Lupus Q&A Revised and Updated, 3rd edition: Everything You Need to Know - Softcover

Lahita, Robert G.; Phillips, Robert H.

Softcover

ISBN 10: 1583335455 ISBN 13: 9781583335451

Publisher: Penguin Publishing Group, 2014

Synopsis

A revised and updated edition of the bestselling resource for lupus patients, their families, and medical professionals
 
A perennial bestseller, Lupus Q&A is the go-to guide for sufferers of a chronic autoimmune disease that affects more than 1.4 million people in the United States alone. Characterized by achy joints and skin rashes, lupus often mimics other diseases, making it tricky to diagnose and treat. In this completely revised and updated edition, Dr. Robert Lahita and Dr. Robert Phillips—leading experts on lupus—discuss topics in a clear, concise, and easy-to-follow Q&A format.
 
Lahita and Phillips review the newest drugs and explore beneficial complementary and alternative treatments, including new data on hormone use. Demystifying everything from diagnosis to the disease’s psychological impact, Lupus Q&A prepares readers to face the challenges ahead—and to restore their health and their lives.

About this title

About the Author:

Robert G. Lahita, M.D., Ph.D., is the chairman of the Department of Medicine at Newark Beth Israel Medical Center. He has been internationally recognized for his clinical research in systemic lupus erythematosus and autoimmune disease, and is the recipient of numerous research grants from the Primus Foundation, the Lupus Foundation of America, and the National Institutes of Health. Dr. Lahita lives in Newark, New Jersey.

Robert H. Phillips, Ph.D., a clinical psychologist, is the director of the Center for Coping on Long Island, NY. He is internationally recognized for his work helping people with chronic medical problems, is the author of more than 35 books, and has presented at meetings all over the world. A former board member of the Lupus Foundation of America, he is on the national board of the American Autoimmune Related Diseases Association.

Excerpt. © Reprinted by permission. All rights reserved.:

Original Foreword

It is my great pleasure to write a foreword to this book, dedicated to the patient with lupus and written by Dr. Robert Lahita, who has done so much for my family, in conjunction with Dr. Robert Phillips.

Autoimmune disease, a topic that is very important to my family, can strike anyone at any age. One of the great mysteries of our time, this category of illness inflicts psychological and physical damage upon its sufferers. Systemic lupus erythematosus, in particular, has caused untold devastation and suffering for lupus patients and their families. It is also a disease that is harder to diagnose than it is to pronounce. This is why it is important to have a book like this one—solid and comprehensive, yet simple and direct. It answers questions for patients, for their families, and perhaps even for their doctors. The authors have taken particular aim at the public interest in this disease that affects women most, but has an impact on people of every gender, of every age, and of every race, and in doing so have given some attention and interest to a disease that affects many but about which so little is known. I know, for this disease has touched, and will doubtless continue to touch, the members of my family.

George joins me in wishing all patients and their families well and in hoping that Lupus Q & A: Everything You Need to Know will help many people to understand and cope with this dreaded disease.

—BARBARA BUSH

Former First Lady

Houston, Texas

Preface

There is no more difficult disease to diagnose, understand, or treat than the disease called systemic lupus erythematosus. This may be because lupus is not one disease but many diseases grouped under one heading. It may also be because the disease can present itself to both physicians and patients in mysterious ways, throwing them off the track, leading them to think of other more common illnesses, and eluding standard diagnostic methods. Whatever the reason, lupus is complex and problematic.

This book was designed to answer the many questions you may have about this disease and its impact on your life. Many of the questions are based on the countless numbers of patients who enter our clinics and offices daily with long lists of questions, the letters that arrive weekly in the mail seeking answers, the questions we hear at conventions or meetings, or the sad phone messages that ask for help because “my doctors don’t understand the disease.” This is not a companion to any textbook. It is written strictly for patients and is based on their needs and questions. The goal was not to educate doctors with the material in this book (although many will find it helpful) but rather to address patients’ very real questions. We also realize that despite the dozens of questions answered in the book, certain areas may have been inadvertently overlooked. However, our goal was to address questions that, according to our experience, are most on the minds of our patients.

Ironically, the disease lupus seems to have gotten more complex, not less, over the years. New knowledge about the immune system and its workings has led to other autoimmune diseases being added to the roster of problems that need resolution. For example, because of difficulties in classification, a disease such as autoimmune phospholipid syndrome is often given the label “lupus.” Some physicians label it lupus in order to give the disease a billing code acceptable for insurance reimbursement! Diseases such as autoimmune phospholipid syndrome have resulted in swelling numbers of lupus patients. The Lupus Foundation of America has estimated that some 2 to 2.5 million Americans believe that they have lupus, and that some 86 percent of Americans have heard of the disease. While these are staggering numbers, one must be cautioned to remember that they may reflect the fact that many illnesses that are not lupus are being called lupus. Hopefully, this book will help to clarify the reasons that the numbers of people with lupus are increasing.

