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.     

What is the best approach to diagnosing large-vessel vasculitis?

Temporal arteritis, including large-vessel giant cell arteritis, and Takayasu's arteritis are the two primary large-vessel vasculitides. Patients with temporal arteritis often present with headache, swollen temporal arteries, impairment of vision or symptoms of polymyalgia rheumatica. Clinical examination includes palpation of the temporal arteries and radial pulses, auscultation of the subclavian and axillary region, and fundoscopy. The presence of jaw claudication, diplopia and temporal artery abnormalities correlates with a high probability of positive histology. Duplex ultrasonography of the temporal arteries delineates a characteristic hypoechoic, oedematous wall swelling, stenoses and occlusions. It detects the same pathologies in the axillary arteries and other arteries in large-vessel giant cell arteritis. Angiography, magnetic resonance imaging, magnetic resonance angiography, electron beam computed tomography, computed tomography angiography and positron emission tomography show characteristic changes in the aorta and its primary branches in large-vessel giant cell arteritis and Takayasu's arteritis. Takayasu's arteritis often begins with diffuse symptoms such as low-grade fever, arthralgia, fatigue and weight loss. Clinical examination is important to detect bruits, pulse reduction and blood pressure differences. Profound experience exists with angiography. Other imaging methods are interesting alternatives as they are less invasive and may depict the inflammatory wall swelling.     

Jaw claudication in primary amyloidosis: unusual presentation of a rare disease

We describe two patients with temporal artery biopsy-proven amyloidosis presenting with symptoms of jaw claudication, visual disturbance, and proximal muscle stiffness suggestive of giant cell arteritis (GCA) and polymyalgia rheumatica. At the onset of disease, neither patient had other characteristic symptoms to suggest primary amyloid. We point out similarities between GCA and primary amyloid that can lead to confusion in diagnosis.     

Large artery involvement in giant cell (temporal) arteritis.

Of 248 patients with giant cell arteritis, 34 had evidence that the disease affected the aorta or its major branches. Symptoms suggestive of large artery involvement were intermittent claudication of an extremity, paresthesias, and Raynaud's phenomenon. Physical findings included absent or decreased large artery pulses and bruits over large arteries. Four patients presented with decreased upper extremity pulses as the initial manifestation of their arteritis. Nine other patients under treatment for temporal arteritis or polymyalgia rheumatica first developed evidence of large artery involvement as corticosteroid therapy was tapered or discontinued. Angiography, performed in 10 patients, was helpful in indicating arteritis rather than atherosclerosis as the cause of large artery disease. Three patients died with aortic rupture, and, at autopsy, widespread giant cell arteritis was found. However, when corticosteroids were given in adequate doses, the response was favorable in most patients; intermittent claudication decreased and the pulses improved.     

Giant Cell Arteritis with Internal Carotid Artery Occlusion in the Absence of Typical Clinical Features

A 65-year-old man presented with a slight headache and transient visual disturbance. Magnetic resonance imaging (MRI) revealed occlusion of the left internal carotid artery (ICA) and acute brain infarctions in both hemispheres, and a blood examination indicated inflammation. Gadolinium enhancement was observed in the walls of the temporal arteries and ICAs. After we diagnosed giant cell arteritis (GCA) by a temporal artery biopsy, aspirin and corticosteroids were administered. The typical symptoms of GCA, such as jaw claudication and temporal artery tenderness, were absent during the entire clinical course, and the findings of contrast-enhanced MRI contributed to the diagnosis.     

Neuro-ophthalmic complications in giant cell arteritis

Giant cell arteritis (GCA) is a medical emergency characterized by systemic inflammation and critical ischemia. Neuro-ophthalmic complications occur early, with permanent vision loss in up to one fifth of patients. This mainly results from failure of prompt recognition and treatment. Diagnosis of GCA is often preceded by unrecognized symptoms, including constitutional upset and jaw claudication. Features predictive of permanent visual loss include jaw claudication and temporal artery abnormalities on physical examination. These patients often do not mount high inflammatory responses. Modern imaging techniques show diagnostic promise, and have led to an increased recognition of major artery involvement in GCA. However, temporal artery biopsy remains the gold standard for investigation. Intimal hyperplasia on histologic examination is associated with neuro-ophthalmic complications. The mainstay of therapy remains corticosteroids. Experience using conventional disease-modifying drugs has been mixed, and biologic therapies require further evaluation for their steroid-sparing potential.     

Giant cell arteritis presenting as limb claudication. Report and review of the literature.

Upper limb claudication and pulselessness is an uncommon presentation of giant cell arteritis (GCA), often resulting in delayed diagnosis. We describe such a case diagnosed by angiography, in which a temporal artery biopsy showed classic GCA, despite the absence of local signs or symptoms. A review of 26 similar cases revealed that in 81% of patients where the only manifestation of GCA was upper limb findings, temporal artery biopsy yielded positive findings. Steroid therapy clinically improved 24/26 patients. These findings suggest that a consideration of temporal artery biopsy early in the investigation will hasten diagnosis and appropriate therapy.     

