1: J Rheumatol. 2003 Oct;30(10):2165-9.

Comment in:
J Rheumatol. 2005 Jun;32(6):1173; author reply 1173.

Temporal arteritis associated with systemic necrotizing vasculitis.

Hamidou MA, Moreau A, Toquet C, El Kouri D, de Faucal P, Grolleau JY.

Department of Internal Medicine and Laboratory of Pathology, University Hospital. Hotel-Dieu, Place Alexis Ricordeau, Nantes 44035, France.
mohamed.hamidou@chu-nantes.fr

OBJECTIVE: To evaluate the clinical and laboratory characteristics of patients with systemic vasculitis associated with temporal artery involvement. METHODS: From a cohort of 120 patients fulfilling American College of Rheumatology criteria for temporal arteritis, we retrospectively identified 7 patients with systemic necrotizing vasculitis associated with histological temporal arteritis. RESULTS: Among the 7 patients, 2 had classic polyarteritis nodosa, one had unclassified systemic vasculitis, one had Wegener's granulomatosis (WG), and 3 had microscopic polyangiitis. The mean age of the patients was 70.2 years, and cranial symptoms revealed the disease in all but one patient. Temporal arteritis was generally associated with extracephalic manifestations suggestive of systemic vasculitis. Antineutrophilic cytoplasmic antibodies were positive in 3 of the 4 patients with small vessel vasculitis. Pathologically, the main temporal artery was involved in all but one patient, with inflammatory infiltrate of vasa vasorum and adventitia associated in 5 with small tributary involvement. Fibrinoid necrosis was rare, observed in 2 specimens; 2 patients with unclassified systemic vasculitis and WG had a classic giant cell arteritis  (GCA) histologic pattern. Only one patient had exclusive involvement of small vessels, surrounding the spared main temporal artery. Muscle biopsies showed histopathological evidence of vasculitis in 2 patients, skin biopsy in one, and vein biopsy in the other. CONCLUSION: Temporal artery involvement in systemic necrotizing vasculitis was generally associated with extracranial clinical features suggestive of systemic vasculitis. Temporal artery biopsy is a simple tool for diagnosis of vasculitis, but the histopathological findings do not always discriminate between necrotizing vasculitis and classic GCA.

2: Arthritis Rheum. 2001 Jun;44(6):1387-95.

Small-vessel vasculitis surrounding a spared temporal artery: clinical and pathological findings in a series of twenty-eight patients.

Esteban MJ, Font C, Hernandez-Rodriguez J, Valls-Sole J, Sanmarti R, Cardellach F, Garcia-Martinez A, Campo E, Urbano-Marquez A, Grau JM, Cid MC.

Hospital Clinic, University of Barcelona, Institut d'Investigacions, Biomediques August Pi i Sunyer, Spain.

OBJECTIVE: Occasionally, a temporal artery biopsy reveals small-vessel vasculitis (SVV) surrounding a spared temporal artery, the significance of which is unclear. We analyzed the final diagnosis in a series of patients with this condition and tried to identify histopathologic features with potential usefulness in predicting the ultimate diagnosis. METHODS: We performed a clinical and histopathologic review of 28 patients in whom SVV surrounding a spared temporal artery was the first histologic finding that led to the diagnosis of vasculitis. For comparison purposes, we analyzed the pattern of small vessel involvement in 30 patients with biopsy-proven giant cell arteritis (GCA). RESULTS: GCA was considered the most likely diagnosis in 12 patients, based on the absence of clinical evidence of additional organ involvement and normal findings on muscle biopsy and electrophysiologic study. Three patients had systemic necrotizing vasculitis (SNV), based on the demonstration of typical lesions on subsequent muscle, nerve, or kidney biopsy. After extensive evaluation, 4 patients remained unclassifiable. Nine patients were incompletely studied. Fibrinoid necrosis was significantly more frequent in patients with SNV (P = 0.0022), whereas involvement of vasa vasorum was more frequent in patients classified as having GCA (P = 0.022). No differences in the pattern of small vessel involvement were found in patients with SVV surrounding a spared temporal artery who were classified as having GCA compared with patients with biopsy-proven GCA. Granulocytes were observed at similar frequency in all conditions. CONCLUSION: SVV may be the only abnormal feature in a temporal artery biopsy and the only histologic evidence of vasculitis. The diagnosis of GCA can be reasonably established in most of these patients when there is no apparent evidence of additional organ involvement. However, when fibrinoid necrosis is observed or the temporal artery vasa vasorum are not involved, SNV must be extensively excluded.

3: Arthritis Rheum. 1994 Jul;37(7):1007-12.

Giant cell arteritis in Iceland. An epidemiologic and histopathologic analysis.

Baldursson O, Steinsson K, Bjornsson J, Lie JT.

University Hospital, Reykjavik, Iceland.

OBJECTIVE. To investigate the incidence and clinical and histopathologic features of giant cell (temporal) arteritis (GCA) in the Caucasian population of Iceland. METHODS. All patients diagnosed between 1984 and 1990 were included. Case ascertainment for the study was done in 2 ways: 1) a computerized search from all hospitals and primary care clinics for the diagnosis of GCA, and 2) a review of all temporal artery biopsies performed during the 7-year period.
RESULTS. One hundred thirty-three patients with GCA were identified. All fulfilled the 1990 American College of Rheumatology criteria for the classification of GCA. The incidence rate for the population 50 years and older was 27/100,000 (36/100,000 and 18/100,000 for women and men, respectively). Clinical findings included the following: mean age at diagnosis 72.5 years for women and 70.3 years for men, new headache 63.2%, abnormal temporal artery on palpation 43.6%, mean erythrocyte sedimentation rate 88 mm/hour, symptoms of polymyalgia rheumatica 48.1%, and visual disturbances 14.3%. A total of 744 patients underwent temporal artery biopsy during the 7-year period; 16.8% had a positive biopsy result. All 133 patients with the diagnosis of GCA underwent a temporal artery biopsy; 94% had a positive result. Histopathologic findings from the positive biopsies included a fragmented internal elastic lamina in 99.2%, giant cells in 65.6%, and fibrinoid necrosis in 12%. CONCLUSION. Compared with previous epidemiologic surveys, this study shows a high incidence of biopsy-proven GCA in Iceland.

4: J Rheumatol. 1992 Aug;19(8):1312-4.

Concurrent temporal arteritis and Churg-Strauss syndrome.

Vidal E, Liozon F, Rogues AM, Cransac M, Berdha JF, Liozon E.

Department of Internal Medicine, University of Limoges, France.

We describe the case of a 41-year-old man who presented with clinical and histopathologic evidence of temporal artery lesions associated with the Churg-Strauss syndrome. Pathological examination of the temporal artery showed panarteritis without giant cell formation or fibrinoid necrosis. We review the world literature concerning the vasculitides with features that overlap giant cell arteritis (GCA) and polyarteritis nodosa (PAN) and classify into 2 sub-groups PAN with unusual temporal artery localization and GCA with variably disseminated arterial injuries. These cases emphasize the fact that not all arteritis involving the temporal arteries is GCA. Only 3 cases with temporal artery involvement and concurrent Churg-Strauss syndrome have been published.