temporal_arteritis

Background

Giant cell arteritis is the other name for this vasculitis. It is probably a more accurate name since arteries other than the temporal artery are affected. It is a disease of the middle aged to elderly with the superficial temporal and ophthalmic arteries most commonly involved, but other extracranial branches of the carotid arteries may be involved. The clinical presentation depends upon the vessel involved but include visual disturbances including blindness, headache, jaw pain, and neurological disturbances. Rarely, skin changes may occur with necrosis and ulceration associated with tenderness. A hallmark of the disease is an elevated erythrocyte sedimentation rate (ESR).

There is a rare variant occurring in children and young adults which presents with a lump on the forehead. The ESR is not elevated unlike the adult variant.

In spite of the characteristic histological changes, confirming the diagnosis by artery biopsy may be difficult. There are often skip lesions leading to negative results on the biopsy. Some clinicians feel that if the diagnosis is strongly suspected, a trial of corticosteroid therapy is indicated, even in the absence of a tissue diagnosis. The pathologist receiving a biopsy should get multiple levels and consider obtaining an elastic stain to investigate the integrity of the internal elastic lamina.

OUTLINE

Epidemiology

Disease Associations

Pathogenesis

Laboratory/Radiologic/
Other Diagnostic Testing

Gross Appearance and Clinical Variants

Histopathological Features and Variants

Special Stains/
Immunohistochemistry/
Electron Microscopy

Differential Diagnosis

Prognosis

Treatment

Commonly Used Terms

Internet Links

EPIDEMIOLOGY CHARACTERIZATION

INCIDENCE
Arthritis Rheum. 1988;31:745-749.

Incidence rate of up to 30 cases per year per 100,000 persons older than 50 years in some populations

DISEASE ASSOCIATIONS CHARACTERIZATION

KIMURA'S DISEASE

Juvenile temporal arteritis is a manifestation of kimura disease.

Watanabe C, Koga M, Honda Y, Oh-I T.

Department of Dermatology, Tokyo Medical University, Tokyo, Japan.

Am J Dermatopathol 2002 Feb;24(1):43-9 Abstract quote
An asymptomatic nodule appeared in the right temporal region of an 81-year-old woman. Histopathologic examination confirmed significant thickening of the vascular wall, constriction of the vessel lumen, and infiltration of numerous eosinophils and lymphocytes. Giant cells were not seen. Lymphoid follicles and capillaries surrounded the large vessel. Elastica van Gieson staining revealed a laceration of the internal elastic lamina. Based on these clinical and histologic findings, the patient was diagnosed as having juvenile temporal arteritis (JTA), a disease first proposed by Lie and his colleagues in 1975. Three years later, a new eruption, again asymptomatic, appeared in the posterior region of the patient's right ear. Subsequently, she was referred to our department. Histologic examination of the new lesion confirmed the infiltration of lymphocytes and eosinophils, which was accompanied by numerous lymphoid follicles, and the proliferation of endothelial cells and capillaries from the deep dermis to the subcutaneous tissue. The patient was diagnosed as having Kimura disease, which is a persistent and recurrent illness.

We hypothesized that JTA was a partial expression of Kimura disease and investigated whether past cases of JTA could be considered Kimura disease. As a result, we found that most cases of JTA could indeed be considered Kimura disease. Furthermore, we examined the vascular changes in the routinely and elastic fiber-stained sections of three cases with Kimura disease and two cases with angiolymphoid hyperplasia with eosinophilia. The results showed occlusive vascular changes in most samples from these cases, supporting the hypothesis that JTA is an accessory lesion of Kimura disease.

PATHOGENESIS CHARACTERIZATION

Cell mediated immune attack is favored

HERPES SIMPLEX VIRUS

High Prevalence of Herpes Simplex Virus DNA in Temporal Arteritis Biopsy Specimens

James F. Powers, PhD, Shahinaz Bedri, MD, Shakir Hussein, MD, Robert N. Salomon MD, and Arthur S. Tischler, MD

Am J Clin Pathol 2005;123:261-264 Abstract quote

Giant cell arteritis (GCA) affecting the cranial arteries is a disease of unknown cause that causes blindness, stroke, and other morbidity. Its sudden onset and segmental distribution are suggestive of diseases that involve viral reactivation, and cranial arteries are known to be innervated by ganglia that harbor herpes simplex virus (HSV).

We used a high-sensitivity polymerase chain reaction assay to test for HSV DNA in specimens from 39 consecutive temporal artery biopsies performed for suspected GCA. HSV DNA was detected in 21 (88%) of 24 histologically positive and 8 (53%) of 15 histologically negative specimens (P = .027; Fisher exact test). Analysis of 10 renal artery samples from age-matched control subjects using the same assay showed no detectable HSV DNA.

