health

Rare Autoimmune Disease Attacks Blood Vessels

Ask the Doctors by by Eve Glazier, M.D. and Elizabeth Ko, M.D
by Eve Glazier, M.D. and Elizabeth Ko, M.D
Ask the Doctors | March 16th, 2018

Dear Doctor: My brother died from Wegener's disease at age 62. Could you please tell me more about this disease -- what causes it and what can be done? Could there be a connection to the fact that our father died of rheumatoid arthritis at age 64?

Dear Reader: I'm sorry to hear about the loss of your brother -- and understand your desire to learn more about this rare disease. For starters, Wegener's is named after Friedrich Wegener, the German pathologist who defined it. In recent years, Wegener's has been called granulomatosis with polyangiitis (GPA), which more accurately describes the disease. Granulomatosis refers to masses of scar tissue, and polyangiitis refers to inflammation of blood vessels.

An autoimmune condition that affects one to two per 100,000 people per year, GPA is usually linked to an abnormal protein called ANCA, for antineutrophil cytoplasmic antibody. ANCA attacks two types of white blood cells, monocytes and -- more important -- neutrophils.

Neutrophils normally help fight infections by ingesting and killing bacteria. They also help in the inflammatory response to injury. ANCA, however, causes the neutrophils to inflame normal tissue. It also causes them to release chemicals normally reserved for killing bacteria, causing even greater destruction to normal tissue. This dual assault affects the smaller blood vessels of the body, especially in the respiratory system and in the kidneys; symptoms can progress either slowly (over several months) or rapidly (over a matter of days).

The hallmark of the disease is vascular inflammation of the organs, most notably the lungs, affecting their air spaces and leading to scar tissue that forms nodules within them. In a severe form of GPA, the small blood vessels leak blood into the air spaces, causing a sudden drop in a person's oxygen level. This is turn leads to a coughing-up of blood and the failure of the respiratory system.

In most cases -- 90 percent -- GPA also affects the sinuses, nose, ears, mouth or throat. These symptoms can appear similar to those caused by common nasal congestion, sinusitis or a runny nose. But as the disease progresses, it can cause ulcers in the nose and mouth, hearing loss and the destruction of nose cartilage, resulting in nasal deformities.

The kidneys don't escape attack either. Because blood is filtered by the small vessels of the kidneys, inflammation of these blood vessels compromises the kidneys' filtering mechanism. Early damage can be diagnosed through tests that detect protein in the urine, but ultimately about 80 percent of people with GPA will develop severe kidney problems within two years of diagnosis -- and many will have kidney failure.

GPA can also damage the eyes, the skin and the nervous system.

Because the disease is, in effect, an autoimmune attack, treatment focuses on suppressing the immune system with steroids and other drugs. Although these drugs have side effects, they're effective in controlling the disease. Once remission occurs, patients can switch to milder immune-suppressing drugs. Note that the disease can reoccur, however, sometimes induced by infection.

Why the disease develops is unknown. It's possible that an infection may lead to the formation of ANCA, causing GPA; genetic factors may play a role as well. In fact, one study found similar genetic changes in GPA and rheumatoid arthritis, so your father's history of rheumatoid arthritis may be a factor. As you can see, however, more research is needed. You're not alone in wanting answers.

(Send your questions to askthedoctors@mednet.ucla.edu, or write: Ask the Doctors, c/o Media Relations, UCLA Health, 924 Westwood Blvd., Suite 350, Los Angeles, CA, 90095. Owing to the volume of mail, personal replies cannot be provided.)

health

Preoperative Self-Care Can Quicken Patients' Recovery Time

Ask the Doctors by by Eve Glazier, M.D. and Elizabeth Ko, M.D
by Eve Glazier, M.D. and Elizabeth Ko, M.D
Ask the Doctors | March 15th, 2018

Dear Doctor: I'm 75 years old and am scheduled to have minor surgery next month. My doctor has been pushing me to walk every day and to perform core exercises -- not to mention eating right -- ahead of my operation. Does this really matter?

Dear Reader: Indeed, it does. Recent studies show that elderly patients are at increased risk for a range of complications during surgery and during the recovery process as well. But it's not just about age. By comparing pre-operative assessment data to how patients fare during surgery and recovery, researchers have been able to establish a definitive link between frailty and poor surgical outcomes. These range from complications during the procedure, slow or incomplete recovery afterward, and a net decline in health and cognition in the weeks and months after the surgery.

And younger readers, take note. This correlation turns out to hold true for patients of any age. In fact, the new thinking is that frailty is a more important indicator than chronological age when evaluating patients for elective surgery.

However, frailty, which is basically a reduction of the physical and mental reserves that help us bounce back from setbacks due to illness or injury, is far more common in older adults. Additional factors also make surgery more of a challenge for older adults. These include ongoing conditions or illnesses that place added stress on the body. Problems with vision, hearing or mobility have an effect on both surgery and recovery. A range of prescription medications can increase the risk of certain complications. And issues related to cognitive function, mood and social circumstances play a role in overall well-being.

