Challenges in Managing Takayasu Arteritis
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Takayasu Arteritis is a chronic inflammatory disease that affects large blood vessels. The document discusses challenges in managing the disease course and activity over time. It summarizes guidelines for determining disease activity versus remission using clinical features, biomarkers and imaging. Medical management focuses on controlling inflammation with steroids, DMARDs and biologics. Surgery is indicated for complications like aortic aneurysms or critical stenosis when disease is inactive.
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Challenges in Managing Takayasu Arteritis
- 1. Challenges in Managing TAKAYASU ARTERITIS SIDNEY ERWIN T. MANAHAN, MD, FPCP, FPRA Internal Medicine - Rheumatology
- 2. Scenarios • Among patients in “clinical remission” and on stable medical therapy, what do we do if there is disease progression? • Among patients with fixed stenosis not amenable to medical treatment, when do we send for intervention?
- 3. Early Onset Granulomatous Arteritis Cryoglobulinemic Vasculitis (CV) IgA Vasculitis (IgAV) Urticarial Vasculitis (HUV) Polyarteritis Nodosa (PAN) Kawasaki Disease (KD) Microscopic Polyangiitis (MPA) Granulomatosis with Polyangiitis (GPA) Eosinophilic Granulomatosis with Polyangiitis (EGPA) TAKAYASU ARTERITIS (TAK) Giant Cell Arteritis (GCA) Behcet’s Disease (BD) Cogan’s Syndrome (CS)
- 4. Disease Course SYSTEMIC VASCULAR BURNOUT (Pre-vasculitic) DISEASE ACTIVITY Inflammatory Features Vascular Insufficiency Constitutional (stenosis, Remission Symptoms aneurysms) TIME COURSE
- 5. Diagnosing Takayasu Arteritis 1990 ACR 1996 Sharma Modified • Age <40years • Left midsubclavian artery lesion • Limb claudication • Right midsubclavian artery • Decreased brachial pulse lesion • SBP difference >10mmHg • Characteristic s/sx for >1 month • Bruit over the subclavian or aorta • ESR >20mm/Hr • Arteriogram abnormality • Carotid artery tenderness • Hypertension • Aortic regurgitation or Annuloaortic ectasia • Pulmonary artery lesion • Left mid CCA lesion • Distal inominate artery lesion • Descending thoracic aorta lesion • Abdominal aorta lesion • Coronary Artery lesion
- 6. Biomarkers in Diagnosis Ishihara T, et al. Circulation J 2012: doi: 10.1253/circj.CJ-12-0131
- 7. TAK Disease Course • Control Inflammation • Relieve symptoms • Limit extent of vessel involvement DISEASE ACTIVITY 80% 20% • Monitor disease activity • Corrective vascular interventions TIME COURSE Ma J, et al. J Vasc Surg 2010; 51: 700-6. Subramanyan R, et al. Circulation 1989, 80: 429-37
- 8. Biomarkers of Activity Useful in Monitoring Not Useful • ESR • Fibrinogen, Haptoglobin • CRP (>2050ng/ml) • CRP • Serum Amyloid A (SAA) • -Acid glycoprotein • C4 Binding Protein (C4BP) • Serum Amyloid P • Pentraxin3 (PTX3) • C4a, C3c • Matrix Metalloproteinase-9 • Transthyretin (MMP-9) • 1-microglobin • MMP-2, MMP-3 Ishihara T, et al. Circulation J 2012: doi: 10.1253/circj.CJ-12-0131. Ma J, et al. J Vasc Surg 2010; 51: 700-6.
- 9. Biomarkers of Activity Biomarker Active TA Inactive TA Controls P-value ESR 39.1 + 24.8 15.2 + 9.6 11.3 + 5.1 <0.05 Elevated ESR (%) 83 28 0 SAA 95.9 (51.9) 49.2 (82) 23.9 (50.1) <0.005 C4BP 88.5 (72.6) 61.7 (57.7) 32.6 (32.1) <0.005 CRP 6.65 (18.1) 2.3 (5.75) 2.28 (1.58) 0.116 C4a 13.3 (13.6) 14.9 (10.4) 16 (23.9) 0.784 C3c 689.8 + 263 780.8 + 231 793 + 225 0.513 Values listed as Mean + SD or Median (Interquartile range) Ma J, et al. J Vasc Surg 2010; 51: 700-6.
