Mesangioproliferative glomerulonephritis associated with retinitis pigmentosa.

Abstract

SUMMARY Two patients with retinitis pigmentosa and glomerulonephritis are described. The patients were unrelated and had identical renal lesions, mesangioproliferative glomerulonephritis. This may not be a chance association, and simple dip-stick urine analysis in patients with primary retinal degenerations may lead to earlier identification of renal disease.The association between primary retinal degeneration and renal disease has been well described,'" but usually in the context of inherited syndromes with multiple abnormalities, such as the Laurence-MoonBiedl syndrome, which are often apparent at or soon after birth.We describe two unrelated patients who were diagnosed as having retinitis pigmentosa during their third decade and who had different renal presentations of the same underlying glomerular lesion in adult life. Neither had a family history of blindness or nephritis, and though the patient in case 2 also had a disorder of cardiac conduction and neuronal deafness there were no features suggestive of a recognised syndrome in either case. Case reports CASE 1A 65-year-old woman had been registered as partially sighted for many years, her ophthalmological diagnosis being retinitis pigmentosa. She presented in 1979 with proteinuria, hypertension, and microscopic haematuria. On demonstration of highly selective proteinuria a presumptive diagnosis of minimal change glomerulonephritis was made. The proteinuria and oedema remitted with diuretics, cyclophosphamide, and steroids, which were withdrawn after nine months.She was referred to the renal unit five years later with a relapse of her nephritis. The 24-hour urinary protein excretion was 7 g and creatinine clearance was 18 ml/min. Urine microscopy demonstrated an active sediment, with casts and red cells; a test for Correspondence to Dr P A Kalra, 80 Islington Street, Ponsonby, Auckland, New Zealand.antinuclear factor (ANF) was negative, and immunoglobulins were within the normal ranges. A diuresis was obtained with a salt-poor albumin infusion and diuretics, and she was also restarted on prednisolone. Renal A 26-year-old man had been diagnosed as hypertensive at the time of total hip replacement for secondary osteoarthrosis. Blood pressure required treatment with nadolol, minoxidil, and nifedipine for control.Five years later he was admitted to the coronary care unit with severe central chest pain unassociated with arrhythmia. He was a stocky, unusual looking man with hypertrichosis and a broad, flat facies; he appeared to be of normal intelligence. Chromosome analysis was normal. Serial ECG recordings, though demonstrating type A Wolfe-Parkinson-White syndrome and left ventricular hypertrophy, provided no evidence of myocardial infarction, and there was no increase of serum enzymes. After a second episode of pain, normal coronary arteries were shown at angiography. He was noted to have chronic renal failure with a creatinine clearance of 67 ml/min. The 24-hour urinary protein excretion was 5-4 g, antinuclear factor (ANF) negative, serum IgA and IgG both...