CME INDIA Case Presentation by Dr. C. B. Prasad, M.D. (medicine) D. T. M&H, Chairman API Sitamarhi branch. Executive committee member, API Bihar. Tara Diabetes care and research centre, Sitamarhi.
CME INDIA Case Study
- A case of new onset of headache with jaw pain in a 68-year-old lady
- This is the photograph of temples both sides.
- ESR – 100 mm/h.
- C-reactive protein (CRP 126 mg/L (normal range <10).
- She had no fever and no sign of infectious focus.
- She experienced a sudden headache and jaw pain (29/6/2022).
- Both temporal arteries were thickened with low pulsation.
- No history of visual defect.
- What is the diagnosis?
Dr. N. K. Singh:
- Here vision is not affected.
- Looks Temporal arteritis.
- Many cases present with profound visual loss secondary to an ischaemic optic
- Ask for double vision, you can get if third, fourth or sixth cranial nerve palsy.
- Do a comprehensive pulse assessment, bruit auscultation and inter-arm
systolic blood pressure difference may be if left ventricular disease.
- In any scenario where GCA is suspected, do palpate temporal arteries. You may find prominence, beading and reduced or absent pulse. In this case, low pulsation mentioned.
- In this case ESR is raised, but in 10% cases, it could be normal.
- These days, do take a history of COVID. It may lead to diagnostic delay for those with GCA. While acute-onset headache, fever, fatigue, myalgia and elevated inflammatory markers are typical common presentations, dry cough and isolated dyspnoea are rare in GCA.
Dr. Satish, Kumar, Cardiologist, Bokaro:
- Temporal Arteritis.
Dr. N. K. Singh:
- The diagnostic criteria for giant cell arteritis, as laid down by the American College of Rheumatology, demands the fulfilment of at least two of the following five criteria (Some additional also):
- Age more than 50 years.
- New headache.
- Superficial temporal artery (STA) tenderness or decreased pulsation.
- Elevated ESR more than50 mm in the first hour.
- Abnormal findings on temporal artery biopsy.
- These days, Duplex sonography of the temporal arteries is an emerging viable technique. It suggests by demonstration of a characteristic dark halo around the artery, is emerging as a viable alternative. It is complemented to the gold standard in non-invasive diagnosis.
Credit: Rachel Piccus, Michael Stormly Hansen, Steffen Hamannet al. DOI: 10.7861/clinmed.2022-004.Royal college of physician 2022.
Dr. Diwakar, DNB (Med):
- Temporal arteritis, 1: New onset of headache after 55 years 2: Raised ESR 3: palpable temporal artery or pulsations.
Dr. Chandan Sharma, MD, Dibrugarh:
- Giant cell arteritis.
Dr. Venkatesh Molio Maregoan, Goa:
- Temporal arteritis.
Dr. R. K. Gupta, Yamuna Nagar:
- Temporal Arteritis.
Dr. C. B. Prasad:
|Most important is to start treatment after diagnosis to prevent vision loss. Lastly gold standard for final diagnosis is biopsy.|
|Cortisone treatment time is just like door to needle time in ACS.|
|Diagnosis was confirmed when she was started on prednisolone 40mg daily and after 5days she showed marked improvement.|
Dr. G. B. Sattur, Hubli:
- Tocilizumab is recommended by FDA. But steroids are main stay not only for pain reduction but to prevent blindness. They need long term follow up as steroids may be needed long term and Polymyalgia rheumatica can accompany at any stage.
Dr. S. K. Goenka, Begusarai:
- Aspirin also needs to be considered.
CME INDIA Learning Points
- Patients presenting with new-onset visual loss or diplopia should be referred on the same day for an ophthalmology assessment.
- About half of patients diagnosed with GCA have polymyalgia rheumatica (PMR).
- Remember GCA is classically distinguished by cranial ischemia-related symptoms (Such as headache, jaw claudication, and scalp tenderness), although large-vessel involvement leading to complications such as aortic aneurysm is becoming more widely recognized.
- Where there is a strong clinical suspicion of GCA, start Glucocorticosteroid.It should be given without waiting for the tests to return.
