Ocular Immunology & Inflammation, 2014; 22(5): 391–393 ! Informa Healthcare USA, Inc.. ISSN: 0927-3948 print / 1744-5078 online DOI: 10.3109/09273948.2013.849351
LETTER TO THE EDITOR
Giant Cell Arteritis Presenting with Uveitis* Stephanie N Slemp,
Ocul Immunol Inflamm Downloaded from informahealthcare.com by University of Colorado Libraries on 12/28/14 For personal use only.
1
MD
1
, Sarah E Martin, MD1, Richard A Burgett, Eyas M Hattab, MD1
MD
2
, and
Department of Pathology and Laboratory Medicine and 2Department of Ophthalmology, Indiana University School of Medicine, Indianapolis, IN, USA
ABSTRACT Giant cell arteritis, also known as temporal arteritis, is the most common primary vasculitis affecting the nervous system. Early recognition of this treatable condition is essential to avoid potentially devastating complications. Giant cell arteritis occurs in adults older than 50 years and affects large and medium-sized arteries, especially the external and internal carotid arteries and their branches. Severe inflammation of the vessel wall may result in obstruction of the lumen and end-organ ischemia. Typical giant cell arteritis symptoms include headache, scalp tenderness, jaw claudication, and polymyalgia rheumatica. Ischemia induced by the arteritis can lead to blindness. Herein, we describe a rare case of giant cell arteritis in a patient who initially presented with uveitis, thus eluding timely diagnosis and prompt therapy. Keywords: Giant cell arteritis, temporal arteritis, uveitis
LETTER TO THE EDITOR
presumptive diagnosis of endophthalmitis followed by prompt pars plana vitrectomy with vitreous biopsy, vitreous culture, and intravitreal antibiotics. Cultures and cytology were negative for infectious organisms and malignancy. He developed inferior exudative retinal detachment with possible underlying choroidal mass. Posterior segment inflammation precluded a clear view of the process and B-scan ultrasonography demonstrated scleral thickening suggestive of posterior scleritis. Review of systems was positive for generalized fatigue and diffuse headache, in addition to right eye redness and pain. Complaints of headache prompted testing for Westergren erythrocyte sedimentation rate (ESR) and C-reactive protein. Both were elevated, measuring 70 mm/hr and 5.8 mg/dL, respectively. Temporal artery biopsy revealed an intense mixed inflammatory infiltrate involving all three layers of the vessel wall. The infiltrate was composed of a mixture of lymphocytes, plasma cells, histiocytes and giant cells. Fibrinoid necrosis with neutrophils was noted. Luminal occlusion with recanalization was also evident (Figure 1A–C).
Giant cell arteritis, also known as temporal arteritis, is the most common primary vasculitis affecting the nervous system and most often presents with headache, polymyalgia rheumatica, and fatigue and jaw claudication.1,2 Herein, we describe a case of giant cell arteritis with an unusual clinical presentation that was not initially recognized and resulted in an adverse clinical outcome.
CASE REPORT A 76-year-old man with history of pseudophakia and exudative macular degeneration, treated with intravitreal Avastin injections, presented with temporal headache and jaw pain, decreased vision to the counting fingers level and right eye pain for four months. He reported no episodes of fever or malaise. A disciform macular scar was noted on initial presentation. However, vitreous inflammation led to a
*Presented, in part, at the 89th annual meeting of the American Association of Neuropathologists, Charleston, SC, June 20–23, 2013. Received 22 August 2013; revised 17 September 2013; accepted 24 September 2013; published online 17 October 2013 Correspondence: Eyas M Hattab, MD Department of Pathology and Laboratory Medicine Indiana University School of Medicine 350 West 11th Street, IUHPL 4014 Indianapolis, IN 46202 Phone: 317-491-6317 Fax: 317-491-6419 E-mail: [email protected]
391
Ocul Immunol Inflamm Downloaded from informahealthcare.com by University of Colorado Libraries on 12/28/14 For personal use only.
392 Stephanie N Slemp et al.
FIGURE 1. (A) A cross-section of the temporal artery shows an intense inflammatory infiltrate involving all three layers of the vessel wall (H&E). (B) Luminal occlusion with recanalization (arrow) and fibrinoid necrosis are also present (H&E). (C) The inflammatory infiltrate consists of a mixture of lymphocytes, plasma cells, histiocytes, and giant cells (arrow) (H&E). (D) A Verhoeff–van Gieson stain highlights an extensively disrupted internal elastic lamina (arrow).
Verhoeff–van Gieson stain highlighted an extensively disrupted and focally obliterated internal elastic lamina (Figure 1D). A diagnosis of GCA was rendered. The patient was placed on high-dose oral prednisone and topical prednisolone acetate. His headache and jaw pain dramatically improved in the first 24 hours and promptly resolved. ESR and C-reactive protein normalized. His scleritis quickly responded to treatment. His findings were significantly improved in one month and resolved by two months. Followup five months after diagnosis of GCA showed no recurrence of scleritis on a dose of only 5 mg prednisone daily, and ophthalmic examination demonstrated resolution of intraocular inflammation and scleritis.
DISCUSSION We described a patient with posterior uveitis and a possible underlying posterior scleritis who subsequently was diagnosed with GCA two weeks later. Although sterile or culture negative endophthalmitis is possible, its persistence after vitrectomy and the strong laboratory and pathologic findings suggest that GCA was not coincidental in this case. Uveitis as a
presenting feature of GCA is very rare and, to our knowledge, has only been reported in three other patients.3-5 These patients, along with ours, were all initially treated with topical steroids, delaying the correct diagnosis and treatment anywhere from three weeks to one year.3-5 Most patients had slight improvement in visual symptoms with topical treatment; however, headache or polymyalgia prompted further investigation.3,4 One patient was initially treated with both topical and systemic steroids for a diagnosis of bilateral panuveitis.5 Her symptoms improved, and it was not until tapering of the steroids that she had recurrence of symptoms one year later. In both our patient and the patient described by Bandini et al., there was unfortunately no improvement in vision after appropriate diagnosis and treatment of GCA.3 However, the disciform macular scar that was noted on initial presentation may have been responsible for the persistent poor visual acuity in our patient. In two cases, as well as ours, ESR was found to be elevated after initial treatment with topical steroids failed to improve symptoms.3,4 Elevated ESR associated with symptoms of GCA, prompted temporal artery biopsy in all cases. Although temporal artery biopsy is an invasive procedure, the highly Ocular Immunology & Inflammation
Giant Cell Arteritis Presenting with Uveitis treatable nature of promptly diagnosed GCA and potentially devastating consequences of a delayed diagnosis cannot be denied. Awareness that posterior uveitis and scleritis may be the presenting findings of GCA should prompt clinicians to ask about other symptoms of GCA and consider obtaining sedimentation rate, C-reactive protein, and temporal artery biopsy in select patients.
Ocul Immunol Inflamm Downloaded from informahealthcare.com by University of Colorado Libraries on 12/28/14 For personal use only.
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.
!
2014 Informa Healthcare USA, Inc.
393
REFERENCES 1. Salvarani C, Cantini F, Boiardi L, et al. Polymyalgia rheumatica and giant-cell arteritis. N Engl J Med. 2002; 347:261–271. 2. Zhou L, Luneau K, Weyand CM, et al. Clinicopathologic correlations in giant cell arteritis: a retrospective study of 107 cases. Ophthalmology. 2009;116: 1574–1580. 3. Bandini F, Benedetti L, Ceppa P, et al. Uveitis as a presenting sign of giant cell arteritis. J Neuroophthalmol. 2005;25:247–248. 4. Dasgupta B, Pitzalis C, Panayi GS. Inflammation of the uveal tract as a presenting feature of temporal arteritis. Ann Rheum Dis. 1989;48:964–965. 5. Rajesh CV, Cole M. Panuveitis as a presenting feature of giant cell arteritis. Br J Ophthalmol. 2000;84:340.
