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Exudative Retinal Detachment and Scleritis in Polyarteritis
EXUDATIVE RETINAL D E T A C H M E N T AND SCLERITIS IN POLYARTERITIS R I C H A R D A. K I E L A R , Lexington,
Scleritis associated with polyarteritis is uncommon. There are only three reported cases of scleritis with exudative retinal detachment associated with polyarteritis in which the globes have been examined histologically. 1 - 3 This case is reported because of its rarity. CASE REPORT A 64-year-old white man was first seen in the medical clinic April 1968 because of a 13.5-kg (30 lb) weight loss and leg pain. General physical exam ination was unremarkable. Blood pressure was 110/ 70 mm Hg. Near visual acuity was reported "normal and equal" in each eye, as was external ocular examination and ophthalmoscopy. Laboratory ex amination revealed a white blood cell count of 14,000/mm 3 with a normal differential count, mild microcytic hypochromic anemia, and microscopic hematuria. Chest x-ray film, upper gastrointestinal series, barium enema, intravenous pyelogram, and blood urea nitrogen tests were normal. A gastrocnemius muscle biopsy was performed and inflammation of medium and small arteries was present on histologie examination (Fig. 1). A diagno sis of polyarteritis was made, and he was placed on 40 mg of prednisone every other day, gradually decreased to 10 mg every other day over the next eight months, and this treatment was continued until August 1973. During that time he felt better and gained weight. Sedimentation rates varied be tween 17 and 51 g/100 ml, and repeat urinalyses showed microscopic hematuria. Blood pressure re mained normal. He was first seen in the ophthalmology clinic in August 1973, with a history of a painful red right eye with loss of vision over the past several months. Visual acuity was R.E.: light perception, and L.E.: 6/6 (20/20). Scleritis was present temporally, ap peared to be deep, and extended from the corneoscleral limbus to 8 mm posteriorly. Moderate flare and cells were present in the anterior chamber. Intraocular pressures were 13 mm Hg in the right eye, and 18 mm Hg in the left eye. Gonioscopy revealed open angles. A total retinal detachment with shifting subretinal fluid without holes was present in the right eye. Examination of the left eye From the Department of Ophthalmology, Univer sity of Kentucky Medical Center, Lexington, Ken tucky. Reprint requests to Richard A. Kielar, M.D., De partment of Ophthalmology, University of Kentucky Medical Center, Lexington, KY 40506.
M.D.
Kentucky was normal. A diagnosis of scleritis with exudative retinal detachment was made, and the eye was treated with a retrobulbar corticosteroid injection and 40 mg of prednisone daily for ten days, with little change in the ocular findings. The patient did not return until March 1974 when right visual acuity was no light perception, with marked flare and cell, posterior synechiae, and scierai inflammation and thinning. A cataract obscured the fundus. He had returned to his previous prednisone dose of 10 mg every other day during this seven-month interval, and had developed no new systemic findings since his original physical examination in April 1968. Due to persistent pain in this blind eye, his eye was enucleated in September 1974. RESULTS
Gross examination—The eye was fixed in formalin. External abnormalities were limited to a slight haziness of the cornea and an oval area of scierai thinning ex tending temporally from the corneoscleral limbus to 5 mm posterior to the corneoscleral limbus. The globe was opened in a horizontal plane. The retina was detached from the ora serrata to the equator by a white translucent homoge nous fluid. A similar detachment was pres ent in the posterior pole. The peripheral choroid appeared slightly thickened. No other abnormalities were noted. Microscopic—The eye was imbedded in paraffin and multiple sections were stained with hematoxylin and eosin. A superficial vascular pannus with numer ous chronic round cells was present in the cornea. There were scattered polymorphonuclear leukocytes throughout the stroma, most marked at the corneoscleral limbus, and midstromal blood vessels with perivascular round cells. The anteri or sciera was involved with a granulomatous inflammation consisting of large areas of scierai necrosis, chronic round cells, epithelioid cells, and scattered giant cells. Marked destruction of scierai fibers extended from the corneoscleral limbus to
694
VOL. 82 NO. 5
POLYARTERITIS
695
Fig. 1 (Kielar). Muscle biopsy specimen has inflammatory cells in arterial wall (hematoxylin and eosin, x40).
