'Royal Eye Hospital, Manchester, M13 9WH, 2Torbay Hospital, Lawes Bridge,. Torquay, 3Royal Devon and Exeter Hospital (Wonford), Barrack Road, Exeter,.
JOURNAL OF THE ROYAL SOCIETY OF MEDICINE
Volume 88
October 1995
Ocular and renal sarcoidosis S Shah FRCS(Edin) FRCOphth1 A Nicholls MRCP3
M D Cole FRCOphth2
J R Soc Med 1995;88:597P-598P
CASE PRESENTED TO SECTION OF OPHTHALMOLOGY, 9 JUNE 1994
Keywords: sarcoidosis; retina; kidney; vasculitis; lacrimal gland
Sarcoidosis commonly presents in young adults with bilateral hilar lymphadenopathy, lung parenchymal disease and/or skin lesions. Ocular symptoms are the presenting feature in up to 10% of cases1, but eye involvement can be demonstrated in around a quarter of all patients2. Renal disease is much rarer, and may manifest with hypercalcaemic nephropathy, granulomatous interstitial nephritis, tubular dysfunction or glomerulonephritis. Eye and renal involvement are rarely found together, and may be confused with Wegener's granulomatosis or polyarteritis. We report a case of a young man who developed renal failure due to sarcoidosis soon after presenting with uveitis. The case illustrates an unusual combination of systemic features that may not be widely recognized3. CASE HISTORY A 46-year-old white man presented with a 3-week history of linear interference and blurring of vision in his left eye
preceded by black spots. His general practitioner had prescribed chloramphenicol without effect. He had no history of pain, redness, photophobia or myopia. He had been generally unwell for I month and had suffered itching and lymphadenopathy for 3 months. A year previously, hypromellose had been given for dry eyes, thought to be part of Sjogren's syndrome (rheumatoid factor positive). Penicillamine had been started but was withdrawn because of proteinuria. A malignant melanoma overlying his scapula had been completely excised around this time. There was a history of classical migraine. Visual acuity was 6/4 OD and 6/5 OS. The anterior chambers of both eyes were quiet and the lacrimal glands were not enlarged or tender. The left eye showed a moderate number of vitreous cells. Fundoscopy showed a mixture of arteriolar and venous disease with widespread 'Royal Eye Hospital, Manchester, M13 9WH, 2Torbay Hospital, Lawes Bridge, Torquay, 3Royal Devon and Exeter Hospital (Wonford), Barrack Road, Exeter, England, UK Correspondence to: Mr S. Shah
Figure 1 Fundal fluroescein angiography showing only slight vascular leakage
retinal periphlebitis, predominantly at the posterior pole, with mild macular oedema and early optic disc swelling. The right eye was normal. The blood pressure was 180/110. Urinalysis showed haematuria, proteinuria and glycosuria. Urine microscopy showed red and white cells with scanty granular casts; urine culture was sterile. Full blood count, erythrocyte sedimentation rate (ESR) and blood glucose were normal. The serum urea was 20 mmol/l and creatinine 414mol/l. Chest X-ray showed prominence of the left hilum significantly changed from 4 years previously. Antinuclear antibody and anti-neutrophil cytopasmic antibody (ANCA) were negative; rheumatoid factor was positive; calcium and serum angiotensinconverting enzyme (ACE) were normal. A renal biopsy performed the next day revealed granulomatous interstitial nephritis with little glomerular necrosis. Fundal fluorescein angiography (Figure 1) showed only a little vascular leakage, suggesting that the inflammatory component to the retinal vascular disease was very small. An initial diagnosis of Wegener's granulomatosis involving the lacrimal gland, retina and kidney was made. High dose corticosteroids and oral cyclophosphamide were started and the serum creatinine fell to 160mol/l within 3 months. The eye involvement resolved totally. The malignant melanoma then recurred locally after 4 months of immunosuppression. By this time the ANCA test had returned negative and it was appreciated that the initial hilar adenopathy might have represented sarcoidosis. The renal histology was reviewed, and considered more typical of sarcoid than Wegener's granulomatosis. The steroids
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were tapered and azathioprine was substituted for cyclophosphamide. Despite this, lymph node melanoma recurred after a total of a year of immunosuppression, so all immunosuppressive therapy was discontinued. Six months later the patient was well with no eye disease, no tumour, and stable kidney function (creatinine 135mol/l). DISCUSSION
