Diffuse interstitial pneumonia and pulmonary hypertension: a novel manifestation of chronic granulomatous disease.
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.
Khaldi
H
H
APHP, Service de Pneumologie, Service de Pneumologie Hôpital Bichat, Abderrahmen Mami, Ariana , Tunisie.
Marchand-Adam
S
S
Kannengiesser
C
C
Fabre
A
A
Debray
M P
MP
Danel
C
C
Gougerot-Pocidalo
M-A
MA
El Benna
J
J
Humbert
M
M
Aubier
M
M
Crestani
B
B
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
D004232:Diuretics; D010726:Phosphodiesterase Inhibitors; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D005665:Furosemide; D000068677:Sildenafil Citrate