the area overlying the pars plana on the temporal side of the globe. A similar process was present to a lesser extent at the nasal corneoscleral limbus (Fig. 2). The sciera posterior to the nasal corneo scleral limbus was markedly thickened by diffuse granulomatous inflammation and fibrosis, with only minute foci of necro sis, which extended to the equator (Fig. 3). The involvement was most marked on the inner aspect of the scierai shell. A similar process was present temporally to a lesser extent. Multinucleated giant cells were present in the scierai spur. The adjacent trabecular meshwork, iris root, ciliary body, and anterior choroid were infiltrated with chronic round inflamma tory cells, many in a perivascular loca tion. Cystic changes were present in the nonpigmented epithelium of the pars plana with extension of inflammatory
Fig. 2 (Kielar). Anterior scierai thinning (arrows) and marked scierai thickening posterior to the equa tor (hematoxylin and eosin, x l ) .
696
AMERICAN JOURNAL OF OPHTHALMOLOGY
NOVEMBER, 1976
Fig. 3 (Kielar). Granulomatous scleritis with epithelioid and giant cells (hematoxylin and eosin, xlOO).
cells into the epithelium. A thin incom plete pigmented pupillary membrane was present. Fibrous connective tissue re placed the outer choroidal architecture from the ora serrata to the equator. The choroid posterior to the equator had adventitial thickening of one large choroidal blood vessel in the region of the macula. The retina was totally detached with eosinophilic subretinal fluid, most marked in the periphery. Scattered pigmented and nonpigmented macrophages were present in the subretinal fluid at the macula, and a thin fibrous strand between the visual receptors and subretinal fluid was present at this site. The rods and cones of the entire retina were degenerated. Cystic changes were present in the outer nuclear and bipolar cell layer in the posterior pole, with scattered eosinophilic exudates in the outer nuclear and outer plexiform layers near the temporal edge of the optic
disk. A full-thickness macular hole was present. Scattered drusen, disruption, and proliferation of retinal pigment epitheli um were present. Scattered retinal blood vessels—some were veins—had perivascular accumulations of chronic round cells which, in areas, extended through the internal limiting membrane onto the inner retinal surface and along the poste rior hyaloid membrane. The optic nerve was normal. Posterior migration of the lens epithelium and globular liquefaction of anterior and posterior cortex were pres ent. The angle was open. Pathologic diagnosis was granuloma tous scleritis; chronic nongranulomatous iridocyclitis and choroiditis; exudative retinal detachment with macular hole; perivasculitis of intrascleral, iris, ciliary body, and retinal blood vessels; acute and chronic stromal keratitis; and posterior subcapsular and cortical cataract.
