Background Aortitis is a several and potentially lethal clinical condition. It is usually oligosymptomatic or may present with non-specific symptoms that complicate and delay the diagnosis. On the other hand, treatment of aortitis is not established.
Objectives The objetive of this study is to describe clinical manifestations, laboratory studies and treatment of patients with aortitis in a single center.
Methods We have reviewed the medical records of patients who had positive Positron Emission Tomography (PET) for aortitis fron January 2013 to December 2015 in the Rheumatology Department of a University Hospital. We included 13 cases and we recorded epidemiological, clinical data, laboratory results, treatment and evolution.
Quantitative variable results were expressed as mean±standard deviation (SD) or median (interquatile range-IQR). Qualitative variable results were expressed as percentage. The analysis was performed with the SAS System for Windows V 9.2.
Results In the last 3 years 13 cases of aortitis were diagnosed (3 in 2013, 3 in 2014 and 7 in 2015). The male to female ratio was 3/10. Mean age±SD was 72.23±12.36 years [range, 51–83]. The underlying conditions were: giant cell arteritis (GCA) (n=7), polymyalgia rheumatica (PmR) (n=2), idiopathic (n=2), relapsing polychondritis (n=1) and Sjögren syndrome (n=1). The median interval between the diagnosis of the underline disease and the aortitis was 7 months [1–20].
The most common manifestations were constitutional syndrome (n=5), inflammatory back pain (n=4), pain irradiated to lower limbs (n=4), atypical polymyalgia (n=1) and fever (n=1).
All patients exhibited increased acute phase reactants except for the two patients with idiopathic aortitis. Erythrocyte sedimentation rate (ESR) mean was 62±33.84 mm/1st hour and C Reactive Protein (CRP) median level mas 3.3 [2.91–5.13] mg/dl. Five patients presented anaemia.
At the time of diagnosis, 9 patients were receiving prednisone (mean dose, 18.89±19.3 mg/day), 4 were with methotrexate (MTX) (mean dose, 15±5.77 mg/week) and the remaining 3 were not receiving any drug.
Treatment of aortitis was based on the onset or increase in prednisone dose (mean dose, 34.17±9.73 mg/day) in all cases except for one patient who needed surgery for aortic aneurysm. MTX was initiated in 5 patients and its dose was increased in 4 more (mean maximum dose, 14.72±6.43 mg/week). Three patients were treated with methilprednisolone i.v. (0.5 g/day, 3 consecutive days). Tocilizumab (TCZ) was added to therapy in 2 cases due to refractory disease.
In the first visit, two to four months later, the ESR mean was 25.92±24.88mm/1st hour and CRP median was 0.32 [0.16–1.11] mg/dl. In successive clinical controls, after a mean follow-up of 10±4.32 months, clinical and laboratory improvement was maintained.
Four patients had a new PET-TAC control 6–16 months later, 3 of them presented complete remission and another one partial remission.
Conclusions According to our data, clinical and laboratory data are often nonspecific in aortitis. The increase in the diagnosis cases in the last years is probably related to a higher index of suspicion. The presence of GCA or PmR refractory to standard treatment or unexplained raised ESR or CRP, may be red flags for suspecting an aortitis. Treatment with moderate doses of corticosteroids and MTX was effective in most of our patients. In 2 refractory cases, TCZ was useful.
Disclosure of Interest None declared