The combination of renal and eye disease in sarcoidosis is excessively rare, having been described only once before in a series of four cases4. This contrasts with the relative frequency of eye involvement in Wegener's granulomatosis5, and explains our initial diagnosis of this condition rather than sarcoidosis. Uveitis is the commonest ocular pathology in sarcoidosis, but other manifestations include: infiltration of the conjunctiva; reduced lacrimal secretion due to infiltration of the lacrimal glands; involvement of the retina and optic nerve head; deposition of calcium salts in the bulbar conjunctiva and cornea (band keratopathy); and infiltration of the orbital fat3. Ocular changes may be symptomless and discovered only by examination6. Reduced lacrimal secretion has been shown to be present in 12.6% of cases but only 4.3% are symptomatic. However, of those cases with ophthalmic involvement, 46% have lacrimal involvement2. Usually, lacrimal gland sarcoidosis is accompanied by easily detected evidence of sarcoidosis elsewhere. Occasionally as in one case of Cook et al.7, it is the first evidence of the multi-system disease. Retinal sarcoid encompasses focal periphlebitis, venous occlusion, acute retinopathy and
neovascularization8'9. The kidneys may be affected in several ways by
sarcoidosis10. The most frequent cause of impairment of renal function is hypercalcaemiall12 which is usually reversible. Granulomatous interstitial nephritis is well recognized, but excessively rare, with only a handful of cases reported in the literaturel l-16. Various forms of glomerulonephritis have also been reportedl'. Favourable responses to corticosteroids can be expected if renal function is impaired by hypercalcaemia or granulomatous infiltration provided permanent damage has not already occurred17'18. This case report demonstrates some of the rarer aspects of sarcoidosis. It shows the limited diagnostic value of serum ACE when the disease is focal as the serum ACE is related to the total mass of granulomaP9. It also demonstrates the dilemma of immunosuppression in the face of recently
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diagnosed malignancy, particularly one (such as melanoma) that may be under some form of immune surveillance or control. REFERENCES 1 Sitzbach LE, James DG. Course and prognosis of sarcoidosis around the world. Am J Med 1974;57:847 2 Karma A, Huhti E. Cause and outcome of ocular sarcoidosis. Am J Ophthalmol 1988;106:467-72 3 Karma A. Ophthalmic changes in sarcoidosis. Acaa Ophthalmol 1979;57(suppl): 141 4 Rosenbaum JT. Bilateral anterior uveitis and interstitial nephritis. Am J Ophthalmol 1988;105:534-7 5 Stavrou P, Deutsch J, Rene C, Laws DE, Luqmani RA, Murray PI. Ocular manifestations of classical and limited Wegener's granulomatosis. QJ Med 1993;86:719-25 6 Scadding JG, Mitchell DN. Sarcoidosis. Cambridge: Chapman & Hall Ltd, 1985:207-26 7 Cook J, Brubaker R. Lacrimal sarcoidosis. Arch Ophthalmol 1972;88:513-17 8 Sanders MD, Schilling JS. Retinal, choroidal and optic disc involvement in sarcoidosis. Trans Ophthalmol Soc UK 1976;96: 140-4 9 Spalton D. Fundus changes in sarcoidosis. Trans Ophthalmol Soc UK 1979;99:167-9 10 Kenouch S, Mery J-P. Sarcoidosis. In: Cameron S, Davison AM, Grunfeld J-P, Kerr D, Ritz E, eds. Oxford Textbook of Clinical Nephrology, Vol 1. Oxford: Oxford University Press, 1992;576-82 11 Lebacq E, Desmet V. Renal involvement in sarcoidosis. Postgrad MedJ 1970;46:526-9 12 Macsearraigh E, Twomey M. Sarcoidosis with renal involvement. Postgrad MedJ 1978;54:528-32 13 Wamberque F, Duchatele P, Riberi P, et a]. Localization renal specifique de la sarcoidose. J Urol Niphrol (Paris) 1978;84:859-65 14 Allegri L, Olivetti G, David S, et al. Sarcoid granulomatous nephritis with isolated and reversible renal failure: A case report. Nephron 1980;25:207-8 15 Korzets Z, Schneider M, Taragan R, et a). Acute renal failure due to sarcoid granulomatous infiltration of the renal parenchyma. AmJ Kidney Dis 1985;6:250-3 16 Matsuo N, Yu K, Hasegawa 0, et al. A case of sarcoid granulomatous interstitial nephritis improved by steroid therapy. Jpn J Nephrol 1991 ;33:703-8 17 Grateau G, Hannedouche T, Dehaine V, et a]. Interstitial granulomatous sarcoid nephritis revealed by renal failure. Four cases. Presse Med 1988;17:1393-5 18 McCurley T, Salter J. Renal insufficiency in sarcoidosis. Arch of Pathol Lab Med 1990;114:488-492 19 Baarsma GB, La Hey E. Predictive value of serum angiotensinconverting enzyme and lysozyme levels in the diagnosis of ocular sarcoidosis. Am J Ophthalmol 1987; 104:211 -15
(Accepted 4 October 1994)
