Polymyalgia rheumatica and giant cell arteritis. The dilemma of therapy

Polymyalgia rheumatica, next to rheumatoid arthritis the most common inflammatory rheumatic disorder of the elderly, is a nonspecific clinical syndrome involving pain in the shoulder and pelvic girdles. Giant cell arteritis appears to localize in elastin-containing arteries and can cause similar myalgias. A relationship exists between the two diseases, as evidenced by their frequent coexistence in the same patient. The symptoms of polymyalgia rheumatica respond to low-dose corticosteroid therapy, while giant cell arteritis requires higher doses to prevent blindness due to involvement of the temporal artery. The key decision in therapy, therefore, concerns the dose and duration of use of steroid for polymyalgic symptoms. In this decision, prevention of the catastrophic complications of giant cell arteritis and avoidance of needless side effects of high-dose steroid therapy in the elderly are competing considerations.     

Management of giant cell arteritis and polymyalgia rheumatica

Giant cell arteritis and polymyalgia rheumatica are closely related disorders that affect persons more than 50 years of age and cause substantial morbidity. Patients with giant cell arteritis typically have a localized headache, nonspecific systemic symptoms, temporal artery tenderness and a high erythrocyte sedimentation rate (ESR). The diagnosis is confirmed by characteristic pathologic findings on temporal artery biopsy. Patients with polymyalgia rheumatica usually have similar nonspecific systemic symptoms, proximal muscle pain and stiffness, and an elevated ESR. The diagnosis is based on the clinical findings. Both disorders are treated with corticosteroids: high dosages for giant cell arteritis (prednisone in a dosage of 40 to 60 mg per day) and lower dosages for polymyalgia rheumatica (prednisone in a dosage of 10 to 20 mg per day). Symptom relief in response to treatment is rapid and reinforces the diagnosis. After normalization of the ESR, the corticosteroid is tapered, with the patient monitored closely for symptom recurrence. Most patients require corticosteroid therapy for two to three years and experience one or more treatment complications.     

Factor VIII-von Willebrand factor in giant cell arteritis and polymyalgia rheumatica

Levels of three factor VIII-von Willebrand factor components (von Willebrand antigen, ristocetin cofactor, and factor VIII coagulant) were higher in specimens of plasma from 27 patients with giant cell arteritis and 18 patients with polymyalgia rheumatica than in specimens from 21 normal control subjects. Values in patients with active giant cell arteritis were higher than those in patients with either inactive giant cell arteritis or active polymyalgia rheumatica. Levels of factor VIII-von Willebrand factor components tended to decline gradually after disease activity had been suppressed by corticosteroid therapy and therefore may be indicators of vascular damage. These levels, however, did not revert to normal rapidly in response to corticosteroid therapy as did the patients' symptoms and the usual laboratory measurements indicative of inflammation; thus, measurements of these components are unlikely to be useful in day-to-day management of these diseases. Electrophoretic analysis suggested that the elevated values are due to increased amounts of normal factor VIII-von Willebrand factor rather than to the presence of an abnormal molecule.     

Polymyalgia rheumatica and giant cell arteritis

Polymyalgia rheumatica and giant cell arteritis are common, closely related vasculitic conditions that almost exclusively occur in patients older than 50 years. They may be manifestations of the same underlying disease and often coexist. Patients with polymyalgia rheumatica usually present with acute onset of stiffness and pain in the shoulder and pelvic musculature, which may be accompanied by fever, malaise, and weight loss. If untreated, polymyalgia rheumatica may result in significant disability. Giant cell arteritis may manifest as visual loss or diplopia, abnormalities of the temporal artery such as tenderness or decreased pulsation, jaw claudication, and new-onset headaches. Erythrocyte sedimentation rate and temporal artery biopsy help make the diagnosis. Giant cell arteritis requires urgent diagnosis because without treatment it may lead to irreversible blindness. Patients with either condition also may have nonspecific symptoms. Corticosteroids are the mainstay of therapy for both conditions, with higher doses required for treatment of giant cell arteritis. Duration of corticosteroid therapy can be five years or longer before complete clinical remission is achieved. Monitoring for corticosteroid-associated side effects such as osteoporosis and diabetes, as well as for relapses and flare-ups, is key to chronic management. The prognosis for either condition, if treated, is good.     

