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Occult giant cell arteritis: ocular manifestations.

PURPOSE: To report the incidence, visual symptoms, and ocular signs of occult giant cell arteritis in patients who initially presented with visual symptoms and ocular signs of giant cell arteritis. Occult giant cell arteritis was defined as ocular involvement by giant cell arteritis without any systemic symptoms and signs of giant cell arteritis. METHODS: In a prospective study from 1973 to 1995, we investigated 85 patients who had ocular involvement caused by giant cell arteritis and whose diagnosis of giant cell arteritis was confirmed on temporal artery biopsy. At the initial visit, patients were questioned specifically on systemic and ocular symptoms and signs of giant cell arteritis at or before the onset of visual disturbance. Erythrocyte sedimentation rate (Westergren) and C-reactive protein level were evaluated before the start of systemic corticosteroid therapy. RESULTS: Eighteen (21.2%) of 85 patients had occult giant cell arteritis. There was no significant difference in age and sex distribution between patients with and without systemic symptoms of giant cell arteritis. Although both groups of patients had abnormal erythrocyte sedimentation rate and C-reactive protein level, there was a significant difference in erythrocyte sedimentation rate (P < .0001) and C-reactive protein level (P=.0133), these being relatively lower in patients with occult giant cell arteritis. The ocular symptoms in the 18 patients with occult giant cell arteritis were visual loss of varying severity in 18 (100%), amaurosis fugax in six (33.3%), diplopia in two (11.1%), and eye pain in one (5.6%). Ocular ischemic lesions consisted of anterior ischemic optic neuropathy in 17 (94.4%), central retinal artery occlusion in two (11.1%), and cilioretinal artery occlusion in two (of 11 patients with satisfactory fluorescein angiography [18.2%]). The ocular symptoms and ischemic lesions were seen in a variety of combinations. CONCLUSIONS: Because occult giant cell arteritis is a potential cause of blindness, its early diagnosis is the key to preventing blindness; it is important to recognize that 21.2% of patients with giant cell arteritis and visual loss do not have any systemic symptoms of giant cell arteritis. Thus, in persons older than 55 years, amaurosis fugax or visual loss, development of an acute ocular ischemic lesion (particularly arteritic anterior ischemic optic neuropathy), and abnormal C-reactive protein level, with or without elevated erythrocyte sedimentation rate and systemic symptoms, should raise a high index of suspicion for giant cell arteritis.

Aged↗

Risk factors and predictive models of giant cell arteritis in polymyalgia rheumatica.

OBJECTIVE: To identify in polymyalgia rheumatica the best set of predictors for a positive temporal artery biopsy and to define predictive models with either a high or low probability of giant cell arteritis (GCA). PATIENTS AND METHODS: Retrospective study of 227 patients, 137 with polymyalgia rheumatica unassociated with arteritis (group A) and 90 with polymyalgia associated with biopsy-proven giant cell arteritis (group B or training set). Data on demographic features, clinical and laboratory abnormalities were collected. Risk factors for arteritis were estimated by nonlinear logistic regressions. Simple predictive models were constructed with those predictors more related to arteritis by multivariable analysis. These models were then tested in group B and in 89 cases of arteritis without polymyalgia rheumatica (group C or test set). RESULTS: The best predictors of arteritis were a new headache odds ratio (OR) 13.6 (95% confidence interval [CI] 4.7 to 39.3); age at onset < 70 years OR 0.11 (CI 0.04 to 0.35); abnormal temporal arteries OR 4.2 (CI 1.3 to 13.7); raised liver enzymes OR 2.9 (CI 1.1 to 7.8), and jaw claudication OR 4.8 (CI 1.0 to 22.7). Amaurosis was only observed in patients with arteritis. Three subsets had a very high risk of arteritis: (1) Patients with recent headache, abnormal arteries, and > or = 70 years at disease onset: sensitivity 44%, positive predictive value (PPV) 93%, likelihood ratio (LR) 20.3; (2) patients with a new headache, jaw claudication, and abnormal arteries: sensitivity 34.4%, PPV 96.9%, LR 47.2; and (3) those, that in addition to the last 3 features, were > or = 70 years of age at disease onset: sensitivity 26.7%, PPV 100%. We could also identify a subset with a very low risk of arteritis constituted by patients < 70 years, without headache, and with clinically normal temporal arteries: sensitivity 1.1%, PPV 1.7%, LR 0.03. In group C or the test set, these four predictive models correctly identified 57.3%, 29.2%, 23.6, and 3.4% of patients, respectively. CONCLUSIONS: In polymyalgia rheumatica it is feasible to identify subsets with a very high likelihood of GCA. Although in some of these subsets the diagnosis of arteritis is almost certain, we suggest that even then it should be confirmed by temporal artery biopsy. By contrast, in those patients with polymyalgia < 70 years and without cranial features of giant cell arteritis, the risk of vasculitis is so low that the biopsy could be initially avoided and the patient treated with low-dose corticosteroids.

