The left temporal artery showed a hypoechoic wall thickening with a diameter of up to 1. This finding is suggestive of giant cell arteritis , especially in patients with polymyalgia rheumatica. Support Radiopaedia and see fewer ads. Updating… Please wait. Unable to process the form.

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Introduction: The Horton's disease, or temporal arteritis, is the most common autoimmune systemic vasculitis in adults, especially the old-aged. Objective: To review the literature about the several aspects of the Horton's disease, and confirm the otorhinolaryngologic clinical manifestations. Literature's Review: The disease's clinical manifestations start after 50 years old, and it's more frequent near 72 years old. The intense headache, temporal tumefaction, mandibular claudication and visual loss are the main signals and symptoms.

There may occur dysacusis, necrosis of the tongue and odynophagia. Considerations: Lots of aspects of the Horton's disease do not have their due elucidation and, even being a disease that affects many adults, it doesn't have deep investigations in great quantity.

A better understanding of this disease will lead to the increment of cure possibilities and reduction of morbidity in the patients, especially in ophthalmologic and otorhinolaryngologic areas. Clinical Aspects of the Temporal Arteritis. Its clinical manifestations depend upon the location of the relative or absolute ischemia of the territories irrigated by the affected vessels 3. Clinically, most cases have an insidious beginning, which explains the delay until the diagnosis that many times is observed in this disease 5.

The vision loss for ischemic optical neuropathy is the most feared complication, in addition to cerebral infarct and aortic aneurysm. In the otorhinolaryngologic focus, there may occur neurosensorial hearing loss and lingual necrosis 4 6. The diagnosis is eminently clinical and laboratorial, with histopathological confirmation, and its treatment is made with long-term corticotherapy 7.

The mortality for temporal arteritis results mainly from secondary factors and seldom from direct factors, with the aortic rupture. However both mortality and morbidity associated to such vasculitis are higher and higher, specially due to its clinical manifestations, the age of the population affected and the relative therapeutic aggressivity.

Because it is a multisystemic disease of late diagnosis and growing morbimortality, the accomplishment of a literature review is necessary whose objective is to clarify its clinical signs and symptoms, as well and confirm its otorhinolaryngologic manifestations both before the poorly broadcast knowledge of this disease and its relevance.

METHOD The review was made by means of researches on online databases, which provided articles and information for the production of such bibliographic review, and the sources and references to be accessed at any time for research and improvement, taking into account the scientific literature is constantly being renewed. It was Initially described in by Hutchinson, who observed it in the shape of a very painful inflammation of the temporal artery in an year-old patient.

Some years after, Horton and collaborators made the clinical and histopathological correlations of the disease and named it temporal arteritis. Later on, the term "Horton's Disease" ended up being consecrated. In , both mono and binocular vision loss was registered for the first time as a complication in the affected patients.

In , Paulley e Hughes described many forms of the disease. Since then, more varied types of clinical manifestations, prognostics and complications were observed 2. Epidemiology The Horton's Disease is the most prominent vasculitis in adults older than 50 years, with peak of incidence between 70 and 80 years 3 , and it's of about cases per Women have from 2 to 6 times more chances to be affected than men 1 , probably due to hormonal factors; however we observed that the female sex prevalence is associated with the rheumatic polymialgia and not in its pure form 5.

The percentage is also higher in persons with a history of smoking. The disease doesn't have preference for racial type, but is slightly higher in Caucasians, specially Scandinavian descendents, which probably relates to the distribution of the HLA-DR4 antigen among the races.

Etiopathogenesis Until today the etiopathogenesis has not been fully clarified. It's known it's a reaction depending on T-cells activated by antigen and that the giant cells hurt the inner coat and also damage the vessels elasticity, since they damage the vessels whose wall contains elastic tissue, and suggest that the elastin becomes antigenic in time and starts an immunomediated inflammatory reaction; however, no anti-elastin antibodies were found 8.

Nevertheless, an important feature is the fact the intracranial arteries are saved for they don't have elastic tissue. So we can say the more elastic tissue there is in the artery, the greater the probability to develop Giant Cells Arteritis 7, 8. It's worth remarking that the affection of the orbitary cavity arteries also has a relation with the quantity of elastic tissue the different arteries contain in its walls. While the ophthalmic and ciliary posterior arteries contain a moderate quantity of elastic tissue, the retina central artery doesn't contain much elastin, mainly when it penetrates the optical nerve sheath, and specially after perforating the nerve substance 9.

