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Wegener's granulomatosis: a diagnostic dilemma. Case presentation
CASE REPORT
Wegener's granulomatosis: a diagnostic dilemma. Case presentation Peter T. Huang,* MD, FRCSC; Jamie Bhamra,:j: BSc; John T. Huang, t MD, FRCSC
W
egener' s granulomatosis is a rare autoimmune and vasculitic disease characterized by endo thelial inflammation of the blood vessel wall. Vascu litic changes primarily involve small-calibre vessels of the arterial system. In most cases the lungs, kidneys, and upper and lower respiratory tract are involved. Glomerulonephritis, necrotizing granulomas in the lungs and generalized focal necrotizing vasculitis may all be established in the disease. 1 It is this focal necro tizing vasculitis that leads to other systemic problems, such as skin, joint and ocular involvement. However, any organ system may be involved. Initial symptoms may be obscure and may vary from one patient to another. Most patients experience respi ratory tract symptoms, joint pain, weakness and fatigue. 2 Yet the symptoms are usually vague and non specific. Elevation of the titre of antineutrophil cyto plasmic antibody is helpful in the diagnosis. 3 Its sensi tivity ranges from 50% during the onset of the disease to 100% in the generalized phase. 3 We describe a patient with an extensive history of progressive inflammatory autoimmune disease local ized primarily to the face and head. Internal medicine specialists, otolaryngologists, dentists, oral surgeons, rheumatologists, family physicians and ophthalmolo
*Department Head and tDirector of Undergraduate Medical Edu cation, Division of Ophthalmology, Faculty of Medicine, University of Calgary, Calgary, Alta. *Senior medical student, University of Calgary, Calgary, Alta. Originally received July 23, 2002 Accepted for publication Nov. 14, 2002 Reprint requests to: Dr. John T. Huang, Bow River Medical Specialists, Suite 313, 4411 16th Ave. NW, Calgary AB T3B OM3; fax (403) 286-3316; [email protected] This article has been peer-reviewed. Can J Ophthalmol 2003;38:63--6
Wegener's granulomatosis-Huang et al
gists all played significant roles over the course of his disease, leading to the diagnosis of Wegener's granu lomatosis. The focus of this report is the unusual ocu lar presentation. CASE REPORT
A 61-year-old white man, a former high school teacher, presented with left otalgia and otorrhea, which was treated with betamethasone-gentamicin drops and orally administered cloxacillin. Five months later he experienced right maxillofacial pain, which was treated with dental extraction and oral antibiotic therapy. Seven months after the initial onset of symptoms he presented with fever, facial pain and blurred vision. He reported decreased distant visual acuity bilaterally over the preceding 9 days. He had no significant histo ry of ocular surgery, manifestations or disease. Vitritis and uveitis were found bilaterally. His medications at presentation included clindamycin, used over the prior month for recurring oral infections (inflammation, pain and swelling), and rofecoxib, used for the previ ous 3 to 4 weeks for tendonitis. His visual acuity was 20/60-2 (refraction -2.00 +0.5 x 9) in the right eye and 20/50-2 (refraction plano +0.50 X 180) in the left eye. The intraocular pressure (IOP) was 16 mm Hg in the right eye and 20 mm Hg in the left. Given the patient's history, topical daily latanoprost and prednisolone therapy were prescribed. Serologic testing showed antibodies to rubella IgG and measles IgG. Antibodies to rubella and measles IgM and to par vovirus IgG and IgM were not detected. Fluorescein angiography demonstrated bilateral cys toid macular edema (Fig. 1). No obvious epiretinal membrane was noted to suggest a tractional compo nent to the macular edema. The perifoveal vasculature appeared somewhat irregular and had a telangiectatic appearance, which suggested that perifoveal telangiec tasia accounted for the macular edema.
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Fig. 1-Fluorescein angiograms of right eye of patient with Wegener's granulomatosis. Left: Early phase. Perifoveal vasculature is irregular and telangiectatic. Right: Late phase. Late leakage demonstrates cystoid macular edema.
