Coronary Bypass Surgery: Purpose, Procedure and Recovery

Understanding The Connection Between Lupus And The Lungs

Cheryl Russell of Highland Beach, FL, doesn't indulge in what she calls "symptom chasing." After more than three decades with lupus, she refuses to head to the doctor every time she has joint pain or some other problem.

"I learned early on that if I do that, I just end up going to specialist after specialist and having test after test, chasing symptoms that will disappear on their own," says Russell, who raised two now-grown children with her husband, Jake. "I let things have time to work themselves out before jumping on them." 

Trouble began in June 2011 when she noticed she could no longer take a deep breath. She couldn't yawn, and sneezing felt weird. She didn't say anything to her husband, her children, or her doctor. By July, her breathing was worse, and her feet started to swell. Still Russell stayed silent, unaware of the possible connection between lupus and lung disorders and intent on not spoiling a road trip to New England.

The couple had almost made it home from their three-week vacation when things came to a head. In the middle of the night in South Carolina, Russell woke up in their hotel room with intense chest pain and extreme difficulty breathing. She was intent on seeing her own doctors, so the couple hightailed it—so fast they got a speeding ticket—to the emergency room at Boca Raton Regional Hospital. The cause of Russell's breathing difficulties and her swollen feet was a potentially deadly blood clot in her lungs. She also had a rare lung condition called bronchiolitis obliterans with organizing pneumonia, or BOOP.

After eight days in the hospital, doctors sent Russell home with prescriptions for prednisone, a blood thinner, and an amped-up dose of the immunosuppressive drug CellCept®. Although Russell is now doing better, she still can't walk and talk at the same time.

The whole experience has been eye-opening. "Even for someone like myself who's been involved with lupus for so many years, I had never heard of anyone having any lupus-related lung problems," says Russell, who hopes a regimen of Benlysta® infusion therapy will let her get back to her favorite pastimes of cooking and gardening.

It surprises many people to learn that lung issues are common among people with lupus. According to the Johns Hopkins Lupus Center, about 50% of people with lupus will experience lung issues during the course of their disease.

In fact, lung complications rank just behind problems with joints, skin, and blood cell counts, says Ben deBoisblanc, M.D., a professor of medicine and physiology at the Louisiana State University Health Sciences Center in New Orleans.

Research has shown that at some point, most people living with lupus will show signs of lung involvement. "The more severe the lupus you have, the more likely you are to have lung problems," he says. 

Lung involvement in lupus

Although the underlying connective tissue disease is the root cause of lupus-related lung problems, the exact mechanism differs for each of the most common conditions.

The most frequent lung problem that affects people with lupus is pleuritis, also known as pleurisy. In this condition, the pleura—a membrane that covers the exterior of the lungs and the interior of the chest cavity—becomes inflamed. Although the pleura normally produces a small amount of fluid to lubricate the space between the chest wall and the lungs, that fluid can build up excessively. Pleural effusion, which occurs when the fluid between the lungs and the chest wall leaks out, can cause a lot of pain.

With lupus pneumonitis, the inflammation is within the lung tissue itself. The cause is usually an infection by bacteria, viruses, or fungi. It can also be caused by an autoimmune injury to the lung, deBoisblanc adds.

Blood clots in the lungs, known as pulmonary emboli, present another possible complication. "Lupus can cause blood clots to form and break loose and go to the lungs," deBoisblanc explains.

Some people with lupus have a condition called antiphospholipid antibody syndrome that puts them at even higher risk of pulmonary emboli. With this disorder, the body produces antibodies against normal blood proteins, which can cause clots to form in the arteries.

Less common—except among people who have both lupus and other types of connective tissue disorders such as scleroderma—is pulmonary hypertension, or high blood pressure in the blood vessels within the lungs.

The disorder is still a bit mysterious, says Steven M. Kawut, M.D., director of the pulmonary vascular disease program at the Perelman School of Medicine at the University of Pennsylvania. 

"We don't know the pathophysiology," he says. What doctors do know is that the high blood pressure in the lungs' blood vessels strains the right side of the heart and can cause shortness of breath, swelling of the lower extremities, and, eventually, heart failure.

Other lung complications include chronic diffuse interstitial lung disease, which can scar the lung and prevent oxygen from moving easily from the lungs into the blood; pulmonary hemorrhage, or bleeding into the lungs; and "shrinking lung" syndrome, which causes a sense of breathlessness and the feeling that the lungs are not able to expand.

