The Lupus Book: A Guide for Patients and Their Families, Third Edition (26 page)

When ascites is diagnosed, I usually arrange to have some of the fluid re-

moved for microscopic analysis and culture to help identify its source and cause, which, in turn, suggests appropriate management. Exudative ascites can be mistaken for a ‘‘surgical abdomen,’’ resulting in unnecessary surgery. If an infection is ruled out, ascites is treated with anti-inflammatory medication, gentle water pills (diuretics), and occasionally periodic drainage. Infections are treated with antibiotics.

MALABSORPTION

Have you ever felt that the food you eat isn’t getting into your system? Severe diarrhea with very low serum protein (especially albumin) levels is rare in SLE, but when it does occur, it should tip off the lupus specialist that protein is being lost through the intestine as a consequence of malabsorption. Abdominal pain

may also be present. Among those who suffer from malabsorption, 90 percent

are children; less than 1 percent of the adults with SLE malabsorb their food.

Protein-losing enteropathy
, as this symptom complex is called, is treated with corticosteroids.

LESS FREQUENT COMPLICATIONS: MESENTERIC

VASCULITIS, INFARCTION, AND BOWEL HEMORRHAGE

Jackie had a severe case of lupus, being treated with 40 milligrams of

prednisone a day, which her doctor wanted to raise. Jackie, however, had

become diabetic and developed high blood pressure already. Since she was

still getting used to having lupus, she was not ready for the increase in

medication. One morning, she developed a fever, severe abdominal pain,

and bloody diarrhea. Her doctor hospitalized Jackie and called in a surgical

consultant. The diagnosis of an acute abdomen was made and she was taken

to surgery. In surgery, her doctors discovered vasculitis along with a small

perforation that resulted from steroid use. Nutrition became a major prob-

lem, and peritonitis set in that seemed resistant to all antibiotics. Her sit-

uation is critical and her doctors are not sure she will make it. They are

hopeful that an experimental approach will help.

The Impact of Lupus upon the GI Tract and Liver

[139]

If cramping and abdominal pain are associated with vomiting, fever, and

bloody stools, an immediate call to the doctor is in order.
Mesenteric vasculitis
, or inflammation of the blood supply to the small and large intestines, is one of the most serious complications of lupus. It calls for urgent intervention, since it is life-threatening. Even in the best of hands, this condition carries up to an 80

percent mortality rate. Estimates suggest that 1 to 3 percent of lupus patients may develop this complication. Patients on steroids are especially at risk, since the hormone thins the lining of the bowel and makes it more susceptible to

perforation.

The blood supply to the bowel can also sustain blood clots in patients with

antiphospholipid antibodies (Chapter 21), which also leads to
mesenteric infarction
. This implies that not enough oxygen is getting to the bowel tissue because of an interruption in the blood supply. Both mesenteric vasculitis and

antiphospholipid antibodies can induce mesenteric infarction.

Since the body cannot survive for more than a week or two with infarcted or

‘‘dead’’ bowel, surgical removal of this tissue is necessary. The best way for a doctor to approach mesenteric vasculitis or infarction is to be on the lookout

for it. Few rheumatologists who regularly treat lupus patients see this compli-

cation more than once every 2 to 3 years. Additionally, if vasculitis is evident, costicosteroids are given; if a clot is present, the patient is given anticoagulants after surgery.

THE BILIARY TREE

Bile is formed in the liver, stored in the gallbladder, and drained into the duodenum of the small intestine through bile ducts. Systemic lupus rarely affects

this region except for the pancreas. The pancreas is a secretory and endocrine

organ. It makes digestive enzymes that help the intestine break down food and

empty it into the duodenum through the biliary tree. As an endocrine organ, the pancreatic islets manufacture insulin.

WHAT ABOUT THE PANCREAS?

