suits in the formation of transudative ascites. How- ever, the sole occurrence of exudative ascites rarely incurs the suspicion of sarcoidosis, and we feel it.
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CASE REPORT
Primary Peritoneal Sarcoidosis L E S T E R E. R O B E R T S O N , JR, MD, and J O H N T. C U N N I N G H A M , MD, F A C P KEY WORDS: sarcoidosis; ascites.
Sarcoidosis is a granulomatous disease of u n k n o w n etiology that affects multiple organ systems (1); its manifestations are protean, with involvement having been described in virtually every organ. However, serosal involvement is rare. Pericardial (2, 3), pleural (4, 5), and peritoneal involvement (6-11) have been reported. Ascites as a complication of sarcoidosis has been observed and is typically due to severe hepatic involvement and portal hypertension (12-16) or severe pulmonary involvement and right heart failure (17), which most frequently resuits in the formation of transudative ascites. However, the sole occurrence of exudative ascites rarely incurs the suspicion of sarcoidosis, and we feel it should be included in the differential diagnosis of excudative ascites. CASE R E P O R T A 14-year-old black male who had been previously well was referred for the evaluation of unexplained ascites. Preadmission evaluation included normal routine laboratory studies and chest x-ray. An abdominal ultrasound and CT scan were remarkable only for ascites. On questioning, the patient reported a gradual increase in abdominal girth over the past month. He was otherwise asymptomatic and denied fever, chills, night sweats, malaise, change in bowel habits, cough, or dyspnea. There was no antecedent history of abdominal surgery, blood transfusion, foreign travel, tuberculous exposure, substance abuse, or jaundice. Admission physical examination revealed a healthy appearing pubescent black male. Vital signs and oral temperature were normal. Examination above the neck was normal. The chest was clear, and the heart sounds were normal. Tense ascites was present. There was no abdominal tenderness, hepatosplenomegaly, lymphadenopathy, or stigmata of liver disease. Manuscript received March 6, 1990; revised manuscript received May 16, 1990; accepted May 24, 1990. From the Department of Internal Medicine and Gastroenterology Division, Medical University of South Carolina, Charleston, South Carolina. Address for reprint requests: Dr. John T. Cunningham, Medical University of South Carolina, Gastroenterology Division, 171 Ashley Avenue, Charleston, South Carolina 29425.
Laboratory studies included: hematocrit, 43%; white blood cells, 5.7• 103 with a normal differential analysis; platelets, normal; and prothrombin time, 11.6 sec (control 12.1 sec). The electrolytes, blood sugar, blood urea nitrogen, creatinine, and urine analysis were normal. The total serum protein was 8.2 g/100 ml; albumin 4.8 g/100 ml; total bilirubin 0.2 mg/100 ml; aspartate aminotransferase 24 units/liter; and alkaline phosphatase 194 IU/liter (normal for age). Serum angiotensin converting enzyme levels were slightly elevated at 34 ng/ml/hr. The chest radiograph was normal. Paracentesis demonstrated a sanguinous exudate (red blood cells 10.5x 103/ml, white blood ceils 1.6• 10S/ml, with 13% polys, 54% lymphocytes, and 33% mesothelial cells and a protein of 5.9 g/100 ml. Review of outside CT scan demonstrated diffuse thickening of the jejunal wall. This was reaffirmed with a small bowel follow through. Jejunal biopsy with a Crosby capsule revealed normal mucosa. The possibility of ascitic bowel wall edema was considered. A PPD was nonreactive and control antigens were reactive. At this point diagnostic peritoneoscopy was performed. Peritoneoscopy showed the visceral and parietal peritoneum to be studded with 1 to 2-mm white nodules (Figure 1). The serosa over the small bowel and stomach were thickened; loops of bowel were matted together. The liver was reddish-brown with a round edge and covered by a thick fibrinous peel. The falciform ligament was normal in appearance, and the spleen was not visualized. No endoscopic evidence of portal hypertension was present. Biopsy of the peritoneum demonstrated multiple welldefined epithelioid granulomas containing multinucleated giant cells (Figure 2). Surrounding the granuloma and intervening stroma were numerous eosinophils, plasma ceils, and lymphocytes. There was no evidence of necrosis. Special stains for myobacteria, fungi, and bacteria were negative. Culture of the biopsy specimen and ascitic fluid for organisms was negative. Despite a normal-appearing chest radiograph, bronchoscopy was performed. Miliary nodules highl3; consistent with a diagnosis of sarcoidosis were seen in the trachea and right mainsterm bronchus. Transbronchial biopsies revealed multiple noncaseating epithelioid granulomas. All special stains and cultures were negative. Therapy with prednisone 30 mg/day was instituted with gradual resolution of the ascites; after six weeks, the dose was slowly tapered and discontinued after complete resolu-
Digestive Diseases and Sciences, Vol. 35, No. 12 (December 1990) 0163-2116/90/1200-1545506.00/0 9 1990PlenumPublishingCorporation
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Fig 1. Peritoneoscopic view demonstrating multiple small white nodules (arrows) studding the visceral peritoneum.