- 10. The IDEAL Imaging Modality in TAK • Facilitate early diagnosis • Provide assessment of disease extent • Provide assessment of inflammatory activity • Demonstrate response to treatment • Distinguish vs. atherosclerotic plaques Mason J. Nature Rev Rheum 2010.
- 11. Performance of Imaging Modalities CT/ MR High Resolution 18F-FDG PET Angiography Ultrasound Scan Early diagnosis Disease extent Disease activity Evaluate response Differentiate vs. atherosclerosis Monitor every No SINGLE modality 6 MONTHS for evidence of provides all the progressive vascular disease information required. Modalities may have distinct or complementary roles in care. Mason J. Nature Rev Rheum 2010.
- 12. Determining TAK Activity National Institutes of Health (NIH) Criteria • Systemic Features • Elevated ESR or CRP • Symptoms of Vascular Ischemia • Typical Angiographic Features New onset OR Worsening of any two of the above criteria reflects disease activity. Kerr GS, et al. Ann Int Med 1994.
- 13. Determining TAK Activity REMISSION SUSTAINED REMISSION • Absence of symptoms • Remission criteria for AT • Normal inflammatory LEAST 6 months markers • Steroid dose <10mg/day • No new imaging findings
- 14. Controlling Disease Activity Prednisone DMARDs BIOLOGICS Japan Guidelines MTX • Starting dose: 20-30 mg/d AZA Infliximab* • Maximum dose: 60 mg/d CsA Etanercept* CYC American College of Rheumatology Tocilizumab • Max starting dose: 60 mg/d MMF * Open-label trials JCS Joint Working Group. Circ J 2011; 75: 474-503. Mukhtyar C, et al. Ann Rheum Dis 2008; doi: 10.1136/ard.2008.088351. Johnston SL, et al. J Clin Path 2002; 55: 481-486
- 15. Medical Management of TAK Prednisone 0.5 – 1 mkd Difficult to taper Is disease MTX 7.5 – 25mg/wk AZA 2 mkd INACTIVE? CsA 3 mkd (Can taper steroids) CYC PO 50-100 mg/d (IV 300-750 mg/m2 /mo) Ineffective MMF 1.5 – 3 mg/d Ineffective Infliximab 5mg/kg/dose Ineffective Etanercept 25 mg 2/wk Tocilizumab 8 mg/kg/mo JCS Joint Working Group. Circ J 2011; 75: 474-503. Johnston SL, et al. J Clin Path 2002; 55: 481-486
- 16. TAK Surgery Best done during Inactive Phase • Prevent restenosis, anastomotic failure, thrombosis, hemorrhage and infection If Urgent surgery during Active Phase • ESR <30mm/hr • CRP <1mg/dl JCS Joint Working Group. Circ J 2011; 75: 474-503. Johnston SL, et al. J Clin Path 2002; 55: 481-486
- 17. Indications for TAK Surgery • Aortic root dilation >50mm on CT • Aortic coarctation • Aortic valve regurgitation >75% EF • Dilatation of branches of aorta >30mm • Symptomatic cerebral ischemia • Critical stenosis of >3 cerebral vessels • Cardiac ischemia w/ confirmed CAD • Renal artery lesions – esp those with HF, unstable angina, renovascular HPN, decreased renal function JCS Joint Working Group. Circ J 2011; 75: 474-503. Johnston SL, et al. J Clin Path 2002; 55: 481-486
- 18. Summary • Reviewed the course of Takayasu Arteritis • Discussed definitions of disease activity and remission – Role of biomarkers – Role of imaging studies • Presented the medical management of Takayasu Arteritis • Enumerated indications for surgical intervention