DISCUSSION: In addition to our case, we identified nine published cases of WD with pulmonary involvement without GI symptoms. Including the present case the ten cases comprised eight men and two women, with a mean age of 49.5 years. WD and pulmonary tuberculosis have been diagnosed in this patient. Hypothesis that patients with WD are cellular immune defects could explain the association with tuberculosis, which could partially explain why women and all races are susceptible to this disease. This patient received the diagnosis before the onset of intestinal manifestations. In patients presenting with lung involvement associatedwith arthralgia and fever, sarcoidosis is a possible alternative diagnosis. In cases of suspected WD with or without pulmonary involvement, duodenal biopsy with PAS staining is indicated, even in the absence of intestinal clinical symptoms. In our case, the bronchial location of the bacterium was confirmed by immunohistochemical analysis with anti-T whipplei antibodies.