- Use of tocilizumab, is based on randomised controlled trial evidence of its efficacy in achieving remission with significant glucocorticoid sparing over the course of 12 months.
- Tests, including non-invasive imaging, in conjunction with clinical expertise are the current gold standard for diagnosis of giant cell arteritis.
What is this?
- Giant cell arteritis (GCA) is a systemic granulomatous vasculitis.
- It affects the medium and large-size arteries.
- It may present with a range of ophthalmic findings.
- GCA is commonly found in elderly patients.
- It is very rare in Asian and Middle-Eastern populations.
- Genetic studies have identified increased risk associated with HLA-DRB1*04.
What is the Current understanding of the immunopathology?
- It implicates age-related dysfunction of the immune system and its interactions with the aging blood vessel wall rather than an immune response to any one specific antigen.
- If there is a constellation of genetic risk factors and environmental triggers, dendritic cells in the adventitia recruit T cells which leads to:
- Cytokine production.
- Interferon gamma production.
- Inflammation including macrophages which can differentiate into giant cells.
- This inflammation remodels the vessel wall, leading to ischemia of the downstream organs.
How it presents to Ophthalmology?
- Arteritic anterior ischemic optic neuropathy is the most common ophthalmic manifestation.
- Others are:
- Central or branch retinal artery occlusion.
- Ophthalmic artery occlusion.
- Cranial neuropathies causing diplopia.
- Rarely anterior segment ischemia and anisocoria may also occur.
Diagnostic Workup in Nutshell
- It is ultimately a clinical diagnosis.
- It is usually supported with lab results, pathology, and/or imaging.
- Temporal artery biopsy (TAB) remains the gold standard diagnostic test
- Sensitivity of TAB is debated and practice patterns still vary with respect to sample length and whether unilateral or simultaneous bilateral biopsies are performed.
- Some studies have reported higher sensitivity of ultrasounds over TAB
- MRI and even PET CT protocols offer additional options for less invasive diagnostic testing.
Know it too:
Colour Doppler ultrasound (CDUS)
- Now recommended as an initial, non-invasive investigation.
- CDUS assesses arterial wall anatomy, lumen patency and blood flow.
- Positive findings include the halo sign and the compression sign.
- The halo sign (homogeneous, hypoechogenic circumferential vessel wall
- Sensitivity 68%.
- Specificity of 91%, It increases to 100% when found bilaterally.
- The Halo Score has recently been developed to quantify vascular inflammation and is useful to establish a diagnosis of GCA as well as characterise the risk of ocular ischaemia.
- The compression sign – The ability of a normal artery to extinguish with pressure as opposed to a vasculitic artery that will not. (It is useful to distinguish a true versus a false halo.
- CDUS has reduced the requirement for TAB Temporal artery biopsy. TAB is influenced by biopsy length, the variation in histological interpretation, biopsy
cuts and glucocorticoids.
- Histological findings – Extensive inflammatory infiltrates, granulomas and nucleated giant cells.
Positron emission tomography – computed tomography (PET-CT)
- PET-CT on a time-of-flight scanner with fluorine-fluorodeoxyglucose (F-FDG)
administration and 1 mm CT slice thickness allows assessment of disease activity from FDG vessel uptake.
- F-FDG-PET – It is recommended for patients presenting with suspected involvement of thoracic and vertebral vessels where CDUS cannot adequately image these deeper vessels.
Magnetic resonance imaging (MRI) can also be used in the diagnosis of GCA
- A meta-analysis of 6 studies estimated.
- Sensitivity 73%.
- Specificity of 88% for MRI compared to clinical diagnosis.
- It suggests that MRI is comparable to TAB in diagnostic value, and may spare TAB in patients with normal MRI findings.
- “Black-blood” is an MRI vessel wall imaging technique that creates high-contrast images of blood vessel walls. It can be particularly useful for diagnosis of GCA.
