sildenafil-citrate and Granulomatous-Disease--Chronic

The present authors report the case of an adult with chronic granulomatous disease who developed an unusual lung fibrosis associated with severe pulmonary hypertension. Histological analysis of a lung biopsy showed a diffuse infiltration with pigmented macrophages without granulomas, which particularly involved the pulmonary arterial and venular walls. Clinical and histological findings were suggestive of pulmonary veno-occlusive disease. Such a clinical association has not been previously described in the literature and might be due to the persistent expression of gp91phox at a very low level. In conclusion, the present case report illustrates a novel manifestation of chronic granulomatous disease.

Topics: Adult; Biopsy; Bronchoalveolar Lavage; Bronchoscopy; Diagnosis, Differential; Diuretics; Furosemide; Granulomatous Disease, Chronic; Hemodynamics; Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Male; Phosphodiesterase Inhibitors; Piperazines; Purines; Respiratory Function Tests; Sildenafil Citrate; Smoking; Sulfones; Tomography, X-Ray Computed