Aged↗

Ultrasonography of the temporal, periorbital and carotid arteries in the diagnosis of giant cell arteritis and its neuroophthalmological complications.

AIM: Evaluation of the diagnostic contribution of colour duplex sonography of the superficial temporal and the carotid arteries and Doppler sonography of the periorbital arteries by analysing the results in patients with giant cell arteritis with and without neuroophthalmological complications and patients with other diagnoses and neuroophthalmological complications. METHODS: In a case control study, ultrasonographic findings in patients with neuroophthalmological complications (25 giant cell arteritis, 23 non giant cell arteritis) were compared to those of 62 patients suffering from giant cell arteritis without neuroophthalmological complications. Concentric hypoechogenic mural thickening (a so-called halo) was considered as an ultrasonographic finding typical of giant cell arteritis. Absent or retrograde signals not corresponding to carotid occlusive disease were classified as Doppler sonographic findings typical of giant cell arteritis of the periorbital arteries. RESULTS: There are higher rates of abnormalities in Doppler sonography of the periorbital arteries as well as halos in combination with stenosis of the temporal arteries in patients with giant cell arteritis with neuroophthalmological complications. This suggests that in patients with giant cell arteritis and neuroophthalmological complications, the haemodynamic situation is more severely disturbed. Patients suffering from giant cell arteritis with and without neuroophthalmological complications had the same rate of temporal artery abnormalities on colour duplex sonography (72 vs. 71 %), whereas abnormalities in the periorbital arteries were clearly different (40 vs. 8 %). Only 1 patient with giant cell arteritis without neuroophthalmological complication had anomalies of the carotid arteries typical of giant cell arteritis. In patients with other diagnoses and neuroophthalmological complications, the rate of anomalies in temporal artery colour duplex sonography and Doppler sonography of the periorbital arteries was 9 % and 0 %. CONCLUSION: Colour duplex sonography of the superficial temporal and carotid arteries and Doppler sonograpy of the periorbital arteries are complementary methods and should be part of the evaluation of all patients suspected to suffer from giant cell arteritis.

Aged↗

Diagnosing giant cell temporal arteritis.

Arteritis is a complex and totally underestimated clinical entity that may present in several ways. Due to the diversity of clinical features and the frequent overlapping of clinical syndromes, many different classifications are available for arteritides, but the simplest classification for the practicing physician is the broad division of arteritis into giant cell arteritis and non-giant cell arteritis. Giant cell arteritis encompasses two distinct clinical entities that are pathologically indistinguishable from each other; but both involve arteritis of median and large-sized arteries characterized by an infiltration of giant cells. These include temporal arteritis and Takayasu's arteritis. The non-giant cell arteritis encompasses a greater variety of uncommon diseases that are often associated with other systemic processes such as systemic lupus erythematosus or periarteritis nodosa, and it generally results in ischemic organ dysfunction that is amenable only to medical treatment. Less commonly, however, non-giant cell arteritis may become manifested as acute arterial occlusion caused by circulating anticoagulants, abdominal apoplexy as intra-abdominal bleeding from rupture of micro aneurysms of artery branches, gastrointestinal bleeding from local necrosis of the gastrointestinal tract, or deep-vein thrombosis secondary to hypercoagulable states. Although temporal arteritis has generated a voluminous body of literature, its precise etiology remains elusive. This study summarizes our experience in 15 cases of temporal arteritis and one case is presented to increase knowledge about this disease entity.

