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Show Journal of Neuro- Ophthalmology 15( 1): 36- 38, 1995. © 1995 Raven Press, Ltd., New York Invasive Aspergillosis A Complication of Treatment of Temporal Arteritis Robert E. Wiggins, Jr., M. D. Temporal arteritis is a systemic necrotizing vasculitis for which the only effective treatment is systemic corticosteroids. A review of the literature suggests that there is a significant mortality rate in untreated patients but that those patients who receive adequate corticosteroid treatment rarely experience a reduced life expectancy. I had the opportunity to evaluate a patient with temporal arteritis who eventually died from disseminated aspergillosis 6 weeks after beginning corticosteroid treatment. A high index of suspicion for infections with opportunistic organisms should be maintained in patients with temporal arteritis receiving corticosteroids. Key Words: Temporal arteritis- Aspergillosis. Temporal arteritis is a systemic necrotizing vasculitis that is frequently associated with severe visual loss. Death may occur, particularly in the acute untreated phase, as a result of inflammation of the aorta or cerebral vessels ( 1). The prompt initiation of corticosteroid therapy usually relieves the systemic manifestations and prevents devastating visual loss. Although steroid- related side effects are frequently described in patients treated from temporal arteritis, reports of death are rare. This case report describes a patient with biopsy-proven temporal arteritis who succumbed to disseminated aspergillosis as a consequence of corticosteroid immunosuppression. In this patient, a marked elevation of a previously normal erythrocyte sedimentation rate ( ESR) during treatment was indicative of an opportunistic infection rather than reactivation of the vasculitis. CASE REPORT From the Asheville Eye Medical and Surgical Associates, Asheville, North Carolina, U. S. A. Address correspondence to Dr. Robert E. Wiggins Jr., 495 Biltmore Avenue, Asheville, NC 28801, U. S. A. An 80- year- old white female reported that she had experienced four episodes of transient visual loss in the right eye over the previous 24 hours. She also complained of severe temple pain over the previous several weeks as well as malaise, scalp tenderness, and jaw claudication. Past medical history included controlled hypertension and hypothyroidism. Visual acuity was 20/ 70 in the right eye and 20/ 50 in the left eye. Bilateral cataracts were present. There was no afferent pupillary defect, and the fundi were normal. There were no carotid bruits. The temporal arteries were nodular and markedly tender to palpation. An ESR was 87. A complete blood count was normal as was a recent chest radiograph. A presumptive diagnosis of amaurosis fugax secondary to temporal arteritis was made. 36 INVASIVE ASPERGILLOSIS 37 The patient underwent a temporal artery biopsy that day after receiving 250 mg of intravenous methylprednisolone. She was subsequently started on 80 mg of prednisone per day. The temporal artery biopsy demonstrated " severe acute and chronic arteritis." Within 24 hours of initiation of treatment, the patient noted resolution of her headache and jaw claudication, and she had no further episodes of visual loss. The ESR had decreased to 9 after 2 weeks of treatment and the prednisone was decreased to 70 mg per day. However, after 3'/ 2 weeks of treatment, the patient became confused. She was found to be hypoxic with new bilateral pulmonary infiltrates. The ESR was increased to 110 and the white blood cell count ( WBC) was 6,300. She was hospitalized. The pulmonary infiltrates were thought to be secondary either to an infectious process or to uncontrolled vasculitis. She failed to respond to broad spectrum, intravenous antibiotics, and a subsequent lung biopsy showed invasively growing septate hyphae consistent with aspergillosis. The patient was begun on Amphotericin B but had progressive neurologic deterioration. A head magnetic resonance scan now showed multiple ring enhancing cerebral abscesses as compared with a normal scan on admission ( Fig. 1). The patient died 2 weeks after admission from complications of disseminated aspergillosis. At autopsy, Aspergillus fumiga-tus was identified in all lobes of the lung with FIG. 1. T1- weighted, gadolinium enhanced axial MRI scan. dissemination to the brain, kidneys, and thyroid gland. DISCUSSION Temporal arteritis is a systemic vasculitis, which has been associated with an increased mortality in the initial phase of the illness. Death in these cases is usually due to inflammation of the cerebral vessels, cardiac vessels, or the aorta, and usually occurs in untreated or