Protein-Losing Enteropathy in Systemic Lupus erythematosus

Digestive Diseases and Sciences, Vol. 34, No. 1 (January 1989), pp. 132-135

CASE REPORT

Protein-Losing Enteropathy in Systemic Lupus Erythematosus Diagnosis and Monitoring Immunosuppressive Therapy by Alpha-l-Antitrypsin Clearance in Stool K E N T G. B E N N E R , MD and A N T H O N Y M O N T A N A R O , MD protein-losing enteropathy; systemic lupus erythematosus; ct-l-antitrypsin clearance; hypoalbu-

KEY WORDS:

minemia.

Hypoalbuminemia occurs commonly in patients with chronic diseases and is usually attributed to decreased protein ingestion, suppressed endogenous production due to liver disease, or renal protein loss. Mild to moderate suppression of serum albumin levels in patients with systemic lupus erythematosus (SLE) is usually attributed to renal loss or disease activity, but several recent reports have described S L E patients with profound symptomatic hypoalbuminemia in the absence of nephrotic syndrome, constrictive pericarditis, or starvation and presumed to be caused by protein-losing enteropathy (1). Suggested potential mechanisms of enteric protein loss include disordered mucosal cell metabolism, mucosal ulceration, and defects in intestinal lymphatics attributable to congenital deformity, increased central venous pressure, or partial lymphatic obstruction (2). We describe a patient with severe protein-losing enteropathy as the initial manifestation of systemic lupus erythematosus who was diagnosed and monitored on immunosuppressive therapy by the recently described method of alpha-l-antitrypsin clearance in stool. CASE R E P O R T The patient is a 22-year-old woman of Chinese ancestry who was admitted to hospital for anasarca. Ten months Manuscript received March 6, 1987; revised manuscript received October 6, 1987; accepted December 24, 1987. From the Divisions of Gastroenterology and Allergy and Immunology, Department of Medicine, Oregon Health Sciences University, Portland, Oregon. Address for reprint requests: Dr. Kent G. Benner, Division of Gastroenterology, OHSU, L-461, 3181 SW Sam Jackson Park Road, Portland, Oregon 97201. 132

prior to presentation, periorbital edema and pruritus developed and cutaneous angioedema was suspected. Four months later, increasing facial and periorbital edema and chemosis occurred and investigation revealed a serum albumin of 3.8 g/dl and an ESR of 54 mm/hr. Hydrochlorothiazide was prescribed and edema transiently improved. Rapid development of marked bilateral lower extremity edema over the subsequent three months was accompanied by increased fatigue, decreased exercise tolerance, and a 30-pound weight gain. Endocrinological evaluation revealed a clinically euthyroid patient with a free T4 of 0.4 ng/dl (0.7-1.8 ng/dl) and a normal thyroid stimulating hormone level of 10.6 mIU/ml (0.012.0 mIU/ml), which was felt to represent "sick euthyroid syndrome." At the time of admission, there was no history of diarrhea, and the patient was otherwise systemically well. She denied use of prescription or nonprescription medication. Physical examination revealed a blood pressure of 100/72, weight of 183 pounds, anasarca, bilateral periorbital edema and chemosis. No skin rashes or lymphadenopathy were noted. Chest examination showed dullness to percussion and decreased fremitus over the lower lung fields bilaterally. Her abdomen was distended with striae and shifting dullness. There was no hepatomegally or stigmata of chronic liver disease. There was 4+ pitting edema over the abdomen, presacral area, and lower extremities bilaterally. The hematocrit was 41.7% and leukocyte count was 7600/mm 3 with a normal differential count. Serum electrolytes were: sodium, 135 meq/liter; potassium, 4.6 meq/liter; chloride, 108 meq/liter; bicarbonate, 25.0 meq/ liter; calcium, 6.6 meq/liter; phosphate, 3.9 meq/liter. Total serum protein by electrophoresis was 3.5 g/dl with an albumin of 0.8 g/dl. Serum cholesterol was 660 mg/dl and triglyceride was 370 mg/dl. Westergren erythrocyte sedimentation rate was 118 mm/hr. Urinalysis was unremarkable with a negative dipstick for protein, and a 24-hr urine collection documented no significant proteinuria and normal renal function. Serologic testing included: Digestive Diseases and Sciences, Vol. 34, No. 1 (January 1989)

0163-2116/89/0100-0132506.00/0 9 1989Plenum Publishing Corporation

PROTEIN-LOSING ENTEROPATHY IN LUPUS

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Fig 1. Clinical response of the patient's protein-losing enteropathy to prednisone and azathioprine immunosuppression as indicated by alpha-l-antitrypsin clearance in stool, serum albumin, and body weight during three years of follow-up. ANA, (positive), 1:480 with a speckled pattern; anti-ds DNA binding, 9.7% (0.0-9.0%); serum C'3 complement, 25 mg/dl (70-176 mg/dl); serum C'4 complement,