Vol. XXV, No. 10 Printed in U.S.A.
JOURNAL OF THE AMERICAN GERIATRICS SOCIETY Copyright © 1977by the American Geriatrics Society
Temporal Arteritis T. J. MURRAY, MD, FRCP(C)*
Dalhousie University and Camp Hill Hospital, Halifax, Nova Scotia ABSTRACT: Temporal arteritis (granulomatous inflammation) usually involves the temporal and and ophthalmic arteries, but may be part of a more widespread inflammation of the medium and large vessels. The patient usually presents with an associated group of constitutional symptoms (fever, malaise, anorexia, weight loss, anemia) and rheumatic complaints (polymyalgia rheumatica). The diagnosis should be considered in any patient over 55 years old in whom these symptoms develop or in whom there is evidence of recent onset of headache, visual loss or localized arterial involvement. The diagnosis is also to be considered when the erythrocyte sedimentation rate is over 50 mm/hr, and the presence of arteritis is confirmed by temporalartery biopsy findings. Visual loss may occur in 50 percent of affected patients; other serious complications are less common. A strong clinical suspicion of temporal arteritis will permit diagnosis of the more uncommon and atypical presentations of the syndrome. Although cases of temporal arteritis may be self-limited, treatment is imperative because of the threat of blindness. Patients respond well to steroid (prednisone) therapy, which should be maintained for a prolonged period. Temporal arteritis (giant-cell, or cranial arteritis) is a granulomatous inflammatory disease of the medium sized arteries, manifested primarily with temporal and ophthalmic artery involvement. It was first described by Jonathan Hutchinson in 1890 (l) and was clearly defined as an entity by Horton et al in 1932 (2). The usual presenting symptoms are headache, visual disturbance and constitutional symptoms of malaise, fever, anorexia and weight loss. Today, the disease is known to be much more widespread than was initially believed; it is now regarded as one end of the spectrum of collagen vascular diseases.
nosed, largely because no serious complications develop. The self-limiting nature of the disorder also restricts its diagnosis.
Etiology Temporal arteritis is part of the autoimmune spectrum of diseases referred to as collagen vascular diseases and it may involve a foreign-body reaction to some nonspecific agent or irritation (4). Recent work showed anti-IgG activity of IgA together with complement in biopsy specimens from patients with temporal arteritis; this strongly indicates that antigen/antibody complexes or otherwise denatured immunoglobulins are present in tissue (5). A definite relationship between the histologic stages and the presence of anti-IgG activity in the tissue lesions was also shown.
Incidence Recent studies indicate an average annual incidence rate of 3/100,000, with women affected four times as frequently as men (3). Clearer definition of the syndrome and its milder variations suggests that its true incidence is considerably higher; a number of cases do not come to the physician's attention and others are not diag-
Pathologic features The granulomatous arteritis primarily occurs in medium sized vessels, usually the temporal and ophthalmic arteries on both sides. Bilateral involvement is frequent although the presenting signs are often unilateral (6). Other vessels may be affected, e.g., any of the branches from the
* Professor of Medicine, Dalhousie University, Halifax. Address for correspondence: T. J. Murray, MD, Chief of Medicine, Camp Hill Hospital, Halifax, Nova Scotia, Canada B3H 3G2.
450
October 1977
TEMPORAL ARTERITIS
aorta including the coronary, subclavian, carotid, renal, mesenteric and iliac arteries (4). Although the arteritis may be focal, in that it involves only certain vessels, "skip lesions" also occur in the affected artery (7). Cohen and Smith (6) found that bilateral arteritis was the rule, but Klein et al (7) demonstrated occasional unilateral diseases. Granulomatous arteritis involves the inner layers of the media with cellular infiltration of lymphocytes, and some neutrophils, plasma cells, eosinophils and macrophages. The number of giant cells, characteristic of the disease, varies within the wall. Some areas show necrosis, whereas others show connective tissue repair. The loose connective tissue may obstruct the vessellumen and enlarge the temporal artery to give it its cordlike, pulseless feel on palpation. In some cases, there is fragmentation ofthe internal elastic lamina. Although the vessel may be totally occluded, recanalization can occur later when the disease burns itself out.
Clinical manifestations The typical picture is that of an elderly patient with a history of many months of general malaise, weight loss, anorexia, and mild recurrent fever. Most such patients present with unilateral headache, and some with visual loss. Examination reveals a generally unwell person with a pulseless, cordlike temporal artery and redness and tenderness over that area of the scalp. Laboratory studies show a high erythrocyte sedimentation rate (often over 100 mm/hr), anemia and other hematologic characteristics of chronic disease. Biopsy of the temporal artery demonstrates the characteristic giant-cell arteritis. The patient responds dramatically to treatment with steroids. Headache. The headache varies but usually is unilateral and felt superficially in the scalp. The pain may be described as aching, jabbing, or throbbing. It may also be experienced over the frontal and occipital regions; onset is acute in some cases but gradual in others. Although the headache is not characteristic in itself, temporal arteritis should be suspected in any elderly patient who presents with recent onset of headache, particularly if the patient has constitutional symptoms. An unusual symptom, jaw pain associated with talking or chewing, is seen in 50 percent of cases and may occur without the temporal headache. Visual symptoms. If untreated, about 50 per-
cent of patients with temporal arteritis develop visual symptoms and sometimes blindness. Although the syndrome may otherwise be a benign and self-limited disorder which can burn itself out within six months, the possibility of blindness makes temporal arteritis a disease that it is important to recognize early and treat as an emergency. Occasionally, there are recurrent transient visual symptoms, but loss of vision is usually acute, and often becomes bilateral. When the visual symptoms are fully developed, response to therapy is poor and a residual visual deficit or blindness is common. Mental changes. Occasionally, confusion or dementia develops as part of the progressive symptomatology of temporal arteritis, particularly if the patient has borderline cerebral function which has deteriorated further in association with the constitutional symptoms of mild fever, malaise, anorexia and weight loss. Involvement of the intracranial vessel is possible, as is ischemia due to involvement of the carotid and vertebrobasilar arteries. Fever. Some patients with temporal arteritis present with low-grade fever of unknown origin. Cerebrovascular symptoms. Acute cerebral infarction has been described. Along with myocardial infarction these lesions account for 12 percent of deaths among patients with temporal arteritis(8). Polymyalgia rheumatica is now recognized as part of the syndrome of temporal arteritis. In 50 percent of affected patients the temporal artery biopsy findings are positive. These patients present with widespread muscle and joint pains, joint stiffuess, and the other constitutional-symptoms associated with temporal arteritis. Joint swelling and muscle weakness are unusual; stiffuess is the chief symptom and finding. The syndrome is often migratory and aggravated by damp weather. It tends to cause greater discomfort at night. The laboratory findings are the same as those in temporal arteritis. Patients respond to anti-inflammatory agents, but since temporal arteritis may still develop during treatment with these drugs (9), steroids must be used. Skin lesions. Local changes over the temporal arteries are common, e.g., redness, tenderness and slight swelling. Necrosis of the scalp and even the 'tongue may occur (10). Cardiac manifestations. Involvement of the coronary arteries is another cause of death in these patients. Occasionally, recurrent angina is observed, and this may be refractory to treatment until steroids are used.
451
T. J. MURRAY
Limb involvement. Peripheral arterial insufficiency can occur in cases of temporal arteritis. Gangrene may be more common than previously believed, because some cases are regarded as Buerger's disease, in which condition giant cells are also found. Other possible symptoms of temporal arteritis are intermittent claudication, paresthesia and Raynaud's phenomenon (7). Diagnosis The diagnosis must be suspected in more subtle clinical situations than previously recognized for this disorder. Any of the foregoing symptoms or problems in a patient over 55 years of age, particularly if associated with an erythrocyte sedimentation rate (ESR) over 50 mm/hr, should make one suspect temporal arteritis. Although the clinical picture may be characteristic, and a pulseless, cordlike and inflamed temporal artery may be present, some laboratory investigations are useful in confirming the diagnosis. The ESR is the simplest and most useful indicator initially; it is almost always over 50 mm/hr and often over 100. Other less specific laboratory findings include anemia, hypochromasia, rouleaux formation, a low concentration of serum iron, mild leukocytosis, toxic changes in the leukocytes, and a low concentration of serum albumin with an elevated level of alpha globulin (10). Other observations have included an increase in blood fibrinogen, thrombocytosis, and eosinophilia. Selective arteriographs of the external carotid artery may show characteristic segmental narrowing of the temporal artery (11). However, the most important diagnostic procedure is the temporal artery biopsy. This should be performed in every suspected case, and a long segment of the temporal artery should be taken. Although the biopsy procedure may relieve the headache, the patient must still be treated with steroids to avoid possible blindness later.
