Giant cell arteritis

Giant cell arteritis is a granulomatous inflammation of the aorta and its major branches, predominantly the extracranial branches of the carotid artery, with frequent damage to the temporal artery. The disease usually occurs in patients older than 50 years and is often combined with rheumatic polymyalgia.

[1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11]

Epidemiology

Giant cell arteritis is affected mainly by people of the European race. The incidence varies widely from 0.5 to 23.3 cases per 100,000 population over 50 years. Its increase in older age groups is noted. Women are sick somewhat more often than men (ratio 3: 1). The disease is more common in Northern Europe and America (especially among Scandinavian emigrants) than in the southern regions of the globe.

[12], [13], [14], [15], [16], [17], [18], [19], [20], [21], [22]

How does giant cell arteritis appear?

Sometimes the disease giant cell arteritis begins acutely, patients can clearly mark the day and hour of the onset of the disease, but in most cases the symptoms of the disease appear gradually.

To the constitutional signs of the disease include fever (subfebrile and febrile), which is often for a long time the only symptom of the disease, heavy sweat, general weakness, anorexia, weight loss (up to 10 kg and more for several months), depression.

Vascular disorders depend on the localization of the process to the arterial channel. When the temporal artery suffers a constant acute acute bilateral headache develops with localization in the frontal and parietal region, tenderness when touched to the skin of the skull, swelling, swelling of the temporal arteries and weakening of their pulsation. Headache in the defeat of the occipital artery is localized in the occipital region.

The pathology of the maxillary artery leads to "intermittent claudication" of the masticatory muscles, causeless toothache or "intermittent chromosomes" of the tongue (when the process is localized in the lingual artery). Changes in the external carotid artery lead to swelling of the face, impaired swallowing and hearing

Inflammation of the arteries, blood-supplying eyes and eye muscles, can lead to a visual impairment, often irreversible, which is the first symptom of the disease. Described ischemic choriorentinitis, corneal edema, iritis, conjunctivitis, episcleritis, scleritis, anterior ischemic optic neuropathy. A transient decrease in vision (amavrosis fugax) and diplopia is very characteristic. The development of blindness is the most formidable early complication of giant cell arteritis.

There are pathological changes (aneurysm) of the aorta (mainly the thoracic region) and large arteries, as well as a symptom of tissue ischemia.

Rheumatic polymyalgia occurs in 40-60% of patients, and in 5-50% with a temporal artery biopsy, signs of an inflammatory process are found.

Joint damage occurs in the form of symmetric seronegative polyarthritis resembling rheumatoid arthritis in the elderly (involving mainly knee, wrist and ankle joints, less often proximal interphalangeal and metatarsophalangeal joints), or mono-oligoarthritis.

Various symptoms of the pathology of the upper respiratory tract occur in 10% of patients and may be the first signs of the disease. Prevalent non-productive cough in the background of fever. Significantly, there are pains in the chest and throat. The latter are often significantly expressed and, apparently, are caused by the defeat of the branches of the external carotid artery, predominantly a. Pharyngea ascendens. Inflammatory changes rarely affect the lungs directly. There are only separate descriptions of such cases.

How to recognize giant cell arteritis?

The most important laboratory features reflecting giant cell arteritis are the marked increase in ESR and CRP. However, some patients report normal values of ESR. A more sensitive indicator of activity is an increase in the concentration of CRP and IL-6 (more than 6 pg / ml).

Ultrasound with high resolution does not allow differentiating inflammatory lesions of vessels from atherosclerotic. With MRI, you can find the presence of inflammatory changes in the thoracic aorta, the formation of an aneurysm.

In radiography and CT of the lungs, basal interstitial fibrosis, diffuse reticular changes in the pulmonary pattern, multiple nodules, aneurysms of the thoracic aorta are detected. The results of bronchoalveolar lavage in patients with clinical signs of the pathology of the respiratory system, but without x-ray changes, reveal signs of T-lymphocytic alveolitis with a predominance of CD4 + lymphocytes.

Diagnosis of giant cell arteritis is based on the classification criteria of ARA. The disease should be suspected in all patients over 50 with severe headaches, visual impairment, symptoms of rheumatic polymyalgia, a significant increase in ESR and anemia. To confirm the diagnosis, it is advisable to conduct a biopsy of the temporal artery. However, since giant cell arteritis often develops focal segmental vascular lesions, negative biopsy results do not completely exclude this diagnosis. In addition, this does not serve as a basis for not prescribing glucocorticosteroids.

Differential diagnostics

Differential diagnosis of giant cell arteritis is carried out with a wide range of disease, with symptoms of rheumatic polymyalgia and damage to large vessels. These include rheumatoid arthritis and other inflammatory joint diseases in the elderly, shoulder joint damage (humeropathy periarthritis), inflammatory myopathies. Malignant neoplasms, infections, hypothyroidism (autoimmune thyroiditis), Parkinson's disease, systemic amyloidosis. Atherosclerotic vascular lesion.

[23], [24], [25], [26], [27], [28], [29], [30], [31], [32], [33]

How to treat giant cell arteritis?

If there is a suspicion of giant cell arteritis and excluding other diseases (tumors, etc.), glucocorticoid therapy should be started immediately to prevent the development of blindness and irreversible damage to the internal organs.

Glucocorticosteroids - the main method of treatment for giant cell arteritis. Prednisolone is also used at a dose of 40-60 mg / cyto in several ways until the normalization of ESR and the disappearance of symptoms. Reduce the dose of 2.1 s mg / cy every 2 weeks to 20 mg / day, then 10% every 2 weeks to 10 mg / day, then 1 mg every 4 weeks 1d whether there are no visual impairments or lesions of large vessels, an adequate starting dose of prednisolone may be less than 20 mg / day. In the process of decreasing the dose of prednisolone, the symptom is carefully monitored, the ESR is monitored every 4 weeks during the first Snake, then every 12 sowing within 12-18 months after completion of treatment.

In the severe course of giant cell arteritis, the dose of glucocorticoids should be increased to 60-80 mg / day, or pulse therapy with methylprednisolone followed by a transition to a maintenance dose of prednisolone 20-30 mg / day, or methotrexate (15-17.5 mg / week). When appointing methotrexate, it is necessary to remember the possibility of developing pneumonitis on its background.

The duration of therapy is assessed individually for each patient. If within 6 months against the background of taking prednisolone at a dose of 2.5 mg / day clinical symptoms of the disease are absent, treatment can be stopped. Admission of acetylsalicylic acid at a dose of 100 mg / day reduces the risk of developing blindness and cerebrovascular accidents.

What is the prognosis of giant cell arteritis?

In general, the prognosis for the life of patients on giant cell arteritis is favorable. The five-year survival rate is almost 100%. However, there is a serious risk of development of various complications of the disease, primarily damage to the arteries of the eyes, leading to a partial or complete vision.