tion. The patient is asymptomatic at six months' follow-up with no recurrence. DISCUSSION Sarcoidosis is a systemic disease characterized by the presence of noncaseating epithelioid granulomas in involved tissues (1). Since the earliest descriptions of sarcoidosis (18, 19) many authors have reported on the varying clinical manifestations and organ involvement in this protean disease. Clinically the lungs, reticuloendothelial system, eyes, skin, and liver are the most frequently involved sites. 20 As characteristic of many other organs, hepatic involvement is usually asymptomatic and may be characterized only by asymptomatic elevation of hepatic enzymes (21, 22). However, pathologic evidence is common, and hepatic granulomas have been described in up to 94% of liver biopsies (23). In contrast serosal involvement is rare. As a result, ascites, pleural effusion, and pericardial effusion are so uncommon that coincidental disease must be considered. However, pericardial, pleural, and peritoneal sarcoidosis does occur as a result of direct organ extension (13). Longcope and Freiman state that with the exception of direct extension
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from the pericardium, pleura, or mediastinum "the serous surfaces of the body cavities appear as though almost immune to the disease" (13). Causes of ascites may be divided into pathologic processes that are intrinsic and extrinsic to the peritoneum (24). Ascites in sarcoidosis typically is a result of granulomatous disease outside the peritoneum. Severe granulomatous liver involvement and portal hypertension can cause ascites in sarcoidosis (12-16). Associated hypoproteinemia may be a contributing factor. In addition ascites has been reported in sarcoidosis as a consequence of right heart failure from severe pulmonary hypertension (17). Primary peritoneal sarcoidosis has been described (6-11), and, in the earliest reports, the granulomatous involvement was localized around thickened surgical scars, and a local sarcoid-tissue reaction with extension from the scar could not be excluded (6, 7). In five of six previously reported welldescribed cases of diffuse peritoneal involvement, the diagnosis of sarcoidosis was established prior to demonstration of peritoneal involvement (8, 9, 11). Laparotomy was the most common method of diagnosis (6-10). Ascites, both bloody and nonbloody has been demonstrated (11). Remarkably, the ascites resolved in all of the cases, often in weeks and frequently without the addition of steroids. The etiology of this young patient's ascites was elusive until peritoneoscopy demonstrated miliary nodules, raising the possibilities of peritoneal tuberculosis, fungal infection, carcinomatosis, and sarcoidosis. Sarcoidosis is an exclusionary diagnosis supported by the finding of culture-negative noncaseating epithelioid granulomas. The nonreactive tuberculin skin test, negative special stains, and numerous negative cultures reasonably excluded other causes. Although no pulmonary radiographic evidence of sarcoidosis was present, characteristic endobronchial lesions were observed and granulomas were found on transbronchial biopsy. This patient was remarkable for his young age and lack of associated findings typical for sarcoidosis. Our patient received a sixweek course of prednisone with resolution of the ascites and has done well without clinical evidence of recurrence for almost one year. SUMMARY
A 14-year-old male was admitted for the evaluation of tense exudative ascites. Despite thorough evaluation, the diagnosis remained cryptic until Digestive Diseases and Sciences, Vol. 35, No. 12 (December 1990)
PRIMARY P E R I T O N E A L SARCOIDOSIS
Fig 2. Histologic peritoneal biopsy with: A low-power photomicrograph demonstrating a well-formed granuloma; B high-power photomicrograph with multinucleated giant cells, epitheliod cells, and lymphocytes.
peritoneoscopy revealed diffuse studding of the entire visualized peritoneum with multiple miliary nodules, and peritoneal biopsy demonstrated multiple noncaseating epithelioid granulomas. After other causes were excluded, a diagnosis of sarcoidosis was considered and confirmed with classic endobronchial findings at bronchoscopy. Involvement of the peritoneum with sarcoidosis is rare and, to our knowledge, only one other case describes this as the initial manifestation of this disease. Digestive Diseases and Sciences, Vol. 35, No. 12 (December 1990)
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