ISOLATED OPHTHALMOLOGIC RELAPSE IN A CASE OF SUSPECTED MICROSCOPIC POLYANGIITIS Luis Caminal-Montero,* Luis Trapiella-Martínez,* Jose´ B. Díaz-Lo´pez,* Marta Fonolla´-Gil†
Purpose: To report a case of suspected microscopic polyangiitis (MPA) with isolated retinal vasculitis not described previously. Methods: Case report. Results: A 50-year-old man was admitted to the hospital because of retinal vasculitis. The patient had been diagnosed with systemic vasculitis type MPA 8 years earlier. Cranial magnetic resonance imaging and transcranial Doppler ultrasonography did not reveal other vascular lesions. ANCA and MPO were detected, but results of systemic evaluation were normal. The patient was treated with intravenous methylprednisolone and cyclophosphamide followed by oral prednisone. Visual acuity improved rapidly, leading to discontinuation of corticosteroid therapy after 2 months. Conclusion: Retinal vasculitis can be a sign of systemic disease and, in this case, was the single manifestation of a relapse. In some cases of recurrent isolated retinal vasculitis, a short treatment course of immunosuppressive and systemic steroids can be enough for resolution. RETINAL CASES & BRIEF REPORTS 2:24 –26, 2008
From *Unidad de Enfermedades Autoinmunes Siste´micas, Servicio de Medicina Interna, and †Servicio de Oftalmologı´a, Hospital Universitario Central de Asturias, Oviedo, Spain.
of isolated retinal vasculitis after an 8-year follow-up period of clinical remission.
M
A 50-year-old man was admitted to the hospital because of headache, blurred vision, and a superior scotoma in his right eye in 2004. The patient had been diagnosed with systemic vasculitis 8 years earlier. He had developed central nervous system involvement, cardiomyopathy, hypertension, and renal failure, with normal funduscopic examination findings. Results of testing for antineutrophil antibodies were negative. Further laboratory evaluation revealed negative results of testing for antinuclear and anticardiolipin antibodies as well as negative results of tests for hepatitis C and hepatitis B viruses and human immunodeficiency virus. Otolaryngology consultation did not reveal abnormal findings, and no lung nodules were found by computed thoracic tomography. Renal percutaneous biopsy was performed, and only interstitial nephritis was found. Muscle biopsy showed nonspecific medium-sized arteritis, and visceral digital-subtraction arteriography disclosed microaneurysms in renal arteries consistent with vasculitis. After lung hemorrhage, lung biopsy was performed, and diffuse alveolitis without granuloma or vasculitis was shown by histologic examination. He received immunosuppressive therapy with intravenous highdose methylprednisolone and cyclophosphamide with improvement. Right hemiparesis and hypertension were the only sequelae.
Case Report
icroscopic polyangiitis (MPA) is a small-vessel necrotizing vasculitis characterized by the presence of perinuclear antineutrophil antibodies with antigen specificity to myeloperoxidase. It is differentiated from other small-vessel vasculitides by the absence of granulomatous inflammation, asthma, and eosinophilia. Capillaries, venules, and arterioles in any organ can be affected, a fact that results in a wide variety of signs and symptoms.1 Ocular manifestations are rare and include scleritis, conjunctivitis, peripheral ulcerative keratitis, conjunctival ulcerated nodules, central scotomas, hypopyon, iridocyclitis, and retinal vasculitis.2– 4 We describe a 50-year-old man with MPA who developed relapse Reprint requests: Luis Caminal-Montero, Servicio de Medicina Interna, Hospital Universitario Central de Asturias, Celestino Villamil, s/n, 33005 Oviedo, Spain; e-mail: [email protected]
24
ISOLATED OPHTHALMOLOGIC RELAPSE IN A CASE OF SUSPECTED MICROSCOPIC POLYANGIITIS
25
Fig. 1. Funduscopic examination showing retinal edema and cottonwool spots.