Wendy thought she would die. Although her lupus was under reasonably

good control with steroids and Imuran, she never knew back pain could be

as bad as this was. It occurred suddenly after an evening of drinking beer

with her college roommate, who had just broken up with Wendy’s former

boyfriend. At 3 A.M., Wendy noticed the sudden onset of searing back pain

and knew she had overdone it. Her roommate took her, screaming in pain,

to the emergency room, where her serum amylase was 2000 and a diagnosis

of acute pancreatitis was made. Dr. Dietz was not sure if the pancreatitis

was from her prednisone, Imuran, alcohol abuse, or lupus. Her rheumatol-

ogist was asked to see Wendy. She stopped all medication except for in-

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Where and How Can the Body Be Affected by Lupus?

travenous steroids; she also ordered intravenous fluids, pain medicine, and

nasogastric suction. It took a week for Wendy to start coming around, and

the culprit was not active lupus but either Imuran (which was not resumed)

or life-style habits. Wendy was told that she could not have any alcohol at

any time for the next few years, and she has adhered strictly to this regimen.

I hope you never experience the severe midabdominal pain radiating to your

back that Wendy had, which is associated with nausea, vomiting, and fever. In

lupus, several mechanisms contribute to this extremely painful form of pancre-

atic inflammation known as
pancreatitis
.

Certain agents used in the management of lupus have been found to provoke

pancreatitis. These include corticosteroids, azathioprine (Imuran), and thiazide diuretics (e.g., hydrochlorothiazide, Dyazide). Vasculitis of the pancreatic blood supply is the second most common cause of pancreatitis in SLE. One can also

develop pancreatitis for the same reasons that otherwise healthy people do—

alcohol abuse, gallstones, and physical trauma to the back where the pancreas

is.

Ultrasounds or CT scans are obtained to look for gallstones or ‘‘cysts’’ in

the pancreas (pancreatic pseudocysts), which could be perpetuating the problem.

The management of pancreatitis is quite problematic. Since pancreatic vas-

culitis is treated with steroids and steroid-induced pancreatitis is treated with steroid withdrawal, this serious process is first approached with general measures until the cause is determined. These include giving you nothing by mouth, supplying intravenous hydration, putting a tube through your nose that goes into

the intestine to remove secretions, and administering pain medicine. If previ-

ously prescribed, thiazide diuretics and azathioprine are discontinued. If generally active lupus is evident, steroid doses can be briefly but greatly increased to treat presumed pancreatic vasculitis. Should steroids be found culpable, they are tapered but cannot be abruptly discontinued. In spite of aggressive measures,

recurrence is common and the process can go on for months.

HOW DOES LUPUS AFFECT THE LIVER?

How can you tell if your liver is involved? Most of the time, no symptoms or

signs are evident until advanced disease is present. On occasion, right-sided

upper abdominal pain of distension, fevers, or a yellow appearance are clues.

Lupus activity in one form or another in the liver is evident in numerous ways

that are delineated below.

Involvement of the liver in SLE is a frequently misunderstood complication

of the disease. It can be affected as a result of both lupus and medications used to treat inflammation. The concepts of what constitutes
autoimmune (lupoid)
The Impact of Lupus upon the GI Tract and Liver

[141]

hepatitis
has undergone many changes since it was first described in the 1950s.

This section attempts to reconcile our differing perceptions of what ‘‘lupus in the liver’’ really means.

Enlargement of the liver
, or hepatomegaly, is found in 10 percent of patients with SLE. The liver is rarely tender unless the enlargement is so great that the capsule or covering of the organ is stretched. The most common causes of large

livers in lupus include autoimmune hepatitis, ascites, congestive heart failure, or a complication of a large spleen, whose materials drain into the liver.

Jaundice
, the condition we think of as turning a patient yellow, is seen in 1

to 4 percent of patients with lupus. Manifested by high serum levels of bilirubin, which are responsible for that yellow pigmentation and itching, jaundice results from autoimmune hemolytic anemia, viral hepatitis, cirrhosis, or bile duct obstruction from gallstones, tumor, or pancreatitis. Occasionally, certain medications, including NSAIDs and azathioprine, may produce jaundice.

Hepatic vasculitis
, or inflammation of the small and medium-sized arteries of the liver, is extremely rare and is noted in just one lupus patient per thousand.