American College of Rheumatology Recommends
|Recommendation: For patients with suspected GCA, we conditionally recommend an initial unilateral temporal artery biopsy over bilateral biopsies.|
|For patients with suspected GCA, we conditionally recommend a long-segment temporal|
artery biopsy specimen (>1 cm) over a short-segment temporal artery biopsy specimen (<1 cm).
|For patients with suspected GCA, we conditionally recommend obtaining a temporal artery biopsy specimen within 2 weeks of starting oral glucocorticoids over waiting longer than 2 weeks for a biops.|
|For patients with suspected GCA, we conditionally recommend temporal artery biopsy over temporal artery ultrasound for establishing a diagnosis of GCA.|
|For patients with suspected GCA, we conditionally recommend temporal artery biopsy over mag- netic resonance imaging (MRI) of the cranial arteries for establishing a diagnosis of GCA.|
|For patients with suspected GCA and a negative temporal artery biopsy result (or results), we conditionally recommend noninvasive vascular imaging of the large vessels with clinical assessment to aid in diagnosis over clinical assessment alone.|
|For patients with newly diagnosed GCA, we conditionally recommend obtaining non-invasive vascular imaging to evaluate large vessel involvement.|
How to manage?
Do it immediately
- Start immediate glucocorticoid prescription.
- It is recommended where GCA is confirmed or strongly suspected.
- Initial Dose: Oral prednisolone 40–60 mg daily.
- If symptoms of cranial ischaemia, including visual disturbance, are present: Use a 500–1,000 mg intravenous prednisolone induction dose for 3 days followed by a reduction course.
Alert for Long term
- To prevent long-term steroid-related complications: Follow a GC-tapering regimen over 6–24 months according to response (symptoms and inflammatory markers).
- Relapse of GCA is common.
- Treat flares are by increasing GC dose, elongating treatment.
- When to use methotrexate? It is recommended alongside a GC-tapering regimen in those with a high risk of GC toxicity or relapse. It is needed to reduce the cumulative GC dose and prevent relapse.
- There is class 1 evidence to support the use of tocilizumab, an interleukin-6 inhibitor, in combination with GC-tapering to reduce GC-toxicity and prevent relapse.
|Management recommendations by ACR 2021|
|For patients with newly diagnosed GCA without manifestations of cranial ischemia, we condi-tionally recommend initiating treatment with high-dose oral glucocorticoids over intravenous (IV) pulse glucocorticoid.|
|For patients with newly diagnosed GCA with threatened vision loss, we conditionally recommend initiating treatment with IV pulse glucocorticoids over high-dose oral glucocorticoids.|
|For patients with newly diagnosed GCA, we conditionally recommend dosing oral glucocorti-coids daily over an alternate-day schedule.|
|For patients with newly diagnosed GCA, we conditionally recommend initiating treatment with high-dose oral glucocorticoids over moderate-dose oral glucocorticoid.|
|For patients with newly diagnosed GCA, we conditionally recommend the use of oral gluco-corticoids with tocilizumab over oral glucocorticoids alone.|
|For patients with GCA with active extracranial large vessel involvement, we conditionally|
recommend treatment with oral glucocorticoids combined with a nonglucocorticoid immunosuppressive agent over oral glucocorticoids alone.
|The optimal duration of therapy with glucocorticoids for GCA is not well established|
and should be guided by the patient’s values and preferences.
|In patients with newly diagnosed GCA, we conditionally recommend against the use of a|
hydroxymethylglutaryl-coenzyme A reductase inhibitor (“statin”) specifically for the treatment of GC.
|For patients with GCA who have critical or flow-limiting involvement of the vertebral or carotid arteries, we conditionally recommend adding aspirin.|
|For patients with GCA who experience disease relapse while receiving moderate-to-high–|
dose glucocorticoids, we conditionally recommend adding a nonglucocorticoid immunosuppressive drug.
|For patients with GCA who experience disease relapse with symptoms of cranial ischemia, we conditionally recommend adding a nonglucocorticoid immunosuppressive agent and increasing the dose of glucocorticoids over increasing the dose of glucocorticoids alone.|
|For patients with GCA who experience disease relapse with cranial symptoms while receiving gluco-corticoids, we conditionally recommend adding tocilizumab and increasing the dose of glucocorticoids over adding methotrexate and increasing the dose of glucocorticoid.|
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