The development of ascites due to peritoneal involvement in sarcoidosis is a rare occurrence and in previous reports has been described only as serous fluid. We present the findings in two patients in whom sarcoidosis was associated with bloody ascites where no other cause could be demonstrated.
This 42-year old black woman developed bilateral parotid swelling in 1971. A chest roentgenogram showed bilateral hilar adenopathy. Both mediastinoscopy and liver biopsy revealed noncaseating granulomas. A tuberculin skin test (purified protein derivative of tuberculin [PPD]) was negative. The patient did well for two years without medications. In December of1973, she was admitted for repair of a ruptured appendix and periappendicular abscess. She subsequently developed a strangulated loop of small bowel which required surgical reduction in July 1974. Splenomegaly was noted. In December 1974, the patient developed abdominal swelling, slight shortness of breath, and night sweats without fever. She denied alcohol abuse, exposure to hepatotoxins, or tuberculosis.
On admission the patient was noted to be emaciated. The skin was clear without adenopathy. There was dullness at both pulmonary bases. Nodules were palpated in the abdominal wall, and there was massive ascites. A chest roentgenogram showed moderate hilar adenopathy and possible granulomas in both pulmonary fields. Upper gastrointestinal roentgenograms showed no evidence of varices. The spleen was enlarged on scan. The level of lactic dehydrogenase (LDH) was 574 units/L (reference range, 170 to 330 units/L). Testing with PPD was again negative. Paracentesis revealed bloody fluid with 148,000 red blood cells and 577 lymphocytes per milliliter. The protein level of the fluid was 6.5 g/dl, the LDH level was 300 units/L, and the amylase level was 62 units/L (serum reference range, 20 to 140 units/L).
Bone marrow biopsy showed noncaseating granulomas with negative auramine-rhodamine, acid-fast, bacterial, and fungal stains. At laparotomy, 7 L of bloody ascitic fluid were removed. The entire abdominal cavity was studded with 1-mm white nodules. Microscopic examination of biopsies of falciform ligament, ligamentum teres, and liver showed multiple noncaseating granulomas negative for acid-fast bacteria and fungi on special stains. The patient was started on isoniazid (INH) and streptomycin, but they were discontinued when cultures for mycobacteria were negative. Some ascites persisted for about eight years.
In 1984, the patient underwent repair of a ventral hernia, hysterectomy, and bilateral salpingo-oophorectomy for uterine leiomyomas. No ascites was seen at operation. Noncaseating granulomas were present in the hernia sac, ovaries, fallopian tubes, and the uterine serosa. The granulomas were somewhat hyalinized as compared with those present ten years earlier. Stains for organisms were again negative.
This 29-year-old black woman developed blurred vision in 1968 and was diagnosed as having uveitis secondary to sarcoidosis. She was treated successfully with systemic steroids. Three years later, she developed epigastric discomfort. Splenomegaly was diagnosed, and a cervical lymph node biopsy revealed granulomas consistent with sarcoid. The patient was again given a course of steroids. Subsequently, she was followed for anemia, hepatosplenomegaly, and urinary sediment containing red and white blood cells.
In December 1971, the patient was started on therapy with prednisone for hypercalciuria. She then noted swelling of her abdomen over four or five days without other symptoms. On admission for evaluation, her vital signs were stable. The skin was clear, and there was no adenopathy. The findings from cardiac and pulmonary examinations were normal. The patients abdomen was tensely distended, with a marked fluid wave, and the liver was not palpable. The spleen was felt 4 to 5 cm below the left costal margin. Renal calculi were noted on abdominal roentgenogram. Testing with PPD was negative. At paracentesis, pink ascitic fluid contained red blood cells at a level greater than 10®/ml and white blood cells at 400/ ml, with 11 percent polymorphonuclear cells, 67 percent lymphocytes, and 22 percent monocytes. The protein level was 5.6 g/dl, and the amylase level was 55 units/L (serum reference range, 20 to 140 units/L). Tuberculosis and fungal stains were negative. At laparoscopy, 3,800 ml of bloody ascitic fluid was removed. The surface of the liver and enlarged spleen were studded with yellowish lesions consistent with sarcoid involvement but were not biopsied.
The patient was discharged on therapy with prednisone, isoniazid (INH), and spironolactone. Over the next four months, her ascites completely resolved, and the size of the spleen decreased markedly. The dosage of steroids was tapered, but the isoniazid was continued for one year despite negative cultures. When last seen ten years later, the patient had no recurrence of her ascites and had been symptomatic only for renal stones.
Sarcoidosis is known to involve serosal surfaces, including pleura, pericardium, and peritoneum, and secondary serous efiusions have been noted in these areas. Less commonly, pleural and pericardial efiusions have been bloody. This report documents that bloody peritoneal efiusions may also occur in sarcoidosis.
Although the red blood cells in the ascites may originate from vessels damaged by rapid formation of new granulomas, this remains to be proven. The clinical course of sarcoid-induced ascites is surprisingly benign, and the condition resolved in five of the seven reported cases within a few weeks (Table 1). This is strikingly different from that seen in patients with ascites secondary to portal hypertension complicating hepatic sarcoidosis, where bleeding from the esophageal varices and death are common.
In any case, sarcoidosis should be added to the list of those diseases which in their clinical course may produce peritoneal involvement and bloody ascites.
Table 1—Ascites with Sarcoidosis
|Case, Sex, Race, Age (Yr)||
|Histologic findings of Noncaseating Granulomas||Treatment and Follow-Up|
|1, F, В, 391||Skin; pulmonary adenopathy||
|6 L of turbid yellow fluid||None||Omentum; peritoneal cavity; intestine||No treatment; died 4 yr later of cor pulmonale; no ascites at autopsy|
|2, M, ?, 24*||Parotid gland||
|4 L of yellow fluid||Negative||Peritoneum; lymph nodes; liver||Sodium restriction; ascites for 1 yr; controlled with prednisone|
|3, F, W, 28s||None||
|20 L of serous fluid||Negative||Spleen; pancreas; lymph nodes; liver||Anti-TV therapy; no recurrence at 2.5 yr|
|4, F, W, 634||None||Positive for therapy; active TV 30 yr ago||2 L of serous fluid||Negative||Liver; peritoneum; hernia sac||Anti-TV therapy 30 yr ago; no recurrence at 2 yr|
|5, M, W, 67*||None||Negative; positive 2 yr later||Unstated amount of turbid fluid||Negative||Peritoneum||Prednisone, 1 yr; ascites resolved; asymptomatic at 2 yr|
|6, F, B, 42||Parotid gland; liver pulmonary hilar lymph nodes||Negative||7 L of bloody fluid||Negative||Bone marrow; falciform ligament; liver; fallopian tubes; ovaries; uterine serosa; hernia sac||Anti-TV therapy, 6 wk; persistent ascites for 8 yr; no ascites past 2 yr|
|7, F, B, 29||Pulmonary hilar lymph nodes; uveitis||Negative||4 L of bloody fluid||Negative||Cervical lymph nodes||Anti-TV therapy, 1 yr; prednisone, 0.5 yr; no recurrent ascites at 10 yr|