Fig. 3. Absence of retinal edema and cotton-wool spots after immunosuppressive therapy.
At the present admission, visual acuity was 20/32 in the right eye and 20/20 in the left eye; the cornea, anterior chamber, iris, and lens were normal. Intraocular pressure was 12 mmHg bilaterally. Fundus examination showed cotton-wool spots and retinal edema in the right eye (Fig. 1). Fluorescein angiography showed occlusion of the inferior temporal branch retinal artery with staining of the involved arterial segment (Fig. 2). Cranial magnetic resonance imaging and transcranial Doppler imaging did not reveal new vascular lesions. Perinuclear antineutrophil antibodies to myeloperoxidase were detected (7.63 U/mL [normal value, ⬍4 U/mL]), but results of systemic evaluation and routine hematologic and biochemical tests were normal. The patient was treated with intravenous methylprednisolone (1 g daily for 3 days) and cyclophosphamide (15 mg/kg), followed by a 2-month tapering course of oral prednisone. Visual acuity in the right eye improved to 20/25, and a new fluorescein angiogram showed arterial filling without staining (Figs. 3 and 4). During a 12-month follow-up period, no remarkable ocular signs were observed, and there were no other systemic vasculitis features.
Discussion
Fig. 2. Fluorescein angiography showing occlusion of the inferior temporal branch retinal artery with staining of the involved arterial segment
Accurate diagnosis of specific types of vasculitides can be problematic. Classic polyarteritis nodosa vasculitis was initially diagnosed for our patient. The current approach to subclassification of systemic vasculitis proposed that polyarteritis nodosa and MPA are differentiated by the presence or absence of smallvessel involvement.1 After lung hemorrhage and alveolar capillaritis that indicated some form of smallvessel vasculitis, we diagnosed our patient with MPA. MPA is categorized as perinuclear antineutrophil antibody–associated vasculitis, usually without microaneurysms. Nevertheless, up to 10% of patients with MPA are negative for antineutrophil antibodies, and microaneurysms are also rarely seen on visceral angiograms.1,5
Fig. 4. Fluorescein angiography showing arterial filling without staining after treatment.
26
RETINAL CASES & BRIEF REPORTSℜ
Visual loss is an ocular emergency, and the development of isolated retinal vasculitis in a patient with previous systemic vasculitis makes a diagnosis challenging. Retinal vasculitis may be associated with a wide variety of infections and systemic inflammatory diseases. When the arteries are predominantly involved, as in our case, systemic lupus erythematosus, polyarteritis nodosa, Churg–Strauss syndrome, infection with herpes simplex virus or varicella-zoster virus, and syphilis are the primary entities to consider.6 The treatment of immune retinal vasculitis depends on the severity of the sight-threatening intraocular inflammation.2 The rapid improvement with immunosuppressive therapy without evidence of other systemic disease led us to discontinue administration of cyclophosphamide and corticosteroids in 2 months. Retinal vasculitis can be a sign of systemic disease, but in the case reported here, it was the single manifestation of a relapse. To our knowledge, this is the
●
2008
●
VOLUME 2
●
NUMBER 1
first report of relapse of isolated retinal vasculitis in a patient with MPA. Key words: microscopic polyangiitis, systemic vasculitis, ocular manifestations. References 1. 2. 3. 4.
5.
6.
Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med 1997;337:1512–1523. McCluskey P, Powell RJ. The eye in systemic inflammatory diseases. Lancet 2004;364:2125–2133. Herbort CP, Cimino L, Abu E, Asrar AM. Ocular vasculitis: a multidisciplinary approach. Curr Opin Rheumatol 2005;17:25–33. Mihara M, Hayasaka S, Watanabe K, et al. Ocular manifestations in patients with microscopic polyangiitis. Eur J Ophthalmol 2004;15:138–142. Lhote F, Cohen P, Genereau T, et al. Microscopic polyangiitis: clinical aspects and treatment. Ann Med Interne (Paris) 1996; 147:165–177. Kathiresan S, Kelsey PB, Steere AC, et al. Case14-2005: a 38-year-old man with fever and blurred vision. N Engl J Med 2005;352:2003–2012.
