Amyloidosis is a group of diseases which have in common the accumulation of abnormal protein in various organs. It is best known in Pennsylvania as the illness that afflicted Governor Robert Casey and Mayor Richard Caligiuri. The symptoms experienced by patients depend on the type of protein in a particular case and the organs involved.

Rheumatologists first became interested in amyloidosis because it can occur as a result of any chronic inflammatory process, such as rheumatoid arthritis, ankylosing spondylitis, or chronic infections. Only a minority (<1%) of rheumatic disease patients develop this complication, and usually only those whose inflammation is not well controlled. Amyloidosis most often affects the kidneys, resulting in increased amounts of protein in the urine and kidney failure. It may also involve the adrenal glands or intestines. The diagnosis can be made by biopsying the involved organ. A simple way to make the diagnosis is to remove a small amount of fat from under the skin. This type of amyloidosis, called secondary amyloidosis (also called AA amyloid), is treated by suppressing the inflammatory process as aggressively as possible. A trial is under way to test a drug which may dissolve this type of amyloid, hopefully leading to improved organ function.

The most common form of amyloid is AL, or light chain-associated amyloid. This condition results from an abnormal protein produced by cells in the bone marrow called plasma cells. It is a severe form of amyloidosis which can involve almost any organ, but especially the heart, kidney, lung, nerve, and intestine. To destroy the cells that produce the abnormal protein, patients are treated with chemotherapy or bone marrow transplantation.

Familial amyloidosis is the result of accumulation of a protein resulting from the process of mutation. The symptoms of a particular form of familial amyloidosis depends on the ethnic origin of the family involved and even on the individual family involved. One of the common forms (although it is very rare) is found among Portuguese families and mainly involves the nerves and heart. Because the abnormal protein is produced in the liver, this form can be treated with liver transplantation.

Amyloid proteins of many types are of great interest to scientists trying to find the cause of a variety of illnesses. Alzheimer’s disease is a progressive debilitating neurologic disease characterized by memory loss. Biopsies of the brain in patients with Alzheimer’s show clumps of nerve cells enveloped in amyloid protein, which may be the result of chronic inflammation. Amyloid is found in the pancreas of those with adult onset diabetes and may play a role in that disease. One form of amyloidosis develops in patients on kidney dialysis and can result in shoulder pain, carpal tunnel syndrome, and abnormal fractures.

Successful treatment of amyloidosis depends on early and precise diagnosis to determine the exact form of protein involved.

Alan M. Berg, M.D.

activities that are made more difficult by arthritis. For couples in this situation, it may be beneficial for the spouse to learn more about arthritis. Researchers at the University of Pittsburgh have in fact found that some individuals with OA feel more confident in managing their symptoms if their spouse or significant other attends a course with them on strategies for managing arthritis. Furthermore, many spouses report that learning more about OA through a course helps them to be more supportive.

There are many options in the Pittsburgh area for learning about arthritis and its treatment. If you or your spouse/significant other have been diagnosed with OA and you want to learn more about strategies for managing arthritis, please call (412) 624-5532 or write to the Arthritis Education Project, 121 University Place, Pittsburgh, PA 15260.

Lynn Martire, Ph.D.

Rod Rutkowski, social worker at the Arthritis Rehabilitation Unit at UPMC St. Margaret for 20 years, received the Addie Thomas Service Award, the national award given to an ARHP member in recognition of "meritorious service to the rheumatology community." Rod was cited for his many contributions over the years, including his work for the Arthritis Foundation, the ARHP (national president several years ago) and his dedication to his patients. Over 10 years ago he organized a now nationally recognized "Men with Arthritis" support group at UPMC St. Margaret Hospital.

This year, the UPMC Arthritis Network has grown to 28 rheumatologists serving the greater Pittsburgh area. Each of the practice groups has gained one physician. We are pleased to introduce them to you now.

Christiane L. Arsever, M.D., has joined Arthritis and Internal Medicine Associates-UPMC. After earning her bachelor’s degree and her medical degree from the University of Michigan, Ann Arbor, Dr. Arsever completed a residency in internal medicine at Northwestern University Medical Center, Chicago, and a three-year fellowship in rheumatology at the Rackham Arthritis Research Unit of the University of Michigan Medical Center, Ann Arbor. In 1998-99, Dr. Arsever was listed in The Best Doctors in America. Dr. Arsever is board-certified in internal medicine and rheumatology. Her office is located at 3500 Fifth Avenue, 4th Floor, Pittsburgh, PA 15213, telephone (412) 682-2434.