On repeat examination the patient reported further sub jective improvement in his vision, but the visual acuity was measured at 20/80-1 in the right eye and 20/40-2 in the left. Idiopathic perifoveal telangiectasia was ob served, confirming the angiographic findings (Fig. 2). A course of oral prednisone therapy was started, beginning with 50 mg/d for 1 week and tapering by 10 mg each week thereafter to a target dosage of 10 mg/d. The daily latanoprost and prednisolone therapy were continued. Three weeks later the retinal service received the patient and reported that the cystoid macular edema had responded to the steroid therapy. The vision had improved to about 20/20 bilaterally. Trace anterior uveitis and vitritis were present in both eyes. Of note, a new cotton-wool spot was identified in the left infero nasal macula.
The following week the visual acuity was 20/30-2 bilaterally. The IOP was controlled (19 mm Hg and 17 mm Hg in the right and left eyes respectively). The course of prednisone therapy was complete. The patient continued with latanoprost and prednisolone eye drops daily. Two weeks later the visual acuity had improved to 20/20 in the right eye and 20/20-1 in the left. The IOP values were 13 mm Hg and 12 mm Hg respectively. The latanoprost drops were stopped, but the pred nisolone drops were continued daily. However, the patient's systemic problems had worsened, and he was admitted to hospital. He reported ocular discomfort. A left facial mass had appeared, and he had severe diffi culty opening his jaw and had lost a considerable amount of weight. Biopsy of the mass showed only
Fig. 2-Fundus photographs of right eye (left) and left eye (right), showing blunt foveal reflexes secondary to cystoid macular edema.
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Wegener's granulomatosis-Huang et al nonspecific granulomatous changes. Plasmocytosis with monoclonal gammopathy of unknown signifi cance, anemia and an elevated titre of antinuclear anti body were also present. The otolaryngology service performed bilateral intranasal antrostomy for chronic sinusitis. Tissue biopsy showed a plasmacytic infil trate. The medication regimen now included cefurox ime axetil (500 mg twice daily), metronidazole (two tablets twice daily), prednisone (60 mg/d) and cele coxib (200 mg/d). The visual acuity remained stable, at 20/25+ 1 in the right eye and 20/20-2 in the left eye, as did the IOP. The patient was seen by a rheumatolo gist, but no definitive diagnosis was made. After discharge, the patient was readmitted for steroid-induced psychosis and mania. Diplopia had developed, and bilateral sixth nerve palsy and upward gaze palsy were noted. The visual acuity and IOP remained stable. Laboratory studies during the clinical course showed monoclonal peaks and a markedly increased erythrocyte sedimentation rate. The patient was again seen by the rheumatologist, who now confirmed that the presentation was consis tent with Wegener' s granulomatosis. About 8 months after initial presentation to the oph thalmologist, reevaluation showed subjective and ob jective impairment in vision. Prednisolone eye drops, cyclophosphamide (125 mg) and prednisone (10 mg given orally) were used daily. The IOP remained nor mal (16 mm Hg in the right eye and 18 mm Hg in the left). At the most recent examination the patient's visual acuity was 20/40-1 in the right eye and 20/30 in the left eye. COMMENTS
The fact that Wegener' s granulomatosis can present in many different ways makes it a diagnostic dilemma. In many cases the patient must be followed for 5 to 15 months before the diagnosis can be established. One of the key indications of the disease is ocular involve ment, which occurs in at least 50% of patients. I .4 The type of vasculitis that occurs usually involves the small to medium-sized arteries of the head and neck. 5 Eye involvement may be the only presentation of Wegener's granulomatosis. Alternatively, it may be the initial presentation, followed by systemic disease, or ocular problems may present after the systemic manifestations are clearly established. In our patient ocular manifestations became apparent about 10 months after the onset of systemic conditions. The