POLYARTERITIS
VOL. 82 NO. 5 DISCUSSION
The term polyarteritis designates a vari ety of clinical states in which necrotizing inflammation of arteries is present. It af fects arteries of various areas of the body and thus has protean signs and symp toms. Based on histologie and clinical findings, Black 4 subdivided it into classic polyarteritis nodosa, hypersensitivity angiitis, granulomatous angiitis, rheumatic angiitis, and cranial arteritis. Wise 5 reported ocular findings in about 10 to 20% of cases, with diverse ocular involvement. Ocular involvement in polyarteritis most commonly involves the choroidal vessels. 5 An exudative retinal detachment may occur when systemic hy pertension is present, even without cho roidal vascular involvement. 6 Scleritis is uncommon in polyarteritis. When it occurs, it appears most frequent ly in the variant known as Wegener's granulomatosis. In this group, described in detail by Cogan, 7 the scleritis is accom panied by a marginal stromal keratitis, furrow, or ulcer. The posterior segment of the globe is usually minimally involved, and no exudative detachment is present. My patient had no respiratory tract dis ease, and thus does not fit into this subclassification of polyarteritis. Scleritis with an associated exudative retinal detachment has been described in cases of rheumatoid arthritis, 8 polyarteri tis, 1 - 3 and in cases with no demonstrable systemic disease. 9 There are only three reported cases of scleritis with exudative retinal detachment associated with poly arteritis that have been examined histologically. 1 - 3 Histologie findings in the present case are consistent with previous case reports. In all cases a severe diffuse necrotizing granulomatous scleritis extended from the corneoscleral limbus to the equator with involvement of underlying uveal tract. The choroidal blood vessels that were not adjacent to the scierai inflamma tion were uninvolved. Perivascular round
697
cell involvement with obliteration of uveal vessels adjacent to areas of scleritis has been a feature of these cases, and while it is consistent with a diagnosis of polyarteritis, it is not pathognomonic, as similar changes have been reported by Wolter and Bentley 8 and Sevel 10 in scleri tis associated with rheumatoid arthritis. Perivascular round cell involvement of intrascleral, iris, ciliary body, and ret inal vessels in this case are nonspecific changes that can occur in a variety of intraocular inflammations. Sheehan, Harriman, and Bradshaw 1 1 reported the only case with histologie evidence of retinal arterial involvement with necrotizing in flammation and infiltration of eosinophils that was specific for polyarteritis. Although a macular hole can be respon sible for a retinal detachment, it is unlike ly to be the cause of the retinal detach ment in this case, as visual acuity was equal in both eyes four years before onset of visual symptoms, and visual loss, ocu lar pain, and findings of scleritis with retinal detachment occurred in close proximity. The systemic findings in the three previous case reports, 1 - 3 and the pres ent case of scleritis with retinal detach ment in polyarteritis, are similar (Table). These patients tended to have minimal systemic findings at the time of ocular involvement. Constitutional symptoms of malaise, weight loss, mild anemia, microscopic hematuria, and elevated sed imentation rate were frequently present. Respiratory tract disease, severe kidney disease and hypertension (except in one case), and arthritis were noticeably ab sent. Eosinophilia was inconstant. Mus cle biopsy when performed established the diagnosis. Time of onset of ocular involvement after systemic symptoms were present varied from several weeks to six months in the other cases, 1 - 3 but was four years in the present case. Survival time also varied: two patients died within one year
698
AMERICAN JOURNAL OF OPHTHALMOLOGY
NOVEMBER, 1976
TABLE SYSTEMIC FINDINGS A T O N S E T O F OCULAR I N V O L V E M E N T
Weight Loss
Elevated Sedimentation Rate
Anemia
Kidney
Ingalls 1
+
+
+
0 Microscopic hematuria
Cury, Beakey, and Payne 2 Sevel and Moore 3
+
+
0
+
+
+
Hematuria Not done casts albuminuria
Present study
+
+
+
Microscopic hematuria
Case Report
later
0
from onset of symptoms, one patient is alive five years after onset, and my patient is alive six years since diagnosis. The case reported by Cury, Beakey, and Payne 2 was diagnosed as allergic granu lomatous angiitis; however, this patient had no pulmonary involvement, and per haps does not fit into this particular subclassification of polyarteritis. Those cases of scleritis associated with retinal detachment that are not associated with classical rheumatoid arthritis should be suspected of having a form of poly arteritis in which systemic findings are often minimal. If anemia, eosinophilia, elevated sedimentation rate, microscopic hematuria, or weight loss are present, a muscle biopsy is indicated in an attempt to establish the diagnosis. SUMMARY