Aged↗

The role of the thymus in development of necrotizing arteritis in transgenic rats carrying the env-pX gene of human T-cell leukemia virus type-I.

Necrotizing arteritis mimicking polyarteritis nodosa occurred in transgenic rats carrying the env-pX gene of human T-cell leukemia virus type I. To investigate the pathogenesis of necrotizing arteritis in these rats (env-pX rats), adoptive transfers of spleen cells and bone marrow cells were done from env-pX rats before they developed arteritis to nontransgenic rats. Necrotizing arteritis occurred in lethally irradiated nontransgenic rats reconstituted by env-pX spleen cells, thus indicating that the env-pX transgene in affected vessels may not be essential for the development of arteritis. In contrast, arteritis was not induced in nontransgenic recipients by adoptive transfers of env-pX bone marrow cells, which suggested that T cells derived from the env-pX thymus may play a role in the development of arteritis. To clarify if the process of differentiation of T cells in the env-pX thymus is crucial to develop necrotizing arteritis, reciprocal exchange of thymus frameworks was done between env-pX and nontransgenic rats. Necrotizing arteritis occurred in nontransgenic rats with an env-pX thymus framework, whereas development of arteritis was suppressed in env-pX rats in which the thymus framework was replaced with a nontransgenic one. This collective evidence shows that the thymus is directly associated with the development of necrotizing arteritis in env-pX rats.

Animals↗

Efficacy of unilateral versus bilateral temporal artery biopsies for the diagnosis of giant cell arteritis.

PURPOSE: To determine the utility of unilateral versus bilateral temporal artery biopsies in detecting the pathologic changes of giant cell arteritis. METHODS: We performed a retrospective study to determine the utility of unilateral versus bilateral temporal artery biopsies in detecting the pathologic changes of giant cell arteritis. The pathologic reports of consecutive temporal artery biopsy specimens received at the Wilmer Ocular Pathology Laboratory over a 28-year period from 1968 to 1996 were reviewed. RESULTS: Of 908 specimens examined from 758 patients, 300 specimens were simultaneous bilateral biopsies from 150 patients, 72 specimens were bilateral sequential biopsies from 36 patients, and the remaining 536 specimens were unilateral biopsies from 536 patients. Of the 186 patients who had bilateral simultaneous or nonsimultaneous biopsies, 176 had identical diagnoses on both sides. In four patients, no artery was obtained on one side. In each of the remaining six patients, five of whom had bilateral simultaneous biopsies and one of whom had bilateral sequential biopsies performed 8 days apart, the biopsy specimen from one side was interpreted as showing only arteriosclerotic changes with no evidence of active or healed arteritis, whereas the other specimen was interpreted as showing either probable healed arteritis (three specimens) or possible early arteritis (three cases). In none of the six patients with differing diagnoses between the two sides was one side interpreted as showing definite, active giant cell arteritis. Five of the six patients were subsequently determined to have giant cell arteritis, based on a combination of clinical findings, erythrocyte sedimentation rate, and response to treatment with systemic corticosteroids. CONCLUSIONS: The results of this study indicate that performing a bilateral simultaneous or sequential temporal artery biopsy improves the diagnostic yield in at least 3% of cases of giant cell arteritis, whereas in 97% of cases, the two specimens show the same findings. Thus, in patients in whom only one artery can be biopsied, there is a high probability of obtaining the correct diagnosis. Nevertheless, although the improvement in diagnostic yield of bilateral temporal artery biopsies is low, the consequences of both delayed diagnosis and treatment of giant cell arteritis as well as the use of systemic corticosteroids in patients who do not have giant cell arteritis are of such potential severity that consideration should always be given to performing bilateral temporal artery biopsies in patients suspected of having the disease.

