*For medical professionals reading only, please compare the classification standards in 1990. What is the difference?

*For medical professionals only for reference

Compared with the classification standards in 1990, what is the difference?

Written by 丨Volume

Giant cell arteritis (GCA), once called temporal arteritis , cranial arteritis , granulomatous arteritis , and later gradually realized that any larger artery in the body can be involved. It is a systemic vasculitis named after pathological characteristics. The most common manifestations of this disease are systemic symptoms (including fatigue, fatigue, appetite deficiency, weight loss, low fever and other discomforts). recently developed headaches, visual symptoms, intermittent mandibular movement disorder triple signs and rheumatoid polymyalgia.

Systemic + Multiple Systems involve

Systemic Symptoms

Almost all patients have at least one systemic symptom, including fatigue, fatigue, appetite deficiency, weight loss, low fever and other discomforts. There is no certain pattern of fever, most of which are low and moderate (about 38℃) fever, and about 15% of patients can reach up to 39~40℃; a few GCA patients only have systemic symptoms and the erythrocyte sedimentation rate of is increased by .

Organ involvement

Organ involvement is mostly caused by inflammation of the lesion and stenosis of the lumen, resulting in insufficient blood supply to the corresponding organ tissues and impaired function. The symptoms vary according to the affected blood vessel location and duration of the disease, and the severity of the disease is different. Common ones include:

1. Head: The carotid artery and its branches such as superficial temporal, occipital, vertebral artery, etc. are involved in head symptoms. is the most common headache and is the most common symptom other than systemic symptoms of GCA. Up to 85% of patients may experience headaches, and about half of patients have headaches as the first symptom of .

Headache characteristics:

① Most often manifests as newly occurring, one or two-sided temporal pulsating pain, and may also involve the occipital or other parts.

②Heart pain is cut, burning or persistent swelling and pain, and can also occur intermittently.

③Insensitive to painkillers, it can aggravate when touched or combed.

④ Occasionally hemorrhagic necrosis on the scalp.

⑤50% of patients have scalp tenderness or palpable painful nodules, and nodules are more diagnostic value if distributed along the temporal artery. Typical temporal artery involvement is characterized by thickening and hardening of the artery, angrily opening, and increased pulsation, and may also cause weakening or disappearance of the pulse due to vascular occlusion.

2. Eyes: often manifested as black swelling, unclear vision, double vision , partial blindness or total blindness. 20% of patients with GCA have eye involvement and blindness as the first symptoms. If blindness on one side of is not actively treated, the contralateral side may be involved in within 1 to 2 weeks.

3. Intermittent mandibular movement disorder: About 60% of patients suffer from facial arteritis, vascular stenosis, and local blood supply, resulting in spasm of the mandibular muscle, intermittent chewing discomfort, chewing pain, chewing pause and jaw skew, etc., also known as jaw lameness, which has a high specificity for GCA diagnosis; it is rare to have dysphagia, , sluggish taste, and unclear speech due to tongue muscle movement disorder; there are also reports of tongue infarction.

4.Nervous system : About 30% of patients have multiple neurological symptoms, with different manifestations, and both the central or peripheral nerves may be involved. The most common are transient ischemic attack (TIA), stroke or neuropathy,

5. Cardiovascular system manifestations: About 1/3 of GCA patients may have other major artery involvement , and in the absence of typical triplets, only major artery involvement, with or without systemic symptoms. GCA can also involve distal limbs, coronary artery , mesenteric artery, etc., which can be manifested as Raynaud phenomenon and limb claudication. Coronary artery lesions can lead to myocardial infarction, heart failure, myocarditis and pericarditis.

6. Others: GCA less involves the respiratory system, and mental symptoms are depression or confusion.

1990-year ACR classification standard still applies now?

Currently, it mostly uses the GCA classification standard ^[1] formulated by the ACR in 1990. When applying this classification standard, it is necessary to note: when large vasculitis is diagnosed, this classification standard is used to diagnose GCA; before diagnosis, other diagnoses similar to vasculitis should be ruled out.

Table 1: GCA classification standards formulated by ACR in 1990

If the above five standards meet 3 or more, GCA can be diagnosed.

However, about 40% of GCA patients have no temporal artery involvement. According to the 1999 ACR classification criteria, these patients failed to meet the GCA diagnosis, suggesting that the above classification criteria are deficient in diagnosis of GCA; nearly 25% of patients with positive temporal artery biopsy cannot meet this classification criteria. In addition, it has been more than 30 years since the GCA classification standard formulated by the ACR in 1990. The 1990 standard was formulated before the widespread use of non-invasive and advanced vascular imaging technologies. Therefore, new classification standards are needed to meet the needs of clinical diagnosis of .

2022 ACR/EULAR proposes a new classification standard

2018 EULAR proposes that for patients suspected of GCA, early imaging examination is recommended as a supplement to diagnostic indicators. In this year's new classification standard of ACR/EULAR ^[2], positive temporal artery biopsy or halo signs on temporal artery ultrasound is a very valuable indicator. FDG-PET shows that aortic activity also has certain characteristics for the diagnosis of GCA. These two are newly included indicators in the new version of the standard. The sensitivity of the new classification standard reached 87%, and the specificity reached 95%.

Compared with the original 1990 GCA classification criteria, the 2022 ACR/EULAR GCA classification criteria showed higher sensitivity while maintaining specificity similar to the 1990 standard. In particular, the new standard can correctly classify more patients with large blood vessel GCA subtype.

Knowledge Classroom

When the temporal artery wall swells, it appears on ultrasound images as the low echo band surrounding the lumen thickens, and its cross-sectional image is called "halo sign". Compared with temporal artery biopsy, the sensitivity of "halo sign" to diagnose GCA is 40% to 100%, and the specificity is 68% to 100%. If "halo signs" are detected in both temporal arteries, their diagnostic specificity is higher. "Hyp" can also occur in granulomatous polyangiitis, tuberculosis and and other lesions.

Figure 1: 2022 New GCA Classification Standards

Treatment

Glucocorticoid is a first-line drug for the treatment of GCA; in order to further control vascular inflammation, reduce the dosage of glucocorticoids and reduce the risk of disease recurrence, it should be treated with immunosuppressant .

Table 2: Drug treatment of GCA

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References:

[1]Hunder GG, Bloch DA, Michel BA, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990;33(8):1122-8.

[2]Ponte C, et al.2022 American College of Rheumatology/EULAR classification criteria for giant cell arteritis. Ann Rheum Dis 2022;81:1647–1653.

This article review丨Chen Xinpeng Associate Chief Physician

Editor丨Carser

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