La périartérite noueuse,un diagnostic alternatif à l’artérite gigantocellulaire devant une artérite temporale

IntroductionWeight loss, myalgias, neurologic manifestations and arterial hypertension are common features of polyarteritis nodosa (PAN) at diagnosis. Temporal arteritis is a rarer manifestation of PAN, more suggestive of giant cell arteritis (GCA).CaseWe report the case of a 77-year-old woman who presented with fatigue, weight loss, fever, neck pain, jaw claudication and cough, diagnosed with giant cell arteritis. Diagnosis was reconsidered in favour of a medium and small-sized vessels necrotizing vasculitis corresponding to PAN because of steroid dependence, mononeuritis and suggestive histological features.ConclusionAlthough temporal arteritis is suggestive of GCA, other causes of temporal arteritis can be identified with temporal artery biopsy.     

Giant cell arteritis associated with pericarditis and pancreatic insufficiency in a patient with psoriatic arthritis

A 63-year-old woman with psoriatic arthritis developed arthralgias and shoulder girdle myalgias which were controlled with amitriptyline. Some months later she presented with headache, jaw claudication, weight loss, and chest pain. Anemia of chronic disease, cholestasis, steatorrhea, and pericardial effusion were noted. Giant cell arteritis (GCA) was diagnosed on temporal artery biopsy and prednisone was begun. Her symptoms rapidly abated but steatorrhea continued. It is suggested that these problems were related to GCA. Physicians need to be alert to the diverse presentations of GCA.     

Facial edema as an earlier presenting sign of giant cell arteritis. Possible relationship with angioedema

Giant cell arteritis (GCA) is a chronic granulomatous vasculitis of unknown etiology occurring in the elderly. New-onset headache, scalp tenderness, jaw claudication, temporal artery abnormalities on physical examination, visual symptoms and associated polymyagia rheumatica represent the most typical and frequent features of the disease. However, facial edema is being more commonly recognized as a presenting symptom that may herald the disease. We present a case with facial edema as initial symptom and discuss if this rare symptom of GCA is due to hereditary or acquired angioedema.     

THREE YEAR FOLLOW-UP OF A CASE OF GIANT CELL ARTERITIS PRESENTING WITH A CHRONIC COUGH AND UPPER LIMB ISCHAEMIC SYMPTOMS

A case of giant cell arteritis presenting in an atypical fashionwith respiratory and upper limb ischaemic symptoms is described.The subclavian and axillary arterial involvement is demonstratedin this case, and followed up with repeated angiographic examinationsover the next 3 yr. Despite resolution of all symptoms and returnof pulses and a recordable blood pressure in the ischaemic upperlimb, there was no angiographic evidence of resolution of theaxillary artery stenoses. KEY WORDS: Limb claudication, Respiratory tract involvement, Giant cell arteritis     

Großgefäßvaskulitis

Giant cell arteritis (GCA) and Takayasu arteritis (TA) are the two diseases characterized as large vessel vasculitis (LVV) and are autoimmune diseases with granulomatous inflammation that affect medium and large sized arteries. These diseases are accompanied by symptoms of systemic inflammatory reactions typically including fatigue, weight loss and low grade fever as well as elevation of the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels. More specific symptoms include headache and visual symptoms for GCA and arm or leg claudication, renal hypertension and angina pectoris for TA. Imaging studies to demonstrate inflammatory vascular wall lesions and biopsy of the temporal artery for GCA are the most relevant diagnostic procedures. Treatment relies mainly on glucocorticoids. Methotrexate seems to have a moderate glucocorticoid-sparing effect but evidence for other immunosuppressants, including azathioprine, tocilizumab and cyclophosphamide is limited. Revascularization methods might also be required in TA.     

Temporal arteritis without an elevated erythrocyte sedimentation rate. Case report and review of the literature

An elevated erythrocyte sedimentation rate is regarded as a hallmark of temporal arteritis. Thirty-five cases of biopsy-proved temporal arteritis without an elevated erythrocyte sedimentation rate are identified, and a 36th case is described. All patients had age-adjusted normal Westergren sedimentation rates, 16 with sedimentation rates of 20 mm per hour or less and 20 with sedimentation rates of 21 to 40 mm per hour. Twenty-two patients had sufficient clinical information for analysis and comparison with reported series of patients with biopsy-proved temporal arteritis with an elevated Westergren sedimentation rate. Headache (41 percent), temporal artery abnormalities (41 percent), and visual symptoms (36 percent) were the most common manifestations in patients without an elevated sedimentation rate. Headache (41 percent versus 75 percent, p less than 0.05) and jaw claudication (9 percent versus 43 percent, p less than 0.025) were found less often in the patients without an elevated sedimentation rate. History and physical examination are essential in the diagnosis of temporal arteritis with a normal Westergren sedimentation rate.     