Aged↗

Color duplex ultrasonography in the diagnosis of temporal arteritis.

BACKGROUND: The diagnosis of temporal arteritis usually requires a biopsy of the temporal artery. We examined the usefulness of color duplex ultrasonography in patients suspected of having temporal arteritis. METHODS: In this prospective study, all patients seen in the departments of rheumatology and ophthalmology from January 1994 to October 1996 who had clinically suspected active temporal arteritis or polymyalgia rheumatica were examined by duplex ultrasonography. The final diagnoses, made according to standard criteria, were temporal arteritis in 30 patients, 21 with biopsy-confirmed disease; polymyalgia rheumatica in 37; and negative histologic findings and a diagnosis other than temporal arteritis or polymyalgia rheumatica in 15. We also studied 30 control patients matched for age and sex to the patients with arteritis. Two ultrasound studies were performed and read before the biopsies; one ultrasonographer was unaware of the clinical information. RESULTS: In 22 (73 percent) of the 30 patients with temporal arteritis, ultrasonography showed a dark halo around the lumen of the temporal arteries. The halos disappeared after a mean of 16 days (range, 7 to 56) of treatment with corticosteroids. Twenty-four patients (80 percent) had stenoses or occlusions of temporal-artery segments, and 28 patients (93 percent) had stenoses, occlusions, or a halo. No halos were identified in the 82 patients without temporal arteritis; 6 (7 percent) had stenoses or occlusions. For each of the three types of abnormalities identified by ultrasonography, the interrater agreement was > or =95 percent. CONCLUSIONS: There are characteristic signs of temporal arteritis that can be visualized by color duplex ultrasonography. The most specific sign is a dark halo, which may be due to edema of the artery wall. In patients with typical clinical signs and a halo on ultrasonography, it may be possible to make a diagnosis of temporal arteritis and begin treatment without performing a temporal-artery biopsy.

Aged↗

Von Willebrand factor in the outcome of temporal arteritis.

OBJECTIVE: To determine fluctuation in circulating von Willebrand factor (vWF) in the outcome of patients with temporal arteritis. METHODS: Plasma vWF antigen concentrations were measured in 65 patients with biopsy proven temporal arteritis at different disease activity stages, in 12 with isolated polymyalgia rheumatica, and in 16 controls. Fourteen temporal arteritis patients underwent serial determinations during the course of their disease. RESULTS: vWF concentrations were significantly raised in temporal arteritis (mean 220 [arbitrary units], range 96 to 720) and in polymyalgia rheumatica (mean 196, range 103 to 266) compared with healthy controls (mean 98, range 75 to 137) (P < 0.05). Although vWF values tended to be higher in temporal arteritis, no significant differences were found between temporal arteritis and polymyalgia rheumatica patients nor between temporal arteritis patients with ischaemic complications (mean 269, range 130 to 720) and those who presented with polymyalgia rheumatica or constitutional symptoms only (mean 179, range 140 to 220). The highest levels were obtained in patients with associated, mainly infectious, diseases (mean 631, range 240 to 1680). Raised vWF values found in active temporal arteritis patients (mean 220, range 96 to 720) persisted within the first two years after the beginning of treatment (mean 244, range 102 to 510) but tended to normalise in patients in long term remission (mean 143, range 50 to 260). CONCLUSIONS: Persistent elevation of vWF during early remission of temporal arteritis might represent an endothelial activation status induced by a remaining inflammatory microenvironment rather than a marker of endothelial cell injury. In long term remission, decreasing vWF concentrations might reflect progression of inflammatory lesions to a healing stage.

