It is also important to take note of the size of the esophagus on CT if the patient may have connective tissue disease. This patient has a dilated esophagus, and manometry studies show markedly abnormal esophageal function with decreased motility and increased reflux events. The differential becomes one of the connective tissue disorders, with scleroderma being the primary consideration, but with mixed connective tissue disorders also being possible. We haven’t ruled out the moldy basement being a contributor, but it seems less likely in this instance. Taken together, the changes in the clinical setting and given the fact that we have biochemical data to suggest that there may be elevated ANA and ESR, these features are more in keeping with a non-idiopathic cause.

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Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline