BRIEF REPORTS
Presumptive autonephrectomy in scleroderma Medical management of scleroderma renal crisis has received recent attention (1-3) and in the acute phase with severe hypertension due to renal ischemia, urgent bilateral nephrectomy may not be necessary if an antihypertensive regimen can control arterial pressure. For patients in the acute phase of this disease, we report a unique means of blood pressure control: presumptive autonephrectomy . A 47-year-old woman, with a 6-month history of probable scleroderma based on skin biopsy in the absence of proximal scleroderma, experienced several days of extreme fatigue followed by lightheadedness and nosebleeds. She was referred to the New York Hospital with a blood pressure of 220/120. Admission examination revealed proximal scleroderma with sclerodactyly, bibasilar rales without a third heart sound, and arteriolar narrowing with spasm but without hemorrhages or exudates on fundoscopy. Within 18 hours of admission, 60 mg of propranolol had been administered, and the diastolic pressure fell below 100 mm Hg. However, she complained of back pain and developed fever. The serum creatinine rose from 2.7 to 6.0 mg/dl within the first 48 hours and with persistent oliguria despite fluid and diuretic challenge, peritoneal dialysis was initiated. Transaminase and lactic dehydrogenase levels rose sharply with peak values 72 hours after admission (SGOT 668, SGPT 2250, LDH 757 at 24 hours; SGOT 5340, LDH 1369 at 28 hours; SGOT 6074, SGPT 4770, LDH 9958 at 72 hours; and 526, 1575, 902 respectively at 120 hours). The LDH isoenzyme pattern was consistent with a renal parenchymal source. CPK, alkaline phosphatase, and y-glutamyl-transpeptidase remained normal; indirect bilirubin rose as disseminated intravascular coagulation led to a fall in the hematocrit and a rise in the reticulocyte count 2 days after the peak transaminase and LDH values. The patient’s blood pressure became progressively less severe, requiring no propranolol by the seventh hospital day. In stable condition, without evidence of vascular spasm, she continued with peritoneal dialysis and occasional propranolol but 5 weeks later died of a respiratory arrest. Bilateral nephrectomy in the management of scleroderma renal crisis has been advocated in some situations (4), but others have suggested that intensive antihypertensive therapy alone is adequate (1-3). Our patient did not require urgent surgical nephrectomy to stabilize her clinical status, but she did experience massive enzyme elevations with a renal LDH pattern fol-
305
lowed by resolution of her hypertension. Although permission for pathologic confirmation was not obtained, renal infarction with autonephrectomy is the most likely synthesis of our patient’s course-a unique means of blood pressure control in scleroderma. ROBERTP. KIMBERLY, MD SUSANBRESSMAN,MD LUISTAPIA, MD MICHAEL D. LOCKSHIN, MD The New York Hospital-Cornell University Medical Center New York, New York
REFERENCES Brown RS, McCluskey RT: Clinicopathological conference. N Engl J Med 2992373-875, 1978 Wasner C, Cooke CR, Fries JF: Successful medical treatment of scleroderma renal crisis. N Engl J Med 2992373875, 1978 Mitnick PD, Feig FU: Control of hypertension and reversal of renal failure in scleroderma. N Engl J Med 299:871-872, 1978 LeRoy EC, Fleischmann RM: The management of renal scleroderrna. Experience with dialysis, nephrectomy and transplantation. Am J Med 64:974-978, 1978 Address reprint requests to Robert P. Kimberly, MD, The Hospital for Special Surgery, 535 East 70 Street, New York, N Y 10021.
Submitted for publication November 7, 1978; accepted November 7, 1978.
Cryoglobulins in the procainamide-induced lupus syndrome Drug-induced systemic lupus erythematosus is a well recognized syndrome and is most commonly caused by procainamide (1). The syndrome has been associated with glomerulonephritis (2), hypocomplementemia (3), and a circulating anticoagulant (4). Although cryoglobulins are well recognized in idiopathic SLE ( 9 , to date no case of cryoglobulinemia in drug-induced lupus has been reported. Furthermore, no association has been made between any drug and the development of circulating cryoglobulins. We report the finding of a circulating cryoglobulin in a patient with procainamideinduced SLE.