Polymyalgia rheumatica and temporal arteritis

Polymyalgia rheumatica and temporal arteritis are a clinical syndrome and clinicopathologic entity, respectively. Polymyalgia rheumatica occurs more commonly than temporal arteritis, with approximately half of all patients with temporal arteritis having the polymyalgia rheumatica syndrome. Both conditions are found in the population over 50 years of age and are associated with an elevated ESR. The etiology of both is unclear, although genetic, and potentially, environmental factors may play significant roles. Both conditions respond to corticosteroid therapy, but patients with temporal arteritis require significantly higher doses to control symptoms and to prevent blindness.     

Behavior of prealbumin in the acute phase of polymyalgia rheumatica treated with 6-methylprednisolone

Summary Serum levels of prealbumin, fibronectin, fibrinogen, α₁-acid glycoprotein, C-reactive protein, immunoglobulins, and white blood cell count were prospectively studied in 33 patients affected by polymyalgia rheumatica during the first 45 days of treatment with 6-methylprednisolone. Almost all parameters considered, except for fibronectin and IgM, settled within the normal range fairly quickly, while prealbumin showed a specular course compared with the other reactants. This behavior reflected the improvement of clinical symptoms registered in all patients after steroid treatment. Finally, the genesis of the low baseline prealbumin levels found in polymyalgia rheumatica/giant cell arteritis and their behavior during treatment are discussed.     

Temporal arteritis: an approach to suspected vasculitides

Temporal arteritis, also known as giant cell arteritis, is the most common vasculitis in adults. Classic symptoms include polymyalgia rheumatica, new-onset headache, jaw claudication, and visual symptoms such as diplopia and amaurosis fugax. Elevated erythrocyte sedimentation rate is a common laboratory finding in temporal arteritis, and abnormalities on temporal artery biopsy are the gold standard for diagnosis. Rapid treatment with steroids can prevent permanent vision loss, which is the worst ischemic complication of the disease. It is important for primary care physicians to be able to recognize the signs and symptoms of this disease and begin treatment rapidly.     

Giant cell arteritis and polymyalgia rheumatica.

The close relationship between giant cell arteritis and polymyalgia rheumatica has not been clearly explained. These disorders affect the same patient population and often coexist in the same person. Monitoring of the erythrocyte sedimentation rate is a useful tool in both diagnosis and treatment. Management with varying doses of prednisone has proved effective in resolving symptoms.     

Polymyalgia arteritica: a clinical review

Abstract. In a series of thirty-seven consecutive patients with polymyalgia arteritica, twenty-five had polymyalgia rheumatica and twelve had cranial arteritis. Some failed to respond promptly to low doses of prednisolone and it is recommended that the initial dose should be in the order of 40 mg daily. An ESR above 40 mm in the first hour was present in four patients 3 months after admission; three were found to have rheumatoid disease and one pulmonary tuberculosis.
Symptomatic relapses occurred in fourteen patients on twenty-one occasions and all responded to an increase in the daily dose of maintenance prednisolone. Most occurred in the first year and were attributable to an excessively rapid reduction in steroid therapy. Relapses occurring in patients on a stable dose of prednisolone were commonly associated with the development of rheumatoid disease. In elderly patients who have relapsed, or who have had arteritic complications, life-long prednisolone therapy appears justifiable.     

Giant cell arteritis mimicking multiple myeloma; diagnosed by PET scan

This case report describes a patient who presented with severe anemia, monoclonal gammopathy, a high erythrocyte sedimentation rate and significant weight loss. These features were highly suggestive of multiple myeloma. Bone marrow aspiration was negative for myeloma on two occasions. A positron emission tomography (PET) scan showed extensive 2-flourodeoxy-glucose uptake in the vascular tree consistent with arteritis. A temporal artery biopsy established the diagnosis of giant cell arteritis (GCA). There were no typical symptoms of GCA, such as headache, visual disturbance, or polymyalgia rheumatica. The patient was treated with steroids, which resulted in the resolution of anemia, monoclonal gammapathy, and other symptoms.     

