Giant cell arteritis

Giant cell arteritis is a chronic vasculitis affecting medium and large diameter arteries.

Typically affecting elderly that can potentially cause vision loss. Studies have demonstrated that early recognition and initiation of treatment can improve visual prognosis in patients with GCA 1).

GCA in the Indian Subcontinent (ISC) is rare.

Moorfields Eye Hospital and National Hospital for Neurology and Neurosurgery, London, serves an ethnically diverse population, including a significant population of patients of ISC descent. They hypothesisez that patients of ISC descent are no less likely than others to present with symptoms suggestive of GCA and therefore to undergo temporal artery biopsy (TAB).

Biopsy proven GCA occurs in patients of ISC descent, but rarely. Full investigation for GCA continues to be appropriate where it is suspected, regardless of ethnicity 2)

Simultaneous occurrence of giant cell arteritis and cerebral amyloid angiopathy 3).

Onset is usually insidious, although occasionally it may be abrupt.

Details of some findings:

1. H/A: the most common presenting symptom. May be nonspecific or located in one or both temporal areas, forehead, or occiput. May be superficial or burning with paroxysmal lancinating pain

2. symptoms relating to ECA blood supply (strongly suggestive of GCA, but not pathognomonic): jaw claudication, tongue, or pharyngeal muscles

3. ophthalmologic symptoms: due to arteritis and occlusion of branches of ophthalmic artery or posterior ciliary arteries

a) symptoms include: amaurosis fugax (precedes permanent visual loss in 44%), blindness, visual field cuts, diplopia, ptosis, ocular pain, corneal edema, chemosis

b) blindness: incidence is ≈ 7%, and once it occurs, recovery of sight is unlikely

4. systemic symptoms

a) nonspecific constitutional symptoms: fever (may present as FUO in 15% of cases), anorexia, weight loss, fatigue, malaise

b) 30% have neurologic manifestations. 14% are neuropathies including mononeuropathies and peripheral polyneuropathies of the arms or legs

c) musculoskeletal symptoms

● PMR is the most common (occurs in 40% of patients)

● peripheral arthritis, swelling & pitting edema of hands & feet in 25%

● arm claudication from stenosis of subclavian and axillary arteries

d) thoracic aortic aneurysms: 17 times as likely in GCA. Annual CXRs are adequate for screening

5. temporal arteries on physical examination may exhibit tenderness, swelling, erythema, reduced pulsations, or nodularity. Normal in 33%

6. the presence of systemic symptoms correlates with a lower incidence of blindness or stroke

Vestibulocochlear symptoms as the initial presentation of giant cell arteritis 4).

The superficial temporal artery is often affected in giant cell arteritis and biopsied if the diagnosis is suspected.

Laboratory studies

1. ESR > 40 mm/hr (usually > 50) by Westergren method (if > 80 mm/hr with above clinical syn- dromes, highly suggestive of GCA). ESR is normal in up to 22.5%

2. C-reactive protein: another acute phase reactant that is more sensitive than ESR. Has the advant- age that it can be performed on frozen sera

3. CBC: may show mild normochromic anemia

4. rheumatoid factor, ANA, and serum complement usually normal

5. LFTs abnormal in 30% (usually elevated alkaline phosphatase)

6. tests for rheumatoid factor and ANA are usually negative

7. temporal artery angiography not helpful (angiography elsewhere indicated if suspicion of large artery involvement exists)

8. CT: usually not helpful, one report described calcified areas corresponding to the temporal arteries

9. temporal artery biopsy:

sensitivity ≈ 90% (reported range is 9–97%)

specificity near 100%

predictive value ≈ 94%

1. periarteritis nodosa (PAN)

2. hypersensitivity vasculitis

3. atherosclerotic occlusive disease

4. malignancy: shares common symptoms of low grade fever, malaise, and weight loss

5. infection

6. trigeminal neuralgia

7. ophthalmoplegic migraine

8. dental problems

A 80-year-old male patient with hypertrophic pachymeningoencephalitis that may be associated with temporal arteritis. The patient presented with a 2-week history of latent paresis and ataxia affecting his right hand. He had been diagnosed with temporal arteritis 12 years earlier. Brain MRI showed an enhancement of the left-sided frontoparietal meninges with oedema of the adjacent tissue of the precentral cortex and postcentral cortex. A leptomeningeal biopsy was performed. An autoimmune-mediated immunoglobulin G4-associated hypertrophic pachymeningoencephalitis was diagnosed. The patient received a high-dose corticosteroid therapy and his symptoms gradually improved. This results suggest that hypertrophic pachymeningoencephalitis may occur as a complication of giant cell arteritis and may cause central neurological deficits by cerebral perifocal oedema 5).

A 75-year-old man with type 2 diabetes mellitus presented with complete vision loss in his right eye and severe headaches for the past 24 hours. He had been treated for suspected giant cell arteritis (GCA) with high-dose corticosteroids which were being tapered to stop after an inconclusive right temporal artery biopsy and an erythrocyte sedimentation rate (ESR) value of 8. His current acute presentation, however, raised further concern for partially treated GCA and precipitated treatment with pulsed methylprednisolone. The patient, taking metformin, developed diabetic ketoacidosis and was transferred to the intensive care unit where a swollen, painful right eye with chemosis and complete ophthalmoplegia was subsequently revealed to be secondary to cavernous sinus thrombosis. Rhino-orbital skin necrosis with positive samples for the organism Rhizopus on eventual orbital exenteration revealed angioinvasive fungal infection, mucormycosis, to be the cause. Bhatt et al., discussed here the lessons learnt, and how best to treat a susceptible cohort within our ageing western population 6).

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Baig IF, Pascoe AR, Kini A, Lee AG. Giant cell arteritis: early diagnosis is key. Eye Brain. 2019 Jan 17;11:1-12. doi: 10.2147/EB.S170388. eCollection 2019. Review. PubMed PMID: 30697092; PubMed Central PMCID: PMC6340646.
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Boisch G, Duda S, Hartmann C, Weßling H. Hypertrophic pachymeningoencephalitis associated with temporal giant cell arteritis. BMJ Case Rep. 2018 Sep 27;2018. pii: bcr-2018-225304. doi: 10.1136/bcr-2018-225304. PubMed PMID: 30262524.
Bhatt H, Zilani G, Hayhurst C. Orbitocerebral mucormycosis and intracranial haemorrhage: a role for caution with steroids in suspected giant cell arteritis. BMJ Case Rep. 2018 Jul 15;2018. pii: bcr-2017-224086. doi: 10.1136/bcr-2017-224086. PubMed PMID: 30012677.
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