elderly man, non diabetic, non hypertensive, with established IPF prompted this communication. CASE PRESENTATION: Male, 77 years, presented with progressive dyspnoea in June 2008. Serial clinical and relevant investigative profile ruled out airway and cardiovascular disorders, anaemia, HIV, tuberculosis, bronchiectasis or lung cancer. IPF was diagnosed by HRCT (Fig 1). He was relatively stable while on home oxygen, anti-inflammatory agents including azathioprine and low dose prednisolone. In the recent past he developed anorexia, dysphagia for solids and progressive weakness. Barium swallow (Fig 2) done in March 2012 at our centre revealed obstruction. By esophagogastroduodenoscopy, biopsy of the esophagus specimen showed squamous cell carcinoma of esophagus. He was evaluated by thoracic surgeon who did not suggest surgery and hence he was reffered to palliative care centre locally, for symptomatic, supportive and palliative care. DISCUSSION: Esophageal involvement is described in secondary ILD (eg. systemic sclerosis) but not as a feature of IPF. Lung cancer has been found to have an association with IPF. Though esophageal cancer could be incidental, dysphagia can be mistaken for association related to ILD. CONCLUSIONS: Survival of patients with IPF is related to early diagnosis before progression to significant fibrosis and treatment early with home oxygen and anti-inflammatory drugs. Relatively stable clinical state even in the eighth decade was made possible by regular follow-up and therapy as cited above. Absence of other age related morbidities was in his favour.