Functional profile of tissue-infiltrating and circulating CD68+ cells in giant cell arteritis. Evidence for two components of the disease.

Macrophages represent a critical component in the inflammatory lesions of giant cell arteritis. By combining immunohistochemistry and in situ hybridization, we have analyzed the functional heterogeneity of tissue-infiltrating macrophages in patients with untreated vasculitis. 20% of macrophages in temporal artery tissue synthesized IL-6-specific mRNA and produced IL-6 and IL-1 beta proteins. IL-6 and IL-1 beta production was not limited to CD68+ cells in the lymphoid aggregates but was a feature of CD68+ cells dispersed throughout the tissue. 50% of tissue-infiltrating CD68+ cells synthesized 72-kD type IV collagenase. Only a small subset of CD68+ cells produced cytokines as well as collagenase, indicating functional specialization or distinct differentiation stages of CD68+ cells in the inflamed tissue. Activation of CD68+ cells was not restricted to tissue-infiltrating cells. Expression of IL-6 and IL-1 beta was found in 60-80% of circulating monocytes of patients with untreated giant cell arteritis, whereas collagenase production was restricted to tissue macrophages. IL-6 and IL-1 beta production by the majority of circulating monocytes was a shared feature of patients with giant cell arteritis and polymyalgia rheumatica but was not found in rheumatoid arthritis. These data suggest that giant cell arteritis has two components of disease, an inflammatory reaction in vessel walls and a systemic activation of monocytes. Systemic monocyte activation can manifest independently without vasculitis as exemplified in patients with polymyalgia rheumatica.     

Polymyalgia rheumatica and temporal arthritis

Polymyalgia rheumatica and temporal arteritis are closely related inflammatory conditions that affect different cellular targets in genetically predisposed persons. Compared with temporal arteritis, polymyalgla rheumatica is much more common, affecting one in 200 persons older than 50 years. Temporal arteritis, however, is more dangerous and can lead to sudden blindness. The diagnosis of polymyalgia rheumatica is based on the presence of a clinical syndrome consisting of fever, nonspecific somatic complaints, pain and stiffness in the shoulder and pelvic girdles, and an elevated erythrocyte sedimentation rate. Temporal arteritis typically presents with many of the same findings as polymyalgia rheumatica, but patients also have headaches and tenderness to palpation over the involved artery. Arterial biopsy usually confirms the diagnosis of temporal arteritis. Early diagnosis and treatment of polymyalgia rheumatica or temporal arteritis can dramatically improve patients' lives and return them to previous functional status. Corticosteroid therapy provides rapid and dramatic improvement of the clinical features of both conditions. Therapy is generally continued for six to 24 months. Throughout treatment, clinical condition is assessed periodically. Patients are instructed to see their physician immediately if symptoms recur or they develop new headache, jaw claudication or visual problems.     

Polymyalgia rheumatica. Clinical features and management

Polymyalgia rheumatica is a clinical syndrome that occurs almost exclusively in older patients. It is characterized by muscle aching and stiffness and an elevated erythrocyte sedimentation rate. It must be distinguished from other common disorders, particularly polymyositis, fibromyalgia, and other chronic inflammatory or neoplastic diseases. A temporal artery biopsy should be done in patients with confirmed polymyalgia rheumatica and suspected coexistent temporal arteritis, which can have serious manifestations. Patients with polymyalgia rheumatica respond dramatically to low doses of corticosteroids, although prolonged daily treatment often may be needed.     

Visual complications of polymyalgia rheumatica (polymyalgia arteritica).

Four case histories are reported in which patients with polymyalgia rheumatica (polymyalgia arteritic) developed evidence of cranial arteritis (in one case two years and in one six months) following withdrawal of steroid therapy after apparent cure. In three cases partial or complete loss of sight has resulted. Steroid therapy should not only be introduced rapidly at appropriate dosage levels as soon as the diagnosis is made but should not be reduced or discontinued prematurely.     

