Four weeks before she died of a complication of lupus erythematosus, Flannery O’Connor wrote: “The wolf, I’m afraid, is inside tearing up the place. I’ve been in the hospital 50 days already this year.”

By the time of her next recorded letter, three days later, she had received the rite of extreme unction.

I never met O’Connor, but I have never seen a patient with lupus without thinking of those words and of the woman.

The word “lupus” was appropriated whole to the medical vocabulary directly from the Latin; it means “wolf.” This autoimmune-system disease affects virtually every organ in the body. About 60 percent of patients have a red (erythematous) rash on the face, over the bridge of the nose and on the cheeks below the eyes, in a “butterfly” pattern — a shape similar to the facial markings of a wolf.

EVALUATING A CASE

I saw a young woman with lupus today. She doesn’t have the rash. But she does have extensive kidney disease and what appears to be a massively enlarged heart and, in just two months, she has gained 50 pounds, all of it water. She is, in essence, waterlogged, her tissues swimming in excess fluid, her heart bobbing and squeezing like a fist within a sac of liquid.

Her kidney disease is such that she is losing huge amounts of protein in the urine. Normal kidneys filter the total blood volume many times each day, losing into the urine only tiny amounts of protein. Her kidneys are leaking protein like a sieve. Without that protein, there is nothing to hold the plasma within the blood. The kidneys also are leaking water into all her tissues, including the pericardial sac that surrounds the heart.

After talking with her at length about her illness, I examine her. A soft, scratchy noise, synchronous with the heartbeat, confirms her inflamed pericardium. Her legs are grossly swollen. I press my fingers gently into the skin over her shins — the tips of my fingers sink in as though they were poking the soft belly of the puffy little doughboy in the TV ad. Several minutes later, as I leave, the deep pits are still there.

Prednisone, a form of cortisone, has been started. The hope is that in massive doses it will help seal the protein leaks of her kidneys and that diuretics will mobilize the 50 pounds of excess fluid. The immediate question is whether to use a needle to draw off the fluid from around her heart. The danger is that the fluid will restrict and restrain the heart’s action, like the cellophane wrapper on a box of Valentine candy, and jeopardize its all- important pumping.

I decide we should not attempt to remove the fluid now, reasoning that it will only reaccumulate: The dike has multiple holes in it — the water would seep back in. The cardiology fellows working with me disagree. They are in favor of the needle. I argue that we should wait for the drugs to work. It is often easier to do something rather than wait, but it’s not always best for the patient. We keep a sterile tray with a long pericardial needle in it at her bedside, just in case.

WHEN THE WOLF IS WITHIN

O’Connor had lupus for about 14 years before she imagined the wolf “tearing up the place.” The disease had begun in late 1950 with, as she spells it, “awthritus.” Arthritis, or joint pain, is present in 90 percent of patients with lupus. Just as common is an overwhelming fatigue, often accompanied by fever, loss of appetite and loss of weight.

The exact cause of lupus is uncertain, but it is known that in the course of the disease the body makes antibodies directed against and damaging to its own organs. The process amounts to a kind of immunological betrayal of its owner by the body, an absolute autoimmune revolution. Lupus is primarily a disease of women (90 percent of cases, with a peak incidence in the childbearing years). O’Connor’s father died of lupus, however, and today’s research confirms that there may be a genetic predisposition.

New drugs to suppress the immune system help slow progression of the disease and reduce the requirements for steroids, such as cortisone, with their side effects. But lupus is capricious, with both relapses and remissions. Statistically, the majority of patients can look forward to a time when they will be relatively free of problems.

But O’Connor had a severe case — clearly, the wolf was inside. About the time her lupus was diagnosed, the efficacy of ACTH and cortisone in arthritis was just becoming available. ACTH, a hormone that causes the body to secrete cortisone, was prescribed for O’Connor in the early 1950s. The effect of ACTH (and cortisone, which she took later) is to blunt the body’s response to the turncoat antibodies. The drugs worked — at a price.

ACTH and cortisone can have significant side effects: thinning of bone (osteoporosis); loss of muscle strength; a “moonlike” shape to the face; exacerbation of duodenal ulcers; altered metabolism; increased susceptibility to infections; mood changes, ranging from a feeling of well-being to depression and even psychosis. O’Connor was well-aware of these side effects as she wrote, “So far as I can see, the medicine and the disease run neck and neck to kill you.” But she also felt better on the medications and in 1960, after 10 years on steroids, she confessed, “It is better to be alive with joint trouble than dead without it. Amen.”

A HEROIC EFFORT

During the next week, as I see my own young lupus patient, I can tell, thank God and prednisone, that she is improving. She has lost more than 30 pounds, all of it water. The pericardial fluid has diminished. She’s going to make it. And she gives me a tired, heroic smile.

Heroic is, I think, the word for O’Connor, too. When the disease struck her she was 25, a graduate of the Iowa Writers’ Workshop, a fledgling writer just beginning to make a name for herself. Despite the wolf, the next 14 years were productive: She won three O. Henry prizes; the National Book Award came posthumously in 1971. Many of her best short stories were written during those years: A Good Man Is Hard to Find in 1953; Good Country People, 1955; Everything That Rises Must Converge, 1961; Revelation in 1964, just months before she died.

O’Connor endured, to use Faulkner’s term, despite the lupus. That she weathered the devastating side effects of ACTH and cortisone, continuing to write, slowly, painfully, makes her seem especially heroic to me. It’s one thing to have an acute, limited illness, to pass the bloody agonizing kidney stone and be done with it. It’s quite another to go to sleep with pain, to dream that it’s gone, then wake up to the same pain waiting like a wild beast at the foot of your bed. And to persevere despite the pain, feeling it flow like electricity out of your forearms along your sore wrists and out to your stiff fingers to discover precisely how the 26 letters of the alphabet plan to marry this time.

Lupus betrayed O’Connor like a sophisticated Trojan horse, one with a wolf inside. Once within the body’s gates the enemy was everywhere. Still, she prevailed even if it was only until her 39th year. And her life and words are a study in keeping on keeping on. It is in that spirit that I move to the next patient.