A 64-year-old white man, treated with systemic corticosteroids for five years, de veloped polyarteritis. He then developed a severe scleritis with an exudative retinal detachment in the right eye, which be came blind and painful and was enucleat ed 23 months after onset of the scleritis. Histopathologic examination of the enu cleated eye revealed granulomatous scle ritis, chronic nongranulomatous uveitis, exudative retinal detachment, and perivasculitis of intrascleral, iris, ciliary body, and retinal blood vessels. Systemic
Elevated Blood Pressure Arthritis
Muscle Biopsy
Eosinophilia
Respiratory Tract
Not done
+
0
0
0
+
+
0
0
0
0
0
+
0
0
0
0
0
+
findings were minimal and limited to elevated sedimentation rate, weight loss, mild anemia, and microscopic hematuria. Respiratory disease, severe kidney dis ease, hypertension, and arthritis were no tably absent. A muscle biopsy established the diagnosis. REFERENCES 1. Ingalls, R. G.: Bilateral uveitis and keratitis accompanying periarteritis nodosa. Trans. Am. Acad. Ophthalmol. Otolaryngol. 55:630, 1951. 2. Cury, D., Beakey, A. S., and Payne, B. F.: Allergic granulomatous angiitis associated with uveoscleritis and papilledema. Arch. Ophthalmol. 55:261, 1956. 3. Sevel, D., and Moore, J. G.: Corneoscleral ulcération in periarteritis nodosa. Br. J. Ophthalmol. 50:651, 1966. 4. Black, R. L.: Polyarteritis nodosa. Postgrad. Med. 31:426, 1962. 5. Wise, G. N.: Ocular periarteritis nodosa. Arch. Ophthalmol. 48:1, 1952. 6. Blodi, F. C , and Sullivan, P. B.: Involvement of the eye in periarteritis nodosa. Trans. Am. Acad. Ophthalmol. Otolaryngol. 63:169, 1959. 7. Cogan, D. G.: Corneoscleral lesions in periarte ritis nodosa and Wegener's granulomatosis. Trans. Am. Ophthalmol. Soc. 53:321, 1955. 8. Wolter, J. R., and Bentley, M.D.: Scleromalacia perforans and massive granuloma of the sciera. Am. J. Ophthalmol. 51:71, 1961. 9. Sears, M. L.: Choroidal and retinal detach ments associated with scleritis. Am. J. Ophthlmol. 58:764, 1964. 10. Sevel, D.: Necrogranulomatous scleritis. Am. J. Ophthalmol. 64:1125, 1967. 11. Sheehan, M. D., Harriman, G. F., and Bradshaw, J. P. P.: Polyarteritis nodosa with ophthalmic and neurological complications. Arch. Ophthalmol. 60:537, 1958.
Scleritis associated with polyarteritis is uncommon. There are only three reported cases of scleritis with exudative retinal detachment associated with polyarteritis in which the globes have been examined histologically. 1 - 3 This case is reported because of its rarity. CASE REPORT A 64-year-old white man was first seen in the medical clinic April 1968 because of a 13.5-kg (30 lb) weight loss and leg pain. General physical exam ination was unremarkable. Blood pressure was 110/ 70 mm Hg. Near visual acuity was reported "normal and equal" in each eye, as was external ocular examination and ophthalmoscopy. Laboratory ex amination revealed a white blood cell count of 14,000/mm 3 with a normal differential count, mild microcytic hypochromic anemia, and microscopic hematuria. Chest x-ray film, upper gastrointestinal series, barium enema, intravenous pyelogram, and blood urea nitrogen tests were normal. A gastrocnemius muscle biopsy was performed and inflammation of medium and small arteries was present on histologie examination (Fig. 1). A diagno sis of polyarteritis was made, and he was placed on 40 mg of prednisone every other day, gradually decreased to 10 mg every other day over the next eight months, and this treatment was continued until August 1973. During that time he felt better and gained weight. Sedimentation rates varied be tween 17 and 51 g/100 ml, and repeat urinalyses showed microscopic hematuria. Blood pressure re mained normal. He was first seen in the ophthalmology clinic in August 1973, with a history of a painful red right eye with loss of vision over the past several months. Visual acuity was R.E.: light perception, and L.E.: 6/6 (20/20). Scleritis was present temporally, ap peared to be deep, and extended from the corneoscleral limbus to 8 mm posteriorly. Moderate flare and cells were present in the anterior chamber. Intraocular pressures were 13 mm Hg in the right eye, and 18 mm Hg in the left eye. Gonioscopy revealed open angles. A total retinal detachment with shifting subretinal fluid without holes was present in the right eye. Examination of the left eye From the Department of Ophthalmology, Univer sity of Kentucky Medical Center, Lexington, Ken tucky. Reprint requests to Richard A. Kielar, M.D., De partment of Ophthalmology, University of Kentucky Medical Center, Lexington, KY 40506.