diagnosis was made after ophthalmologic problems pre sented. Many different types of ocular manifestation may occur in Wegener's granulomatosis, involving both the eye and the orbit. Scleritis, episcleritis, corneal and scleral ulceration, optic neuritis, retinal artery occlu sion, retinal edema, glaucoma, keratitis, uveitis, con junctivitis, subconjunctival hemorrhage and proptosis due to cellulitis or orbital pseudotumour have all been described. 1•6•7 Vasculitis-mediated damage also can affect the cranial nerves, may lead to nasolacrimal duct obstruction or may result in retinal detachment. 3·8·9 Although rare, optic disc vasculitis, retinal vasculitis and cotton-wool spots with or without intraretinal hem orrhages have also been reported. 10 It is possible that these ocular manifestations present with different types of vasculitis as well, yet the incidence in Wegener' s granulomatosis is higher owing to frequent involve ment of the arteries of the head and neck. 10 The most common ocular condition varies from study to study. Harper and colleagues' reported that scleritis was the most frequent ocular finding, where as Bullen and associates 6 found that orbital disease and conjunctivitis were most prevalent. Our patient presented with bilateral anterior uveitis and vitritis. Uveitis must be treated quickly since it can lead to other, sight-threatening complications, such as glauco ma and retinal edema, and can be associated with optic neuropathy. 7 Our patient's medical problems were consistent with Wegener' s granulomatosis. He had a variety of systemic illnesses over a period of 10 months before the onset of blurred vision. It was not until 6 months into treatment of the ophthalmic problems that the definitive diagnosis was made. Fluorescein angiography, performed in our patient to rule out the possibility of macular disease, showed retinal swelling, macular edema and cotton-wool spots. Possible causes of the edema included pseu dophakia, uveitis or the recent administration of rofe coxib. The findings also correlated with the ocular manifestations of Wegener's granulomatosis. During the course of the disease the patient mani fested diplopia, bilateral sixth nerve palsy and vertical gaze palsy. Gaze palsy also correlates with the ocular conditions of Wegener's granulomatosis, owing to vasculitis-mediated damage of the cranial nerves. 8 Other abnormalities, including weight loss, facial growths, jaw pain and sinusitis, complicated the patient's course and diagnosis. Many of the findings were consistent with the subacute clinical presentation
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Wegener's granulomatosis-Huang et al of Wegener' s granulomatosis in the head and neck described by D'Cruz and coworkers. 11 The ocular conditions in our patient were success fully treated with topical prednisone therapy and oral prednisolone and latanoprost therapy. The visual acu ity remained very functional with this therapeutic reg imen. In conclusion, we describe a patient presenting with a combination of nonspecific and clinically significant symptoms. The diagnosis was established only after the appearance of ocular manifestations indicative of an autoimmune, inflammatory etiology and extensive investigation. We feel that it is appropriate to consider the diagnosis of Wegener' s granulomatosis in patients with unusual ocular inflammation. 12 ~ REFERENCES
1. Harper SL, Letko E, Samson CM, Zafirakis P, Sangwan V, Nguyen Q, et al. Wegener's granulomatosis: the relation ship between ocular and systemic disease. J Rheumatol 2001;28(5): 1025-32. 2. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener's granulomatosis: an analysis of 158 patients. Ann Intern Med 1992;116: 488-98. 3. Borruat FX. Neuro-ophthalmic manifestation of rheumato logic and associated disorders. Curr Opin Ophthalmol 1996;7:10-8.
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4. Haynes BF, Fishman ML, Fauci AS, Wolff SM. The ocu lar manifestations of Wegener's granulomatosis. Fifteen years experience and review of the literature. Am. J Med 1977;63:131-41. 5. Fauci AS, Haynes BF, Katz P. Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983;98(1):76-85. 6. Bullen CL, Liesegang TJ, McDonald TJ, DeRemee RA. Ocular complications of Wegener's granulomatosis. Oph thalmology 1983;90:279-90. 7. Kanski JJ. Clinical ophthalmology. 4th ed. New Delhi: Reed Educational and Professional Publishing; 1999. p. 94--151. 8. Jabs DA. The rheumatic diseases. In: Ryan S, editor. The retina. Vol II. St. Louis: CV Mosby; 1989. p. 457-80. 9. Hardwig PW, Bartley GB, Garrity JA. Surgical manage ment of nasolacrimal duct obstruction in patients with Wegener's granulomatosis. Ophthalmology 1992;99: 133-9. 10. Hamideh F, Prete PE. Ophthalmic manifestations of rheumatic diseases. Semin Arthritis Rheum 2001 ;30: 217-41. 11. D'Cruz DP, Baguley E, Asherson RA, Hughes GRV. Ear, nose, and throat symptoms in subacute Wegener's granu lomatosis. BMJ l 989;299:419-22. 12. Hole I, Pahor A, Krajnc I. Wegener's granulomatosis - a diagnostic challenge. Wien Klin Wochenschr 2000; 112: 680-3.