Treatment Temporal arteritis is sometimes benign and self-limiting, but the threat of blindness in about half of the patients with this disorder makes treatment imperative and urgent. A reasonable regimen is 60 mg of prednisone daily for 4 weeks, followed by a reduction of 5 mg every 2 weeks to a maintenance dosage of 15 mg daily. Maintenance therapy is continued for as long as 2 years, but further slow reduction in therapy can be made. If the dosage is reduced too rapidly, or therapy
452
Vol. XXV
discontinued prematurely, recurrence is possible. An alternate suggestion is to use comparable doses but on an alternate-day schedule of administration. An anti-ulcer regimen should also be maintained during the steroid therapy, and other steroid precautions should be followed. The ESR can be used as an indicator of the success of treatment. If the rate drops to less than 15-20mm/hr, the inital dosage of 60 mg ofpred.nisone daily can be reduced sooner. The ESR should be tested a month after the cessation of therapy and also for a few years thereafter, even though the disease seldom recurs after a prolonged symptomless period. Steroid therapy is important, not only to decrease symptoms and shorten the course of the disease, but also to prevent serious complications. Unfortunately, blindness may still occur in some patients. There is little likelihood of reversing the blindness when therapy is started after visual symptoms are well established. Kearns (12) has suggested that hyperbaric oxygen is useful in the emergency treatment of visual loss from temporal arteritis, but there is little experience with this method of treatment; it would be impractical in most instances.
Prognosis A mortality rate of 12 percent has been recorded (8), and there may be many patients who have progressive temporal arteritis and die of coronary or cerebral involvement without proper diagnosis. The next most serious problem is that of visual loss, which occurs in about 50 percent of untreated cases. Otherwise, the disorder tends to have a remarkably good prognosis. Patients respond dramatically to steroid therapy and often the disease runs a self-limited course. REFERENCES 1. Hutchinson J: Diseases of the arteries. No.1. On a peculiar form of thrombotic arteritis of the aged which is sometimes productive of gangrene, Arch Surg 1: 323, 1890. 2. Horton BT, Magath TB and Brown GE: An undescribed arteritis of the temporal vessesl Proc Staff Meet Mayo Clin 7: 700, 1932. 3. Hauser WA, Ferguson RH, Holley KE et al: Temporal arteritis in Rochester, Minnesota, 1951 to 1967, Mayo Clin Proc 46: 597, 1971. 4. Andrews JM: Giant cell ("temporal") arteritis: a disease with variable clinical manifestations, Neurology 16: 963, 1966. 5. Waaler E, Tender 0 and Milde E-J: Immunological and histological studies of temporal arteries from patients with temporal arteritis and/or polymyalgia rheumatica, Acta Path Microbiol Scandinav (A) 84: 55, 1976.
October 1977
TEMPORAL ARTERITIS
6. Cohen DN and Smith TR: Skip areas in temporal arteritis: myth versus fact, Tr Am Acad Ophth Otolarygn 78: 772,1974. 7. Klein RG, Campbell RJ, Hunder GG et al: Skip lesions in temporal arteritis, Mayo CUn Proc 51: 504, 1976. 8. Hamrin B, Jonsson N and Landberg T: Involvement of large vessels in polymyalgia arteritica, Lancet 1: 1193, 1965. 9. Easterbrook WM, Baxter DW and Martin JR: Temporal arteritis developing during indomethacin therapy of po-
lymyalgia rheumatica, Canad MAJ 97: 296, 1967. 10. Kinmont PDC and McCallum DI: Skin manifestations of giant cell arteritis, Brit J Dermatol 76: 299, 1964. 11. Gillanders LA, Strachan RW and Blair DW: Temporal arteriography. A new technique for the investigation of giant cell arteritis and polymyalgia rheumatica, Ann Rheum Dis 28: 267, 1969. 12. Kearns TP: Annual review: neuro-ophthalmology, Arch Ophthalmol 74: 710, 1965.
