Comparison of methotrexate and azathioprine as the first-line steroid-sparing immunosuppressive agents in patients with Takayasu's arteritis.

BACKGROUND: Immunosuppressive (IS) agents are recommended for the first-line treatment of patients with active Takayasu's arteritis (TAK) together with glucocorticoids (GCs). However, there is limited data comparing the efficacy and outcomes of different IS agents for this purpose. OBJECTIVES: In this study, we aimed to compare the outcomes of two most frequently used first-line IS agents, namely methotrexate (MTX) and azathioprine (AZA) in TAK patients. METHODS: TAK patients who received any IS agent in addition to GCs as the initial therapy were included in this multicentre, retrospective cohort study. Clinical, laboratory and imaging data of the patients were assessed. In addition, a matched analysis (cc match) using variables 'age', 'gender' and 'diffuse aortic involvement' was performed between patients who received MTX or AZA as the first-line IS treatment. RESULTS: We recruited 301 patients (F/M: 260/41, mean age: 42.2 ± 13.3 years) from 10 tertiary centres. As the first-line IS agent, 204 (67.8 %) patients received MTX, and 77 (25.6 %) received AZA. Less frequently used IS agents included cyclophosphamide in 17 (5.6 %), leflunomide in 2 (0.5 %) and mycophenolate mofetil in one patient. The remission, relapse, radiographic progression and adverse effect rates were similar between patients who received MTX and AZA as the first-line IS agent. Vascular surgery rate was significantly higher in the AZA group (23% vs. 9 %, p = 0.001), whereas the frequency of patients receiving ≤5 mg/day GCs at the end of the follow-up was significantly higher in the MTX group (76% vs 62 %, p = 0.034). Similarly, the rate of vascular surgery was higher in AZA group in matched analysis. Drug survival was similar between MTX and AZA groups (median 48 months, MTX vs AZA: 32% vs 42 %, p = 0.34). IS therapy was discontinued in 18 (12 MTX, 6 AZA) patients during the follow-up period due to remission. Among those patients, two patients had a relapse at 2 and 6 months, while 16 patients were still on remission at the end of a mean 69.4 (±50.9) months of follow-up. CONCLUSIONS: Remission, relapse, radiographic progression and drug survival rates of AZA and MTX were similar for patients with TAK receiving an IS agent as the first-line f therapy. The rate of vascular surgery was higher and the rate of GC dose reduction was lower with AZA compared to MTX at the end of the follow-up.

Mass-like lesions as a rare form of neuro-Behçet's disease: A case report and review of the literature.

Cerebral mass-like lesion (MLL) is a rare form of Neuro-Behçet's (NB) disease. There is currently no detailed knowledge on this issue in the literature. Our aim was to describe a Behçet's disease (BD) patient with MLL, followed by a clinical analysis in light of the available literature regarding BD patients who suffered from an MLL or tumefactive lesion in the brain. We conducted a review of the English literature to analyse data on MLL in BD. The Pub-Med, Web of Science, Proquest and Ovid databases were searched for articles or abstracts using the term "Behçet's disease" combined with one of the following terms: mass-like lesion, tumour-like lesion and tumefactive lesion. We compared clinical and laboratory features of BD patients with MLL with NB patients. We found 12 cases plus our case (6 male, 7 female; mean age: 40 years) with BD who developed MLL alongside BD. Five out of 13 BD patients (38%) had a history of BD before the onset of neurological symptoms. In 8 patients (62%), BD was diagnosed after the onset of neurological involvement. Headache, hemiparesis, dizziness, aphasia, nausea and vomiting were the presenting manifestations of NB patients with MLL. Genital ulceration, eye involvement, skin lesion and arthritis/arthralgia were less commonly reported in NB patients with MLL compared to NB patients without MLL. NB disease should be considered in the differential diagnosis of cerebral MLL even when other cardinal manifestations of BD are absent. Mucocutaneous manifestations, eye and joint involvement may be seen less often in these patients.

Retrospective evaluation of 22 patients with Takayasu's arteritis.

Takayasu's arteritis (TA) is a rare, idiopathic, inflammatory, granulomatous vasculitis that affects the aorta and its primary branches. Clinical features and the pattern of arterial involvement show differences in different regions of the world according to ethnic influences. Our aim in this retrospective study was to evaluate the demographic, clinic, laboratory, and angiographic findings of 22 patients with TA followed by our clinic and also compare our results with series from the literature. The hospital files of the 22 patients followed by our clinic between 1998 and 2009 were retrospectively evaluated. We also compared our results with the series from the literature that we were able to reach by US National Library of Medicine, National Institute of Health. Gender distribution, age at diagnosis, and type of aortic involvement were similar with the study from Turkey. Different clinical manifestations of Takayasu's arteritis have been described in different ethnic groups. We also want to underline the coincidence of TA and other rheumatic diseases such as sarcoidosis, SLE, RA, and psoriatic arthritis, different from other published series.