Jaw claudication in primary systemic amyloidosis.

Amyloid deposits in the temporal artery were observed 40 years ago, but the presence of vascular ischemic symptoms in patients with amyloidosis has been infrequently recognized. We examined 22 patients who had typical jaw claudication with biopsy-proven primary amyloidosis. In none was vasculitis a contributing cause of the claudication. However, two patients were misdiagnosed initially as having temporal arteritis and polymyalgia rheumatica and were treated with corticosteroids, which resulted in significant toxicity. Subsequent temporal artery biopsy revealed extensive amyloid deposits in both patients. Jaw claudication was associated with other ischemic vascular symptoms, such as arm or calf claudication. The median survival for the subset of patients with amyloidosis and jaw claudication was 42 months, and that for the entire group of patients with amyloidosis was 12 months. Appropriate staining of temporal artery biopsy specimens is necessary for the correct diagnosis in such cases.

Clinical features of GCA/PMR.

Giant cell arteritis (GCA) is a common vasculitis of unknown cause that affects persons in middle age and older. Its incidence rises with increasing age. The inflammatory lesions involve larger arteries that contain an abundance of elastic tissue. Although cranial symptoms such as headache, tender scalp, jaw claudication and visual symptoms are common, the disease presents in many different fashions, often with symptoms not directly related to the arteries. These latter presentations include fever, severe malaise, polymyalgia rheumatica, high erythrocyte sedimentation rate and anemia, thrombocytosis, sore throat, and hepatic dysfunction. GCA appears to have a self-limited course, but is also characterized by relapses and recurrences. Visual loss due to occlusion of the optic arteries is the most important early manifestation and aortic aneurysm is the most important late complication. Patients respond promptly to varying doses of glucocorticoids but drug side effects are common.

Giant cell (cranial) arteritis: a clinical review.

Giant cell arteritis is a disease of the elderly which is more common than previously recognized. It is important to be aware of this condition because treatment effectively relieves symptoms and prevents serious complications. The disease is suggested when an elderly patient complains of constitutional symptoms, headache, jaw claudication, or the musculoskeletal manifestations of polymyalgia rheumatica. Abnormalities in temporal arteries or other cranial arteries, or evidence of large vessel involvement may be detected by physical examination. A markedly elevated sedimentation rate in association with other clinical features of the disease strongly suggests giant cell arteritis, but a biopsy should be performed to confirm the diagnosis. Corticosteroid therapy should be started promptly in high doses in order to prevent blindness. Prolonged treatment with lower dose corticosteroids is generally necessary for up to 1 to 2 years, and sometimes longer, for continued symptomatic relief. Long-term follow-up of treated patients has demonstrated no detectable effect on survivorship.

Dysarthria associated with giant cell arteritis.

Over a one month period, a 74-year-old man developed typical features of giant cell arteritis (GCA) including visual changes, headache, scalp tenderness, and an elevated erythrocyte sedimentation rate. In addition, he had reproducible painless dysarthria that was precipitated by chewing or prolonged talking and was relieved by resting the jaw. The dysarthria with chewing along with the other classical symptoms of GCA subsided with treatment. To our knowledge this is the first report of painless dysarthria associated with GCA.

Giant cell arteritis involving the facial artery.

Jaw claudication in giant cell (temporal) arteritis (GCA) is believed to be due to vasculitic obstruction or stenosis of the arteries supplying the muscles of mastication, notably the facial and internal maxillary arteries and their branches. However, histologic documentation of this is rarely available because GCA is usually diagnosed by temporal artery biopsies. We describe a patient with jaw claudication and other clinical features of GCA in whom a facial artery biopsy confirmed involvement by GCA.

Application of neural networks to the classification of giant cell arteritis.

OBJECTIVE: Neural networks are a group of computer-based pattern recognition methods that have recently been applied to clinical diagnosis and classification. In this study, we applied one type of neural network, the backpropagation network, to the diagnostic classification of giant cell arteritis (GCA). METHODS: The analysis was performed on the 807 cases in the vasculitis database of the American College of Rheumatology. Classification was based on the 8 clinical criteria previously used for classification of this data set: 1) age > or = 50 years, 2) new localized headache, 3) temporal artery tenderness or decrease in temporal artery pulse, 4) polymyalgia rheumatica, 5) abnormal result on artery biopsy, 6) erythrocyte sedimentation rate > or = 50 mm/hour, 7) scalp tenderness or nodules, and 8) claudication of the jaw, of the tongue, or on swallowing. To avoid overtraining, network training was terminated when the generalization error reached a minimum. True cross-validation classification rates were obtained. RESULTS: Neural networks correctly classified 94.4% of the GCA cases (n = 214) and 91.9% of the other vasculitis cases (n = 593). In comparison, classification trees correctly classified 91.6% of the GCA cases and 93.4% of the other vasculitis cases. Neural nets and classification trees were compared by receiver operating characteristic (ROC) analysis. The ROC curves for the two methods crossed, indicating that the better classification method depended on the choice of decision threshold. At a decision threshold that gave equal costs to percentage increases in false-positive and false-negative results, the methods were not significantly different in their performance (P = 0.45). CONCLUSION: Neural networks are a potentially useful method for developing diagnostic classification rules from clinical data.

The therapeutic impact of temporal artery biopsy.

To evaluate the clinical usefulness of temporal artery biopsy in the diagnosis of giant-cell arteritis we followed up all 134 residents of Olmsted County, Minnesota, who had temporal artery biopsies between 1965 and 1980. Initial biopsies were positive for giant-cell arteritis in 46 cases and negative in 88. A history of jaw pain or claudication and the findings of a palpably abnormal temporal artery were significantly more common in the patients whose biopsy specimens showed giant-cell arteritis. Over a median follow-up period of 70 months (range 1-192) only 8 of the 88 biopsy-negative patients had clinical courses requiring long-term, high-dose corticosteroid therapy for giant-cell arteritis. In this population-based study temporal artery biopsy correctly predicted the subsequent need for corticosteroid therapy in 94% of cases: these findings indicate that biopsy should be done before patients are committed to long-term corticosteroid therapy.

The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis.

Criteria for the classification of giant cell (temporal) arteritis were developed by comparing 214 patients who had this disease with 593 patients with other forms of vasculitis. For the traditional format classification, 5 criteria were selected: age greater than or equal to 50 years at disease onset, new onset of localized headache, temporal artery tenderness or decreased temporal artery pulse, elevated erythrocyte sedimentation rate (Westergren) greater than or equal to 50 mm/hour, and biopsy sample including an artery, showing necrotizing arteritis, characterized by a predominance of mononuclear cell infiltrates or a granulomatous process with multinucleated giant cells. The presence of 3 or more of these 5 criteria was associated with a sensitivity of 93.5% and a specificity of 91.2%. A classification tree was also constructed using 6 criteria. These criteria were the same as for the traditional format, except that elevated erythrocyte sedimentation rate was excluded, and 2 other variables were included: scalp tenderness and claudication of the jaw or tongue or on deglutition. The classification tree was associated with a sensitivity of 95.3% and specificity of 90.7%.