Large- vessel diseases- uparna 2.pptx

large vessel diseae

1. Non-atherosclerotic Disorders of large blood vessels By: Uparna Heenatigala Group 10

2. TAKAYASU ARTERITIS Takayasu arteritis, aka pulseless disease, is a systemic inflammatory condition which leads to damage of the medium and large arteries and their branches. It P rimarily affects: Aorta Main branches of the aorta Pulmonary artery Which leads to stenosis, a rterial wall thickening, Fibrosis, Thrombus formation , of these large arteries. Pathologic changes include: Stenosis Dilation Aneurysm formation Occlusion The etiology of Takayasu arteritis is largely unknown. At its core, it is an inflammatory disease, and it is thought that autoimmune cell-mediated immunity may be responsible for the disease. Eventual transmural fibrous thickening of the arterial walls is what ultimately leads to ischemic changes and formation of alternating areas of pseudoaneurysm

3. Classification Classified into six types based on severity and affected arteries: Type I – Affects aorta and aortic arch vessels Type IIa – Affects ascending aorta, aortic arch, and branches Type IIb – Affects ascending aorta, aortic arch, and thoracic descending aorta Type III – Affects thoracic descending aorta, abdominal aorta, and/or renal arteries Type IV – Affects abdominal aorta and/or renal arteries Type V – Combines Type IIb and Type IV features

4. CLINICAL FEATURES Prepulseless Phase Early-stage symptoms include: Fever Anorexia Weight loss General malaise Arthralgias (joint pain) Malnutrition This stage is difficult to diagnose because it lacks specific vascular symptoms . Chronic Phase Disease becomes inactive or "burned out" More characteristic features appear, depending on the affected arteries Common clinical signs: Bruits (vascular sounds) Vascular insufficiency

5. Clinical Features Based on Involved Vascular Bed Hypertension (renal artery stenosis) Retinopathy Aortic regurgitation Cerebrovascular symptoms Angina & congestive heart failure Abdominal pain or gastrointestinal bleeding Pulmonary hypertension Extremity claudication (pain due to reduced blood flow)

6. Diagnosis Gold standard: Angiography Detects narrowing or occlusion of the aorta & primary branches Identifies focal or segmental changes in large arteries Clinical suspicion is needed to diagnose Takayasu arteritis, and thus a good, complete history and physical exam is a necessity. Imaging is vital, and CTA ( Computed tomographic angiography) has recently become the standard for initial staging of disease distribution (previously it was conventional angiography) in Takayasu arteritis. This standard is based on the 1994 Tokyo International Conference Classification of Takayasu arteritis. CTA will allow for visualization of vessel wall thickening and luminal narrowing . Laboratory Findings Possible elevations in: Erythrocyte sedimentation rate (ESR) C-reactive protein (CRP) White blood cell count Anemia may be predominant in some cases

7. Treatment Strategies Medical Management (First-Line Treatment) Corticosteroids (e.g., Prednisone) – Reduce inflammation Cytotoxic drugs (e.g., Methotrexate, Azathioprine, Cyclophosphamide) – Used for severe or steroid-resistant cases Biologic agents (e.g., TNF inhibitors, IL-6 inhibitors like Tocilizumab) – Help suppress the immune system Surgical Treatment (For Severe Cases Only) Surgery is reserved for patients with advanced arterial damage who do not respond to medications. Bypass surgery – Rerouting blood flow around blocked arteries Angioplasty – Widening narrowed arteries using a balloon or stent Aortic valve replacement – If aortic regurgitation is severe

8. Giant Cell Arteritis (Temporal Arteritis) Also known as temporal arteritis or cranial arteritis A chronic, systemic, inflammatory vasculitis Primarily affects large- and medium-sized arteries, especially branches of the external carotid artery Most commonly affects the superficial temporal artery Can cause serious complications, including blindness and aortic aneurysms Pathophysiology Immune-mediated inflammatory process Affects aorta and its major branches. Mainly (temporal, ophthalmic, vertebral) Inflammatory cells invade arterial walls Leads to intimal thickening and luminal narrowing Multinucleated giant cells form in vessel walls Granulomatous inflammation develops Results in ischemia (reduced blood supply) to affected areas. Can lead to severe complications like vision loss and stroke