It responds to corticosteroids.

Budd-Chiari syndrome
results from a blood clot in the portal veins, which drain materials from the liver into the vena cava. Those patients with antiphospholipid antibodies appear to be uniquely at risk for developing these clots.

Additionally, hepatic artery clots may occur. Untreated Budd-Chiari syndrome

can lead to ascites, elevated liver pressure (called portal hypertension), and liver failure. The preferred treatment of Budd-Chiari syndrome is anticoagulation

(blood thinning).

Ascites
, already discussed, may also reflect liver failure.

Why Are Liver Blood Tests Abnormal?

Abnormal liver function tests
may be found in 30 to 60 percent of patients with lupus at some point and cause no symptoms. Blood enzyme evaluations included

in routine blood panels such as the AST (also called SGOT), ALT (also called

SGPT), alkaline phosphatase, and GGT may be elevated from a variety of mech-

anisms.

First of all, nearly all NSAIDs as well as acetominophen (Tylenol) can elevate

these enzymes, and lupus patients—for unclear reasons—appear to be particu-

larly susceptible to this. These abnormalities are usually of little consequence and generally represent false alarms, unless they are greater than three times

normal. Also, active lupus can elevate these enzymes. Most nonsteroidals can

be stopped for a week or two and the enzymes rechecked. If they remain in-

creased, the possibilities for this elevation include hepatitis, infection, biliary disease, cancer, pancreatitis, alcoholism, or active lupus.

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Where and How Can the Body Be Affected by Lupus?

What Is Autoimmune (Lupoid) Hepatitis?

Amanda did not feel right. She complained of vague right-sided upper

abdominal discomfort for several months before seeing her doctor. Her

examination demonstrated tenderness in this area, a low-grade fever, and

distension. Blood tests revealed sky-high liver function studies. She was

referred to a gastroenterologist, who embarked upon a workup that included

a liver biopsy. It demonstrated chronic active hepatitis. Since Amanda did

not have a history of viral hepatitis or alcohol abuse and since her hepatitis

virus, cytomegalovirus, toxoplasmosis, and other tests were negative, auto-

antibodies were examined. These were consistent with autoimmune hepa-

titis. Amanda responded to steroids at first, but after 3 years began devel-

oping abdominal swelling, rectal bleeding, and mental slowing. Imuran was

added to her prednisone as an immune suppressive regimen, to which

Amanda responded. A special diet was strictly enforced and mild water

pills were prescribed. This prolonged matters for another year, until it was

evident that Amanda was experiencing liver failure. At that point she un-

derwent a liver transplant and is doing fine 3 years later on prednisone,

Imuran, and cyclosporine A.

Lupoid hepatitis
is a complicated and controversial entity. Described in 1955

and coined by Ian Mackay in 1956, lupoid hepatitis has undergone many

changes in definition. The overwhelming majority of patients who were told

they had lupoid hepatitis between 1955 and 1975 would not fulfill current

criteria. Initially thought of as the presence of chronic active hepatitis (‘‘hepatitis’’ means inflammation of the liver) with lupus erythematosus cells, the

term
autoimmune hepatitis
seemed more appropriate, since few of these patients had typical clinical lupus. Patients with autoimmune hepatitis first notice right upper abdominal pain along with malaise, nausea, aching, and low-grade

fevers. Loss of appetite, light-colored stools, and dark urine may also be present.

The development of diagnostic tests to detect hepatitis A, B, and C has

changed our concepts of autoimmune hepatitis. The current working definition

of autoimmune hepatitis is (1) liver disease consistent with chronic active hepatitis; (2) absence of evidence for active hepatitis virus A, B, or C infection; and (3) a positive ANA or other autoantibodies associated with the syndrome.

Even using these criteria, only 10 percent of patients at the Mayo Clinic with

autoimmune hepatitis fulfilled the American College of Rheumatology (ACR)

criteria for lupus (Chapter 2). Many of the physical findings we associate with SLE (rashes, other organ involvement) are usually absent. Since lupus patients

have compromised immune systems and can develop a viral hepatitis, take med-