453
JOURNAL OF THE AMERICAN GERIATRICS SOCIETY Copyright © 1977by the American Geriatrics Society
Temporal Arteritis T. J. MURRAY, MD, FRCP(C)*
Dalhousie University and Camp Hill Hospital, Halifax, Nova Scotia ABSTRACT: Temporal arteritis (granulomatous inflammation) usually involves the temporal and and ophthalmic arteries, but may be part of a more widespread inflammation of the medium and large vessels. The patient usually presents with an associated group of constitutional symptoms (fever, malaise, anorexia, weight loss, anemia) and rheumatic complaints (polymyalgia rheumatica). The diagnosis should be considered in any patient over 55 years old in whom these symptoms develop or in whom there is evidence of recent onset of headache, visual loss or localized arterial involvement. The diagnosis is also to be considered when the erythrocyte sedimentation rate is over 50 mm/hr, and the presence of arteritis is confirmed by temporalartery biopsy findings. Visual loss may occur in 50 percent of affected patients; other serious complications are less common. A strong clinical suspicion of temporal arteritis will permit diagnosis of the more uncommon and atypical presentations of the syndrome. Although cases of temporal arteritis may be self-limited, treatment is imperative because of the threat of blindness. Patients respond well to steroid (prednisone) therapy, which should be maintained for a prolonged period. Temporal arteritis (giant-cell, or cranial arteritis) is a granulomatous inflammatory disease of the medium sized arteries, manifested primarily with temporal and ophthalmic artery involvement. It was first described by Jonathan Hutchinson in 1890 (l) and was clearly defined as an entity by Horton et al in 1932 (2). The usual presenting symptoms are headache, visual disturbance and constitutional symptoms of malaise, fever, anorexia and weight loss. Today, the disease is known to be much more widespread than was initially believed; it is now regarded as one end of the spectrum of collagen vascular diseases.
nosed, largely because no serious complications develop. The self-limiting nature of the disorder also restricts its diagnosis.
Etiology Temporal arteritis is part of the autoimmune spectrum of diseases referred to as collagen vascular diseases and it may involve a foreign-body reaction to some nonspecific agent or irritation (4). Recent work showed anti-IgG activity of IgA together with complement in biopsy specimens from patients with temporal arteritis; this strongly indicates that antigen/antibody complexes or otherwise denatured immunoglobulins are present in tissue (5). A definite relationship between the histologic stages and the presence of anti-IgG activity in the tissue lesions was also shown.
Incidence Recent studies indicate an average annual incidence rate of 3/100,000, with women affected four times as frequently as men (3). Clearer definition of the syndrome and its milder variations suggests that its true incidence is considerably higher; a number of cases do not come to the physician's attention and others are not diag-
Pathologic features The granulomatous arteritis primarily occurs in medium sized vessels, usually the temporal and ophthalmic arteries on both sides. Bilateral involvement is frequent although the presenting signs are often unilateral (6). Other vessels may be affected, e.g., any of the branches from the
* Professor of Medicine, Dalhousie University, Halifax. Address for correspondence: T. J. Murray, MD, Chief of Medicine, Camp Hill Hospital, Halifax, Nova Scotia, Canada B3H 3G2.
450
October 1977
TEMPORAL ARTERITIS
aorta including the coronary, subclavian, carotid, renal, mesenteric and iliac arteries (4). Although the arteritis may be focal, in that it involves only certain vessels, "skip lesions" also occur in the affected artery (7). Cohen and Smith (6) found that bilateral arteritis was the rule, but Klein et al (7) demonstrated occasional unilateral diseases. Granulomatous arteritis involves the inner layers of the media with cellular infiltration of lymphocytes, and some neutrophils, plasma cells, eosinophils and macrophages. The number of giant cells, characteristic of the disease, varies within the wall. Some areas show necrosis, whereas others show connective tissue repair. The loose connective tissue may obstruct the vessellumen and enlarge the temporal artery to give it its cordlike, pulseless feel on palpation. In some cases, there is fragmentation ofthe internal elastic lamina. Although the vessel may be totally occluded, recanalization can occur later when the disease burns itself out.
