title: Diffuse interstitial pneumonia and pulmonary hypertension: a novel manifestation of chronic granulomatous disease.
abstract: 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.
authors.affiliation: APHP, Service de Pneumologie, Service de Pneumologie Hôpital Bichat, Abderrahmen Mami, Ariana , Tunisie.
authors.forename: H
authors.initials: H
authors.lastname: Khaldi
authors.forename: S
authors.initials: S
authors.lastname: Marchand-Adam
authors.forename: C
authors.initials: C
authors.lastname: Kannengiesser
authors.forename: A
authors.initials: A
authors.lastname: Fabre
authors.forename: M P
authors.initials: MP
authors.lastname: Debray
authors.forename: C
authors.initials: C
authors.lastname: Danel
authors.forename: M-A
authors.initials: MA
authors.lastname: Gougerot-Pocidalo
authors.forename: J
authors.initials: J
authors.lastname: El Benna
authors.forename: M
authors.initials: M
authors.lastname: Humbert
authors.forename: M
authors.initials: M
authors.lastname: Aubier
authors.forename: B
authors.initials: B
authors.lastname: Crestani
mesh_terms: D000328:Adult; D001706:Biopsy; D018893:Bronchoalveolar Lavage; D001999:Bronchoscopy; D003937:Diagnosis, Differential; D004232:Diuretics / Q000627:therapeutic use; D005665:Furosemide / Q000627:therapeutic use; D006105:Granulomatous Disease, Chronic / Q000150:complications*; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D010879:Piperazines / Q000627:therapeutic use; D011687:Purines / Q000627:therapeutic use; D012129:Respiratory Function Tests; D000068677:Sildenafil Citrate; D012907:Smoking / Q000009:adverse effects; D013450:Sulfones / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed
chemical_list: D004232:Diuretics; D010726:Phosphodiesterase Inhibitors; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D005665:Furosemide; D000068677:Sildenafil Citrate