M.D.
Kentucky was normal. A diagnosis of scleritis with exudative retinal detachment was made, and the eye was treated with a retrobulbar corticosteroid injection and 40 mg of prednisone daily for ten days, with little change in the ocular findings. The patient did not return until March 1974 when right visual acuity was no light perception, with marked flare and cell, posterior synechiae, and scierai inflammation and thinning. A cataract obscured the fundus. He had returned to his previous prednisone dose of 10 mg every other day during this seven-month interval, and had developed no new systemic findings since his original physical examination in April 1968. Due to persistent pain in this blind eye, his eye was enucleated in September 1974. RESULTS
Gross examination—The eye was fixed in formalin. External abnormalities were limited to a slight haziness of the cornea and an oval area of scierai thinning ex tending temporally from the corneoscleral limbus to 5 mm posterior to the corneoscleral limbus. The globe was opened in a horizontal plane. The retina was detached from the ora serrata to the equator by a white translucent homoge nous fluid. A similar detachment was pres ent in the posterior pole. The peripheral choroid appeared slightly thickened. No other abnormalities were noted. Microscopic—The eye was imbedded in paraffin and multiple sections were stained with hematoxylin and eosin. A superficial vascular pannus with numer ous chronic round cells was present in the cornea. There were scattered polymorphonuclear leukocytes throughout the stroma, most marked at the corneoscleral limbus, and midstromal blood vessels with perivascular round cells. The anteri or sciera was involved with a granulomatous inflammation consisting of large areas of scierai necrosis, chronic round cells, epithelioid cells, and scattered giant cells. Marked destruction of scierai fibers extended from the corneoscleral limbus to
694
VOL. 82 NO. 5
POLYARTERITIS
695
Fig. 1 (Kielar). Muscle biopsy specimen has inflammatory cells in arterial wall (hematoxylin and eosin, x40).
the area overlying the pars plana on the temporal side of the globe. A similar process was present to a lesser extent at the nasal corneoscleral limbus (Fig. 2). The sciera posterior to the nasal corneo scleral limbus was markedly thickened by diffuse granulomatous inflammation and fibrosis, with only minute foci of necro sis, which extended to the equator (Fig. 3). The involvement was most marked on the inner aspect of the scierai shell. A similar process was present temporally to a lesser extent. Multinucleated giant cells were present in the scierai spur. The adjacent trabecular meshwork, iris root, ciliary body, and anterior choroid were infiltrated with chronic round inflamma tory cells, many in a perivascular loca tion. Cystic changes were present in the nonpigmented epithelium of the pars plana with extension of inflammatory
Fig. 2 (Kielar). Anterior scierai thinning (arrows) and marked scierai thickening posterior to the equa tor (hematoxylin and eosin, x l ) .
696
AMERICAN JOURNAL OF OPHTHALMOLOGY
NOVEMBER, 1976
Fig. 3 (Kielar). Granulomatous scleritis with epithelioid and giant cells (hematoxylin and eosin, xlOO).
cells into the epithelium. A thin incom plete pigmented pupillary membrane was present. Fibrous connective tissue re placed the outer choroidal architecture from the ora serrata to the equator. The choroid posterior to the equator had adventitial thickening of one large choroidal blood vessel in the region of the macula. The retina was totally detached with eosinophilic subretinal fluid, most marked in the periphery. Scattered pigmented and nonpigmented macrophages were present in the subretinal fluid at the macula, and a thin fibrous strand between the visual receptors and subretinal fluid was present at this site. The rods and cones of the entire retina were degenerated. Cystic changes were present in the outer nuclear and bipolar cell layer in the posterior pole, with scattered eosinophilic exudates in the outer nuclear and outer plexiform layers near the temporal edge of the optic
disk. A full-thickness macular hole was present. Scattered drusen, disruption, and proliferation of retinal pigment epitheli um were present. Scattered retinal blood vessels—some were veins—had perivascular accumulations of chronic round cells which, in areas, extended through the internal limiting membrane onto the inner retinal surface and along the poste rior hyaloid membrane. The optic nerve was normal. Posterior migration of the lens epithelium and globular liquefaction of anterior and posterior cortex were pres ent. The angle was open. Pathologic diagnosis was granuloma tous scleritis; chronic nongranulomatous iridocyclitis and choroiditis; exudative retinal detachment with macular hole; perivasculitis of intrascleral, iris, ciliary body, and retinal blood vessels; acute and chronic stromal keratitis; and posterior subcapsular and cortical cataract.