We conclude that detectable HSV DNA is correlated with histologically confirmed GCA in this patient population.

LABORATORY/
RADIOLOGIC CHARACTERIZATION

ESR

Giant cell arteritis with low erythrocyte sedimentation rate: frequency of occurence in a population-based study.

Salvarani C, Hunder GG.

Rheumatology Service, Arcispedale S. Maria Nuova, Reggio Emilia, Italy.
Arthritis Rheum 2001 Apr;45(2):140-5 Abstract quote
OBJECTIVE: To determine the frequency of a low erythrocyte sedimentation rate (ESR) in patients with giant cell arteritis (GCA) and evaluate their clinical features in a defined population.

METHODS: A total of 167 patients with GCA were identified in the population of Olmsted County, Minnesota, between the years 1950 and 1998 using methods described in previous studies. All fulfilled American College of Rheumatology criteria for GCA.

RESULTS: In 9 of the 167 patients the ESR was less than 40 mm/hour (Westergren method) at diagnosis. These patients had less frequent systemic symptoms and visual symptoms than the others. No patient with low ESR developed blindness. Other manifestations were similar in those with low and those with high ESR. The response of symptoms to prednisone treatment was within 1 week, and after a median of 25 days of therapy the median ESR dropped from 19 mm/hour to 3 mm/hour. The median duration of glucocorticoid therapy in the 9 patients was 21.5 months and median followup after diagnosis was 12.5 years. Over a long period of observation (median 44 years) in the 9 patients with low ESR, 9 inflammatory events other than GCA were observed in 7 patients. The ESR was normal in 7 of these 9 other events.

CONCLUSION: A low ESR in active GCA is not a rare occurrence. Causes may include localized arteritis in some patients and an inability to mount an acute phase serologic response in others.

HISTOLOGICAL TYPES CHARACTERIZATION

General
Granulomatous arteritis with Langhans and foreign body type giant cells engulfing fragmented elastic fibers. There is a mixed inflammatory cell infiltrate which can include lymphocytes, histiocytes, and eosinophils. An elastic stain will reveal fragmented and destroyed internal elastic lamina leading to the elastic fiber fragments. Older lesions may have intimal fibrosis with thickening of the wall with luminal narrowing. In spite of the name of this disease, giant cells are not required for the diagnosis.

In the juvenile cases, there is an eosinophilic arteritis with thrombosis. Associated lymphocytes and plasma cells are present but no giant cells.

Mayo Clin Proc. 1971;46:597-602.
Arch Ophthalmol. 1984;102:901-903.

False-negative biopsy rate, as determined by correlation with clinical findings, reported as 42% to 61% in published series

Temporal artery biopsy: is there any value in examining biopsies at multiple levels?

J Clin Pathol. 2000;53:131-136.

Analysis of the cost-effectiveness of examining temporal artery biopsy specimens

Only 1 of 132 initially normal biopsy specimens and 2 of 14 cases with chronic perivascular inflammation revealed giant cell arteritis after examining the tissue at multiple levels

In an additional 15 cases, chronic perivascular inflammation, but not vasculitis, was found on deeper sections.

Conclusions:
Routine examination of temporal artery biopsy specimens at multiple levels does not appreciably increase the diagnostic yield of the test, although the authors acknowledged that selective cases may benefit from further sectioning

Chronic perivascular inflammation did not seem to be elucidated further by additional sectioning (vasculitis was not found in a particularly high number of cases).

VARIANTS

The Significance of Perivascular Inflammation in the Absence of Arteritis in Temporal Artery Biopsy Specimens
Am J Clin Pathol 2001;115:342-347

We retrospectively compared 81 temporal artery biopsy specimens demonstrating perivascular inflammation without evidence of temporal arteritis and 76 specimens demonstrating no inflammation.

Patients with perivascular inflammation included 43 women (mean age, 71.2 years). Nineteen patients met the 1990 American College of Rheumatology (ACR) criteria for the diagnosis of temporal arteritis.

All patients demonstrated chronic perivascular inflammation consisting primarily of lymphocytes. Granulomas were noted in 4 specimens. Internal elastic lamina disruption, intimal fibroplasia, and dystrophic calcification were noted in 86 arteries examined. Fibrosis or scarring of the vessel walls was observed in 10 specimens. Corticosteroid therapy was beneficial to 33 of 56 patients.

In patients with no evidence of inflammation (50 women; mean age, 66.6 years), 21 met ACR criteria for temporal arteritis. Histologically, disruption of the elastic lamina was noted in 75 of 81 arteries biopsied, intimal fibroplasia in 66, microcalcifications in 5, and fibrosis or scarring in 5. In this group, 47 patients received corticosteroid therapy; clinical improvement was noted in 28.