Acute Disease↗

[The significance of color duplex ultrasonography for the diagnosis of temporal arteritis].

We examined the usefulness of color duplex ultrasonography in patients suspected of having temporal arteritis. Five patients, who were all aged 70 or older, developed a new onset of localized headache with temporal artery abnormalities, and had an elevated erythrocyte sedimentation rate of > 100 mm/hour. The final diagnoses were temporal arteritis in three patients, polymyalgia rheumatica in one, and probable healed temporal arteritis in one. Color duplex ultrasonography showed stenoses, which were confirmed histologically as well, in the superficial temporal artery of all patients. The characteristic findings of active temporal arteritis were, however, demonstrated in only three biopsy specimens, and in the remaining two the stenoses were thought to be related to previous arteritis. The hypoechoic halo, which has been reported to be a characteristic finding of color duplex ultrasonography in active temporal arteritis, was detected in only one patient with active temporal arteritis and another one with probable healed temporal arteritis. No stenoses were demonstrated in the superficial temporal arteries of 30 control subjects (20 with at least one risk factor of atherosclerosis and 10 without it). Color duplex ultrasonography can therefore be considered a powerful method for detecting stenoses in the superficial temporal artery. Its ability to identify their etiology is, however, unsatisfactory, so that temporal artery biopsy remains undoubtedly the most reliable test for etiological evaluation. We thus recommend color duplex ultrasonography as a supplementary method for the diagnosis of temporal arteritis, because it can provide useful information concerning the appropriate site of temporal artery biopsy.

Aged↗

Giant cell and Takayasu arteritis.

PURPOSE OF REVIEW: Giant cell arteritis and Takayasu arteritis are well known large vessel vasculitides with an unknown etiology. As they have similar clinical features, this short article reviews recent advances in clinical and pathophysiological findings, focusing in particular on papers published in the past year. RECENT FINDINGS: Delayed gadolinium-enhanced magnetic resonance imaging showed delayed hyperenhancement in the aortic wall in Takayasu arteritis. This technique may be useful in monitoring disease activity or inflammation in the arterial wall and can be used for small vessels such as temporal arteries in giant cell arteritis with high-resolution imaging. Evidence is accumulating that antitumor necrosis factor-alpha monoclonal antibody therapy can be useful for patients refractory to corticosteroid and/or immunosuppressant treatment. Functional promoter polymorphisms of genes encoding inducible nitric oxide synthase and I-kappaB-like protein were suggested to be associated with susceptibility to giant cell arteritis and Takayasu arteritis, respectively. SUMMARY: Advances in imaging technique will make it possible to evaluate inflammatory activity of the vascular lesions and provide a useful guide for treatment of giant cell arteritis and Takayasu arteritis. Further understanding of the pathophysiological mechanism may contribute to the development of new medicine targeting critical factors in the pathogenesis, such as antitumor necrosis factor-alpha agents.

Antibodies, Monoclonal↗

[Association of sarcoidosis and Takayasu's arteritis: an additional case report].

INTRODUCTION: Takayasu arteritis has been described in association with various auto-immune disorders (mainly inflammatory digestive tract diseases). However, only few cases of Takayasu arteritis associated with sarcoidosis have been reported, raising the question of an association by chance. EXEGESIS: We report the case of a 26-year old woman with a 1-year history of sarcoidosis, who presented with a right painful upper limb, revealing inflammatory humeral, axillary and subclavian arteritis related to Takayasu arteritis. The patient was successfully treated with steroids. CONCLUSION: Our case report suggests that both Takayasu arteritis and sarcoidosis may be related, and that Takayasu arteritis or Takayasu arteritis-like granulomatous vasculitis may be, in fact, a complication of sarcoidosis. It also indicates that a complete vascular clinical examination should be performed in patients with sarcoidosis, in order to detect asymptomatic underlying inflammatory arteritis.

Adrenal Cortex Hormones↗

Ocular manifestations of giant cell arteritis.

