302 Brief Case Reports
1, 2000
Rapid Onset of Massive
Ascites as the Initial Presentation
of Systemic Lupus Erythematosus
Pete J. Weinstein, M.D., and Charles M. Noyer, M.D.
Division of Gastroenterology and Department of Medicine,
Jacobi Medical Center and Albert Einstein College of Medicine,
Bronx, New York
ABSTRACT
Ascites in systemic lupus erythematosus (SLE) is rarely
massive, and either accompanies the typical manifestations
of active disease or results from nephrotic syndrome, pro-
tein-losing enteropathy, constrictive pericarditis, and condi-
tions unrelated to lupus. Marked ascites has been atrributed
to chronic lupus peritonitis, characterized by the insidious
onset of massive, painless ascites and unrelated to disease
activity. Regardless of the etiology, ascites typically has a
gradual onset and occurs after a diagnosis of SLE has been
made. We describe a young woman presenting with the
rapid development of massive ascites as the initial manifes-
tation of SLE. (Am J Gastroenterol 2000;95:302–303.
© 2000 by Am. Coll. of Gastroenterology)
CASE REPORT
A 33-yr-old woman from Guyana, with no significant med-
ical history, was admitted to Jacobi Medical Center with 1
wk of severe, constant, sharp periumbilical pain and increas-
ing abdominal girth. She noted watery diarrhea, nausea,
anorexia, low grade fever, and a 10-pound weight loss. She
denied a history of hepatitis, jaundice, or alcohol or illicit
drug use, and took no medications. On physical examina-
tion, she appeared uncomfortable, had a temperature of
100.4 °F, tense ascites, and diffuse abdominal tenderness,
but no palpable liver or spleen, adenopathy, rash, jaundice,
peripheral edema, or asterixis. She had loose, brown stool
testing hemoccult positive. White blood cell count was
Figure 1. On the left (A), the appendix at low power has a normal mucosa with evidence of serositis and vasculopathy (hematoxylin and
eosin, ⫻40). High power view on the right (B) reveals vasculopathy (smooth muscle thickening) and vasculitis with thrombosis
(hematoxylin and eosin ⫻100).
AJG – Vol. 95, No.
200/mm3 with 15% eosinophils, Hb 9.3 g/dl, albumin 2.7
mg/dl, cholesterol 156, and ESR 20 mm/h. Liver tests,
amylase, BUN (11.0 mg/dl), creatinine (0.6 mg/dl), and
urinalysis were normal; tests for HbsAg, HCV, and HIV
were negative. She was anergic. A CT scan demonstrated
massive ascites, thickening of the omentum and small bowel
wall, and normal liver, spleen, and pancreas. An echocar-
diogram and Doppler ultrasound of the hepatic, portal, and
mesentric veins were normal. Abdominal paracentesis
yielded clear, yellow fluid with an albumin of 1.9 g/dl
(serum-ascites albumin gradient of 0.8), amylase of 17 IU/L,
and white blood cell count of 278/mm3 (45% neutrophils,
39% lymphs, 16% monos). A repeat ascitic cell count had
17% eosinophils. Ascitic fluid cytology and culture for
bacteria and AFB were negative, as were stool for ova and
parasites, as well as, toxocara and strongyloides IgG4 se-
rologies. After antibiotics and repetitive paracenteses failed
to relieve her symptoms, an exploratory laparotomy was
performed, revealing a large amount of ascites and a hyper-
emic appendix studded with white nodules. The liver ap-
peared normal and a biopsy showed mild fibrosis, portal
inflammation, and no granulomas. The resected appendix
had a normal mucosa, acute and chronic serositis with
marked eosinophil infiltration, and small vessel vasculitis
with thrombosis and smooth muscle thickening (Fig. 1).
Anti– double-stranded DNA and ANA were positive, C3
was 23.7 (normal 50 –160), C4 7.9 (normal 5–35), and CH
10.4 (normal 100 –300). A diagnosis of SLE was made, and
the patient received methylprednisolone. Cyclophospha-
mide was added when pancytopenia, nephrotic range pro-
teinuria, renal insufficiency, and recurring fevers developed.
The patient improved, and was discharged with minimal
ascites and mild renal insufficiency.
DISCUSSION
This patient presented with the acute onset of massive
ascites as the initial manifestation of SLE. Unlike most
AJG – January, 2000
patients with SLE and ascites, our patient, when diagnosed,
had a normal urinalysis and renal function and no typical
manifestations of SLE. This case is unique in that, as shown
by the serositis seen at pathological review, the low serum-
ascites albumin gradient, the exclusion of other causes of
ascites, and the pain preceding the onset of ascites, lupus-
related serositis caused the patient’s massive ascites.
Marked serosal exudation in conjunction with a reduced
peritoneal absorptive capacity could have facilitated the
rapid accumulation of fluid.
Ascites occurs in 8 –11% of patients with SLE, usually
associated with nephrotic syndrome, protein-losing enterop-
athy, constrictive pericarditis, congestive heart failure, or
Budd-Chiari syndrome (1). Cirrhosis, pancreatitis, perito-
neal carcinomatosis, and tuberculous peritonitis should also
be excluded. Serositis, found at autopsy in 63% of patients
with SLE, rarely causes significant ascites (2). Abdominal
pain in patients with active lupus is often attributed to
serositis; yet, patients with histologically documented se-
rositis have minimal ascites (3). Massive ascites has not
been described in this setting. Since Dubois stated, in 1974,
that lupus peritonitis or serositis alone does not cause sig-
nificant ascites, chronic lupus peritonitis has been reported
as causing massive ascites (4 –11). In these patients, SLE
was usually diagnosed years earlier, and disease activity did
not correlate with the course of ascites (6, 8). Abdominal
pain was not a significant feature but when present, was
attributed to massive distention. Ascitic fluid characteristics
included white blood cell counts ranging from 10/mm3 to
1630/mm3 (mean 393/mm3 with predominantly lymphs or
monos), fluid protein ranging from 3.4 mg/dl to 4.7 mg/dl
(mean 3.9 mg/dl), and a serum-ascites albumin gradient
⬍1.1. Several patients had detectable anti-DNA antibodies
and LE cells in the ascitic fluid (10). In the patients under-
going laparotomy or laparoscopy, the peritoneum appeared
hyperemic and thickened, as nodular or adhesive, or as
grossly normal (6, 8 –11). The peritoneal histology revealed
chronic inflammation and small vessel vasculitis. Immuno-
fluorescence studies in several patients showed complement
and immunoglobulin deposits in the walls of peritoneal
blood vessels. Corticosteroid therapy effectively controlled
ascites in most cases, but 50% required an additional im-
munosuppressive agent to manage incomplete responses or
recurrence.
Brief Case Reports 303
Our patient’s presentation does not match the clinical
description of chronic lupus peritonitis, as the ascites accu-
mulated rapidly during an initial lupus flare, the abdominal
pain was severe, and there was no evidence of underlying
SLE. In conclusion, this is a unique case of SLE presenting
with the acute and rapid onset of massive ascites due to
lupus serositis/peritonitis. SLE should be considered in the
differential diagnosis of new onset ascites, after the typical
causes have been excluded.
Reprint requests and correspondence: Charles M. Noyer, M.D.,
Department of Medicine, 7NW, Jack D. Weiler Hospital of the
Albert Einstein College of Medicine, 1825 Easchester Road,
Bronx, NY 10461.
Received Sept. 8, 1998; accepted Feb. 26, 1999.
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