9. Clinical symptoms Prodromal phase with constitutional symptoms: Headache (most common symptom) Fever Malaise Myalgias (muscle pain) Often coexists with polymyalgia rheumatica Possible HLA-related association between the two diseases Complications from Vascular Narrowing & End-Organ Ischemia Visual alterations, including blindness Mural weakness leading to acute aortic dissection (potentially devastating) Ischemic optic neuritis: Occurs in up to 40% of patients May cause partial or complete blindness Considered a medical emergency Cerebral symptoms due to disease extension to carotid arteries Jaw claudication and temporal artery tenderness Aortic lesions (usually asymptomatic until late stages): Thoracic aneurysms Aortic dissections

10. Diagnosis Clinical Criteria (American College of Rheumatology Guidelines) A diagnosis of giant cell arteritis is made when three or more of the following criteria are met: Age ≥ 50 years New-onset localized headache Temporal artery tenderness or decreased pulsation Elevated ESR (erythrocyte sedimentation rate) ≥ 50 mm/hr Abnormal temporal artery biopsy (gold standard) Laboratory & Imaging Tests Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP) → Elevated Complete Blood Count (CBC) → Normocytic anemia , thrombocytosis Temporal artery biopsy → Confirms diagnosis Findings: Granulomatous inflammation Multinucleated giant cells Lymphocyte infiltration Imaging Tests (for large vessel involvement): Ultrasound (Doppler) of temporal artery MRI or CT angiography PET scan (for systemic vascular involvement)

11. Immediate Corticosteroid Therapy (Even Before Biopsy) High-dose prednisone (40-60 mg/day) is started immediately Prevents irreversible vision loss If vision is already affected, IV methylprednisolone may be used Steroid tapering over months to years based on response Regular monitoring of inflammatory markers (ESR, CRP) during treatment Adjunct Therapy Aspirin (low-dose) to reduce stroke risk Methotrexate or Tocilizumab (for steroid-sparing effect) Calcium & Vitamin D (for osteoporosis prevention due to steroids) Prognosis & Long-Term Management Giant cell arteritis is a chronic condition but can be well managed Most patients respond well to corticosteroids Early treatment prevents severe complications like blindness and stroke Relapse can occur, requiring long-term follow-up Treatment & Prognosis

12. If further treatment is needed: In rare cases of temporal arteritis, surgery is needed to repair the arteries.  Bypass grafts tend to occlude if placed during the active phase of temporal arteritis; elective surgery should await disease suppression. Giant cell arteritis prognosis Most people improve quickly once they begin treatment. You will need to remain on steroids for at least 1 to 2 years following a giant cell arteritis diagnosis. In some cases, you may need longer-term treatment. Also, giant cell arteritis can recur even after treatment.

13. REFERENCES https://surgery.ucsf.edu/condition/takayasus-arteritis#:~:text=Narrowed%20or%20occluded%20arteries%20caused,small%20opening%20in%20the%20skin . https://vascular.org/your-vascular-health/vascular-conditions/giant-cell-arteritis Surgical management of giant cell arteritis of the proximal aorta Read at the 69th Annual Meeting of the Southern Thoracic Surgical Association, Fort Lauderdale, Florida, November 9-12, 2022. Author links open overlay panelMotahar  Hosseini MD  a , Alberto Pochettino MD  a , Joseph A.  Dearani  MD  a , Alejandra Castro-Varela MD  b , Hartzell V. Schaff MD  a , Katherine S. King MS  c , Richard C. Daly MD  a , Kevin L.  Greason  MD  a , Juan A.  Crestanello  MD  a , Gabor  Bagameri  MD  a , Nishant Saran MBBS  a Cullen DJ, Apolone G, Greenfield S, et al: ASA physical status and age predict morbidity Kerr, GS, Hallahan, CW, Giordano, J, et al. Takayasu arteritis. Ann Intern Med 1994; 120: 919–929. Google Scholar 2 Brunner, J, Feldman, BM, Tyrrell, PN, et al. Takayasu arteritis in children and adolescents. Rheumatology 2010; 49: 1806–1814. Google Scholar 3 Morales, E, Pineda, C, Martinez-Lavin, M. Takayasu’s arteritis in children. J Rheumatol  1991; 18: 1081–1084. Google Scholar PubMed 4 Watson, L, Brogan, P, Peart, I, et al. Diagnosis and assessment of disease activity in Takayasu arteritis: a childhood case illustrating the challenge. Case Rep Rheumatol  2014; 2014: 603171. Google Scholar

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