Conclusions:
Patients with chronic perivascular inflammation but no arteritis seem no more likely to have temporal arteritis on clinical grounds than similar patients without inflammation on biopsy.

Arthritis Rheum. 1990;33:1122-1128.
Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

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Last Updated February 18, 2005

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Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/ Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/ Immunohistochemistry/ Electron Microscopy
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

PATHOGENESIS	CHARACTERIZATION
Cell mediated immune attack is favored
HERPES SIMPLEX VIRUS
High Prevalence of Herpes Simplex Virus DNA in Temporal Arteritis Biopsy Specimens James F. Powers, PhD, Shahinaz Bedri, MD, Shakir Hussein, MD, Robert N. Salomon MD, and Arthur S. Tischler, MD	Am J Clin Pathol 2005;123:261-264 Abstract quote Giant cell arteritis (GCA) affecting the cranial arteries is a disease of unknown cause that causes blindness, stroke, and other morbidity. Its sudden onset and segmental distribution are suggestive of diseases that involve viral reactivation, and cranial arteries are known to be innervated by ganglia that harbor herpes simplex virus (HSV). We used a high-sensitivity polymerase chain reaction assay to test for HSV DNA in specimens from 39 consecutive temporal artery biopsies performed for suspected GCA. HSV DNA was detected in 21 (88%) of 24 histologically positive and 8 (53%) of 15 histologically negative specimens (P = .027; Fisher exact test). Analysis of 10 renal artery samples from age-matched control subjects using the same assay showed no detectable HSV DNA. We conclude that detectable HSV DNA is correlated with histologically confirmed GCA in this patient population.

HISTOLOGICAL TYPES	CHARACTERIZATION
General	Granulomatous arteritis with Langhans and foreign body type giant cells engulfing fragmented elastic fibers. There is a mixed inflammatory cell infiltrate which can include lymphocytes, histiocytes, and eosinophils. An elastic stain will reveal fragmented and destroyed internal elastic lamina leading to the elastic fiber fragments. Older lesions may have intimal fibrosis with thickening of the wall with luminal narrowing. In spite of the name of this disease, giant cells are not required for the diagnosis. In the juvenile cases, there is an eosinophilic arteritis with thrombosis. Associated lymphocytes and plasma cells are present but no giant cells.
	Mayo Clin Proc. 1971;46:597-602. Arch Ophthalmol. 1984;102:901-903. False-negative biopsy rate, as determined by correlation with clinical findings, reported as 42% to 61% in published series
Temporal artery biopsy: is there any value in examining biopsies at multiple levels?	J Clin Pathol. 2000;53:131-136. Analysis of the cost-effectiveness of examining temporal artery biopsy specimens Only 1 of 132 initially normal biopsy specimens and 2 of 14 cases with chronic perivascular inflammation revealed giant cell arteritis after examining the tissue at multiple levels In an additional 15 cases, chronic perivascular inflammation, but not vasculitis, was found on deeper sections. Conclusions: Routine examination of temporal artery biopsy specimens at multiple levels does not appreciably increase the diagnostic yield of the test, although the authors acknowledged that selective cases may benefit from further sectioning Chronic perivascular inflammation did not seem to be elucidated further by additional sectioning (vasculitis was not found in a particularly high number of cases).
VARIANTS
The Significance of Perivascular Inflammation in the Absence of Arteritis in Temporal Artery Biopsy Specimens	Am J Clin Pathol 2001;115:342-347 We retrospectively compared 81 temporal artery biopsy specimens demonstrating perivascular inflammation without evidence of temporal arteritis and 76 specimens demonstrating no inflammation. Patients with perivascular inflammation included 43 women (mean age, 71.2 years). Nineteen patients met the 1990 American College of Rheumatology (ACR) criteria for the diagnosis of temporal arteritis. All patients demonstrated chronic perivascular inflammation consisting primarily of lymphocytes. Granulomas were noted in 4 specimens. Internal elastic lamina disruption, intimal fibroplasia, and dystrophic calcification were noted in 86 arteries examined. Fibrosis or scarring of the vessel walls was observed in 10 specimens. Corticosteroid therapy was beneficial to 33 of 56 patients. In patients with no evidence of inflammation (50 women; mean age, 66.6 years), 21 met ACR criteria for temporal arteritis. Histologically, disruption of the elastic lamina was noted in 75 of 81 arteries biopsied, intimal fibroplasia in 66, microcalcifications in 5, and fibrosis or scarring in 5. In this group, 47 patients received corticosteroid therapy; clinical improvement was noted in 28. Conclusions: Patients with chronic perivascular inflammation but no arteritis seem no more likely to have temporal arteritis on clinical grounds than similar patients without inflammation on biopsy.