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Letters

Diagnosing and managing polymyalgia rheumatica and temporal arteritis

BMJ 1997; 315 doi: https://doi.org/10.1136/bmj.315.7107.549 (Published 30 August 1997) Cite this as: BMJ 1997;315:549

Sensitivity of temporal artery biopsy varies with biopsy length and sectioning strategy

  1. Cathie Sudlow, Wellcome research fellow in clinical epidemiologya
  1. a Department of Clinical Neurosciences, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU
  2. b Royal United Hospital, Bath BA1 3NG
  3. c Meadow Rise, 3 Lakeside, Swindon SN3 1QE
  4. d Warders Medical Centre, Tonbridge, Kent TN9 1LA
  5. e Southampton University Hospitals, Southampton SO9 4XY
  6. f Queen Alexandra Hospital, Portsmouth PO6 SLY

    Editor—Two recent review articles have discussed the diagnosis and management of giant cell arteritis.1 2 Both mentioned temporal artery biopsy, but the complexities of this deserve further discussion.

    Firstly, temporal artery biopsy is not perfectly sensitive. Medical students learn of the existence of skip lesions and that patients should not be denied steroid treatment for giant cell arteritis that is strongly suspected despite a negative biopsy result. Sensitivity is usually measured against a gold standard test, but for giant cell arteritis no such test exists. Probably the most sensible gold standard is a persisting clinical diagnosis at long term (say, one year) follow up.

    Secondly, clinicians should be aware that the sensitivity of biopsy will depend on quality, particularly biopsy length, and preparation for histological examination; a longer biopsy sample with closely spaced sections is likely to have a higher sensitivity than a short one with widely spaced sections. Supporting evidence for the various opinions about optimal biopsy length and preparation is lacking. The largest published series is from the Mayo Clinic.3 The clinic's recommended practice is to take specimens of substantial length (mean 35 mm) with a further specimen from the artery on the other side if the first gives a negative result on frozen section; despite this, many centres still take considerably shorter specimens.4 5

    My audit of 200 temporal artery biopsy specimens sent to the neuropathology laboratory of the Western General Hospital, Edinburgh, between 1990 and 1996 showed a median biopsy length of only 10 mm. The specimens came from various departments, and the median length did not vary between departments. However, there was a significant difference in length between samples that gave positive and negative results on histological examination (1), which suggests that longer specimens may be more likely to yield a positive result. The only way to resolve the issue is to determine the sensitivity of strategies using different biopsy lengths and sectioning policies, with long term follow up as the diagnostic gold standard.

    Mean length of biopsy sample in patients in whom histological result was normal and those in whom it showed arteritis (figures in parentheses are 95% confidence interval)

    View this table:

    Finally, occasionally the clinical suspicion of giant cell arteritis is so strong that the patient is treated with steroids whatever the biopsy result. The final diagnosis and the treatment policy in such patients depend on the clinical response to steroids, not the biopsy result. To imply that it might be considered negligent to avoid biopsy when it will clearly not alter management1 is surely to advocate a defensive style of medicine that cannot be best for our patients.

    References

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    Oral prednisolone 40 mg daily is not adequate for temporal arteritis once vision is affected

    1. Robin Finlay, Consultant ophthalmologistb
    1. a Department of Clinical Neurosciences, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU
    2. b Royal United Hospital, Bath BA1 3NG
    3. c Meadow Rise, 3 Lakeside, Swindon SN3 1QE
    4. d Warders Medical Centre, Tonbridge, Kent TN9 1LA
    5. e Southampton University Hospitals, Southampton SO9 4XY
    6. f Queen Alexandra Hospital, Portsmouth PO6 SLY

      Editor—A J Swannell's review of the diagnosis and management of polymyalgia rheumatica and temporal arteritis fails to address adequately the management of a patient in whom visual loss due to temporal arteritis is already established. Three such patients have been referred to me from the same rural general practice over 20 years, all of whom became totally and irreversibly blind in both eyes despite treatment with steroids.