C. Kent Kwoh, M.D., is a familiar name to some of us. For eight years, Dr. Kwoh taught at the University of Pittsburgh as a faculty

member in the Division of Rheumatology and Clinical Immunology. He is technically "new" because he rejoins us this year after spending the last six years in Cleveland, specifically at Case Western University in the Division of Rheumatic Diseases, and as a staff physician at the Cleveland VA Medical Center. In 1997, Dr. Kwoh became the director of the Institute for Health Care Research at Case Western and the director of the Program for Health Care Research at the VA. Dr. Kwoh is now Professor of Medicine, serving as the director of Clinical Research, University of Pittsburgh Arthritis Institute. His office is located at 3471 Fifth Avenue, Suite 900 Liliane Kaufmann Building, Pittsburgh, PA 15213, telephone (412) 648-6970.

Pamela R. Neish, M.D., has joined Margolis Rheumatology Associates-UPMC. Dr. Neish earned her bachelor’s and medical degrees from the University of Pittsburgh, after which she completed a residency in internal medicine and a fellowship in the Section of Rheumatology and Immunology at Vanderbilt University Medical Center, Nashville, Tennessee. Dr. Neish is board-certified in internal medicine and rheumatology. Her office is located at 339 Sixth Avenue, 4th Floor, Pittsburgh, PA 15222, telephone (412) 281-1594.

One of our distinguished faculty, Chester V. Oddis, M.D., has recently received a Clinician Scholar Educator Award sponsored by the American College of Rheumatology Research and Education Foundation. This highly competitive award recognizes and supports innovative programs focusing on the education of medical students, residents, and fellows, with a specific emphasis on the mentoring process. Through educational funds provided by this award, Dr. Oddis has devised a teaching experience that will motivate students and young physicians to consider a career in clinical rheumatology and/or basic immunology research focusing on rheumatic diseases.

Dr. Oddis is Associate Professor of Medicine and has served on the University of Pittsburgh faculty for 14 years. He has been recently honored by the University of Pittsburgh Medical School Class of 2002 with the Excellence in Education Award.

William Ridgway, M.D., Assistant Professor of Medicine, Division of Rheumatology and Clinical Immunology, is the recipient of a Pfizer Scholar Grant for new faculty. The grant, which provides $65,000 per year for three years, supports original research in rheumatology and immunology. Dr. Ridgway’s research focuses on autoimmune diseases, including diabetes mellitus.

As part of the UPMC Alive and Well series, two afternoons of programming were dedicated to fibromyalgia. Entitled "Fibromyalgia: What a Pain!", the program was originally scheduled for May 19th only, but the response was so overwhelming that a second afternoon was added on June 16th.

Prior to the lecture series, there was an opportunity for guests to mingle and collect informative brochures, newsletters, and flyers regarding support groups and exercise programs. The event was co-sponsored by the Arthritis Foundation, which generously supplied most of the reading material.

Terence Starz, M.D., opened the program with an overview of fibromyalgia. This was followed by a lecture entitled "Pharmacologic Intervention in Fibromyalgia" given by Doris Cope, M.D. Jane Brandenstein, PT, spoke next regarding the very important role of physical activity. She encouraged doing whatever sort of activity one can manage, because it is better than no activity at all. Rina Itskowitz, OTR/L, provided many useful tips for managing fatigue. She brought along tools to illustrate how modern mechanics can reduce strain on tired muscles. Dina Jones, MS, PT, took the audience through relaxation exercises during her talk on strategies to improve sleep. Ray Hanlon, Ph.D., delivered a lecture on stress management. The lecture series concluded with “Complementary Medicine Approaches to Managing Fibromyalgia” by Ron Glick, M.D. Finally, an expert panel closed the program by answering questions the audience had prepared.

Jennifer Jablon

Physicians in the UPMC Arthritis Network have developed a patient registry to aid their arthritis-related research. The purpose of the Registry is to develop a list of patients who have rheumatic diseases and who are interested in hearing about research studies in which they may be eligible to participate. The Registry is generously supported by a grant from the St. Margaret Memorial Hospital Foundation.