Key words: Wegener's granulomatosis, retina, palsy, multisys tem disease
Wegener's granulomatosis: a diagnostic dilemma. Case presentation Peter T. Huang,* MD, FRCSC; Jamie Bhamra,:j: BSc; John T. Huang, t MD, FRCSC
W
egener' s granulomatosis is a rare autoimmune and vasculitic disease characterized by endo thelial inflammation of the blood vessel wall. Vascu litic changes primarily involve small-calibre vessels of the arterial system. In most cases the lungs, kidneys, and upper and lower respiratory tract are involved. Glomerulonephritis, necrotizing granulomas in the lungs and generalized focal necrotizing vasculitis may all be established in the disease. 1 It is this focal necro tizing vasculitis that leads to other systemic problems, such as skin, joint and ocular involvement. However, any organ system may be involved. Initial symptoms may be obscure and may vary from one patient to another. Most patients experience respi ratory tract symptoms, joint pain, weakness and fatigue. 2 Yet the symptoms are usually vague and non specific. Elevation of the titre of antineutrophil cyto plasmic antibody is helpful in the diagnosis. 3 Its sensi tivity ranges from 50% during the onset of the disease to 100% in the generalized phase. 3 We describe a patient with an extensive history of progressive inflammatory autoimmune disease local ized primarily to the face and head. Internal medicine specialists, otolaryngologists, dentists, oral surgeons, rheumatologists, family physicians and ophthalmolo
*Department Head and tDirector of Undergraduate Medical Edu cation, Division of Ophthalmology, Faculty of Medicine, University of Calgary, Calgary, Alta. *Senior medical student, University of Calgary, Calgary, Alta. Originally received July 23, 2002 Accepted for publication Nov. 14, 2002 Reprint requests to: Dr. John T. Huang, Bow River Medical Specialists, Suite 313, 4411 16th Ave. NW, Calgary AB T3B OM3; fax (403) 286-3316; [email protected] This article has been peer-reviewed. Can J Ophthalmol 2003;38:63--6
Wegener's granulomatosis-Huang et al
gists all played significant roles over the course of his disease, leading to the diagnosis of Wegener's granu lomatosis. The focus of this report is the unusual ocu lar presentation. CASE REPORT
A 61-year-old white man, a former high school teacher, presented with left otalgia and otorrhea, which was treated with betamethasone-gentamicin drops and orally administered cloxacillin. Five months later he experienced right maxillofacial pain, which was treated with dental extraction and oral antibiotic therapy. Seven months after the initial onset of symptoms he presented with fever, facial pain and blurred vision. He reported decreased distant visual acuity bilaterally over the preceding 9 days. He had no significant histo ry of ocular surgery, manifestations or disease. Vitritis and uveitis were found bilaterally. His medications at presentation included clindamycin, used over the prior month for recurring oral infections (inflammation, pain and swelling), and rofecoxib, used for the previ ous 3 to 4 weeks for tendonitis. His visual acuity was 20/60-2 (refraction -2.00 +0.5 x 9) in the right eye and 20/50-2 (refraction plano +0.50 X 180) in the left eye. The intraocular pressure (IOP) was 16 mm Hg in the right eye and 20 mm Hg in the left. Given the patient's history, topical daily latanoprost and prednisolone therapy were prescribed. Serologic testing showed antibodies to rubella IgG and measles IgG. Antibodies to rubella and measles IgM and to par vovirus IgG and IgM were not detected. Fluorescein angiography demonstrated bilateral cys toid macular edema (Fig. 1). No obvious epiretinal membrane was noted to suggest a tractional compo nent to the macular edema. The perifoveal vasculature appeared somewhat irregular and had a telangiectatic appearance, which suggested that perifoveal telangiec tasia accounted for the macular edema.
63
Wegener's granulomatosis-Huang et al
Fig. 1-Fluorescein angiograms of right eye of patient with Wegener's granulomatosis. Left: Early phase. Perifoveal vasculature is irregular and telangiectatic. Right: Late phase. Late leakage demonstrates cystoid macular edema.