Clinical manifestations The typical picture is that of an elderly patient with a history of many months of general malaise, weight loss, anorexia, and mild recurrent fever. Most such patients present with unilateral headache, and some with visual loss. Examination reveals a generally unwell person with a pulseless, cordlike temporal artery and redness and tenderness over that area of the scalp. Laboratory studies show a high erythrocyte sedimentation rate (often over 100 mm/hr), anemia and other hematologic characteristics of chronic disease. Biopsy of the temporal artery demonstrates the characteristic giant-cell arteritis. The patient responds dramatically to treatment with steroids. Headache. The headache varies but usually is unilateral and felt superficially in the scalp. The pain may be described as aching, jabbing, or throbbing. It may also be experienced over the frontal and occipital regions; onset is acute in some cases but gradual in others. Although the headache is not characteristic in itself, temporal arteritis should be suspected in any elderly patient who presents with recent onset of headache, particularly if the patient has constitutional symptoms. An unusual symptom, jaw pain associated with talking or chewing, is seen in 50 percent of cases and may occur without the temporal headache. Visual symptoms. If untreated, about 50 per-
cent of patients with temporal arteritis develop visual symptoms and sometimes blindness. Although the syndrome may otherwise be a benign and self-limited disorder which can burn itself out within six months, the possibility of blindness makes temporal arteritis a disease that it is important to recognize early and treat as an emergency. Occasionally, there are recurrent transient visual symptoms, but loss of vision is usually acute, and often becomes bilateral. When the visual symptoms are fully developed, response to therapy is poor and a residual visual deficit or blindness is common. Mental changes. Occasionally, confusion or dementia develops as part of the progressive symptomatology of temporal arteritis, particularly if the patient has borderline cerebral function which has deteriorated further in association with the constitutional symptoms of mild fever, malaise, anorexia and weight loss. Involvement of the intracranial vessel is possible, as is ischemia due to involvement of the carotid and vertebrobasilar arteries. Fever. Some patients with temporal arteritis present with low-grade fever of unknown origin. Cerebrovascular symptoms. Acute cerebral infarction has been described. Along with myocardial infarction these lesions account for 12 percent of deaths among patients with temporal arteritis(8). Polymyalgia rheumatica is now recognized as part of the syndrome of temporal arteritis. In 50 percent of affected patients the temporal artery biopsy findings are positive. These patients present with widespread muscle and joint pains, joint stiffuess, and the other constitutional-symptoms associated with temporal arteritis. Joint swelling and muscle weakness are unusual; stiffuess is the chief symptom and finding. The syndrome is often migratory and aggravated by damp weather. It tends to cause greater discomfort at night. The laboratory findings are the same as those in temporal arteritis. Patients respond to anti-inflammatory agents, but since temporal arteritis may still develop during treatment with these drugs (9), steroids must be used. Skin lesions. Local changes over the temporal arteries are common, e.g., redness, tenderness and slight swelling. Necrosis of the scalp and even the 'tongue may occur (10). Cardiac manifestations. Involvement of the coronary arteries is another cause of death in these patients. Occasionally, recurrent angina is observed, and this may be refractory to treatment until steroids are used.
451
T. J. MURRAY
Limb involvement. Peripheral arterial insufficiency can occur in cases of temporal arteritis. Gangrene may be more common than previously believed, because some cases are regarded as Buerger's disease, in which condition giant cells are also found. Other possible symptoms of temporal arteritis are intermittent claudication, paresthesia and Raynaud's phenomenon (7). Diagnosis The diagnosis must be suspected in more subtle clinical situations than previously recognized for this disorder. Any of the foregoing symptoms or problems in a patient over 55 years of age, particularly if associated with an erythrocyte sedimentation rate (ESR) over 50 mm/hr, should make one suspect temporal arteritis. Although the clinical picture may be characteristic, and a pulseless, cordlike and inflamed temporal artery may be present, some laboratory investigations are useful in confirming the diagnosis. The ESR is the simplest and most useful indicator initially; it is almost always over 50 mm/hr and often over 100. Other less specific laboratory findings include anemia, hypochromasia, rouleaux formation, a low concentration of serum iron, mild leukocytosis, toxic changes in the leukocytes, and a low concentration of serum albumin with an elevated level of alpha globulin (10). Other observations have included an increase in blood fibrinogen, thrombocytosis, and eosinophilia. Selective arteriographs of the external carotid artery may show characteristic segmental narrowing of the temporal artery (11). However, the most important diagnostic procedure is the temporal artery biopsy. This should be performed in every suspected case, and a long segment of the temporal artery should be taken. Although the biopsy procedure may relieve the headache, the patient must still be treated with steroids to avoid possible blindness later.