Purpose: To report a case of suspected microscopic polyangiitis (MPA) with isolated retinal vasculitis not described previously. Methods: Case report. Results: A 50-year-old man was admitted to the hospital because of retinal vasculitis. The patient had been diagnosed with systemic vasculitis type MPA 8 years earlier. Cranial magnetic resonance imaging and transcranial Doppler ultrasonography did not reveal other vascular lesions. ANCA and MPO were detected, but results of systemic evaluation were normal. The patient was treated with intravenous methylprednisolone and cyclophosphamide followed by oral prednisone. Visual acuity improved rapidly, leading to discontinuation of corticosteroid therapy after 2 months. Conclusion: Retinal vasculitis can be a sign of systemic disease and, in this case, was the single manifestation of a relapse. In some cases of recurrent isolated retinal vasculitis, a short treatment course of immunosuppressive and systemic steroids can be enough for resolution. RETINAL CASES & BRIEF REPORTS 2:24 –26, 2008
From *Unidad de Enfermedades Autoinmunes Siste´micas, Servicio de Medicina Interna, and †Servicio de Oftalmologı´a, Hospital Universitario Central de Asturias, Oviedo, Spain.
of isolated retinal vasculitis after an 8-year follow-up period of clinical remission.
M
A 50-year-old man was admitted to the hospital because of headache, blurred vision, and a superior scotoma in his right eye in 2004. The patient had been diagnosed with systemic vasculitis 8 years earlier. He had developed central nervous system involvement, cardiomyopathy, hypertension, and renal failure, with normal funduscopic examination findings. Results of testing for antineutrophil antibodies were negative. Further laboratory evaluation revealed negative results of testing for antinuclear and anticardiolipin antibodies as well as negative results of tests for hepatitis C and hepatitis B viruses and human immunodeficiency virus. Otolaryngology consultation did not reveal abnormal findings, and no lung nodules were found by computed thoracic tomography. Renal percutaneous biopsy was performed, and only interstitial nephritis was found. Muscle biopsy showed nonspecific medium-sized arteritis, and visceral digital-subtraction arteriography disclosed microaneurysms in renal arteries consistent with vasculitis. After lung hemorrhage, lung biopsy was performed, and diffuse alveolitis without granuloma or vasculitis was shown by histologic examination. He received immunosuppressive therapy with intravenous highdose methylprednisolone and cyclophosphamide with improvement. Right hemiparesis and hypertension were the only sequelae.
Case Report
icroscopic polyangiitis (MPA) is a small-vessel necrotizing vasculitis characterized by the presence of perinuclear antineutrophil antibodies with antigen specificity to myeloperoxidase. It is differentiated from other small-vessel vasculitides by the absence of granulomatous inflammation, asthma, and eosinophilia. Capillaries, venules, and arterioles in any organ can be affected, a fact that results in a wide variety of signs and symptoms.1 Ocular manifestations are rare and include scleritis, conjunctivitis, peripheral ulcerative keratitis, conjunctival ulcerated nodules, central scotomas, hypopyon, iridocyclitis, and retinal vasculitis.2– 4 We describe a 50-year-old man with MPA who developed relapse Reprint requests: Luis Caminal-Montero, Servicio de Medicina Interna, Hospital Universitario Central de Asturias, Celestino Villamil, s/n, 33005 Oviedo, Spain; e-mail: [email protected]
24
ISOLATED OPHTHALMOLOGIC RELAPSE IN A CASE OF SUSPECTED MICROSCOPIC POLYANGIITIS
25
Fig. 1. Funduscopic examination showing retinal edema and cottonwool spots.
Fig. 3. Absence of retinal edema and cotton-wool spots after immunosuppressive therapy.
