Steroid-responsive pulmonary hypertension in a patient with Langerhans' cell granulomatosis (histiocytosis X).
We report the case of a man with biopsy-proved pulmonary Langerhans' cell granulomatosis (histiocytosis X) and pulmonary precapillary hypertension. Pulmonary vascular resistances and mean pulmonary artery pressure decreased with corticosteroid therapy and dyspnea improved.
Benyounes
B
B
Pulmonology Unit, Hŏpital Bichat, Paris, France.
Crestani
B
B
Couvelard
A
A
Vissuzaine
C
C
Aubier
M
M
D000328:Adult; D001794:Blood Pressure / Q000187:drug effects; D005938:Glucocorticoids / Q000627:therapeutic use*; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D011241:Prednisone / Q000627:therapeutic use*; D011652:Pulmonary Circulation / Q000187:drug effects; D011859:Radiography; D014655:Vascular Resistance / Q000187:drug effects
D005938:Glucocorticoids; D011241:Prednisone