Landmark Week for Lupus Patients

Lupus has been dominating headlines this week, and patients have reason to be hopeful about the progress being made in the understanding of this disease. Earlier this week, researchers announced that they may have determined the mechanism that drives the body to attack its own cells and tissues; two new studies in the journal Science Translational Medicinedetermined a cycle of cell death and chronic inflammation involving blood cells called neutrophils. Then yesterday, the first drug to be approved for the treatment of lupus in over 50 years--since the approval of Plaquenil (hydroxychloroquine) and corticosteroids, in 1955--got the green light from the FDA. Marketed under the trade name Benlysta (belimumab) and developed by Human Genome Sciences, the intravenous drug is an inhibitor that may reduce the number of abnormal cells believed to be linked to the disease.
 
The news will impact a wide variety of medical specialists, as proper treatment for lupus can require a cadre of specialists in addition to a family doctor, including rheumatologists, clinical immunologists nephrologists, hematologists, dermatologists, neurologists, cardiologists and endocrinologists, as well as nurses, psychologists and social workers. The positive aspect of this news is simple: a new understanding of the disease has led to a new treatment thanks to the FDA’s approval. Here’s how that happened:
 

• Two clinical studies involving 1,684 lupus patients demonstrated the safety and effectiveness of Benlysta; patients treated with Benlysta and standard therapies experienced less disease activity than those who received a placebo.
• Some patients had a reduced likelihood of severe flares, and some were able to have their steroid doses reduced.

 
Despite these positive results and FDA approval for a new drug, there are still some barriers to diagnosing lupus. There is no single laboratory test to determine if a patient has lupus, and the process can be tedious because of the various ways in which the disease can mask itself as other conditions. Per the Lupus Research Institute, a patient typically must meet four or more of the following “Eleven Criteria” to be considered for a lupus diagnosis:
 

• Malar rash: butterfly-shaped rash across cheeks and nose
• Discoid (skin) rash: raised red patches
• Photosensitivity: skin rash as result of unusual reaction to sunlight
• Mouth or nose ulcers: usually painless
• Arthritis (nonerosive) in two or more joints, along with tenderness, swelling, or effusion. With nonerosive arthritis, the bones around joints don’t get destroyed.
• Cardio-pulmonary involvement: inflammation of the lining around the heart (pericarditis) and/or lungs (pleuritis)
• Neurologic disorder: seizures and/or psychosis
• Renal (kidney) disorder: excessive protein in the urine, or cellular casts in the urine
• Hematologic (blood) disorder: hemolytic anemia, low white blood cell count, or low platelet count
• Immunologic disorder: antibodies to double stranded DNA, antibodies to Sm, or antibodies to cardiolipin
• Antinuclear antibodies (ANA): a positive test in the absence of drugs known to induce it.

 
Needless to say, some patients may have a long road to a proper diagnosis, and misdiagnosis is not uncommon in this patient population. But recent insights into the biological mechanisms behind the disease coupled with a landmark drug approval have shone the spotlight on this disease. With more attention being paid to lupus and progress being made, there is hope that increased public awareness will result in earlier detection of the disease.

 
Do you think that the approval of Benlysta will help raise awareness of lupus among physicians and patients?

Are there signs and symptoms that you look for in addition to the 11 standard criteria when you suspect a patient may have lupus?

What changes, if any, would you like to see made to the diagnostic criteria for lupus?

How important is a multidisciplinary approach when it comes to diagnosing and managing lupus?