Last, a bit of history is necessary to allow readers to understand this disease and its past. Let’s review the “timeline” of lupus.

   · Lupus got its name because it was originally thought to represent the wounds that resulted from being attacked by a wolf. The trademark “butterfly rash” was thought to be from the bite or scratches of a wolf.
   · This butterfly rash on the malar part of the face (above the cheeks) was first mentioned in the thirteenth century.
   · The actual term lupus erythematosus was first mentioned by a fellow named Cazenave in 1851.
   · There was much confusion regarding the diagnosis of lupus until well into the twentieth century. It was often confused with tuberculosis, disseminated gonorrhea, and many skin disorders.
   · Only in the 1930s and 1940s did pathologists look at organs such as the kidneys and skin and realize that common changes in these organs had certain similarities in patients with lupus. Together these formed the typical aspects of lupus.
   · Immunology was in its infancy in the 1930s and 1940s, and the classical description of antibody structure was not to take place for some decades. No one knew the mechanism through which lupus could so globally damage so many organs of the body. No one really understood this newly described illness, which counted rash, kidney failure, and sun sensitivity among its list of characteristics.
   · In the 1940s came the association with the false-positive test for syphilis, the discovery of the LE cell, and the idea that these phenomena might have something to do with “blood proteins.” These proteins were later called antibodies, and it was suggested that they might be reacting with normal tissues. All of these discoveries were important in the understanding and diagnosis of lupus.
   · Coincidentally, the discovery of cortisone in 1948 by Philip Hench provided the first and greatest therapy for lupus.
   · The 1950s brought the fluorescent antinuclear antibody assay, an important test in the diagnosis of lupus, and the discovery of autoantibodies such as Sm and RNP. These autoantibodies form the basis for our understanding of the disease process of lupus—how we diagnose it and a small bit about how the disease affects the body. In addition, the 1950s brought about important insight into the genetics of the disease. All of this added significantly to knowledge about lupus.
   · Since the 1950s, much research has focused on the following areas: molecular genetics (in order to learn more about the immune response), hormones and their importance, and, more recently, the development of several new drugs. Although lupus research has come far in the past twenty-five years, it will take an understanding of the cause of the disease—currently unknown—in order to develop a targeted cure.
   · In the early 1980s a new condition called antiphospholipid syndrome was described that in many cases is inexorably linked to lupus. It is troubling because it causes bleeding and clotting. It is usually a condition of “sticky blood” that can result in blood clots in the lungs or the brain. The addition of factors concerning the syndrome resulted in a revision of the criteria for the classification of lupus.
   · In the first decade of the twenty-first century came a number of significant advances, which are detailed in this book. These include the use of newer biological agents for lupus such as belimumab and rituximab. Newer drugs previously used for transplant of organs came to use as well.

Despite all of the history of this disease, we still have a long way to go in our understanding of lupus and related illnesses. However, we have made strides in the last thirty years that have revolutionized the way we examine many illnesses. Great discoveries are yet to happen. Let us hope that this book will continue to enlighten patients as we move ahead.

—ROBERT LAHITA, M.D., PH.D.

Newark Beth Israel Medical Center

Rutgers, the New Jersey Medical School

Newark, New Jersey

—ROBERT H. PHILLIPS, PH.D.

Center for Coping

Long Island, New York

1

Lupus: An Overview

Lupus is a complex disease. So, of course, once patients are diagnosed with lupus, they have many questions about this puzzling disorder. This chapter will introduce you to the basics of lupus. First, we will answer the question, “Just what is lupus?” We will then discuss the different types of lupus, the causes of the disease, and common myths surrounding the disorder.

The Nature of Lupus

WHAT IS LUPUS?

Lupus is an autoimmune disease. The immune system, which normally protects the body, turns against the body (auto) and attacks it. Lupus has no known cause and, as a result, no known cure. The disease can affect many different systems of the body, and there are many different ways that it can affect people.

WHY IS IT CALLED LUPUS?

In the early twentieth century, most physicians thought lupus was a skin disease. The disease got its name because many patients looked as though they had been bitten or scratched by wolves. Lupus is Latin for “wolf.”

WHY IS LUPUS REFERRED TO AS A CHRONIC DISEASE?

Lupus is considered a chronic disease because it is currently incurable. Once one experiences lupus symptoms, one has lupus forever. However, certain forms of lupus—such as the drug-related form—don’t fit into this incurable category because once the drugs that have “caused” the lupus are withdrawn, the lupus symptoms go away.

