Takayasu arteritis: an update

• Published in: Turkish journal of medical sciences Vol. 48; no. 4; pp. 681 - 697
• Main Authors: Keser, Gökhan, Aksu, Kenan, Direskeneli, Haner
• Format: Journal Article
• Language: English
• Published: Turkey 16-08-2018
• Subjects: Antibodies, Monoclonal, Humanized - therapeutic use; Biomarkers - metabolism; Endovascular Procedures - methods; Guidelines as Topic; Humans; Immunosuppressive Agents - therapeutic use; Leflunomide - therapeutic use; Physical Examination; Prognosis; Radiology, Interventional; Takayasu Arteritis - diagnosis; Takayasu Arteritis - physiopathology; Takayasu Arteritis - therapy; disease activity; diagnosis; large vessel vasculitis; Takayasu arteritis; management
• ISSN: 1300-0144; 1303-6165
• DOI: 10.3906/sag-1804-136

Takayasu arteritis (TAK) is a challenging chronic, granulomatous, large-vessel systemic vasculitis, mostly due to difficulties in
early diagnosis and assessing actual disease activity. Since there are no specific diagnostic laboratory tests, biomarkers, or autoantibodies,
many patients experience considerable delay in diagnosis. Remembering the possibility of TAK together with the use of acute phase
responses and appropriate imaging studies may be helpful for early diagnosis. Since there may be discrepancies between systemic and
vascular wall inflammation, using only acute phase responses is not reliable in assessing current disease activity. Therefore, physical
examination and new imaging findings should also be used to assess current disease activity. Despite its limitations, the Indian Takayasu
Clinical Activity Score (ITAS2010) may also be helpful for this purpose. The rationale of medical treatment is to suppress both vascular and
systemic inflammation with appropriate systemic immunosuppression, including corticosteroids and conventional immunosuppressive
agents. In cases of refractory disease activity, leflunomide and biologic agents such as TNF inhibitors and tocilizumab may be tried.
In selected cases with persistent lesions that cannot be reversed with medical treatment, endovascular interventions including balloon
angioplasty, stent and stent graft replacement, or surgery may be tried. However, such procedures should be performed after suppression
of inflammation, i.e. during inactive disease. Prognosis of TAK is probably getting better with lower mortality rates reported in recent
years, probably due to the use of more effective medical treatments as well as the use of endovascular interventions when necessary and
available.