inadequately treated patients ( 1- 3). Long- term follow- up in several studies has demonstrated no deleterious effect on survivorship among patients receiving adequate treatment with corticosteroids ( 4). In a review of 42 patients diagnosed with temporal arteritis over a 25- year period, Huston and colleagues ( 5) found that " this group of older patients tolerated corticosteroid treatment quite well." The most commonly reported adverse effects were vertebral compression fractures ( 11 patients) and myopathy ( 7 patients). No patients developed severe or life- threatening complications. In contrast, Graham and coworkers ( 6) reported that patients who required a high maintenance dose of corticosteroids had a higher long- term ( greater than 6 months after diagnosis) death rate and suggested that continued steroid therapy might be responsible for the increased mortality. Bisgard and coworkers ( 7) also found an increased mortality rate in steroid- treated patients but attributed the excess mortality to the disease rather than to the medication. Recently, Chmelewski and colleagues ( 8) reported a death from sepsis in a patient receiving corticosteroids 74 months after diagnosis of temporal arteritis. A case of aspergillosis was also reported in that series. Finally, Miller ( 9) reported the development of an opportunistic infection 6 months after initiation of corticosteroid treatment. The patient, a 72- year- old male with temporal arteritis, developed a crypto-coccal meningitis, which was successfully treated. It was noted that a rise in the ESR was initially attributed to a reactivation of the disease and led to an increase in the dose of corticosteroid before the correct diagnosis was ascertained. A marked rise in the ESR was also noted in our patient without an elevation of the WBC. Invasive aspergillosis is associated with a high mortality rate ( 90% in organ transplant patients) ( 10). The route of infection is usually the respiratory tract. The diagnosis in this case was suspected because of the patient's immunocompromised state and her failure to respond to broad- spectrum antibiotics. The patient died despite early diagno- / Neuro- Ophlhalmol, Vol. 15, No. 1, 1995 38 R. E. WIGGINS JR. sis by bronchoscopic biopsy and culture and treatment with Amphotericin B. The only known risk factors in this patient were corticosteroid treatment and advanced age. This report documents a fatal complication related to corticosteroid immunosuppressive treatment of temporal arteritis. A review of the literature suggests that this is a rare complication of treatment and that most steroid- related complications are not life- threatening. It has been suggested that a temporal artery biopsy may not be necessary when typical symptoms of the disease are present in association with an elevated ESR ( 11). However, because of the potential for serious lor even fatal corticosteroid complications, I agree with Miller ( 9) and Kearns ( 12) that, for medical and legal reasons, a biopsy should be obtained in all patients suspected of having temporal arteritis, even when the diagnosis seems certain on clinical grounds. Acknowledgment: I thank Stephen Pollock, M. D., for reviewing this manuscript. REFERENCES 1. Graham E. Survival in temporal arteritis. Trans Ophthalmol Soc UK 1980; 100: 108- 10. 2. Hamilton CR, Shelly WM, Tumulty PA. Giant cell arteritis: including temporal arteritis and polymyalgia rheumatica. Medicine 1971; 50: 1- 27. 3. Nordborg E, Bengtsson BA. Death rates and causes of death in 284 consecutive patients with giant cell arteritis confirmed by biopsy. Br Med ] 1989; 299: 549- 50. 4. Keltner JL. Giant- cell arteritis. Ophthalmology 1982; 89: 1101- 10. 5. Huston KA, Hunder GG, Lie JT, et al. Temporal arteritis: a 25 year epidemiologic, clinical, and pathologic study. Ann Intern Med 1978; 88: 162- 7. 6. Graham E, Holland A, Avery A, et al. Prognosis in giant cell arteritis. Br Med J 1981; 282: 269- 71. 7. Bisgard C, Sloth H, Keiding N, et al. Excess mortality in giant cell arteritis. / Intern Med 1991; 230: 119- 23. 8. Chmelewski WL, McKnight KM, Agudelo CA, et al. Presenting features and outcomes in patients undergoing temporal artery biopsy. Arch Intern Med 1992; 152: 1690- 5. 9. Miller NL. Walsh and Hoyt's Clinical N'euro- ophthalmology, 4th Ed. Baltimore: Williams & Wilkins; 1991: 2623- 6. 10. Tang CM, Cohen J. Invasive aspergillosis: diagnosis, treatment, and prevention. Infec Dis Clin Pract 1992; 1: 217- 23. 11. Paice WE. Giant cell arteritis: difficult decisions in diagnosis, investigation, and treatment. Postgrad Med J 1989; 65: 743- 7. 12. Kearns P. Temporal artery biopsy. Br Med J 1988; 297: 1404. / Neuro- Ophthalmol, Vol. 15, No. 1, 1995 |