Giant cell arteritis: suspect it, treat it promptly

Giant cell arteritis is the most common form of vasculitis affecting older people, and the diagnosis should be considered in older patients who present with the new onset of headache, visual dysfunction, polymyalgia rheumatica, or systemic inflammatory symptoms. Physicians should be familiar with its variety of clinical presentations and the abnormal laboratory findings associated with it.     

Polymyalgia rheumatica: clinical presentation is key to diagnosis and treatment

Polymyalgia rheumatica should be considered in the differential diagnosis in patients over 50 years old who present with bilateral achiness and stiffness in the shoulders or hips or both. It usually responds quickly to once-daily, low-dose prednisone, but some patients require treatment for several years. Polymyalgia rheumatica frequently overlaps with giant cell arteritis, and patients must be followed closely for development of complications from this condition, especially aortitis.     

Tocilizumab: A novel humanized anti-interleukin 6 (IL-6) receptor antibody for the treatment of patients with non-RA systemic,inflammatory rheumatic diseases

Abstract

Tocilizumab is a highly effective therapeutic agent for the treatment of rheumatoid arthritis and systemic juvenile idiopathic arthritis. Furthermore, a large amount of case study data reveals that tocilizumab can be an effective therapy for not only rheumatoid arthritis but also for other mostly rare inflammatory rheumatic diseases. By blocking the interleukin-6 pathway tocilizumab can be a useful therapeutic alternative when conventional treatment fails. It is successful in treating diseases such as the adult-onset Still's disease, amyloidosis, giant cell arteritis, multiple myeloma, polymyalgia rheumatica, relapsing polychondritis, remitting seronegative symmetrical synovitis with pitting edema-syndrome, systemic lupus erythematosus, systemic sclerosis, and Takayasu arteritis. Studies underway are now recruiting patients to acquire further data on treating patients with non-rheumatic arthritis, inflammatory diseases. This review focuses on tocilizumab as a promising agent for treating rare and orphan diseases in rheumatology for which no satisfactory treatment is yet available.     

Giant cell arteritis with multiple cranial nerve palsy and reversible proptosis: a case report

Giant cell arteritis (GCA) often presents with symptoms of headache, jaw claudication, polymyalgia rheumatica, and blurred vision. GCA is relatively rare and may have atypical manifestations in Asians, including multiple cranial nerve palsy and reversible proptosis. A high suspicion of GCA is suggested when any older Asian suffers from headache that is new-onset or different from the previous pattern, even without other typical manifestations of GCA.     

Microheterogeneity of alpha 1-antichymotrypsin in the management of giant-cell arteritis and polymyalgia rheumatica

1. Using crossed immunoaffinity electrophoresis with free concanavalin A in the first dimension, we studied the glycan microheterogeneity of alpha 1-antichymotrypsin in sera from patients with giant-cell arteritis and/or polymyalgia rheumatica, and its variation in the serum of several of these patients during induction of disease remission by prednisone therapy and possible further recurrence of giant-cell arteritis and/or polymyalgia rheumatica. 2. In the serum of patients with active disease we observed increased proportions of concanavalin A nonreactive and concanavalin A weakly reactive fractions. The results were expressed as the ratio of concanavalin A non-reactive fraction plus concanavalin A weakly reactive fraction to concanavalin A reactive fraction, called R alpha 1-ACT. An R alpha 1-ACT higher than 1.8 (upper normal value) was found in 30/31 sera from patients with active disease (sensitivity 97%) and in 2/22 sera from patients with inactive disease (specificity 91%). 3. The erythrocyte sedimentation rate and the serum C-reactive protein level, common markers of biological inflammation, are usually elevated in active giant-cell arteritis and/or polymyalgia rheumatica. The two parameters are commonly used to guide the therapy during the course of the disease, but they have no specificity. An erythrocyte sedimentation rate of greater than 30 mm/h was found in 30/31 sera from patients with active disease (sensitivity 97%) and in 5/22 sera from patients with inactive disease (specificity 77%).(ABSTRACT TRUNCATED AT 250 WORDS)