The good news is that this new research has prompted the American College of Surgeons to launch a nationwide effort for hospitals, health care providers and patients to become aware of the unique challenges that elderly adults face when undergoing surgery and recovery, as well as the steps they can take to mitigate those risks. Supporting data comes from Duke University's Perioperative Optimization of Senior Health (POSH) program, an interdisciplinary team approach to helping older adults prepare for and recover from surgery. This program includes the very behaviors your physician is urging you to adopt.

In an assessment of elderly patients undergoing elective abdominal surgery, those from the POSH program had shorter hospital stays (four days for the POSH group versus six days for the control group) and lower readmission rates (7.8 percent vs. 18.3 percent) over the course of the month following surgery. They also experienced fewer complications and were more likely to be discharged to go home with self-care than were the patients in the control group, who had not taken part in the POSH program.

We hope that you'll take your physician's advice to heart and follow the recommended program. Not only can these behaviors increase the chances for a good outcome following your surgery, but continuing the fitness regimen of daily walks, core exercises and deep breathing after your recovery will improve your quality of life.

(Send your questions to askthedoctors@mednet.ucla.edu, or write: Ask the Doctors, c/o Media Relations, UCLA Health, 924 Westwood Blvd., Suite 350, Los Angeles, CA, 90095. Owing to the volume of mail, personal replies cannot be provided.)

health

Common Adenovirus Has Many Subtypes

Ask the Doctors by by Eve Glazier, M.D. and Elizabeth Ko, M.D
by Eve Glazier, M.D. and Elizabeth Ko, M.D
Ask the Doctors | March 14th, 2018

Dear Doctor: I've been hearing about adenovirus, which is often mistaken as the flu. How can you tell the difference? And is it as serious as the flu?

Dear Reader: Your question is a great reminder, especially during flu season, of how complex and ubiquitous viruses are. They cause a host of illnesses, with the common cold alone blamed on more than 200 identified subtypes of virus and many more that are still unidentified. One main group of viruses is adenovirus.

Adenovirus got its name because it was first isolated in the adenoids, although this isn't the only place it's found. More than 60 types of adenovirus exist, with some causing much different symptoms than others. Serotypes 3, 5, 7, 14 and 21, for example, have been associated with more severe disease.

Adenoviruses most commonly cause upper respiratory symptoms. These include inflammation of the throat, leading to a sore throat, and swelling of the membranes in the nose, leading to runny nose and nasal congestion. Such symptoms are often accompanied by headache, fever, fatigue, muscle pain and stomach pain.

But adenovirus can also lead to conjunctivitis, laryngitis, bronchitis and even pneumonia. Adenovirus-caused pneumonia more often affects those younger than 5 years old, accounting for 15 percent of pneumonias in this age group. Young children can also be affected by subtypes of adenovirus that lead to diarrhea, which can last up to eight to 12 days.

In rare cases, the virus can affect the brain, causing meningitis or encephalitis, or lead to inflammation of the liver and the heart muscle. In people with a compromised immune system or those who have had an organ transplant, adenovirus can lead to more severe disease and possible death.

Adenovirus is a resilient virus. It can survive for long periods on environmental surfaces and -- though bleach, formaldehyde and heat can inactivate it -- the virus is resistant to many disinfectants. It can be transmitted through respiratory droplets spread by sneezing, coughing or contact with secretions. Adenovirus is also shed in the stool for many weeks after an acute infection. Without proper handwashing by all parties, the virus can then be taken in orally by another individual.

Because adenovirus is easily transmissible, it's associated with outbreaks of infection in day care settings and among military recruits. In fact, military recruits are now vaccinated against adenovirus, which has decreased their rate of infection.

Adenovirus is diagnosed by either viral culture or by tests producing more rapid results. The treatment is similar to those for other cold viruses -- fluid intake, rest, acetaminophen or nonsteroidal anti-inflammatory drugs (aspirin, ibuprofen) for headache and medications for diarrhea. For people who are taking drugs to suppress the immune system, the antiviral medication cidofovir can improve survival.

Yes, many of the symptoms are similar to influenza, especially in young children. The fever in those under 5 with adenovirus averages 102.6 degrees. This is similar to influenza. However, influenza is a much deadlier virus, especially among older individuals, causing thousands of deaths per year. Although adenovirus can cause significant illness, it doesn't usually lead to the intensity of sickness and the death rates seen with flu.

Rapid flu tests can help distinguish whether a specific illness is due to influenza or another virus, such as adenovirus, but the point remains: If a person becomes dehydrated or if his or her mental state changes, seek emergency help. Neither illness should be taken lightly.

(Send your questions to askthedoctors@mednet.ucla.edu, or write: Ask the Doctors, c/o Media Relations, UCLA Health, 924 Westwood Blvd., Suite 350, Los Angeles, CA, 90095. Owing to the volume of mail, personal replies cannot be provided.)

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