The secret of giant cell arteritis: Why is it an emergency warning for vision loss?

Giant Cell Arteritis (GCA), commonly known as temporal arteritis, is an autoimmune inflammatory disease that affects the large blood vessels. Symptoms of this disease vary and include headaches, temporal pain, flu-like symptoms, double vision, and difficulty opening your mouth. Of particular concern is that GCA may lead to blockage of the ophthalmic artery, which can cause blindness, or even cause aortic dissection or aneurysm, which are extremely dangerous complications. GCA is often closely associated with polymyalgia rheumatica (PMR), the underlying cause of which remains unclear.

"The pathology of GCA involves inflammation of the small blood vessels that supply the large arteries, primarily affecting arteries around the head and neck, but also in the chest."

The diagnosis of GCA is relatively complex and is usually based on symptoms, blood tests, and medical imaging techniques. It can ultimately be confirmed by temporal artery biopsy. However, in about 10% of cases, temporal artery biopsy results are normal. Treatment is usually with high-dose steroids (eg, fluclomethalin), which are gradually reduced as symptoms improve. It is worth noting that in addition to steroids, bisphosphonates may be used to prevent bone loss or proton pump inhibitors to reduce the risk of gastrointestinal problems.

Symptom Overview

Common symptoms of giant cell arteritis include: cranial distension, fever, headache, scalp sensitivity, jaw cramps when chewing, tongue pain, blurred vision or sudden blindness, and even pain in the limbs. According to statistics, in 76% of cases involving the eye, the ophthalmic artery is involved, leading to arterial anterior optic ischemic neuropathy. Early recognition of these symptoms is crucial to prevent ischemic visual loss. Therefore, GCA is considered a medical emergency that requires immediate attention.

"Early and accurate diagnosis of GCA is crucial because prompt treatment can prevent irreversible loss of vision."

Association with other diseases

Recent studies have shown that varicella-zoster virus (VZV) antigens were detected in 74% of positive temporal artery biopsies, suggesting that VZV infection may trigger an inflammatory response. Furthermore, the coexistence of GCA and polymyalgia occurs in approximately 50% of cases. Although these two diseases have obvious differences on the surface, they can be regarded as different manifestations of the same process in terms of pathogenesis. It is worth mentioning that GCA may affect the aorta, leading to aneurysms and aortic dissection, and the incidence of these complications is quite high.

Diagnosis and imaging examinations

Diagnosis of GCA usually requires a physical examination of the head, during which the temporal arteries will be found to be prominent and may not be pulsatile. Common laboratory test manifestations include: elevated erythrocyte sedimentation rate, C-reactive protein and other indicators, while biochemical tests show abnormal liver function, increased platelet count, etc. Temporal artery biopsy is the gold standard for diagnosis; however, a negative result does not completely rule out the possibility of the disease.

"The halo sign is often seen during ultrasound examination of the temporal artery."

Treatment strategies

Because of the potential consequences of GCA, it is important to take immediate action. Oral steroids are usually the first choice of treatment, with high doses required initially and gradually reduced over the next 12 to 18 months. People are often concerned about the side effects of steroids. Possible short-term side effects include mood changes, necrotizing bone disease, and increased risk of infection, while long-term use can lead to weight gain, diabetes, and osteoporosis. Recently, it has been found that injection of tocilizumab can effectively control the recurrence and onset of GCA.

Epidemiological status

Giant cell arteritis mostly occurs in people over 50 years old, especially those in their 70s. It affects about 1 in 15,000 people each year and is twice as common in women as in men. People of northern European descent are more likely to be affected.

Impact on quality of life

GCA and its treatment have a certain impact on patients' quality of life. Patients are generally deeply disturbed by vision loss, severe pain, and fatigue. Traditional health assessment scales may not fully capture the unique impact of GCA when evaluating it, so a health-related quality indicator specifically for GCA (GCA-PRO) has been developed recently to help understand the profound impact of the disease on life.

Giant cell arteritis is not only a physical challenge for the elderly, but also a psychological and social test. Have you ever wondered how rare diseases like these can quietly hide in our daily lives and yet cause huge impacts in an instant?

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