LARGE VESSEL INVOLVEMENT IN ANTINEUTROPHIL CYTOPLASMIC ANTIBODY- ASSOCIATED VASCULITIS: A SINGLE CENTER EXPERIENCE OVER TWO DECADES
Authors: Mahmut Kaymakci et al.
Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a necrotizing small vessel disease. However, large vessels may be involved. This study characterized 36 patients with AAV and involvement of large arterial vessels.
Arteritis of the temporal artery (TA-AAV) was based on histopathology whereas the diagnosis of aortitis (A-AAV) and periaortitis (PA-AAV) could be either based on radiology or histopathology. 23 had MPO-ANCA and 13 had PR3- ANCA. Mean (SD) age at AAV diagnosis was 63.4 (12.79); 20 (56%) were male. Seventeen patients had TA-AAV, 10 had A-AAV and 9 had PA-AAV. The majority of patients were diagnosed with large vessel involvement and AAV within one-year. Inflammatory markers were elevated at the time of diagnosis of large vessel involvement. Twenty-five (69%) patients had histopathologic confirmation of AAV diagnosis. There was no overlap between TA-AAV, A-AAV and PA-AAV groups. Of the 17 patients with TA-AAV, 6 had inflammation of a temporal artery branch vessel. The most frequent site of involvement in the A-AAV group was the ascending thoracic aorta, whereas in the PA-AAV group, the abdominal aorta was most frequently involved. Glucocorticoids (36/36), rituximab (19/36), and methotrexate (18/36) were the most frequent treatments. During the study period 8 patients died. There was no difference in mortality between this cohort of L-AAV patients and the general population (standardized mortality ratio: 0.99; 95% CI, 0.43- 1.95).
The current trend to confirm diagnosis of large vessel vasculitis using imaging methods instead of histology bears the risk of delayed identification of differential diagnosis such as AAV. In case of atypical clinical presentation such as signs of kidney or lung involvement, clinicians should search for ANCA positivity and consider biopsy of temporal artery.