Know the signs

The good news, according to lung specialists, is that diagnosing these potentially life-threatening conditions is usually straightforward, and treatment is successful for most conditions—especially if caught early. A pulmonologist can help with testing and diagnosis, but often a rheumatologist directs treatment.

When you have lupus, you should watch carefully for signs of pulmonary trouble. Keep an eye out for two common symptoms: shortness of breath and pain with breathing, deBoisblanc says. "Pulmonary issues often affect people with lupus who are younger, and who shouldn't be having significant breathing problems at all," he says.

Of course, each lung condition has its own symptoms and diagnostic methods. For example, a sharp pain in the chest that worsens during deep breathing, coughing, or even laughing suggests pleuritis, which a chest X-ray can confirm.

X-rays can also be useful in diagnosing pneumonitis, which may be suspected if you're coughing a lot, experiencing chest pain and shortness of breath, and running a fever. Your doctor may also order tests of your blood and sputum—the mucus from the lungs. A bronchoscopy looks inside your lungs to see if an infection is the pneumonitis' cause.  

The unexplained shortness of breath, fainting, and chest pain of pulmonary hypertension can be picked up via echocardiography and then right-heart catheterization. Your doctor will first rule out other possible causes, such as sleep disorders and other lung diseases, Kawut says. Chest pain and shortness of breath can also mean pulmonary emboli, as Russell discovered.   

For Paula Rostron of Killingly, CT, lung problems actually led to her lupus diagnosis. In 2004, Rostron noticed she was having palpitations and was out of breath, even when doing something as non strenuous as blow-drying her hair. Her doctor did an EKG and immediately sent Rostron to the hospital after seeing the results. There, she was diagnosed with pulmonary emboli. "Even a tiny blood clot can kill a person, and I had huge blood clots in my lungs," she remembers.

Rostron spent a week in intensive care, then went home with a prescription for blood thinners and was soon diagnosed with antiphospholipid antibody syndrome. She was diagnosed with lupus in 2010, after experiencing migraines, joint pain, and pleuritic symptoms.

Treating lupus and the lungs

Treatment should focus on both the underlying lupus and the specific lung problem, deBoisblanc says. For pleuritis, that means immunosuppressive drugs plus analgesics to soothe the pain. For pneumonitis, treatment typically begins with antibiotics to rule out infection, followed by corticosteroids and immunosuppressants such as Imuran®. Pulmonary emboli necessitate blood thinners, often indefinitely.

Treatment for pulmonary hypertension shows the progress being made in treating lupus-related lung problems.

"Twenty years ago, there were no therapies for pulmonary hypertension," says deBoisblanc, and life expectancy was very limited. "Now we have excellent therapies that allow patients to manage their pulmonary hypertension not too differently than you'd manage any chronic disease."

Renee Mestayer, one of Dr. DeBoisblanc's patients, witnessed the evolution of treatments firsthand. Diagnosed with lupus in 1983, Mestayer, of New Iberia, LA, developed pulmonary hypertension in 1999. Eventually, she could barely walk to the mailbox in front of her house and had to use an oxygen tank.

Mestayer has tried a number of new medications over the past decade in addition to her lupus medications. She now takes the pulmonary hypertension drug Tracleer®, as well as Revatio®, which relaxes the walls of arteries and lowers blood pressure, and Tyvaso®, which also helps keep arteries open.

"I'm very fortunate because of all the new medicines that have come out in the last 10 years," says Mestayer. Although she still tires easily, she can now walk long distances on her treadmill. She credits her medications and her faith for getting her through the experience.

"At the time I was diagnosed, my two boys were in sixth and seventh grade," says Mestayer. "I asked God to let me see them graduate high school—and now I'm a grandma!"

Reviva Receives US Patent Covering Use Of Brilaroxazine To Treat Idiopathic Pulmonary Fibrosis

Reviva Pharmaceuticals Holdings, Inc. (Reviva), a late-stage pharmaceutical company, announced United States (US) Patent 12053477 has been granted by the US Patent and Trademark Office (USPTO) covering use of brilaroxazine for the treatment of idiopathic pulmonary fibrosis (IPF), and for the treatment of pulmonary fibrosis in a subject having chronic obstructive pulmonary disease (COPD), sickle cell anaemia, scleroderma, or lung cancer, adding to its existing patent protection in key market Japan.

