[Pulmonary involvement in Behcet's disease: a positive single-center experience with the use of immunosuppressive therapy].
OBJECTIVE
Behcet's syndrome, or Behcet's disease (BD), is a multisystem pathology, and survival is related to pulmonary involvement. However, it appears that different treatments correlate with different prognoses. The aim of this study was to evaluate clinical and tomographic evolution, as well as the survival, of patients with BD-related pulmonary involvement.


METHODS
A retrospective review of our experience with pulmonary manifestations in patients with BD treated at our institution between January 1, 1988 and April 30, 2006. The clinical, radiological, treatment and survival data were obtained from medical charts.


RESULTS
We identified 9 patients with BD-related pulmonary involvement. The mean age was 34 +/- 11.5 years, and 7 of the patients were male. The radiological findings were as follows: pulmonary artery aneurysm (PAA) in 8 patients; pulmonary embolism in 3 (translating to an incidence of 5.11 cases/100 patient-years); alveolar hemorrhage in one; and pulmonary hypertension in one. The treatment consisted of immunosuppression with prednisone plus chlorambucil (or cyclophosphamide or mycophenolate mofetil) in all patients, with partial or complete resolution of the PAAs. One patient with a PAA and pulmonary hypertension also received sildenafil and warfarin, with good clinical and tomographic response (the first report in the English literature). In our sample, the mean duration of the follow-up period was 6.52 years. The three-year survival rate was 88.8%, as was the five-year survival rate.


CONCLUSIONS
Patients with BD-related pulmonary involvement can present good survival with immunosuppressive therapy, and BD should be borne in mind as a possible cause of pulmonary hypertension and alveolar hemorrhage.
Santana
Alfredo Nicodemos Cruz
AN
Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil. alfredonicodemos@hotmail.com
Antunes
Telma
T
Barros
Juliana Monteiro de
JM
Kairalla
Ronaldo Adib
RA
Carvalho
Carlos Roberto Ribeiro de
CR
Barbas
Carmen Silvia Valente
CS
D000328:Adult; D000783:Aneurysm / Q000000981:diagnostic imaging / Q000209:etiology*; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001528:Behcet Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy; D002699:Chlorambucil / Q000627:therapeutic use; D005260:Female; D006470:Hemorrhage / Q000000981:diagnostic imaging; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000209:etiology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D011650:Pulmonary Alveoli / Q000000981:diagnostic imaging; D011651:Pulmonary Artery / Q000000981:diagnostic imaging; D011655:Pulmonary Embolism / Q000000981:diagnostic imaging / Q000209:etiology*; D011859:Radiography; D012189:Retrospective Studies; D015996:Survival Rate
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D002699:Chlorambucil; D011241:Prednisone