How does the Registry Work?

Patients are invited to participate in the Registry in one of two ways either by receiving a letter at their home address from their physician or by noticing the poster in their physician’s waiting room and completing the Registry consent form during an office visit. Thereafter, the patient’s name, address, date of birth, and rheumatic disease diagnosis are entered into a computer database. This confidential database is maintained by and solely accessible to the registry coordinator. When a researcher decides to do a rheumatic disease study, he or she can apply to the UPMC Arthritis Network Research Committee for permission to utilize this Registry. Before this committee agrees to review a proposal, the investigator must have approval from one of the local Investigational Review Boards (IRBs). These boards are composed of both professional and lay persons whose goal is to make sure that patients are given all of the information that they need (informed consent) before they agree to p articipate in a research study. The Research Committee has two or more representatives of each of the practices in the Network. After Research Committee approval is obtained, the researcher will receive the names and addresses of Registry participants with the appropriate diagnosis. Then either the researcher or a member of his/her research team will contact Registry patients with details of that study.

As participants in the UPMC Arthritis Network Registry, patients are under no obligation to participate in any of the studies. When agreeing to become part of the Registry, patients are merely consenting to being contacted with information about upcoming research studies. Patients may remain members of the Registry regardless of whether they have decided to join any studies in which they are invited to enroll. Registry participants receive both study announcements and informative newsletters such as this one.

Current Studies

The Registry is currently being utilized in eight different studies. Several researchers are testing strategies to improve rheumatoid arthritis and osteoarthritis patients’ ability to comply with treatment. An investigation is being conducted to determine the frequency of cardiovascular disease in women with rheumatoid arthritis. New osteoporosis drugs are being tested with the hope that they will increase bone mineral density. Exercises are being developed to aid in the rehabilitation of knee osteoarthritis. Several popular dietary supplements that are thought to decrease osteoarthritis pain are going through the clinical trials necessary for FDA approval. Preliminary interest has been expressed in future studies of fibromyalgia and gout.

No Obligations

Participants in the UPMC Arthritis Network Registry are under no obligation to participate in the studies they may be contacted about. It is possible to remain a member of the Registry even if every study request is declined. When agreeing to participate in the Registry, patients are simply agreeing to being contacted about upcoming research studies.

Registry Contents

The Arthritis Network Registry now includes over 600 patients with rheumatoid arthritis, over 900 patients with osteoarthritis, and over 650 patients with fibromyalgia. These figures represent only a portion of the population who suffer with these diseases. The Registry is in the process of recruiting patients with osteoporosis and gout. In order to participate, a consent form must be signed and returned as directed. Signing the consent form simply gives researchers who are approved by the Research Committee permission to contact participants regarding their approved research project and the opportunity to participate in it.

Joining the Registry

The Registry can never have too many people in it. In addition to osteoporosis and gout recruitment, the Registry also remains open to all rheumatoid arthritis, osteoarthritis, and fibromyalgia patients. New diseases such as lupus, scleroderma, and polymyositis/dermatomyositis will be added to the Registry in the coming months. Jennifer Jablon, the Registry Coordinator, is available at (412)383-8674 or by e-mail at jablonj@msx.dept-med.pitt.edu to answer any questions.

Jennifer Jablon

The Network and entire regional community mourn the premature loss of Dr. Aldo V. (Vinny) Londino, who died on December 17, 2000. He was a life-long sufferer of cystic fibrosis and had recently had a lung transplant for this condition. Dr. Londino was Associate Professor of Medicine and Pediatrics at the University of Pittsburgh and Chief of Pediatric Rheumatology and Director of the Arthritis Center at Children’s Hospital of Pittsburgh.

Dr. Londino graduated from Allegheny College and Temple Medical School. He completed his internal medicine training at Mercy Hospital of Pittsburgh. Dr. Londino then studied rheumatology at both the University of Massachusetts and Tufts University where he learned both pediatric and adult rheumatology. He returned to Pittsburgh in private practice before becoming a full-time University faculty member in 1985. There he fashioned a reputation as an expert diagnostician, an outstanding teacher, and most importantly a

revered care giver to his adult and pediatric patients. Dr. Londino was recently named Teacher of the Year by the Children’s Hospital pediatric residents and the region’s top pediatric rheumatologist by Pittsburgh Magazine. He was especially adept at making children who were ill feel comfortable and confident because he was always gentle and genuinely upbeat. His quick wit and joke-telling ability were legendary. As in his public life, Dr. Londino’s family life was characterized by love and generosity. He was devoted to his wife, Joanne, and his two sons, Vinny, Jr. and Greg. He taught the values of honesty, kindness, fair play and patience as a youth baseball coach in Ingomar.