PURPOSE: To report the ocular manifestations of giant cell arteritis using the strict criterion of a positive temporal artery biopsy for diagnosis of giant cell arteritis. METHODS: In a prospective study from 1973 to 1995, we investigated 170 patients whose diagnosis of giant cell arteritis was confirmed on temporal artery biopsy. At the initial visit, all patients were questioned regarding systemic and ocular signs and symptoms of giant cell arteritis and underwent ophthalmic, erythrocyte sedimentation rate (Westergren), and C-reactive protein evaluations. Any patient with a high index of suspicion of giant cell arteritis was immediately started on systemic corticosteroid therapy and had temporal artery biopsy performed as soon as possible. RESULTS: Eighty-five (50.0%) of the 170 patients with giant cell arteritis proven by temporal artery biopsy presented with ocular involvement. Ocular symptoms in patients with ocular involvement were visual loss of varying severity in 83 (97.7%), amaurosis fugax in 26 (30.6%), diplopia in five (5.9%), and eye pain in seven (8.2%); ocular ischemic lesions consisted of arteritic anterior ischemic optic neuropathy in 69 (81.2%), central retinal artery occlusion in 12 (14.1%), cilioretinal artery occlusion in 12 (of 55 patients with satisfactory fluorescein angiography [21.8%]), posterior ischemic optic neuropathy in six (7.1%), and ocular ischemia in one (1.2%). In almost every patient with giant cell arteritis, fluorescein fundus angiography disclosed occlusive disease of the posterior ciliary arteries. CONCLUSION: Because giant cell arteritis is a potentially blinding disease and its early diagnosis is the key to preventing blindness, it is important to recognize its various ocular manifestations.

Aged↗

Umbilical arteritis and phlebitis mark different stages of the fetal inflammatory response.

OBJECTIVE: Funisitis, the inflammation of the umbilical cord determined by histologic examination of the placenta, is evidence of a fetal inflammatory response. The inflammatory process may involve the umbilical vein (phlebitis) and one or both umbilical arteries (arteritis) and extend into the Wharton's jelly. This study was conducted to examine whether the pattern of inflammation of the umbilical cord correlates with a biochemical marker of systemic fetal inflammation (umbilical cord plasma interleukin-6) and an adverse neonatal outcome. STUDY DESIGN: This cohort study included 636 cases of preterm delivery (<36 weeks) with or without inflammation of the umbilical cord. Umbilical cord blood was collected at the time of delivery. The aim of pathologic examination was to characterize the extent of umbilical cord inflammation and the involvement of the vein (phlebitis), the involvement of one or both arteries (arteritis), and the presence of inflammation of the Wharton's jelly. Umbilical cord plasma interleukin-6 concentrations were assayed by a sensitive and specific immunoassay. RESULTS: Neonates with umbilical arteritis had a significantly higher median concentration of cord plasma interleukin-6 (median, 111 pg/mL; range, 0.1-19,230 pg/mL) than those without umbilical arteritis (median, 22.5 pg/mL; range, 0.9-511.6 pg/mL; P <.05). Also, severe neonatal morbidity occurred more frequently in infants with arteritis than in those without arteritis (74% vs 50%; P <.05). And finally, the most severe form of inflammation, which involves both arteries, vein, and Wharton's jelly, was associated with the highest median concentration of plasma interleukin-6 observed in this study (median, 182.6 pg/mL; range, 0.1-7,400 pg/mL), whereas inflammation limited to the vein (phlebitis) was associated with a lower concentration of cord plasma interleukin-6 (median, 29.1 pg/mL; range, 0.9-511.6 pg/mL; P <.05). CONCLUSION: Neonates whose placenta demonstrates umbilical arteritis have higher concentrations of umbilical cord plasma interleukin-6 and higher rates of adverse outcome than those without umbilical arteritis.

Arteritis↗

The role of 2-18F-fluoro-2-deoxy-D-glucose positron emission tomography in the diagnosis of giant cell arteritis of the temporal arteries.