LETTER TO THE EDITOR
Giant Cell Arteritis Presenting with Uveitis* Stephanie N Slemp,
Ocul Immunol Inflamm Downloaded from informahealthcare.com by University of Colorado Libraries on 12/28/14 For personal use only.
1
MD
1
, Sarah E Martin, MD1, Richard A Burgett, Eyas M Hattab, MD1
MD
2
, and
Department of Pathology and Laboratory Medicine and 2Department of Ophthalmology, Indiana University School of Medicine, Indianapolis, IN, USA
ABSTRACT Giant cell arteritis, also known as temporal arteritis, is the most common primary vasculitis affecting the nervous system. Early recognition of this treatable condition is essential to avoid potentially devastating complications. Giant cell arteritis occurs in adults older than 50 years and affects large and medium-sized arteries, especially the external and internal carotid arteries and their branches. Severe inflammation of the vessel wall may result in obstruction of the lumen and end-organ ischemia. Typical giant cell arteritis symptoms include headache, scalp tenderness, jaw claudication, and polymyalgia rheumatica. Ischemia induced by the arteritis can lead to blindness. Herein, we describe a rare case of giant cell arteritis in a patient who initially presented with uveitis, thus eluding timely diagnosis and prompt therapy. Keywords: Giant cell arteritis, temporal arteritis, uveitis
LETTER TO THE EDITOR
presumptive diagnosis of endophthalmitis followed by prompt pars plana vitrectomy with vitreous biopsy, vitreous culture, and intravitreal antibiotics. Cultures and cytology were negative for infectious organisms and malignancy. He developed inferior exudative retinal detachment with possible underlying choroidal mass. Posterior segment inflammation precluded a clear view of the process and B-scan ultrasonography demonstrated scleral thickening suggestive of posterior scleritis. Review of systems was positive for generalized fatigue and diffuse headache, in addition to right eye redness and pain. Complaints of headache prompted testing for Westergren erythrocyte sedimentation rate (ESR) and C-reactive protein. Both were elevated, measuring 70 mm/hr and 5.8 mg/dL, respectively. Temporal artery biopsy revealed an intense mixed inflammatory infiltrate involving all three layers of the vessel wall. The infiltrate was composed of a mixture of lymphocytes, plasma cells, histiocytes and giant cells. Fibrinoid necrosis with neutrophils was noted. Luminal occlusion with recanalization was also evident (Figure 1A–C).
Giant cell arteritis, also known as temporal arteritis, is the most common primary vasculitis affecting the nervous system and most often presents with headache, polymyalgia rheumatica, and fatigue and jaw claudication.1,2 Herein, we describe a case of giant cell arteritis with an unusual clinical presentation that was not initially recognized and resulted in an adverse clinical outcome.
CASE REPORT A 76-year-old man with history of pseudophakia and exudative macular degeneration, treated with intravitreal Avastin injections, presented with temporal headache and jaw pain, decreased vision to the counting fingers level and right eye pain for four months. He reported no episodes of fever or malaise. A disciform macular scar was noted on initial presentation. However, vitreous inflammation led to a
*Presented, in part, at the 89th annual meeting of the American Association of Neuropathologists, Charleston, SC, June 20–23, 2013. Received 22 August 2013; revised 17 September 2013; accepted 24 September 2013; published online 17 October 2013 Correspondence: Eyas M Hattab, MD Department of Pathology and Laboratory Medicine Indiana University School of Medicine 350 West 11th Street, IUHPL 4014 Indianapolis, IN 46202 Phone: 317-491-6317 Fax: 317-491-6419 E-mail: [email protected]
391
Ocul Immunol Inflamm Downloaded from informahealthcare.com by University of Colorado Libraries on 12/28/14 For personal use only.