Presumptive autonephrectomy in scleroderma Medical management of scleroderma renal crisis has received recent attention (1-3) and in the acute phase with severe hypertension due to renal ischemia, urgent bilateral nephrectomy may not be necessary if an antihypertensive regimen can control arterial pressure. For patients in the acute phase of this disease, we report a unique means of blood pressure control: presumptive autonephrectomy . A 47-year-old woman, with a 6-month history of probable scleroderma based on skin biopsy in the absence of proximal scleroderma, experienced several days of extreme fatigue followed by lightheadedness and nosebleeds. She was referred to the New York Hospital with a blood pressure of 220/120. Admission examination revealed proximal scleroderma with sclerodactyly, bibasilar rales without a third heart sound, and arteriolar narrowing with spasm but without hemorrhages or exudates on fundoscopy. Within 18 hours of admission, 60 mg of propranolol had been administered, and the diastolic pressure fell below 100 mm Hg. However, she complained of back pain and developed fever. The serum creatinine rose from 2.7 to 6.0 mg/dl within the first 48 hours and with persistent oliguria despite fluid and diuretic challenge, peritoneal dialysis was initiated. Transaminase and lactic dehydrogenase levels rose sharply with peak values 72 hours after admission (SGOT 668, SGPT 2250, LDH 757 at 24 hours; SGOT 5340, LDH 1369 at 28 hours; SGOT 6074, SGPT 4770, LDH 9958 at 72 hours; and 526, 1575, 902 respectively at 120 hours). The LDH isoenzyme pattern was consistent with a renal parenchymal source. CPK, alkaline phosphatase, and y-glutamyl-transpeptidase remained normal; indirect bilirubin rose as disseminated intravascular coagulation led to a fall in the hematocrit and a rise in the reticulocyte count 2 days after the peak transaminase and LDH values. The patient’s blood pressure became progressively less severe, requiring no propranolol by the seventh hospital day. In stable condition, without evidence of vascular spasm, she continued with peritoneal dialysis and occasional propranolol but 5 weeks later died of a respiratory arrest. Bilateral nephrectomy in the management of scleroderma renal crisis has been advocated in some situations (4), but others have suggested that intensive antihypertensive therapy alone is adequate (1-3). Our patient did not require urgent surgical nephrectomy to stabilize her clinical status, but she did experience massive enzyme elevations with a renal LDH pattern fol-
305
lowed by resolution of her hypertension. Although permission for pathologic confirmation was not obtained, renal infarction with autonephrectomy is the most likely synthesis of our patient’s course-a unique means of blood pressure control in scleroderma. ROBERTP. KIMBERLY, MD SUSANBRESSMAN,MD LUISTAPIA, MD MICHAEL D. LOCKSHIN, MD The New York Hospital-Cornell University Medical Center New York, New York
REFERENCES Brown RS, McCluskey RT: Clinicopathological conference. N Engl J Med 2992373-875, 1978 Wasner C, Cooke CR, Fries JF: Successful medical treatment of scleroderma renal crisis. N Engl J Med 2992373875, 1978 Mitnick PD, Feig FU: Control of hypertension and reversal of renal failure in scleroderma. N Engl J Med 299:871-872, 1978 LeRoy EC, Fleischmann RM: The management of renal scleroderrna. Experience with dialysis, nephrectomy and transplantation. Am J Med 64:974-978, 1978 Address reprint requests to Robert P. Kimberly, MD, The Hospital for Special Surgery, 535 East 70 Street, New York, N Y 10021.
Submitted for publication November 7, 1978; accepted November 7, 1978.
Cryoglobulins in the procainamide-induced lupus syndrome Drug-induced systemic lupus erythematosus is a well recognized syndrome and is most commonly caused by procainamide (1). The syndrome has been associated with glomerulonephritis (2), hypocomplementemia (3), and a circulating anticoagulant (4). Although cryoglobulins are well recognized in idiopathic SLE ( 9 , to date no case of cryoglobulinemia in drug-induced lupus has been reported. Furthermore, no association has been made between any drug and the development of circulating cryoglobulins. We report the finding of a circulating cryoglobulin in a patient with procainamideinduced SLE.
