Background In a previous study1 our group has reported a 50% mortality rate among 24 patients with Behcet’s disease (BD) having pulmonary arterial aneurysms (PAA). The delay in diagnosing and initiating treatment for this complication could have a role in this outcome.
Objectives To determine whether the outcome of PAA in our centre has changed since our previous report and to assess the possible factors that might influence the prognosis.
Methods Patients diagnosed as having PAA after 1992 (Group 2) were identified retrospectively. Their clinical findings and outcome were compared with those of the patients in the previous report (Group 1).
Results Fifteen additional BD patients (all male) were diagnosed as having PAA. All were treated with cyclophosphamide and prednisolone. Their mean age (32 ± 1.8 SEM years), disease duration (51.5 ± 6.8 SEM months), pathergy positivity (92%), HLA B5 status (60%) and other clinical manifestations were comparable to those of the patients in Group 1. The interval from the first symptom of PAA until the initiation of the treatment was significantly shorter in Group 2 compared to Group 1 (1.7 ± 0.6 SEM months vs. 6.4 ± 1.5 SEM months; p = 0.026). The mean follow ? up time after the initiation of treatment did not significantly differ between groups (50.9 ± 8.5 SEM months for Group 2 and 45.3 ± 11.4 SEM months for Group 1). There were 2 deaths in Group 2 (13%) and 14 deaths in Group 1 (58%). A Kaplan ? Meier analysis revealed a significantly better survival for patients in Group 2 compared to the patients in Group 1 (log rank = 6.8; p = 0.009). When only the patients who had received cyclophosphamide (with or without prednisolone) were considered in the analysis (17 patients from Group 1 and all patients from Group 2), the outcome was still better for the patients in Group 2 (log rank = 3.4; p = 0.065).
Conclusion Early recognition and most probably cyclophosphamide usage improve the outcome of PAA.
Hamuryudan V, Yurdakul S, Moral F, et al. Pulmonary arterial aneurysms in Behcet’s syndrome: a report of 24 cases. Br J Rheumatol. 1994;33:48–51
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