At the present admission, visual acuity was 20/32 in the right eye and 20/20 in the left eye; the cornea, anterior chamber, iris, and lens were normal. Intraocular pressure was 12 mmHg bilaterally. Fundus examination showed cotton-wool spots and retinal edema in the right eye (Fig. 1). Fluorescein angiography showed occlusion of the inferior temporal branch retinal artery with staining of the involved arterial segment (Fig. 2). Cranial magnetic resonance imaging and transcranial Doppler imaging did not reveal new vascular lesions. Perinuclear antineutrophil antibodies to myeloperoxidase were detected (7.63 U/mL [normal value, ⬍4 U/mL]), but results of systemic evaluation and routine hematologic and biochemical tests were normal. The patient was treated with intravenous methylprednisolone (1 g daily for 3 days) and cyclophosphamide (15 mg/kg), followed by a 2-month tapering course of oral prednisone. Visual acuity in the right eye improved to 20/25, and a new fluorescein angiogram showed arterial filling without staining (Figs. 3 and 4). During a 12-month follow-up period, no remarkable ocular signs were observed, and there were no other systemic vasculitis features.
Discussion
Fig. 2. Fluorescein angiography showing occlusion of the inferior temporal branch retinal artery with staining of the involved arterial segment
Accurate diagnosis of specific types of vasculitides can be problematic. Classic polyarteritis nodosa vasculitis was initially diagnosed for our patient. The current approach to subclassification of systemic vasculitis proposed that polyarteritis nodosa and MPA are differentiated by the presence or absence of smallvessel involvement.1 After lung hemorrhage and alveolar capillaritis that indicated some form of smallvessel vasculitis, we diagnosed our patient with MPA. MPA is categorized as perinuclear antineutrophil antibody–associated vasculitis, usually without microaneurysms. Nevertheless, up to 10% of patients with MPA are negative for antineutrophil antibodies, and microaneurysms are also rarely seen on visceral angiograms.1,5
Fig. 4. Fluorescein angiography showing arterial filling without staining after treatment.
26
RETINAL CASES & BRIEF REPORTSℜ
Visual loss is an ocular emergency, and the development of isolated retinal vasculitis in a patient with previous systemic vasculitis makes a diagnosis challenging. Retinal vasculitis may be associated with a wide variety of infections and systemic inflammatory diseases. When the arteries are predominantly involved, as in our case, systemic lupus erythematosus, polyarteritis nodosa, Churg–Strauss syndrome, infection with herpes simplex virus or varicella-zoster virus, and syphilis are the primary entities to consider.6 The treatment of immune retinal vasculitis depends on the severity of the sight-threatening intraocular inflammation.2 The rapid improvement with immunosuppressive therapy without evidence of other systemic disease led us to discontinue administration of cyclophosphamide and corticosteroids in 2 months. Retinal vasculitis can be a sign of systemic disease, but in the case reported here, it was the single manifestation of a relapse. To our knowledge, this is the
●
2008
●
VOLUME 2
●
NUMBER 1
first report of relapse of isolated retinal vasculitis in a patient with MPA. Key words: microscopic polyangiitis, systemic vasculitis, ocular manifestations. References 1. 2. 3. 4.
5.
6.
Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med 1997;337:1512–1523. McCluskey P, Powell RJ. The eye in systemic inflammatory diseases. Lancet 2004;364:2125–2133. Herbort CP, Cimino L, Abu E, Asrar AM. Ocular vasculitis: a multidisciplinary approach. Curr Opin Rheumatol 2005;17:25–33. Mihara M, Hayasaka S, Watanabe K, et al. Ocular manifestations in patients with microscopic polyangiitis. Eur J Ophthalmol 2004;15:138–142. Lhote F, Cohen P, Genereau T, et al. Microscopic polyangiitis: clinical aspects and treatment. Ann Med Interne (Paris) 1996; 147:165–177. Kathiresan S, Kelsey PB, Steere AC, et al. Case14-2005: a 38-year-old man with fever and blurred vision. N Engl J Med 2005;352:2003–2012.
