LETTERS 936 One of 3 patients with normal parotid sialography in 1974 in whom the study was repeated has now developed punctate sialectasia. Three of the 4 patients with normal salivary scintiscans in the 1974 study have now become abnormal with decreased salivary gland radionuclide uptake as well as *“Tc-pertechnetate excretion in saliva. It is apparent that with the passage of time, lupus patients who had subclinical evidence of Sjogren’s syndrome develop sicca symptoms. It is not unlikely that with longer followup, more or perhaps all of our lupus patients will develop these symptoms, despite continued vigilance and treatment. Although none of the 30 patients included in this reevaluation developed acute salivary gland swelling, we have seen this occur in other patients in our lupus clinic who previously had no other clinical evidence of Sjogren’s syndrome. This occurrence has also been reported by Katz and Ehrlich (3). All New Zealand black mice and their F, black/ white hybrids, which are animal models of SLE, develop lacrimal and salivary gland inflammatory infiltrates after 4 months of age (4). The reason for the high prevalence of Sj8gren’s syndrome in human and murine SLE, as well as in other autoimmune diseases, is not known, but our findings indicate that in systemic lupus erythematosus the frequency of sicca symptoms is cumulative and may be heralded by abnormalities in the more sensitive roentgenographic, radionuclide, or pathologic tests. MD FRANCISCO RAMOS-NIEMBRO, DONATOALARC6N-SEGOVIA, M D Department of Immunology and Rheumatology Instituto Nacional de la Nutricidn Mkxico 22, DF, Mkxico REFERENCES I . Alarc6n-Segovia D, Ibaiiez G, VelAzquez-Forero F, Hernbdez-Ortiz J, Gonzhlez-Jimtnez Y: Sjogren’s syndrome in systemic lupus erythematosus: clinical and subclinical manifestations. Ann Intern Med 81:557-583, 1974 2. Alardn-Segovia D: Symptomatic Sjogren’s syndrome in mixed connective tissue disease. J Rheumatol 3:191-195, 1976 3. Katz WA, Ehrlich GE: Acute salivary gland inflammation associated with systemic lupus erythematosus. Ann Rheum Dis 31:384-387, 1972 4. Kessler HS: A laboratory model for Sjogren’s syndrome. Am J Pathol52:671-685, 1978 LE cells in pleural fluid To the Editor: The presence of LE cells in pleural fluid of patients with systemic lupus erythematosus (SLE), or procainamide and hydralazine induced lupus (PSLE), has been previously reported (1-3). Although the association of SLE and PSLE is frequent, the LE phenomenon in pleural fluid is seldom reported. We recently published a report of a case (2) of PSLE in which the initial manifestation of the disease was the finding of LE cells in pleural fluid. Two further cases of the in vivo LE phenomenon in pleural fluid have been subsequently documented. Case 1. A 71-year-old female experienced an acute myocardial infarction. She was subsequently treated with procainamide HC12 gm daily for 5 months. She then developed right sided pleuritic chest pain. Chest x-ray revealed pleural effusion. Wright stain of the pleural fluid documented the presence of LE cells. LE cells were subsequently found in blood. ANA was positive in 1/SO00 dilution; however, antibodies to double-stranded DNA were absent. The pleural fluid resolved within 2 weeks after cessation of therapy with procainamide. LE cells in blood were negative a month later. Case 2. A 69-year-old female was admitted with complaints of fever and weakness of one month duration. A left pleural effusion was present on chest x-ray. LE cells were visible by a Wright stain of the pleural fluid. The presumptive diagnosis of SLE was confirmed by the presence of LE cells in the blood, a strongly positive reaction for ANA in a 1/10 dilution, positive reaction for double-stranded DNA antibodies in a 1/1 reaction, and a low serum complement (C’3) of 43 mg%. Subsequently, treatment with steroids was initiated and the pleural effusion cleared rapidly. The documentation of LE cells in pleural fluid of 3 patients over a 1-year period in one general internal medicine ward suggests that this phenomenon is more common than has previously been emphasized. The appearance of LE cells in these effusions may result from autoincubation of polymorphonuclear leukocytes in the specific body space. Nucleoprotein is released from stagnating cells and subsequently phagocytized by viable polymorphonuclear leukocytes. Thus the specific milieu of the pleural space could explain the early appearance of LE cells in the pleural fluid in all our cases. We suggest that searching for LE cells in sus- LETTERS 937 pected