Background Non-infectious aortitis associated with polymyalgia rheumatica (PMR) is a rare entity (1). Sometimes the conventional immunosuppressive therapy is not enough, forcing us to find new therapeutic options (2,3). We present our experience in a local hospital.
Objectives To evaluate symptoms, clinic, diagnosis and therapeutic options.
Methods During the years 2010–2015 we search for patients diagnosed with PMR which at the same time were diagnosed with non infectious aortitis. Their medical records were reviewed and the following data were collected: initial clinical diagnosis, diagnosis method used, time elapsed since the onset of symptoms to diagnosis, ESR and CRP at diagnosis and after the use of treatment.
Results 5 patients were found: 4 women and 1 man. The average age onset of initial symptoms was 67.2 (74–58) years and the average delay in the diagnosis of the disease was 26 (1–50) months.
The most common onset clinic was classic PMR symptom. Patients 2, 3, 4 and 5 also showed parietooccipital or parietal headache. Other symptoms were lower limb claudication, temporomandibular joint pain, dyspnea, fatigue and pseudofibromyalgia.
All patients underwent temporal artery biopsy. In patient 5 bilateral temporal artery biopsy was performed.
The definitive diagnosis test was PET (4). In patient 2 PET was compatible with aortitis being angioMRI negative.
The average ESR at the beginning and after the treatment was 87.4 (114–65) and 10.2 (15.6) respectively. The initial and final average CRP (mg /dL) was 8.03 (14.54–1.86) and 1.2 (3 to 0.5) respectively.
All patients underwent autoimmune analytical battery, being this study negative.
1,2,3 and 4 patients performed concomitant weekly methotrexate (MTX) with maximum dose of 25 mg weekly sc. Patients 1 and 5 made concomitant treatment with steroids and azathioprine at doses of 100 and 150 mg daily, respectively.
Patients 1,2,3 and 5 were treated with tozilizumab which was well tolerated and without adverse effects to date. The average time from diagnosis of the disease until the start of treatment was 9.75 months (2,3). Patient 4 at the time of publication of these data remained asymptomatic with weekly MTX 15 mg sc and 5 mg of prednisone.
Conclusions Non-infectious aortitis associated with PMR may go unnoticed due to high variability of presentation. This can make, sometimes, a delayed diagnosis (1). PET seems to be a good tool for the diagnosis (4) and tozilizumab seems to be a suitable treatment for those patients refractory to steroids and DMARDs (2,3). More patients are needed to generalize these data.
Non-infectious aortitis: a report of 32 cases from a single tertiary centre in a 4-year period and literature review. Loricera J, Blanco R, et al. Clin Exp Rheumatol. 2015 Mar-Apr;33(2 Suppl 89):S-19–31.
Tocilizumab in refractory aortitis: study on 16 patients and literature review. Loricera J, Blanco R, et al. Clin Exp Rheumatol. 2014 May-Jun;32(3 Suppl 82):S79–89.
Biologic therapy in ANCA-negative vasculitis. Loricera J, Blanco R, et al. Int Immunopharmacol. 2015 Aug;27(2):213–9.
(18)F-FDG PET/CT for the detection of large vessel vasculitis in patients with polymyalgia rheumatica. Lavado-Pérez C, Martínez-Rodríguez I, et al. Rev Esp Med Nucl Imagen Mol. 2015 Sep-Oct;34(5):275–81.
Disclosure of Interest None declared