      The most recent patient was a man of 87 in previously good health who presented to his general practitioner with typical symptoms and signs. On emergency admission he had no light perception in the right eye and 6/6 vision in the apparently normal left eye. Plasma viscosity was 1.9. He was admitted and given 80 mg prednisolone by mouth daily. After 36 hours, despite great improvement in his headaches, he had become totally blind in the left eye as well, though he had not reported this to the nursing staff. Intravenous administration of 1 g methylprednisolone failed to restore vision.

      His case, and results of an audit of outcome of temporal artery biopsies, were discussed by our unit audit committee. The following management guidelines were agreed:

      • All patients with visual loss due to temporal arteritis should be offered admission

      • 500 mg intravenous methylprednisolone should be given immediately on diagnosis

      • Maintenance treatment with 80 mg prednisolone daily should be given until the situation seems stable and the plasma viscosity is falling

      • Visual acuity should be monitored at four hourly intervals and administration of intravenous methylprednisolone 500 mg repeated if further visual loss occurs.

      Two audits of the results of temporal artery biopsy were done in Bath five years apart. Both showed a rate of positive results of about 5%, the referrals for biopsy coming from a variety of sources. This contrasts with 10 positive cases out of 25 of polymyalgia rheumatica reported by Dixon et al.2 There was some disagreement among the audit committee about appropriate criteria for temporal artery biopsy, but I believe that no reasonable request for a biopsy from another doctor should be refused. Temporal arteritis is a worrying and sometimes uncontrollable condition for which the patient has every right to expect our best endeavours. Oral prednisolone 40 mg daily is not adequate treatment once vision is affected.

      References

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      Urgency in giving steroids in giant cell arteritis is still not widely appreciated

      1. Anthony G Freeman, Honorary consultant physicianc
      1. a Department of Clinical Neurosciences, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU
      2. b Royal United Hospital, Bath BA1 3NG
      3. c Meadow Rise, 3 Lakeside, Swindon SN3 1QE
      4. d Warders Medical Centre, Tonbridge, Kent TN9 1LA
      5. e Southampton University Hospitals, Southampton SO9 4XY
      6. f Queen Alexandra Hospital, Portsmouth PO6 SLY

        Editor—In the comprehensive review of the diagnosis and management of polymyalgia rheumatica and temporal arteritis, A J Swannell, like other authors, persists in referring to temporal arteritis as if it was a separate clinical entity.1 Temporal arteritis is but one of many varied manifestations of giant cell arteritis. Most authorities now link polymyalgia rheumatica and giant cell arteritis under the generic term of polymyalgia arteritica.

        Polymyalgia rheumatica is relatively benign but may later progress to giant cell arteritis—a multisystem pathological process. Giant cell arteritis may also present without an antecedent history of polymyalgia rheumatica.2 One must view with concern, as do Karanjia et al, that in 66 patients with giant cell arteritis admitted to a district general hospital there was an average delay of more than six weeks from the onset of symptoms and diagnosis; this was the case even when eight patients in whom the disease presented insidiously over periods longer than six months were excluded.3

        The urgency in treating patients with giant cell arteritis with steroids is still not widely appreciated. A delay of even a few hours in starting treatment may result in irreversible visual failure. If untreated, some 30% of patients develop serious visual complications due either to obliteration of the central retinal artery or, more commonly, to ischaemic optic neuropathy as a direct result of the ciliary arteries supplying the optic nerve and disc being similarly affected. An elderly patient presenting with sudden loss of vision in one or both eyes should be given an intravenous injection of 10 mg dexamethasone before admission to hospital and before the erythrocyte sedimentation rate or, preferably, plasma viscosity is known. Abandoning measurement of the erythrocyte sedimentation rate in favour of measurement of plasma viscosity has been advocated. This would be premature until viscosity can be measured whenever necessary, 24 hours a day.4

        With initial high doses of steroids, toxic effects, such as iatrogenic Cushing's syndrome and osteoporosis, may rarely occur. This, however, is surely a small price to pay compared with permanent blindness in one or both eyes. Monitoring of the acute phase response is essential. Once taking a low maintenance dose of steroids, patients must be warned that any exacerbation of symptoms, particularly sudden deterioration of vision, demands that the dose should be increased immediately. Details of this procedure should be given to the family doctor.5