body effusions may be helpful in early diagnosis of either SLE or PSLE. RAFAELS.CAREL,MD MENACHEM S. SHAPIRO, MD OLINDACORDOBA, MD ROMAMTI TARAGAN, MD ARONGUTMAN, MD Ramat-Gan, Israel REFERENCES Pandya MR, Agus B, Grady RF: In vivo LE phenomenon in pleural fluid. Arthritis Rheum 19:962-963, 1976 Care1 RS, Shapiro MS, Shoham D, Gutman A: Lupus ery- thematosus cells in pleural effusion. Chest 72:670-672, 1977 Weinstein J: Hypocomplementemia in hydralazine-associated systemic lupus erythematosus.Am J Med 65:553-556, 1978 Adrenocortical carcinoma in a patient with systemic lupus erythematosus treated with azathioprine To the Editor: We have observed the development of a rare malignancy, adrenocortical carcinoma, in a patient with systemic lupus erythematosus (SLE) who was treated with azathioprine. Adrenocortical carcinoma in a patient with SLE has not been described previously, nor has the presence of this malignancy in a patient receiving azathioprine. FP, a 47-year-old white male, was referred to the Brooklyn Veterans Administration Medical Center in June 1977 with a 4-week history of left flank pain, 4.5 kg weight loss, and fever. SLE had been diagnosed in 1959 at the time of the onset of arthritis and a positive LE cell test. Over the succeeding years, he had multiple manifestations of SLE including deforming arthritis, pleural and pericardial effusions, grand ma1 seizures, hemolytic anemia, and proteinuria. Eventually he fulfilled 6 of the 14 American Rheumatism Association criteria for SLE ( I ) . Prednisone (up to a maximum dose of 40 mg/day) was initiated in 1962. Azathioprine 100 mg three times a day was begun in 1974. From that time until admission he received the azathioprine, prednisone 10 mg every other day, diphenylhydantoin sodium 300 mg/day, and phenobarbital 30 mg/day. On physical examination the patient appeared chronically ill. Blood pressure was 100/70 mm/Hg, pulse 1OO/minute, respirations 16/minute, and temper- ature was 38.5OC. There were no stigmata of Cushing's syndrome. Tenderness and fullness were present in the left flank, and the spleen and liver were palpated at the costal margins. Proximal muscle weakness was found in all extremities. Results of the remainder of the examination were normal. Laboratory studies showed a hemoglobin of 8.9 gm%, hematocrit of 26%, and white blood count of 16,800 cells/mm-' with 84% polymorphonuclear leukocytes. Urinalysis was normal. Blood urea nitrogen was 38 mg% and serum creatinine was 2.5 mg%. Serum alkaline phosphatase, lactic dehydrogenase, and leucineaminopeptidase levels were elevated. Antinuclear antibody and LE tests were negative at this time; serum complement levels were normal. Plasma cortisol levels were normal but did not suppress after 48-hour administration of 2 or 8 mg/day of dexamethasone. Chest xray and skeletal survey were normal. Sonography and angiography revealed a left solid suprarenal mass with cystic areas. Liver, spleen, and bone scans revealed no evidence of metastases. At surgery, a 12 cm hemorrhagic tumor of the left adrenal gland was removed. Histologically, the tumor was an undifferentiated carcinoma of the adrenal cortex. One month after surgery, metastases to the skin, brain, and ribs became apparent. Despite therapy with 1,l dichloro-2-(o-chlorophenyl)-2-(p-chlorophenyl) ethane, the patient's condition deteriorated. A right upper lobe pulmonary infiltrate appeared, respiratory insufficiency and hypotension developed, and the patient died. Autopsy permission was denied. Adrenocortical carcinoma has not been reported previously in either SLE or in patients receiving azathioprine. In a study of 484 patients with SLE, a higher incidence of cancer (3.7%) was found than in a control population (2). Only 1 of 18 patients developing malignancy had received an immunosuppressive drug other than corticosteroids. The malignancies seen did not differ in type from those occurring in the control group. Another review of 70 patients with SLE reported an 1 I .4% incidence of malignancy (3). Three-quarters of these patients had been treated with immunosuppressive agents. Azathioprine has been implicated in the 5% incidence of cancer in patients with renal transplants (4). Both the size of the azathioprine dose (300 mg/day in a man weighing 65 kilograms) and the duration of azathioprine therapy (3 years) were exceptional in the patient reported here. It is not possible to be certain of the contribution of azathioprine to the development of this patient's adrenocortical cancer, however
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