POLYARTERITIS
VOL. 82 NO. 5 DISCUSSION
The term polyarteritis designates a vari ety of clinical states in which necrotizing inflammation of arteries is present. It af fects arteries of various areas of the body and thus has protean signs and symp toms. Based on histologie and clinical findings, Black 4 subdivided it into classic polyarteritis nodosa, hypersensitivity angiitis, granulomatous angiitis, rheumatic angiitis, and cranial arteritis. Wise 5 reported ocular findings in about 10 to 20% of cases, with diverse ocular involvement. Ocular involvement in polyarteritis most commonly involves the choroidal vessels. 5 An exudative retinal detachment may occur when systemic hy pertension is present, even without cho roidal vascular involvement. 6 Scleritis is uncommon in polyarteritis. When it occurs, it appears most frequent ly in the variant known as Wegener's granulomatosis. In this group, described in detail by Cogan, 7 the scleritis is accom panied by a marginal stromal keratitis, furrow, or ulcer. The posterior segment of the globe is usually minimally involved, and no exudative detachment is present. My patient had no respiratory tract dis ease, and thus does not fit into this subclassification of polyarteritis. Scleritis with an associated exudative retinal detachment has been described in cases of rheumatoid arthritis, 8 polyarteri tis, 1 - 3 and in cases with no demonstrable systemic disease. 9 There are only three reported cases of scleritis with exudative retinal detachment associated with poly arteritis that have been examined histologically. 1 - 3 Histologie findings in the present case are consistent with previous case reports. In all cases a severe diffuse necrotizing granulomatous scleritis extended from the corneoscleral limbus to the equator with involvement of underlying uveal tract. The choroidal blood vessels that were not adjacent to the scierai inflamma tion were uninvolved. Perivascular round
697
cell involvement with obliteration of uveal vessels adjacent to areas of scleritis has been a feature of these cases, and while it is consistent with a diagnosis of polyarteritis, it is not pathognomonic, as similar changes have been reported by Wolter and Bentley 8 and Sevel 10 in scleri tis associated with rheumatoid arthritis. Perivascular round cell involvement of intrascleral, iris, ciliary body, and ret inal vessels in this case are nonspecific changes that can occur in a variety of intraocular inflammations. Sheehan, Harriman, and Bradshaw 1 1 reported the only case with histologie evidence of retinal arterial involvement with necrotizing in flammation and infiltration of eosinophils that was specific for polyarteritis. Although a macular hole can be respon sible for a retinal detachment, it is unlike ly to be the cause of the retinal detach ment in this case, as visual acuity was equal in both eyes four years before onset of visual symptoms, and visual loss, ocu lar pain, and findings of scleritis with retinal detachment occurred in close proximity. The systemic findings in the three previous case reports, 1 - 3 and the pres ent case of scleritis with retinal detach ment in polyarteritis, are similar (Table). These patients tended to have minimal systemic findings at the time of ocular involvement. Constitutional symptoms of malaise, weight loss, mild anemia, microscopic hematuria, and elevated sed imentation rate were frequently present. Respiratory tract disease, severe kidney disease and hypertension (except in one case), and arthritis were noticeably ab sent. Eosinophilia was inconstant. Mus cle biopsy when performed established the diagnosis. Time of onset of ocular involvement after systemic symptoms were present varied from several weeks to six months in the other cases, 1 - 3 but was four years in the present case. Survival time also varied: two patients died within one year