CAN LUPUS BE BOTH ACUTE AND CHRONIC?

Yes. Lupus is a chronic disease but can have acute episodes. As a chronic disease, lupus often has a slow onset, and it is ongoing and incurable. The immune system—unable to rid itself of what is perceived as a foreign substance—continues to react against the “foreign tissue.” This can be a significant problem, lasting for quite a while—possibly up to many months—and can be accompanied by signs and symptoms of other chronic illnesses.

But a patient can experience acute episodes, times when symptoms can worsen in immediate, abrupt, and occasionally severe ways. These acute manifestations, known as flares, can often occur this way, even in those who have had the disease for many years.

It’s like a simmering pot of soup that all of a sudden bubbles over, or like a volcano that simmers and all of a sudden pops its lid but then goes back down and simmers for another twenty years.

WHAT IS A CONNECTIVE TISSUE DISORDER?

This is the former name for lupus and other related diseases. In the past, any disorder involving the muscles, tendons, and, in some cases, even bones, used to be referred to as a connective tissue disease. It was once believed that the inflammation of connective tissue occurred only from overuse. We now know that connective tissue can be inflamed for a variety of reasons, some of which have nothing to do with overuse.

WHAT IS MIXED CONNECTIVE TISSUE DISEASE?

Mixed connective tissue disease is referred to as an “overlap syndrome” because there seems to be an overlap of several different diseases or symptoms, suggesting more than one disease. There are no specific signs or symptoms of overlap disease. To help doctors make this diagnosis, mixed connective tissue disease was originally associated with the presence of an antibody or specific protein in the blood called ribonucleoprotein antibody (RNP).

Some experts think that this overlap syndrome may be related more to a disease called scleroderma than to lupus. Many people with lupus have the anti-RNP antibody, but one must have very high titers, or strength, of the antibody for mixed connective disease to be present. So the best way to describe mixed connective tissue disease is to call it a hybrid illness.

What Happens in Lupus

WHAT EXACTLY HAPPENS IN LUPUS?

It is difficult to provide an accurate answer to this question. The key to understanding what happens in lupus is understanding the job of the immune system. The immune system is designed to protect the body from foreign invaders called antigens. The problem is that in lupus, the immune system cannot distinguish certain “self” tissues from foreign invaders and thus attacks inappropriate targets. So, for some reason, the immune system has a problem correctly identifying antigens and turns against the body that it is designed to protect.

WHAT ARE THE MAIN COMPONENTS OF THE IMMUNE SYSTEM?

The immune system is primarily made up of three categories of cells—B lymphocytes (commonly called B cells), T lymphocytes (T cells), and phagocytes. These three groups of cells form the “soldiers” of the immune system, designed to protect the body from foreign invaders (antigens).

WHAT EXACTLY IS AN ANTIGEN?

Antigens are any substances, produced inside the body or coming from outside the body, that the immune system recognizes as being foreign—such as germs, bacteria, viruses, or fungi. In other words, an antigen is any substance that can trigger an immune response—an effort made by the immune system to eliminate an unwanted foreign invader. In lupus, the immune system suddenly and for no apparent reason turns against the body’s own cells and tissues, mistaking them for foreign invaders, and tries to destroy them as it would other foreign matter. So the body is now reacting to autoantigens (self-antigens).

WHAT IS AN AUTOANTIGEN?

An autoantigen is a substance that occurs naturally within the body—in other words, it is not really foreign but is a “self” substance—but, for some reason, is identified by the immune system as foreign. The autoantigen then triggers an immune response, and the body fights it the way it would normally fight off foreign substances with antibodies. This is called an autoimmune response.

WHAT ARE ANTIBODIES?

Antibodies are proteins that are among the five major classes of immunoglobulin molecules, protein molecules that work to kill foreign substances. They originate in the immune system’s B cells in response to the presence of the foreign substances (antigens), for the primary purpose of destroying them. B cells mature to become plasma cells, or those cells that make antibodies.

WHAT ARE AUTOANTIBODIES?

As you now know, antibodies are molecules that normally defend the body against foreign substances. Autoantibodies are the names for antibodies that fight healthy tissues within the body. No two patients have autoantibodies that have exactly the same target, although in families, two members with the disease may have autoantibodies that look very similar.

WHAT DO ANTIBODIES DO?

Antibodies attach themselves to foreign substances until the combination of antibody and foreign substance (the immune complex) is engulfed by a scavenger cell, called a phagocyte. This cell usually digests the immune compl...

About this edition

• Publisher: Penguin Publishing Group
• Publication date: 2014
• ISBN 10: 1583335455
• ISBN 13: 9781583335451
• Binding: Paperback
• Number of pages: 304