Brilaroxazine has received Orphan Drug Designation by the US Food and Drug Administration (FDA) for the treatment of IPF.

"We are pleased to further expand our intellectual property (IP) for brilaroxazine which we believe has the broad therapeutic potential to treat inflammatory conditions like IPF driven by underlying disruption in serotonin signalling," said Laxminarayan Bhat, Ph.D., founder, president, and CEO. "This additional patent builds on our existing IP in pulmonary hypertension and is designed to maximize the value of brilaroxazine for the treatment of interstitial lung diseases."

Brilaroxazine is an in-house discovered new chemical entity with potent affinity and selectivity against key serotonin and dopamine receptors implicated in the pathobiology of several conditions including schizophrenia, psoriasis and interstitial lung diseases like pulmonary hypertension, pulmonary arterial hypertension (PAH) and idiopathic pulmonary fibrosis (IPF).

Positive topline data from the global phase 3 RECOVER-1 trial in schizophrenia demonstrated the trial successfully met all primary and secondary endpoints with statistically significant and clinically meaningful reductions across all major symptom domains including reduction in key proinflammatory cytokines implicated in the pathobiology of schizophrenia and comorbid inflammatory conditions at week 4 with 50 mg of brilaroxazine vs. Placebo with a generally well-tolerated side effect profile comparable to placebo and discontinuation rates lower than placebo. Positive data from a clinical drug-drug interaction (DDI) study investigating the potential effect of CYP3A4 enzyme on brilaroxazine in healthy subjects supports no clinically significant interaction when combined with a CYP3A4 inhibitor. Reviva believes that a full battery of regulatory compliant toxicology and safety pharmacology studies has been completed for brilaroxazine. Reviva intends to develop brilaroxazine for other neuropsychiatric indications including bipolar disorder, major depressive disorder (MDD) and attention-deficit/hyperactivity disorder (ADHD).

Additionally, brilaroxazine has shown promising nonclinical activity for inflammatory diseases psoriasis, pulmonary arterial hypertension (PAH) and idiopathic pulmonary fibrosis (IPF) with mitigation of fibrosis and inflammation in translational animal models. Brilaroxazine has already received Orphan Drug Designation by the US FDA for the treatment of PAH and IPF conditions.

Reviva is a late-stage biopharmaceutical company that discovers, develops, and seeks to commercialize next-generation therapeutics for diseases representing unmet medical needs and burdens to society, patients, and their families.

Victor DiLEO Obituary - CO - The Denver Post

DiLEO, Victor Gerard 1957 ~ 2012 54, passed away Saturday, May 19, 2012 at the Denver Hospice as a result of scleroderma and pulmonary hypertension diseases. Victor was born July 19, 1957 in Denver, Colorado to Rose and Pete DiLeo. He attended Mullen High School and received a dergee in marketing at the University of Denver in 1979. He married Marlene Bunch in 2002.

Victor had a very successful career for over 30 years as a financial advisor, retiring from MorganStanleySmithBarney in 2011. Victor was an avid skier, tennis player and golf enthusiast and frequently enjoyed mountain and city bike riding. He also greatly enjoyed watching and attending football, hockey and tennis games. Victor was a charter member and former president of Denver Active 20/30, board member of the American Red Cross, and a board member and Chairman of the Board of the Tennyson Center for Children.

Victor had a great enthusiasm for life, loved to travel with Marlene, family and friends and he never missed an opportunity for adventure. He even made "going for coffee" a special time for all.

Victor will be deeply missed by his beloved wife Marlene and her family, brothers Peter DiLeo and partner Lee Vogt, Tom DiLeo and wife Kathleen, Jim DiLeo, all of Denver, and Tom and Kathleen's son Evan DiLeo of Los Angeles. Victor is also survived by many loving cousins located in Colorado, California, New York, and New Jersey and many wonderful close friends and business associates.

Visitation and rosary will be 7:00pm Wednesday, May 23rd at Horan and McConaty Mortuary, 1091 S. Colorado Blvd, Denver; Funeral Mass 1:00pm Thursday, May 24th at Christ the King Catholic Church, 830 Elm St, Denver. Relatives and friends are welcome to attend both services and a reception at the church hall immediately following the funeral service. Memorial contributions can be made in Victor's name to the Scleroderma Foundation Colorado Chapter, 2280 S Albion St, Denver, CO 80222 or to the Tennyson Center for Children, 2950 Tennyson St, Denver,CO 80212.

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