Polyarteritis Nodosa Mimicking Polymyalgia Rheumatica

We report a case of polyarteritis nodosa with a clinical presentation mimicking polymyalgia rheumatica, as well as pathological findings of non-giant-cell arteritis on temporal artery biopsy with symptoms of jaw claudication. Although certain clinical syndromes have been attributed to specific types of systemic vasculitis, considerable overlap occurs. Obtaining tissue biopsy in cases of vasculitis is mandatory for diagnosis and classification. Received: 22 June 1998 / Accepted: 8 December 1998     

Temporal artery bruits in a patient with giant cell arteritis

Giant cell arteritis (GCA) is a disease of unknown etiology characterized by granulomatous inflammation of medium and large arteries. A 69-year-old man presented with right jaw claudication, intermittent scalp tenderness without headache, and visibly swollen temporal arteries. Results of a right temporal artery biopsy were positive for GCA. Auscultation revealed audible bruits of the temporal arteries. We believe this is the first reported example of bruits of the temporal arteries as a manifestation of GCA. The condition resolved with corticosteroid therapy.     

Inflammatory disease in older adults. Cranial arteritis

Cranial arteritis (CA), also called giant cell arteritis or temporal arteritis, is a vasculitis primarily affecting adults over age 50. It is a large vessel vasculitis, and giant cells classically can be identified on histopathologic examination of temporal arteries, but are not essential for diagnosis. Patients typically present with severe headaches, fatigue, polymyalgia-like symptoms, or ischemic complaints such as jaw claudication. Visual loss is the major feared irreversible outcome and can occur in up to 50% of those with untreated disease. Glucocorticoids, typically high dose prednisone (> or = 60 mg/d) is the first-line treatment and successfully controls the inflammatory disease in the vast majority of patients. Most patients can be tapered off steroids within 6 months to 2 years.     

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.     

Temporal arteritis presenting with gastrointestinal symptoms in a middle aged man

Giant cell arteritis, also known as temporal arteritis, is a vasculitis of unknown etiology that classically involves the wall of the large to medium size. We are reporting a case of a young onset temporal arteritis presenting with gastrointestinal symptoms. The patient was a 48-year-old male who presented with a 2-week history of fever, diffuse abdominal pain, and malaise. He underwent a laparoscopic cholecystectomy after findings of elevated bilirubin and alkaline phosphatase as well as suspicion of porcelain gallbladder on ultrasound (or computed tomography scan). The patient subsequently developed painless, intermittent vision loss and unilateral headaches. A work-up included temporal artery biopsy, which showed marked lymphocytic infiltrate in the arterial wall consistent with temporal arteritis. The presentation of temporal arteritis may be atypical. We are reporting a case of temporal arteritis at a young age presenting mainly with gastrointestinal symptoms.     

Case reports

Giant cell arteritis, also known as temporal arteritis, is a vasculitis of unknown etiology that classically involves the wall of the large to medium size. We are reporting a case of a young onset temporal arteritis presenting with gastrointestinal symptoms. The patient was a 48-year-old male who presented with a 2-week history of fever, diffuse abdominal pain, and malaise. He underwent a laparoscopic cholecystectomy after findings of elevated bilirubin and alkaline phosphatase as well as suspicion of porcelain gallbladder on ultrasound (or computed tomography scan). The patient subsequently developed painless, intermittent vision loss and unilateral headaches. A work-up included temporal artery biopsy, which showed marked lymphocytic infiltrate in the arterial wall consistent with temporal arteritis. The presentation of temporal arteritis may be atypical. We are reporting a case of temporal arteritis at a young age presenting mainly with gastrointestinal symptoms.     

Role of imaging studies in the diagnosis and evaluation of giant cell arteritis in Japanese: report of eight cases

The objective of this study is to clarify the characteristics and imaging results of Japanese patients with giant cell arteritis (GCA). Eight patients with biopsy-proven GCA were enrolled. Their clinical data and imaging results were retrospectively examined from their medical records. All the patients met the criteria for the classification of GCA by the American College of Rheumatology. Although the clinical manifestations are similar to those previously reported, none of the eight patients presented ocular symptoms, and half of them presented jaw claudication. Ultrasonography (US) of temporal artery showed the halo sign in all the patients. Fluorodeoxyglucose positron emission tomography (FDG-PET) was performed in four patients and indicated the presence of aortitis of the patients. US is a quick and noninvasive test to detect inflammation of temporal artery, and FDG-PET is very helpful for early diagnosis of aortitis in GCA. Awareness of the disease and appropriate imaging tests will result in diagnosis of GCA.