On January 21, 2001, the University community held a tribute to Dr. Londino, at which a number of his closest associates had the opportunity to further describe his spirit, accomplishments and legacy and to express their respect and love for this remarkable physician.

Many people with rheumatic diseases experience some type of gastrointestinal (GI) involvement. The GI tract includes the esophagus, stomach, small intestine, large intestine (colon), and rectum. Intestinal problems are especially common in patients with connective tissue diseases such as systemic sclerosis (scleroderma), systemic lupus erythematosus, polymyositis-dermatomyositis, Sjogren’s syndrome and various types of vasculitis.

The function of the GI tract is to move food and liquid from the mouth to the rectum. The esophagus normally propels food we eat into the stomach during the act of swallowing. In the stomach, foods are partially digested by stomach acid. In the small intestine, foods are further broken down into tiny nutrient particles, which are absorbed into the blood stream and used for energy. The colon and rectum are more involved with elimination of waste.

Gastroesophageal reflux is a common problem in the general population but is especially frequent in connective tissue disease sufferers. This term refers to the flow of stomach acid in the wrong direction - into the esophagus. The predominant symptom caused by reflux is heartburn, which patients often describe as a burning sensation near the bottom or to the left of the breastbone, which may radiate toward the throat. It can also be appreciated as a sour taste in the mouth. Rarely, patients complain of a sharp chest pain that is sometimes confused with “angina” from coronary heart disease. Reflux can produce a cough and may be worsened by lying flat too soon after meals, overeating, or bending over. Reflux symptoms are especially common after a patient lies down to sleep, when gravity cannot protect the esophagus. These symptoms can be experienced daily or multiple times per day. Severe reflux may affect the upper esophagus and the muscles of the voice box, leading to persistent horseness.

The muscles of the lower esophagus (near the stomach) and the strong muscle that normally opens only to allow food to enter the stomach from the esophagus (sphincter) are weakened. The stomach lining is designed to tolerate this acid, but the esophagus lining is not. The lower

esophagus can become chronically irritated and if reflux is not controlled, permanently damaged. Chronic acid damage of the lower esophagus can lead to stricture (narrowing), which may need to be mechanically opened (dilated) using an endoscope. It can be complicated by bleeding. Stricture frequently recurs so that repeated dilatation is often necessary. You can limit reflux if you:

1. Eat your food slowly and chew thoroughly.
2. Do not overeat - four to five small meals per day are better than two large meals.
3. Do not eat during the 2-3 hours before bedtime.
4. Avoid high fat foods.
5. Avoid tight or restrictive clothing around your waist.
6. Avoid spicy or acidic foods, colas, chocolate, citrus juice, caffeine, alcohol and tobacco.
7. Do not lie down during the first 30-60 minutes after eating.
8. Elevate the head of your bed 4-6 inches on wooden blocks or telephone books.
9. Do not exercise immediately after eating.
10. Minimize use of anti-inflammatory drugs or, if such medications are necessary but cause increased reflux symptoms, ask your doctor about the new COX-2 inhibitor anti-inflammatory agents.

Your doctor may prescribe medications in an attempt to completely eliminate symptoms. There are four types of medications that are commonly used. Antacids (including Tums), Maalox and others neutralize stomach acid once it is produced. H2 blockers like Tagamet, Zantac, and Pepcid reduce acid production in the stomach to a moderate degree. Proton pump inhibitors such as Prilosec and Prevacid, which are more expensive, block stomach acid production almost completely and are very effective and well-tolerated. Prokinetic drugs like Reglan quicken emptying of the esophagus and stomach. They are taken 30 minutes before meals and at bedtime.

In my role as nurse practitioner in the University Arthritis Center Clinic, I deal with the reflux problems of my patients on almost a daily basis. It is my goal to make all patients reflux-free. Working together, the patient and health care team can accomplish this goal in the great majority of cases.

Arlene Romilly, CRNP