698
AMERICAN JOURNAL OF OPHTHALMOLOGY
NOVEMBER, 1976
TABLE SYSTEMIC FINDINGS A T O N S E T O F OCULAR I N V O L V E M E N T
Weight Loss
Elevated Sedimentation Rate
Anemia
Kidney
Ingalls 1
+
+
+
0 Microscopic hematuria
Cury, Beakey, and Payne 2 Sevel and Moore 3
+
+
0
+
+
+
Hematuria Not done casts albuminuria
Present study
+
+
+
Microscopic hematuria
Case Report
later
0
from onset of symptoms, one patient is alive five years after onset, and my patient is alive six years since diagnosis. The case reported by Cury, Beakey, and Payne 2 was diagnosed as allergic granu lomatous angiitis; however, this patient had no pulmonary involvement, and per haps does not fit into this particular subclassification of polyarteritis. Those cases of scleritis associated with retinal detachment that are not associated with classical rheumatoid arthritis should be suspected of having a form of poly arteritis in which systemic findings are often minimal. If anemia, eosinophilia, elevated sedimentation rate, microscopic hematuria, or weight loss are present, a muscle biopsy is indicated in an attempt to establish the diagnosis. SUMMARY
A 64-year-old white man, treated with systemic corticosteroids for five years, de veloped polyarteritis. He then developed a severe scleritis with an exudative retinal detachment in the right eye, which be came blind and painful and was enucleat ed 23 months after onset of the scleritis. Histopathologic examination of the enu cleated eye revealed granulomatous scle ritis, chronic nongranulomatous uveitis, exudative retinal detachment, and perivasculitis of intrascleral, iris, ciliary body, and retinal blood vessels. Systemic
Elevated Blood Pressure Arthritis
Muscle Biopsy
Eosinophilia
Respiratory Tract
Not done
+
0
0
0
+
+
0
0
0
0
0
+
0
0
0
0
0
+
findings were minimal and limited to elevated sedimentation rate, weight loss, mild anemia, and microscopic hematuria. Respiratory disease, severe kidney dis ease, hypertension, and arthritis were no tably absent. A muscle biopsy established the diagnosis. REFERENCES 1. Ingalls, R. G.: Bilateral uveitis and keratitis accompanying periarteritis nodosa. Trans. Am. Acad. Ophthalmol. Otolaryngol. 55:630, 1951. 2. Cury, D., Beakey, A. S., and Payne, B. F.: Allergic granulomatous angiitis associated with uveoscleritis and papilledema. Arch. Ophthalmol. 55:261, 1956. 3. Sevel, D., and Moore, J. G.: Corneoscleral ulcération in periarteritis nodosa. Br. J. Ophthalmol. 50:651, 1966. 4. Black, R. L.: Polyarteritis nodosa. Postgrad. Med. 31:426, 1962. 5. Wise, G. N.: Ocular periarteritis nodosa. Arch. Ophthalmol. 48:1, 1952. 6. Blodi, F. C , and Sullivan, P. B.: Involvement of the eye in periarteritis nodosa. Trans. Am. Acad. Ophthalmol. Otolaryngol. 63:169, 1959. 7. Cogan, D. G.: Corneoscleral lesions in periarte ritis nodosa and Wegener's granulomatosis. Trans. Am. Ophthalmol. Soc. 53:321, 1955. 8. Wolter, J. R., and Bentley, M.D.: Scleromalacia perforans and massive granuloma of the sciera. Am. J. Ophthalmol. 51:71, 1961. 9. Sears, M. L.: Choroidal and retinal detach ments associated with scleritis. Am. J. Ophthlmol. 58:764, 1964. 10. Sevel, D.: Necrogranulomatous scleritis. Am. J. Ophthalmol. 64:1125, 1967. 11. Sheehan, M. D., Harriman, G. F., and Bradshaw, J. P. P.: Polyarteritis nodosa with ophthalmic and neurological complications. Arch. Ophthalmol. 60:537, 1958.