Giant cell arteritis associated with mononeuritis multiplex and complement-activating 19S IgM rheumatoid factor

Giant cell arteritis is a necrotizing granulomatous arteritis of large arteries, especially the aorta and its branches. Mononeuritis multiplex is a peripheral sensorimotor neuropathy usually producing foot or wrist drop, commonly associated with necrotizing arteritis of small and medium-sized arteries. Rheumatoid vasculitis is an example of the latter type of arteritis associated with high-titer 19S IgM rheumatoid factor typically occurring in patients with long-standing erosive rheumatoid arthritis. This report describes a 71-year-old man with biopsy-proved giant cell arteritis, mononeuritis (foot drop) multiplex, and high-titer complement-activating rheumatoid factor without rheumatoid arthritis. Possible pathogenic relationships are discussed.     

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.     

Giant cell arteritis.

Giant cell arteritis is a generalized inflammatory disorder involving large and medium-sized arteries. The etiology is unknown, although an autoimmune pathogenesis seems probable. In view of the clinical similarities between patients with positive biopsy findings for polymyalgia rheumatica and those with negative biopsy findings, many authors favor the concept that polymyalgia rheumatica is an expression of an underlying giant cell arteritis. There is, however, still controversy as to whether polymyalgia rheumatica and temporal arteritis are different expressions of one and the same disease or two separate, partly overlapping types of giant cell arteritis. A single etiologic factor may be responsible for the two conditions, sometimes expressing itself as polymyalgia rheumatica and sometimes as giant cell arteritis. Recent findings of morphologic similarities in terms of arterial wall atrophy, calcifications, and inflammatory reactions may indicate that polymyalgia rheumatica and temporal arteritis represent different degrees or stages of the same disease.     

Giant cell arteritis.

Giant cell arteritis (GCA), the most common form of systemic vasculitis in adults, preferentially involves large and medium-sized arteries in patients over the age of 50. The classic manifestations are headache, jaw claudication, polymyalgia rheumatica (PMR), and visual symptoms, but 40% of patients present with a wide range of occult manifestations. Early diagnosis and treatment with prednisone can prevent blindness, the most feared complication of GCA. The pathogenesis of GCA is T-cell dependent and antigen driven. Clinical subsets of GCA appear to result from variable cytokine expression. The risk of developing thoracic aortic aneurysm is increased more than 17-fold in patients with GCA. GCA can also involve large arteries, especially the subclavian and axillary arteries. Color Doppler ultrasound, magnetic resonance imaging, and positron-emission tomography scanning are providing insights into the extent and pathogenesis of the disease but have not replaced temporal artery biopsy as the gold standard for securing the diagnosis. Two recently completed double-blind, placebo-controlled trials concerning whether methotrexate plus prednisone is more effective than prednisone alone reached conflicting conclusions.     

Giant cell arteritis of the female reproductive tract associated with temporal arteritis

A woman with giant cell arteritis of the reproductive tract is described. This is the 15th such case reported. This diagnosis led to the subsequent finding of biopsy proven temporal arteritis. The patient's illness responded favorably to corticosteroid therapy.     

Coronary arteritis complicating rheumatoid arthritis.

The case is described of a patient with rheumatoid arthritis (RA) who developed heart failure and vasculitis and died of a myocardial infarction. Autopsy showed vasculitis of several major epicardial coronary arteries. Coronary arteritis in a patient with RA is seldom diagnosed during their lifetime but should be suspected when such a patient develops ischaemic heart disease.     

Giant cell arteritis with visceral angiitis.

We describe a case in which giant cell arteritis coexisted with microscopic polyarteritis nodosa and focal-local glomerulonephritis. We also review previous cases of renal abnormalities in giant cell arteritis. We believe that this association of inflammatory renal disease and giant cell arteritis has not been documented in the past.     

Giant cell arteritis associated with pericarditis and large vessel disease

Giant cell arteritis is well recognised to affect branches of the external carotid artery and to cause systemic symptoms, but may also involve branches of the aorta producing a clinical syndrome that may be difficult to diagnose. A patient is described who presented with limb ischaemia, pericarditis and systemic symptoms. Treatment with corticosteroids improved the systemic manifestations but not the ischaemia. The patient died of bowel infarction four months after treatment was commenced.     

Giant cell arteritis

Giant cell arteritis (GCA) is an immune-mediated chronic vasculitis of large- and medium-sized vessels usually occurring in White individuals aged over 50 years, in Western countries. The pathological hallmark of GCA is granulomatous inflammation of the involved vessels. Headache of new-onset is the most common clinical manifestation, and permanent vision loss is the most feared complication of GCA. The level of clinical suspicion for GCA should be based upon patient age, clinical symptoms, laboratory evaluation, and imaging findings. However, the diagnostic gold standard is achieved by histologic confirmation by temporal artery biopsy. Corticosteroids remain the only proven treatment for GCA and the prognosis for visual recovery is poor.     

Giant cell arteritis

Giant cell arteritis is a medium-vessel vasculitis that affects both men and women. Because the disease commonly presents with nonspecific complaints stemming from cranial arterial insufficiency, the challenge for the physician is recognizing the diagnosis. Recognition of the entity and expeditious initiation of therapy are required to prevent permanent complications, including blindness. There is no pathognomonic finding on physical examination, blood testing, or commonly used radiologic investigations to confirm the diagnosis or establish disease activity. Oral corticosteroids are the mainstay of therapy. Other immune system modulators have no demonstrated efficacy and require further investigation. Percutaneous or surgical revascularization is a viable therapeutic option when the disease is not active.     

Giant cell arteritis

Giant cell arteritis (GCA) is the most common vasculitis of the elderly. The diagnosis can be challenging at times because of the limitation of the American Rheumatology Association (ARA) classification criteria and the significant proportion of biopsy-negative patients with GCA. We discuss the role of advanced imaging techniques, including positron emission tomography (PET) scanning, in establishing diagnosis and improved histopathology techniques to improve the sensitivity of temporal artery biopsy.There have been significant advances in the understanding of the pathogenesis of GCA, particularly the role of cytokine pathways such as the interleukins, IL-6-IL-17 axis, and the IL-12-interferon-γ axis and their implication for new therapies. We highlight that glucocorticoids remain the primary treatment for GCA, but recognize the risk of steroid-induced side effects. A number of pharmacotherapies to enable glucocorticoid dose reduction and prevent relapse have been studied.Early diagnosis and fast-track pathways have improved outcomes by encouraging adherence to evidence-based practice.