        References

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        Repeated measurements of erythrocyte sedimentation rate are not efficient use of time or resources

        1. Paul Roome, General practice registrard
        1. a Department of Clinical Neurosciences, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU
        2. b Royal United Hospital, Bath BA1 3NG
        3. c Meadow Rise, 3 Lakeside, Swindon SN3 1QE
        4. d Warders Medical Centre, Tonbridge, Kent TN9 1LA
        5. e Southampton University Hospitals, Southampton SO9 4XY
        6. f Queen Alexandra Hospital, Portsmouth PO6 SLY

          Editor—A J Swannell has provided a comprehensive review of polymyalgia rheumatica and temporal arteritis.1 The author's own policy is to review patients monthly, titrating the dose of prednisolone against symptoms and the erythrocyte sedimentation rate. I believe that repeated measurements of the erythrocyte sedimentation rate are not an efficient use of time or resources.

          To exclude other diagnoses such as malignancy, it is important to monitor the erythrocyte sedimentation rate to ensure that it returns to normal after the initial presentation. The dose of steroid can then be reduced, as suggested by Swannell and by others,2 but only using symptoms as a guide to the severity of disease. If a relapse occurs then the erythrocyte sedimentation rate should be remeasured to confirm the relapse. In a prospective study of 74 patients, however, the erythrocyte sedimentation rate was normal in 48% of relapses.3 The same study reported that the rate was normal in 80% of visits before a relapse. The authors concluded that the erythrocyte sedimentation rate was not useful in predicting relapse.

          As Swannell mentioned, there is little evidence for the various dosing and monitoring schedules. Few studies have taken place in general practice, where most of the patients are treated. For a condition in which side effects of treatment are common,4 it must be time for a large trial to try to define the optimum management schedule.

          References

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          Patients starting steroids should be given advice on risk of osteoporosis

          1. Peter Hodgkins, Senior ophthalmology registrare,
          2. Richard Hull, Consultant rheumatologistf
          1. a Department of Clinical Neurosciences, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU
          2. b Royal United Hospital, Bath BA1 3NG
          3. c Meadow Rise, 3 Lakeside, Swindon SN3 1QE
          4. d Warders Medical Centre, Tonbridge, Kent TN9 1LA
          5. e Southampton University Hospitals, Southampton SO9 4XY
          6. f Queen Alexandra Hospital, Portsmouth PO6 SLY

            Editor—The review of polymyalgia rheumatica and giant cell arteritis correctly concentrated on the clinical presentation, diagnosis, and treatment with corticosteroids.1 We wish to reinforce the author's brief comment about the risks of osteoporosis.

            Polymyalgia rheumatica and giant cell arteritis occur in the aging population, when the risk of fractures is already rising. Corticosteroid treatment, by causing an early loss in bone mass, leads to a further increase in the risk of fracture. The effect is important, especially as the dose of steroid can be high and treatment prolonged, with over half the patients being treated for over two years. Many patients may also be managed by specialists not fully aware of the risks of osteoporosis.

            An audit of practice in one ophthalmology department showed that little consideration was given to the problem of osteoporosis and that little or no advice was given to the patients starting corticosteroids.2 We recently conducted a survey of all British ophthalmologists, with a response rate of 81%.3 While three quarters of respondents regularly prescribed prednisolone in doses of over 5 mg for at least three months, only one quarter gave patients advice on osteoporosis. A recent government report recommended that at this dose bone densitometry should be considered.4 Few consultants in our survey used bone densitometry.

            We believe that all practitioners prescribing corticosteroids should beware of the risk of osteoporosis, especially in the aging population. All patients starting to take corticosteroids should receive basic advice on dietary calcium and exercise. Postmenopausal women should consider hormone replacement therapy, especially if they have had a hysterectomy. Treatment with calcium and vitamin D3, cyclical etidronate, and calcitriol have been shown to have some effect in preventing bone loss in patients taking corticosteroids.

            Our audit has raised local awareness so that patients starting corticosteroids now receive advice as well as referral to an appropriate clinician if they are to remain on treatment for many months.

            References

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