title: [Pulmonary hypertension and Langerhans' cell granulomatosis: successful treatment with sildenafil and iloprost].
abstract: METHODS
  A 51-year-old woman (Case A) who had suffered from pulmonary Langerhans' cell granulomatosis for four years presented with progressive exertional dyspnea. A 39-year-old man (Case B) with pulmonary Langerhans' cell granulomatosis for eight years presented with right heart failure.
  
  
  METHODS
  In Case A we found fixed airflow obstruction, hypoxemia, pulmonary fibrosis, bronchiectasis, severe precapillary pulmonary hypertension and a reduced right ventricular function, in Case B moderate airflow obstruction, hypoxemia, pulmonary fibrosis, emphysema and severe precapillary pulmonary hypertension.
  
  
  METHODS
  Pulmonary hypertension due to pulmonary Langerhans' cell granulomatosis was assumed in both patients. After treatment with sildenafil, the woman has experienced long-term improvement for 2.5 years so far. An initially planned lung transplantation was therefore postponed. During inhaled iloprost the man also showed hemodynamic and clinical improvement and was stable for several years. Five years later recurrent right heart failure required an increase in iloprost dose; lung transplantation was then performed.
  
  
  CONCLUSIONS
  Although no approved drug is available for pulmonary hypertension in Langerhans' cell granulomatosis specific targeted therapies can lead to hemodynamic and long-term clinical improvement. Treatment could be helpful for selected patients with pulmonary hypertension in Langerhans' cell granulomatosis.
authors: Abteilung Innere Medizin, Missionsärztliche Klinik gGmbH Würzburg, Germany. Matthias.held@missioklinik.de
authors: M
authors: M
authors: Held
authors: B
authors: B
authors: Jany
authors: A
authors: A
authors: Warth
authors: H
authors: H
authors: Wilkens
mesh_terms: D000328:Adult; D004359:Drug Therapy, Combination / Q000379:methods; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D016285:Iloprost / Q000008:administration & dosage*; D008297:Male; D008875:Middle Aged; D010879:Piperazines / Q000008:administration & dosage*; D011687:Purines / Q000008:administration & dosage; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000008:administration & dosage*; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage
chemical_list: D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate; D016285:Iloprost