Treatment Temporal arteritis is sometimes benign and self-limiting, but the threat of blindness in about half of the patients with this disorder makes treatment imperative and urgent. A reasonable regimen is 60 mg of prednisone daily for 4 weeks, followed by a reduction of 5 mg every 2 weeks to a maintenance dosage of 15 mg daily. Maintenance therapy is continued for as long as 2 years, but further slow reduction in therapy can be made. If the dosage is reduced too rapidly, or therapy
452
Vol. XXV
discontinued prematurely, recurrence is possible. An alternate suggestion is to use comparable doses but on an alternate-day schedule of administration. An anti-ulcer regimen should also be maintained during the steroid therapy, and other steroid precautions should be followed. The ESR can be used as an indicator of the success of treatment. If the rate drops to less than 15-20mm/hr, the inital dosage of 60 mg ofpred.nisone daily can be reduced sooner. The ESR should be tested a month after the cessation of therapy and also for a few years thereafter, even though the disease seldom recurs after a prolonged symptomless period. Steroid therapy is important, not only to decrease symptoms and shorten the course of the disease, but also to prevent serious complications. Unfortunately, blindness may still occur in some patients. There is little likelihood of reversing the blindness when therapy is started after visual symptoms are well established. Kearns (12) has suggested that hyperbaric oxygen is useful in the emergency treatment of visual loss from temporal arteritis, but there is little experience with this method of treatment; it would be impractical in most instances.
Prognosis A mortality rate of 12 percent has been recorded (8), and there may be many patients who have progressive temporal arteritis and die of coronary or cerebral involvement without proper diagnosis. The next most serious problem is that of visual loss, which occurs in about 50 percent of untreated cases. Otherwise, the disorder tends to have a remarkably good prognosis. Patients respond dramatically to steroid therapy and often the disease runs a self-limited course. REFERENCES 1. Hutchinson J: Diseases of the arteries. No.1. On a peculiar form of thrombotic arteritis of the aged which is sometimes productive of gangrene, Arch Surg 1: 323, 1890. 2. Horton BT, Magath TB and Brown GE: An undescribed arteritis of the temporal vessesl Proc Staff Meet Mayo Clin 7: 700, 1932. 3. Hauser WA, Ferguson RH, Holley KE et al: Temporal arteritis in Rochester, Minnesota, 1951 to 1967, Mayo Clin Proc 46: 597, 1971. 4. Andrews JM: Giant cell ("temporal") arteritis: a disease with variable clinical manifestations, Neurology 16: 963, 1966. 5. Waaler E, Tender 0 and Milde E-J: Immunological and histological studies of temporal arteries from patients with temporal arteritis and/or polymyalgia rheumatica, Acta Path Microbiol Scandinav (A) 84: 55, 1976.
October 1977
TEMPORAL ARTERITIS
6. Cohen DN and Smith TR: Skip areas in temporal arteritis: myth versus fact, Tr Am Acad Ophth Otolarygn 78: 772,1974. 7. Klein RG, Campbell RJ, Hunder GG et al: Skip lesions in temporal arteritis, Mayo CUn Proc 51: 504, 1976. 8. Hamrin B, Jonsson N and Landberg T: Involvement of large vessels in polymyalgia arteritica, Lancet 1: 1193, 1965. 9. Easterbrook WM, Baxter DW and Martin JR: Temporal arteritis developing during indomethacin therapy of po-
lymyalgia rheumatica, Canad MAJ 97: 296, 1967. 10. Kinmont PDC and McCallum DI: Skin manifestations of giant cell arteritis, Brit J Dermatol 76: 299, 1964. 11. Gillanders LA, Strachan RW and Blair DW: Temporal arteriography. A new technique for the investigation of giant cell arteritis and polymyalgia rheumatica, Ann Rheum Dis 28: 267, 1969. 12. Kearns TP: Annual review: neuro-ophthalmology, Arch Ophthalmol 74: 710, 1965.
453