On repeat examination the patient reported further sub jective improvement in his vision, but the visual acuity was measured at 20/80-1 in the right eye and 20/40-2 in the left. Idiopathic perifoveal telangiectasia was ob served, confirming the angiographic findings (Fig. 2). A course of oral prednisone therapy was started, beginning with 50 mg/d for 1 week and tapering by 10 mg each week thereafter to a target dosage of 10 mg/d. The daily latanoprost and prednisolone therapy were continued. Three weeks later the retinal service received the patient and reported that the cystoid macular edema had responded to the steroid therapy. The vision had improved to about 20/20 bilaterally. Trace anterior uveitis and vitritis were present in both eyes. Of note, a new cotton-wool spot was identified in the left infero nasal macula.
The following week the visual acuity was 20/30-2 bilaterally. The IOP was controlled (19 mm Hg and 17 mm Hg in the right and left eyes respectively). The course of prednisone therapy was complete. The patient continued with latanoprost and prednisolone eye drops daily. Two weeks later the visual acuity had improved to 20/20 in the right eye and 20/20-1 in the left. The IOP values were 13 mm Hg and 12 mm Hg respectively. The latanoprost drops were stopped, but the pred nisolone drops were continued daily. However, the patient's systemic problems had worsened, and he was admitted to hospital. He reported ocular discomfort. A left facial mass had appeared, and he had severe diffi culty opening his jaw and had lost a considerable amount of weight. Biopsy of the mass showed only
Fig. 2-Fundus photographs of right eye (left) and left eye (right), showing blunt foveal reflexes secondary to cystoid macular edema.
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Wegener's granulomatosis-Huang et al nonspecific granulomatous changes. Plasmocytosis with monoclonal gammopathy of unknown signifi cance, anemia and an elevated titre of antinuclear anti body were also present. The otolaryngology service performed bilateral intranasal antrostomy for chronic sinusitis. Tissue biopsy showed a plasmacytic infil trate. The medication regimen now included cefurox ime axetil (500 mg twice daily), metronidazole (two tablets twice daily), prednisone (60 mg/d) and cele coxib (200 mg/d). The visual acuity remained stable, at 20/25+ 1 in the right eye and 20/20-2 in the left eye, as did the IOP. The patient was seen by a rheumatolo gist, but no definitive diagnosis was made. After discharge, the patient was readmitted for steroid-induced psychosis and mania. Diplopia had developed, and bilateral sixth nerve palsy and upward gaze palsy were noted. The visual acuity and IOP remained stable. Laboratory studies during the clinical course showed monoclonal peaks and a markedly increased erythrocyte sedimentation rate. The patient was again seen by the rheumatologist, who now confirmed that the presentation was consis tent with Wegener' s granulomatosis. About 8 months after initial presentation to the oph thalmologist, reevaluation showed subjective and ob jective impairment in vision. Prednisolone eye drops, cyclophosphamide (125 mg) and prednisone (10 mg given orally) were used daily. The IOP remained nor mal (16 mm Hg in the right eye and 18 mm Hg in the left). At the most recent examination the patient's visual acuity was 20/40-1 in the right eye and 20/30 in the left eye. COMMENTS
The fact that Wegener' s granulomatosis can present in many different ways makes it a diagnostic dilemma. In many cases the patient must be followed for 5 to 15 months before the diagnosis can be established. One of the key indications of the disease is ocular involve ment, which occurs in at least 50% of patients. I .4 The type of vasculitis that occurs usually involves the small to medium-sized arteries of the head and neck. 5 Eye involvement may be the only presentation of Wegener's granulomatosis. Alternatively, it may be the initial presentation, followed by systemic disease, or ocular problems may present after the systemic manifestations are clearly established. In our patient ocular manifestations became apparent about 10 months after the onset of systemic conditions. The