The inflammatory process typical of giant cells arteritis is generally superposed to the arteriosclerotic changes found in adults and old-aged. The inflammatory lesions are generally distributed in a sparse and uneven manner along the course of the affected vessels. Mostly important among all changes is the granulomatous inflammatory process in several focuses with the presence of lymphocytic, histiocytes and epitheliod cells along the elastic lamina and involving the medium and adventitious layers.

Multinuclear giant cells are commonly seen in the vicinity of degenerated muscular cells or sometimes close to the elastic fragmented cell. The adventitious layer inflammation is prominent 8, 9. However, there are traces that in addition to the immune system, other factors are involved, such as infectious, environmental, racial origin Caucasian Europeans , degenerative processes relating to aging and genetics, because it's known the patients with Giant Cells Arteritis and its variant Rheumatic Polymialgia have an increased prevalence of antigens of histocompatibility HLA, DL-4 and CW3 7, 8.

The involvement of nerves and nervous terminations on the artery wall and surface is what causes spontaneous pain and the artery pulsation 9. Signs and Symptoms Generally the constitutional symptoms precede the headache, polymialgia and ocular and neurological manifestations.

The systemic symptoms include fever, adynamia, inapetency, weight loss and unspecific myalgias, mainly in the morning, with rigidity of the neck and the musculature of the pelvic and shoulder girdle 8, 9 , as well as night sudoresis and anorexia 5. The rheumatic polymialgia with pain and hardening of the neck, dorsum, pelvic and shoulder girdle, as well as the upper and lower ends proximal muscles is common in the GCA.

The claudication during the walk may also be observed 9. Jaw claudication is an GCA's classical symptom. The patient feels pain when he chaws or talk longer and improves with rest and it occurs in cases where there is facial artery involvement. The tongue and deglutition muscles claudication and chewing muscles spasms, named as lockjaw may also occur 8, 9. Other causes for vision loss in GCA include posterior or retrobulbar ischemic optic neuropathy, much less frequent than NAION, ischemic ocular syndrome and obstruction of the central artery of the retina or its branches.

Ischemia of the anterior segment of the eye with keratopathy, chemosis, scleritis and episcleritis and unevenness of the pupil shape may also occur. Later findings include cataract, secondary glaucoma, rubeosis iridis and nonreactive pupils 8, 9. Campimetric defects and cortical blindness, resulting from the vertebral, carotid and hypofisary arteries involvement have also been reported 9.

Ophthalmoplegia is a frequent manifestation of the GCA and, sometimes, when the tropy measures only a few dioptres, the patients complain of diplopia without the ophthalmoparesis to be detected in the clinical exam.

The contrary, that is, ophthalmoplegia without diplopia, is very common, and it occurs when the vision is significantly reduced. The ophthalmoplegia may precede other more common manifestations of the disease 9. Anisocoria occurs rarely in the GCA, since the ciliary ganglion is rich in anastomoses. However the mydriasis associated with ophthalmoplegia has been reported. The Horner's syndrome may develop following the central, pre-ganglionar or post-ganglionar oculo-sympathicomimetic neurons ischemia 9.

Within the neurologic manifestations, the main complaint is headache which is the GCA's cardinal affection and the most frequent complaint that leads the patient to the doctor. Such headache is from a short time ago, about 15 days to 1 month and is different from other headaches the patient could have had before.

Some report pain in any part of the head or diffusely as in the muscular contraction headache 9. In addition, it may present with a number of other manifestations, such as, depression, mental confusion, dementia, psychosis; infarct or cerebral hemorrhage, transient ischemic episodes; ataxia, trembling, deafness, tinnitus, vertigo manifestations relating to the facial artery ; facial paralysis, syncope, convulsions, meningism, insipid diabetes; transverse myelopathy and peripheral neuropathies 8.

Such other manifestations are not as frequent as headache, but in some cases it may be absent, and there's the presence of other symptoms Arteritis of Hidden Giant Cells.

In this case, the visual loss for anterior ischemic optic neuropathy may be the first manifestation.

The main otorhinolaryngologic manifestations are dry persistent cough, odinophagy, dysphonia, lingual paresthesia and claudication, buzz, glossitis, lingual ischemia and necrosis and neurosensorial dysacusis, which may range from slight to deafness in case the treatment is not duly performed 3, 7, 11 12, Diagnosis The Giant Cells Arteritis diagnosis is mostly clinical. By this format, a patient is deemed to be a carrier of GCA if, at least, three of the five criteria are met.