392 Stephanie N Slemp et al.
FIGURE 1. (A) A cross-section of the temporal artery shows an intense inflammatory infiltrate involving all three layers of the vessel wall (H&E). (B) Luminal occlusion with recanalization (arrow) and fibrinoid necrosis are also present (H&E). (C) The inflammatory infiltrate consists of a mixture of lymphocytes, plasma cells, histiocytes, and giant cells (arrow) (H&E). (D) A Verhoeff–van Gieson stain highlights an extensively disrupted internal elastic lamina (arrow).
Verhoeff–van Gieson stain highlighted an extensively disrupted and focally obliterated internal elastic lamina (Figure 1D). A diagnosis of GCA was rendered. The patient was placed on high-dose oral prednisone and topical prednisolone acetate. His headache and jaw pain dramatically improved in the first 24 hours and promptly resolved. ESR and C-reactive protein normalized. His scleritis quickly responded to treatment. His findings were significantly improved in one month and resolved by two months. Followup five months after diagnosis of GCA showed no recurrence of scleritis on a dose of only 5 mg prednisone daily, and ophthalmic examination demonstrated resolution of intraocular inflammation and scleritis.
DISCUSSION We described a patient with posterior uveitis and a possible underlying posterior scleritis who subsequently was diagnosed with GCA two weeks later. Although sterile or culture negative endophthalmitis is possible, its persistence after vitrectomy and the strong laboratory and pathologic findings suggest that GCA was not coincidental in this case. Uveitis as a
presenting feature of GCA is very rare and, to our knowledge, has only been reported in three other patients.3-5 These patients, along with ours, were all initially treated with topical steroids, delaying the correct diagnosis and treatment anywhere from three weeks to one year.3-5 Most patients had slight improvement in visual symptoms with topical treatment; however, headache or polymyalgia prompted further investigation.3,4 One patient was initially treated with both topical and systemic steroids for a diagnosis of bilateral panuveitis.5 Her symptoms improved, and it was not until tapering of the steroids that she had recurrence of symptoms one year later. In both our patient and the patient described by Bandini et al., there was unfortunately no improvement in vision after appropriate diagnosis and treatment of GCA.3 However, the disciform macular scar that was noted on initial presentation may have been responsible for the persistent poor visual acuity in our patient. In two cases, as well as ours, ESR was found to be elevated after initial treatment with topical steroids failed to improve symptoms.3,4 Elevated ESR associated with symptoms of GCA, prompted temporal artery biopsy in all cases. Although temporal artery biopsy is an invasive procedure, the highly Ocular Immunology & Inflammation
Giant Cell Arteritis Presenting with Uveitis treatable nature of promptly diagnosed GCA and potentially devastating consequences of a delayed diagnosis cannot be denied. Awareness that posterior uveitis and scleritis may be the presenting findings of GCA should prompt clinicians to ask about other symptoms of GCA and consider obtaining sedimentation rate, C-reactive protein, and temporal artery biopsy in select patients.
Ocul Immunol Inflamm Downloaded from informahealthcare.com by University of Colorado Libraries on 12/28/14 For personal use only.
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.
!
2014 Informa Healthcare USA, Inc.
393
REFERENCES 1. Salvarani C, Cantini F, Boiardi L, et al. Polymyalgia rheumatica and giant-cell arteritis. N Engl J Med. 2002; 347:261–271. 2. Zhou L, Luneau K, Weyand CM, et al. Clinicopathologic correlations in giant cell arteritis: a retrospective study of 107 cases. Ophthalmology. 2009;116: 1574–1580. 3. Bandini F, Benedetti L, Ceppa P, et al. Uveitis as a presenting sign of giant cell arteritis. J Neuroophthalmol. 2005;25:247–248. 4. Dasgupta B, Pitzalis C, Panayi GS. Inflammation of the uveal tract as a presenting feature of temporal arteritis. Ann Rheum Dis. 1989;48:964–965. 5. Rajesh CV, Cole M. Panuveitis as a presenting feature of giant cell arteritis. Br J Ophthalmol. 2000;84:340.