OBJECTIVE: As one of the diagnostic criteria for giant cell arteritis affecting the temporal arteries (temporal arteritis) is still biopsy-proven vasculitis of the affected artery, the aim of our study was to evaluate the value of a non-invasive procedure, 2-(18)F-fluoro-2-deoxy-d-glucose (FDG) positron emission tomography (F-18-FDG-PET), in the diagnosis of Horton's disease. METHODS: During a period of 10 months, 22 consecutive patients with the clinical diagnosis of giant cell arteritis and a positive hypoechogenic halo in duplex sonography were re-examined with F-18-FDG-PET. Six patients had giant cell arteritis involving both the large arteries and the temporal arteries; five patients showed giant cell arteritis only in the large arteries without concomitant involvement of the temporal arteries, and the remaining 11 patients showed only involvement of the temporal arteries. All patients were examined by sonography and F-18-FDG-PET, which was performed before treatment with corticosteroids. RESULTS: All patients with positive signs of giant cell arteritis in duplex sonography, i.e. a hypoechogenic halo in the large arteries (thoracic, subclavian, axillary, iliac, aorta), also showed elevated FDG uptake in the same vessels, with complete agreement in the anatomical distribution of changes. When positive sonography was limited to the temporal arteries, FDG-PET was completely negative in the temporal arteries and all other arterial locations. CONCLUSION: PET is not yet suitable for the diagnosis of temporal arteritis and therefore cannot replace invasive biopsy. F-18-FDG-PET is well suited to the demonstration of giant cell arteritis in arteries exceeding 4 mm in diameter.

Aged↗

Temporal artery biopsy in giant-cell arteritis. A reappraisal.

We have reviewed the clinical records and histology of 135 patients who underwent temporal artery biopsy between 1973 and 1978. Biopsies were classified histologically as giant-cell arteritis (17%), atypical arteritis (6%), healed arteritis (2%), arteriosclerosis (67%), atherosclerosis (5%), or normal (3%). Most of the histological diagnoses made at the time of biopsy were confirmed but eight cases which had originally been reported as atypical or healed arteritis were classified as arteriosclerosis when reviewed. All 33 patients with histological evidence of arteritis were accepted as clinical cases of temporal arteritis (31) or polymyalgia rheumatica (2) and treated with steroids. A further 24 patients had negative biopsies (arteriosclerosis or atherosclerosis) but were considered on clinical grounds to have cranial arteritis. They too were treated and made a full recovery. In 43 cases, all of whom had negative biopsies, a final diagnosis was reached which was thought to account for the clinical symptoms (e.g., cerebrovascular accident, rheumatoid disease, migraine, etc.). As less than 60% of patients with clinical temporal arteritis had positive biopsies, we suggest that this procedure could be omitted and replaced by a trial of steroid therapy. Biopsy should be reserved for patients with a strong medical contraindication to steroid therapy, or who fail to respond to treatment promptly.

Adult↗

Giant cell arteritis: strategies in diagnosis and treatment.

PURPOSE OF REVIEW: This review summarizes current diagnostic assessments and therapeutic strategies in giant cell arteritis. Giant cell arteritis or temporal arteritis is a chronic vasculitis of large and medium-size vessels. Concurrent symptoms of proximal muscular ache and morning stiffness, polymyalgia rheumatica, are commonly seen. Recent investigations support the contention that polymyalgia rheumatica and temporal arteritis are two different expressions of the same underlying vasculitic disorder. RECENT FINDINGS: The symptomatology of giant cell arteritis is quite varying. Recently a frequent occurrence of audiovestibular manifestations was demonstrated, which should be actively searched for in the clinical investigation. Although color Doppler ultrasound, MRI, and positron emission tomography have illustrated the widespread nature of giant cell arteritis, none of these techniques may currently replace temporal artery biopsy. Biopsy of the superficial temporal artery is a safe and simple procedure, and remains the gold standard for the diagnosis of giant cell arteritis. The importance of long biopsies and meticulous histologic examination using sub-serial sectioning is emphasized. Numerous recent publications confirm the low diagnostic yield of a second, contralateral biopsy. Corticosteroids remain the cornerstone in the treatment of giant cell arteritis. Although steroid treatment promptly eliminates symptoms of systemic inflammation, its effect on inflammatory morphology is delayed. Consequently, there is a need for new therapeutic strategies. The potential role of aspirin has recently been implicated.