The presence of three or more of these five criteria is associated to a sensitivity of In the initial phase, the exam may reveal only subtle changes such as soreness upon the artery palpation. With the disease's progression, the artery tends to become ingurgitated, tortuous and salient. The area adjacent to the vessel may be edemaciated. Generally upon palpation the artery becomes thick, hard and nodular and the soreness expands to the circumjacent region.

The pulse is commonly diminished or absent in the advanced phases. The retinal examination is normal in an individual with full vision. In cases of ischemic optic neuropathy, in the acute phase, the papilla is edemaciated and with small hemorrhages. In the chronic phase, such changes are replaced for paleness and atrophy 5. The visual loss is an important finding. The visual loss generally results from the anterior ischemic optic neuropathy.

Thus, we should suspect of GCA in patients above 50 years old with this ophthalmologic condition diagnosis, and start the corticotherapy even with normal ESR However within the normality range the ESR doesn't prevent the diagnosis. The reactive protein C is also elevated in the GCA. Other possible laboratorial changes include: Slight normocytic normochromic anemia; discreet leucocytosis; liver function abnormalities, more commonly discreet increases of the of alkaline phosphatase and transaminases; elevation of the plasma fibrinogen levels; alpha-2 globulin, complement and gamma-globulin and albumin reduction 5.

The temporal artery biopsy must be carried out in all patients for whom there is suspicion of GCA based on the anamnesis and physical exam, even if the ESR had been normal 5. We must perform temporal artery unilateral biopsy and keep on with the treatment when the biopsy is positive.

When the biopsy is negative, but the clinical suspicion is high, we should perform contralateral biopsy. In the patients with intermediate clinical suspicion we should request temporal artery biopsy and ESR. This group is composed by patients with any of the criteria of major specificity or with anterior ischemic optic neuropathy. Moreover, any of the following findings represent an intermediate clinical suspicion: High ESR, recent beginning headache in old-aged patient, or clinical changes of the temporal artery.

If both temporal arteries biopsy is negative, other reasons for ESR increase must be researched infectious diseases, neoplasms, diabetes or conjunctive tissue diseases, for example. In patients with normal ESR and temporal artery unilateral biopsy without changes, the GCA diagnosis is not much probable. The microscopic exam reveals a panarteritis with perivascular inflammatory infiltrate, intimate hypertrophy, medium necrosis associated with granulomatous tissue formation, presence of giant cells and light thrombosis.

There is interposition of healthy and other committed areas. Biopsies obtained in initial phases normally reveal a prevalence of inflammatory infiltrate, while in the more advanced phases, we observe more easily the formation of granuloma and giant cells. In the context of temporal arteritis, the peripheral nervous system involvement is not much frequent 3. Therefore, to achieve the giant cells arteritis diagnosis, we must try and make blood tests, biopsy, ophthalmologic exam, angiography, ultrasonography and thyroid function.

The differential diagnosis includes dental problems, trigeminal neuralgia, sinusitis, otitis, blood vessels or ocular muscles alterations, among many other causes of headache Treatment The treatment of patients with temporal arteritis is crucial to avoid vision loss and the therapy should be started based on the clinical suspicion, and not on the biopsy results We use one type of corticosteroid, prednison.

Typically, the treatment begins with mg orally everyday in adults 16 , and may reach mg per day Another drug that may also be used is metylprednisolon 2.


Giant cell arteritis

Giant cell arteritis GCA is a common granulomatous vasculitis affecting medium- to large-sized arteries. It is also known as temporal arteritis or cranial arteritis, given its propensity to involve the extracranial external carotid artery branches such as the superficial temporal artery. Giant cell arteritis is the most common primary systemic vasculitis. It has an incidence of per million persons per year 6. Typically affects older individuals with patients usually being older than 50, with a peak incidence between the ages of 70 and 80 3. There is a recognized female predilection. It is histologically similar to other large vessel vasculitides such as Takayasu arteritis showing granulomatous inflammation of arteries with infiltration predominantly by histiocytes, lymphocytes, and multinucleated giant cells.


Temporal Arteritis

PA: x 90 mmHg; FC e de pulso: 72 bpm. A paciente estava alerta e orientada. Pathological changes in temporal arteries removed from unselected cadavers. J Clin Path. A year epidemiologic, clinical, and pathologic study. Ann Intern Med. The epidemiology of biopsy-positive giant cell arteritis: special reference to changes in the age of the population.

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