Giant Cell Arteritis and Takayasu Arteritis: Diagnosis and Management
SUMMARY:
Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are two distinct types of large vessel vasculitis that can cause significant morbidity and mortality. The American College of Rheumatology/Vasculitis Foundation released updated guidelines in 2021 for the diagnosis and treatment of these diseases based on a systematic review of the literature. With advances in diagnostic tools and emerging therapies, a better understanding of these diseases can lead to improved outcomes for patients with GCA and TAK.
Presentation
Giant Cell Arteritis
- Presents with intracranial and/or extracranial manifestations
- Intracranial: Headache | Scalp tenderness | Jaw claudication | Vision changes
- Extracranial: Distant or absent pulses | Limb claudication | Constitutional symptoms (e.g., fever)
- Typically occurs in: Patients over 50 years old | Northern European descent | Women > Men
- Associated with polymyalgia rheumatic
Takayasu Arteritis
- Granulomatous inflammation of the aorta and its branches
- Claudication | Diminished or absent pulses | Hypertension | Bruits over affected vessels | Constitutional symptoms (e.g., fever)
- More common in younger women (10 to 40 years old is typical)
Note: GCA and TAK can appear very similar in their clinical presentations, histopathology, and imaging work up, but they vary widely in age of presentation. GCA patients are typically over 50 with a peak incidence around 70, and TAK are usually younger than 40.
Diagnosis
- GCA and/or TAK should be suspect in any patient with the above clinical presentations
- Laboratory evaluation: Erythrocyte sedimentation rate (ESR) | C-reactive protein (CRP) | CBC | CMP
- In most cases both ESR/CRP will be significantly elevated and obtaining both increases sensitivity and specificity of testing
- A small subset of patients can have normal ESR/CRP at time of diagnosis
Giant Cell Arteritis
- Temporal artery biopsy is the gold standard for diagnosis of GCA and should be performed as soon as possible, ideally within 1 to 2 weeks of starting treatment
- Treatment should not be delayed in order to obtain biopsy
- Unilateral long segment (>1 cm) biopsy is preferred to increase yield
- If unable to obtain biopsy, or if clinical suspicion is high but biopsy is negative, consider imaging studies such as: Ultrasound | Magnetic resonance angiography (MRA) | Positron emission tomography (PET).
Takayasu Arteritis
- Imaging studies such as MRA or computed tomography angiography (CTA) can help diagnose TAK and assess the extent of vascular involvement.
- Biopsy is not routinely recommended but can be considered in cases where diagnosis is uncertain or when other diagnoses are being considered
Treatment
Giant Cell Arteritis
- Steroids are the cornerstone of treatment for GCA
- Treatment differs based on whether patient has severe disease (e.g., vision loss, stroke, or other signs of cranial ischemia) or non-severe disease (e.g., constitutional symptoms, headache, jaw
claudication) - Non-severe disease
- Initiate high dose daily oral glucocorticoids (GC): Prednisone 1 mg/kg/day up to 80 mg or equivalent
- In addition to GC, Tocilizumab is recommended as initial treatment
- Methotrexate (MTX) can be considered in addition to GC if unable to give Tocilizumab (e.g., due to cost, history of recurrent infections or current infection, liver dysfunction, neutropenia) or GC can be given alone
- Severe disease
- Initiate high dose pulse IV glucocorticoids: IV methylprednisolone 500 to 1,000 mg/day or equivalent for 3 to 5 days
- Tocilizumab should be given in addition to IV steroids
- Adjunctive therapy
- If patient has critical or flow-limiting involvement of the vertebral or carotid
arteries, can consider addition of aspirin to prevent ischemic events - Statins are not recommended for GCA/TAK in patients who otherwise do not need statin therapy (e.g., patients with HLD)
- If patient has critical or flow-limiting involvement of the vertebral or carotid
- Surgical options exist for treatment and should be a decision made between a vascular surgeon and a rheumatologist
- For patients on immunosuppression with clinical worsening, increasing immunosuppression is recommended over surgical intervention
- Immediate surgical intervention should be considered for: Aortic aneurysms at high risk for rupture | Impending/progressive tissue or organ loss
- Steroids should be continued in the periprocedural period if patient has active disease
Takayasu Arteritis
- Steroids are the cornerstone of treatment for TAK
- Severe disease consists of life or organ threatening manifestations (e.g., cardiac ischemia, limb ischemia)
- Newly active and/or severe TAK
- Initiate high dose daily oral glucocorticoids (GC): Prednisone 1 mg/kg/day up to 80 mg or equivalent
- IV pulse glucocorticoids may be considered for patients with life or organ threatening disease, but there is no evidence that this is superior to oral GC in TAK
- Non-steroid immunosuppression should be initiated in addition to GC
- Non-steroid immunosuppression for TAK includes: TNF-alpha inhibitors | MTX | Azathioprine
- In contrast to GCA treatment, tocilizumab is generally not recommended due to lack of data demonstrating efficacy
- Newly active and/or mild TAK
- Low dose oral GC may be considered if patient only has mild symptoms (e.g., only constitutional complaints without limb ischemia)
- GC monotherapy can be considered in mild disease states
- Surgical options exist for treatment and should be a decision made between a vascular surgeon and a rheumatologist
- In general, medical management is preferred over surgical intervention including: Patients with persistent limb claudication symptoms without ongoing active disease | Worsening limb/organ ischemia | HTN with renal artery stenosis | Stenosis of a cranial/cervical vessel without clinical symptoms
- If possible, any planned surgical interventions should be held until patient’s disease is no longer active
- Immediate surgical intervention should be considered for: Coronary artery involvement (e.g., myocardial infarction) | Impending or loss of limb viability | Stroke
- Steroids should be continued in the periprocedural period if patient has active disease
Follow Up
Giant Cell Arteritis
- If the patient worsens while on steroids alone, then a non-steroid immunosuppressive agent should be added (e.g., Tocilizumab preferred over MTX) AND steroid dose should be increased if the clinical worsening causes severe disease
- GCs should be tapered over weeks to months, but optimal duration of treatment depends on patient factors (e.g., disease course, side effects from steroid use) and should be determined on a case-by-case basis
- If patient is in clinical remission, long term monitoring is recommended to assess for relapse
- Monitoring includes: History taking | Examination | Labwork (e.g., ESR/CBC)
- If patient develops elevation in inflammatory markers, immunosuppressive medications should not be escalated in the absence of clinical or imaging changes
Takayasu Arteritis
- If patient is in remission after receiving glucocorticoids for ≥6 to 12 months, it is conditionally recommended that steroids be slowly tapered off while the non-steroid immunosuppression is continued
- Steroids can be continued for longer periods if patient is not in remission or if they experience relapses
- If patient develops asymptomatic progression of a previously identified vascular lesion on imaging without evidence of inflammation, then immunosuppressive therapy should not be escalated
- If patient is in clinical remission, long term monitoring is recommended to assess for relapse
- Monitoring includes: History taking | Examination | Labwork (e.g., ESR/CBC)
- Patient should also receive regular non-invasive imaging to assess for new inflammation (e.g., CT angiography, MR angiography, FDG-PET)
- If patient develops elevation in inflammatory markers, immunosuppressive medications should not be escalated in the absence of clinical or imaging changes
KEY POINTS
- GCA and TAK are two types of large vessels vasculitis that can cause severe limb and organ threatening disease
- The cornerstone of treatment for both is glucocorticoids and additional non-steroid immunosuppression
- Surgical options exist for treatment, but are generally not recommended over medical therapy and require a collaborative approach between vascular surgery and rheumatology specialists
