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Vasculitis of large intestine — Clinical Guidelines

Overview

Large vessel vasculitis (LVV) encompasses conditions such as giant cell arteritis (GCA) and Takayasu arteritis (TA), characterized by inflammation affecting large arteries, leading to complications like vascular stenosis, aneurysms, and systemic symptoms. 6 7

Diagnosis

Clinical Presentation: Headache, jaw claudication, visual disturbances, and constitutional symptoms. 6

Imaging:

- Ultrasound: Recommended as first-line imaging for suspected GCA, including axillary arteries. 2 3 - MRI/MRA: Useful for detailed vascular assessment and monitoring disease activity. 12 - 18F-FDG PET/CT: Provides functional and anatomical imaging, beneficial in cases with inconclusive biopsies or clinical suspicion. 11 16

Biopsy: Temporal artery biopsy remains a gold standard for GCA diagnosis, though less sensitive than clinical criteria. 11

Laboratory Tests: Elevated inflammatory markers (ESR, CRP) are common but non-specific. 7

Management

First-Line Treatments:

- Corticosteroids: High-dose initial therapy, tapering based on clinical response. 7

Adjunctive Therapies:

- Biologic Agents: Tocilizumab, rituximab, and others may be considered in refractory cases or for maintenance therapy. 18 - Conventional Immunosuppressants: Methotrexate, azathioprine, or mycophenolate mofetil for steroid-sparing effects. 18

Special Populations

Elderly: GCA predominantly affects elderly patients; careful monitoring for complications like stroke and vision loss is crucial. 6

Pregnancy: Limited data; management involves balancing maternal health with fetal safety, often requiring individualized corticosteroid regimens. 18

Key Recommendations

Use Ultrasound as First-Line Imaging for suspected GCA, including axillary arteries. (Evidence: Strong 2 3)

Initiate High-Dose Corticosteroids for acute management of LVV. (Evidence: Strong 7)

Consider Biologic Agents in patients with refractory disease or for maintenance therapy to reduce corticosteroid use. (Evidence: Moderate 18)

Regular Monitoring with Imaging (MRI, MRA, PET/CT) to assess disease activity and complications. (Evidence: Moderate 12 16)

Evaluate for Comorbidities and tailor management to prevent complications such as cardiovascular events. (Evidence: Expert opinion 17)

References

1 Misra DP, Mukhtyar CB, Chandwar K, Putman M, Walsh M. The fragility of randomized controlled trials in large vessel vasculitis. Autoimmunity reviews 2025. link 2 Dejaco C, Ramiro S, Bond M, Bosch P, Ponte C, Mackie SL et al.. EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice: 2023 update. Annals of the rheumatic diseases 2024. link 3 Bosch P, Bond M, Dejaco C, Ponte C, Mackie SL, Falzon L et al.. Imaging in diagnosis, monitoring and outcome prediction of large vessel vasculitis: a systematic literature review and meta-analysis informing the 2023 update of the EULAR recommendations. RMD open 2023. link 4 Boleto G, Berti A, Merkel PA, Aydin SZ, Direskeneli H, Dejaco C et al.. Measurement Properties of Outcome Instruments for Large-Vessel Vasculitis: A Systematic Literature Review. The Journal of rheumatology 2023. link 5 Ughi N, Padoan R, Crotti C, Sciascia S, Carrara G, Zanetti A et al.. The Italian Society of Rheumatology clinical practice guidelines for the management of large vessel vasculitis. Reumatismo 2022. link 6 Dhanani U, Zhao MY, Charoenkijkajorn C, Pakravan M, Mortensen PW, Lee AG. Large-Vessel Vasculitis in Ophthalmology: Giant Cell Arteritis and Takayasu Arteritis. Asia-Pacific journal of ophthalmology (Philadelphia, Pa.) 2022. link 7 Maz M, Chung SA, Abril A, Langford CA, Gorelik M, Guyatt G et al.. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis. Arthritis & rheumatology (Hoboken, N.J.) 2021. link 8 Maz M, Chung SA, Abril A, Langford CA, Gorelik M, Guyatt G et al.. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis. Arthritis care & research 2021. link 9 Aydin SZ, Robson JC, Sreih AG, Hill C, Alibaz-Oner F, Mackie S et al.. Update on Outcome Measure Development in Large-vessel Vasculitis: Report from OMERACT 2018. The Journal of rheumatology 2019. link 10 Monti S, Águeda AF, Luqmani R. The use of ultrasound in the management of large-vessel vasculitis: an evolving concept. Clinical and experimental rheumatology 2018. link 11 Braun J, Baraliakos X, Fruth M. The role of 18F-FDG positron emission tomography for the diagnosis of vasculitides. Clinical and experimental rheumatology 2018. link 12 Guggenberger KV, Bley TA. Magnetic resonance imaging and magnetic resonance angiography in large-vessel vasculitides. Clinical and experimental rheumatology 2018. link 13 Dejaco C, Ramiro S, Duftner C, Besson FL, Bley TA, Blockmans D et al.. EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice. Annals of the rheumatic diseases 2018. link 14 Cimmino MA, Camellino D. Large vessel vasculitis: is it more common than usually assumed?. Reumatismo 2017. link 15 Sreih AG, Alibaz-Oner F, Kermani TA, Aydin SZ, Cronholm PF, Davis T et al.. Development of a Core Set of Outcome Measures for Large-vessel Vasculitis: Report from OMERACT 2016. The Journal of rheumatology 2017. link 16 Balink H, Bennink RJ, van Eck-Smit BL, Verberne HJ. The role of 18F-FDG PET/CT in large-vessel vasculitis: appropriateness of current classification criteria?. BioMed research international 2014. link 17 Luqmani R. Large vessel vasculitides: update for the cardiologist. Current opinion in cardiology 2012. link 18 Pipitone N, Olivieri I, Salvarani C. Recommendations of the Italian Society of Rheumatology for the treatment of the primary large-vessel vasculitis with biological agents. Clinical and experimental rheumatology 2012. link 19 Fuchs M, Briel M, Daikeler T, Walker UA, Rasch H, Berg S et al.. The impact of 18F-FDG PET on the management of patients with suspected large vessel vasculitis. European journal of nuclear medicine and molecular imaging 2012. link

Vasculitis of large intestine