diagnosis was made after ophthalmologic problems pre sented. Many different types of ocular manifestation may occur in Wegener's granulomatosis, involving both the eye and the orbit. Scleritis, episcleritis, corneal and scleral ulceration, optic neuritis, retinal artery occlu sion, retinal edema, glaucoma, keratitis, uveitis, con junctivitis, subconjunctival hemorrhage and proptosis due to cellulitis or orbital pseudotumour have all been described. 1•6•7 Vasculitis-mediated damage also can affect the cranial nerves, may lead to nasolacrimal duct obstruction or may result in retinal detachment. 3·8·9 Although rare, optic disc vasculitis, retinal vasculitis and cotton-wool spots with or without intraretinal hem orrhages have also been reported. 10 It is possible that these ocular manifestations present with different types of vasculitis as well, yet the incidence in Wegener' s granulomatosis is higher owing to frequent involve ment of the arteries of the head and neck. 10 The most common ocular condition varies from study to study. Harper and colleagues' reported that scleritis was the most frequent ocular finding, where as Bullen and associates 6 found that orbital disease and conjunctivitis were most prevalent. Our patient presented with bilateral anterior uveitis and vitritis. Uveitis must be treated quickly since it can lead to other, sight-threatening complications, such as glauco ma and retinal edema, and can be associated with optic neuropathy. 7 Our patient's medical problems were consistent with Wegener' s granulomatosis. He had a variety of systemic illnesses over a period of 10 months before the onset of blurred vision. It was not until 6 months into treatment of the ophthalmic problems that the definitive diagnosis was made. Fluorescein angiography, performed in our patient to rule out the possibility of macular disease, showed retinal swelling, macular edema and cotton-wool spots. Possible causes of the edema included pseu dophakia, uveitis or the recent administration of rofe coxib. The findings also correlated with the ocular manifestations of Wegener's granulomatosis. During the course of the disease the patient mani fested diplopia, bilateral sixth nerve palsy and vertical gaze palsy. Gaze palsy also correlates with the ocular conditions of Wegener's granulomatosis, owing to vasculitis-mediated damage of the cranial nerves. 8 Other abnormalities, including weight loss, facial growths, jaw pain and sinusitis, complicated the patient's course and diagnosis. Many of the findings were consistent with the subacute clinical presentation
CAN J OPHTHALMOL-VOL. 38, NO. I, 2003
65
Wegener's granulomatosis-Huang et al of Wegener' s granulomatosis in the head and neck described by D'Cruz and coworkers. 11 The ocular conditions in our patient were success fully treated with topical prednisone therapy and oral prednisolone and latanoprost therapy. The visual acu ity remained very functional with this therapeutic reg imen. In conclusion, we describe a patient presenting with a combination of nonspecific and clinically significant symptoms. The diagnosis was established only after the appearance of ocular manifestations indicative of an autoimmune, inflammatory etiology and extensive investigation. We feel that it is appropriate to consider the diagnosis of Wegener' s granulomatosis in patients with unusual ocular inflammation. 12 ~ REFERENCES
1. Harper SL, Letko E, Samson CM, Zafirakis P, Sangwan V, Nguyen Q, et al. Wegener's granulomatosis: the relation ship between ocular and systemic disease. J Rheumatol 2001;28(5): 1025-32. 2. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener's granulomatosis: an analysis of 158 patients. Ann Intern Med 1992;116: 488-98. 3. Borruat FX. Neuro-ophthalmic manifestation of rheumato logic and associated disorders. Curr Opin Ophthalmol 1996;7:10-8.
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4. Haynes BF, Fishman ML, Fauci AS, Wolff SM. The ocu lar manifestations of Wegener's granulomatosis. Fifteen years experience and review of the literature. Am. J Med 1977;63:131-41. 5. Fauci AS, Haynes BF, Katz P. Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983;98(1):76-85. 6. Bullen CL, Liesegang TJ, McDonald TJ, DeRemee RA. Ocular complications of Wegener's granulomatosis. Oph thalmology 1983;90:279-90. 7. Kanski JJ. Clinical ophthalmology. 4th ed. New Delhi: Reed Educational and Professional Publishing; 1999. p. 94--151. 8. Jabs DA. The rheumatic diseases. In: Ryan S, editor. The retina. Vol II. St. Louis: CV Mosby; 1989. p. 457-80. 9. Hardwig PW, Bartley GB, Garrity JA. Surgical manage ment of nasolacrimal duct obstruction in patients with Wegener's granulomatosis. Ophthalmology 1992;99: 133-9. 10. Hamideh F, Prete PE. Ophthalmic manifestations of rheumatic diseases. Semin Arthritis Rheum 2001 ;30: 217-41. 11. D'Cruz DP, Baguley E, Asherson RA, Hughes GRV. Ear, nose, and throat symptoms in subacute Wegener's granu lomatosis. BMJ l 989;299:419-22. 12. Hole I, Pahor A, Krajnc I. Wegener's granulomatosis - a diagnostic challenge. Wien Klin Wochenschr 2000; 112: 680-3.
Key words: Wegener's granulomatosis, retina, palsy, multisys tem disease