Giant Cell Arteritis↗

Giant-cell arteritis. Histological, immunohistochemical and electronmicroscopic studies.

Biopsies from the temporal artery of 32 patients suspected of giant-cell arteritis were evaluated retrospectively by light microscopy, histochemical, and immunohistochemical methods, as well as by transmission electron microscopy (TEM). At the clinical follow-up the 32 patients included four clinical groups: temporal arteritis (8 patients), polymyalgia rheumatica (10 patients), rheumatoid arthritis (4 patients), and a group of miscellaneous diseases unrelated to inflammatory rheumatic diseases (10 patients). There were a number of similarities between age-related alterations in the arteries and the changes in giant-cell arteritis. The most important differences were the inflammatory cellular infiltration of the media, the perifocal accumulation of fibronectin, and the occurrence of deposits of fibrin/fibrinogen and fibrin/fibrinogen degradation products. In addition, alpha-2 macroglobulin, lysozyme and factor VIII were also noted in giant-cell arteritis. The alterations in giant-cell arteritis show a number of similarities to the changes following experimental vascular injury of the rabbit aorta. The nature of the findings in human giant-cell arteritis, as well as the similarity to the experimental arteritis, indicate that giant-cell arteritis may reflect a non-specific reaction to injury, independent of the cause of the disease.

Aged↗

Tissue cytokine patterns in patients with polymyalgia rheumatica and giant cell arteritis.

OBJECTIVE: To analyze temporal artery specimens from patients with giant cell arteritis and polymyalgia rheumatica for the presence of inflammatory cytokines and to ascertain whether a specific cytokine pattern exists for the two conditions. DESIGN: Case series of patients having temporal artery biopsy procedures. SETTING: The outpatient clinic and the research laboratories of the Division of Rheumatology, Mayo Clinic. PATIENTS: 34 patients having temporal artery biopsy procedures: 15 patients had giant cell arteritis, 9 had polymyalgia rheumatica without evidence of vasculitis, and 10 had neither polymyalgia rheumatica nor vasculitis. MEASUREMENT: Temporal artery specimens were analyzed for in vivo presence of cytokine messenger RNA (mRNA) by polymerase chain reaction with cytokine-specific primer sets. RESULTS: Vasculitic lesions in giant cell arteritis samples were characterized by in situ production of interleukin-1 beta, interleukin-6, and transforming growth factor-beta 1 mRNA (indicative of macrophage activation) and by interferon-gamma and interleukin-2 mRNA (indicative of selective T-cell activation). However, macrophage- and T-cell-derived cytokines were also detected in temporal artery biopsy specimens from patients with polymyalgia rheumatica. Tissue-infiltrating T cells in giant cell arteritis and polymyalgia rheumatica samples each had distinctive lymphokine profiles. Although interferon-gamma was found in 67% of giant cell arteritis samples, polymyalgia rheumatica samples had only interleukin-2. CONCLUSIONS: Patients with polymyalgia rheumatica have vascular involvement. Patients with polymyalgia rheumatica and giant cell arteritis share in situ production of mRNA specific for macrophage-derived cytokines. T cells recruited to vasculitic lesions in patients with giant cell arteritis predominantly produce interleukin-2 and interferon-gamma. Patients with polymyalgia rheumatica do not have interferon-gamma production, suggesting that interferon-gamma may be involved in the progression to overt arteritis.

Adult↗