Lupas

Lupas (Latin for wolf) is yet another disease we respiratory therapists treat inside the hospital, especially when there is lung involvement.

It's a disease initially described in the 13th century by Dr.physician Rogerius "who used it to describe erosive facial lesions that were reminiscent of a wolf's bite," according the the Lupas Foundation. Various skin lesions were described as early as the mid 19th century, and in 1856 it was described in the Atlas of Skin Diseases.

In 1872 Moriz Kaposi described "lupus erythematosus" as a more severe disease that just as skin disease. He wrote: "...lupus erythematosus ... may be attended by altogether more severe pathological changes ... and even dangerous constitutional symptoms may be intimately associated with the process in question, and that death may result from conditions which must be considered to arise from the local malady."

Now we know that Lupas is an autoimmune disease that results in the immune system not only attacking foreign substances like bacteria and viruses, but tissues and organs too.

According to the Mayo clinic, "this leads to inflammation and damage to various parts of the body, including the joints, skin, kidneys, heart, lungs, blood vessels and brain."

While the cause in unknown, it's believed to be a combination of genetic and environmental factors. One may be born with the Lupas gene, although not develop symptoms until something in the environment triggers the Lupas gene.

It usually effects black, Hispanic or Asian women between the ages of 15 and 40, although is can effect people of all ages, including infants. The Mayo Clinic notes that it used to be considered life threatening, "but diagnosis and treatment of lupus has improved considerably."

The Mayo Clinic notes four types of Lupas, the first two originally described in 1872 by Kaposi:

1. Systemic lupus erythematosus: The most common, and can affect nearly any part of your body, most commonly the skin, joints, lungs, kidneys and blood.


2. Discoid lupus erythematosus: Affects only the skin (also called Discoid lupus or cutaneous lupus). These patients experience a circular rash on the face, neck and scalp. Some may develop systemic lupus erythematosus.


3. Drug-induced lupus erythematosus: Results from taking certain medicines and goes away when you stop taking these medicines.


4. Neonatal lupus: Rare and affects newborn babies. A mother with certain antibodies that are linked to autoimmune diseases can pass them to the developing fetus — even if the mother has no signs or symptoms of an autoimmune disease. The antibodies can cause neonatal lupus. A baby with neonatal lupus may experience a rash in the weeks following birth that may last several months before disappearing. More-serious cases can cause a problem with the electrical system of the heart (congenital heart block).

Regarding Symptoms, the Mayo Clinic notes the following:

"No two cases of Lupas are alike. Signs and symptoms may come on suddenly or develop slowly, may be mild or severe, and may be temporary or permanent. Most people with lupus have mild disease characterized by episodes — called flares — when signs and symptoms get worse for a while, then improve or even disappear completely for a time."

Symptoms will depend on body part infected, and may include:

• Fatigue


• Fever


• Weight loss or gain


• Joint pain, stiffness and swelling


• Butterfly-shaped rash (malar rash) on the face that covers the cheeks and bridge of the nose


• Skin lesions that appear or worsen with sun exposure


• Mouth sores


• Hair loss (alopecia)


• Fingers and toes that turn white or blue when exposed to cold or during stressful periods (Raynaud's phenomenon)


• Shortness of breath


• Chest pain


• Dry eyes


• Easy bruising


• Anxiety


• Depression


• Memory loss

While the exact reason is unknown, direct sunlight may bring on lupus skin lesions or even trigger an internal response in some patients. Scientists think this may have something to do with the sun causing skin cells to express certain proteins, according to Mayo Clinic.

"Antibodies that are normally present in the body then latch onto these proteins, initiating an inflammatory response. Damaged skin cells also seem to die more frequently in people with lupus, leading to even more inflammation," the Mayo Clinic notes.
There are also certain drugs that can cause Lupas if a patient is on them for a long time, and this is why physicians must be leary of these medicines, and know patient history when prescribing these medicines, which may include antipsychotic chlorpromazine; high blood pressure medications, such as hydralazine (Apresoline); the tuberculosis drug isoniazid and the heart medication procainamide (Pronestyl, Procanbid), among others.

Another cause of Lupas might be chemical exposure at work, although this is hard to prove. Those who inhale silica and mercury may be at increased risk. Smoking cigarettes may also cause Lupas.

Another interesting probably cause that is not well understood is an infection with Epstein-Barr virus.

The Mayo Clinic notes that "Almost everyone has been infected with a common human virus called Epstein-Barr virus. Epstein-Barr virus causes nonspecific signs and symptoms, such as fever and sore throat. Once the initial infection subsides, the virus remains dormant in the cells of your immune system unless something reactivates the virus. For reasons that aren't clear, recurrent Epstein-Barr infections seem to increase the risk of developing lupus."
Lupas may infect the following organs:

1. Kidneys: 75% of patients develop kidney damage within first 2 years of diagnosis, and kidney failure is the leading cause of death among Lupas patients. Patients must have their creatinine level, GFR, BFUN, Sodium level, potassium, and Magnesium levels checked at regular doctor's office visits to monitor kidney function. Of the symptoms listed above, the following are signs of liver failure: generalized itching, chest pain, nausea, vomiting, ankle or leg edema (swelling), and weight gain.

2. Central nercous system: 80% of Lupas patients experience some form of "cognitive dysfunction, such as confusion, memory problems and difficulty expressing their thoughts. Symptoms include: headaches, dizziness, behavioral changes, hallucinatinos, seizures

3. Blood and blood vessels: It can cause anemia, increased risk of bleeding or clotting. It may also cause inflammation of the blood vessels which is serious and causes 7% of deaths.
4. Lungs: Our area of concern. Inflammation of the chest cavity lining (pleurisy) can cause pain with breathing. Lupas may effect PFT results, but rarely are they severe and often produce no symptoms at all.

5. Heart: 40% develop hardened arteries (atherosclerosis), compared to 15% of those who do not have lupas. It can also cause inflammation of heart muscle (myocarditis and endocarditis). Doctors must monitor blood pressure, cholesterol, make sure patients do not smoke, and that they get regular exerrcise.

6. Infection: Lupas and the drugs used to treat it (corticosteroids and cytotoxic drugs) make these patients susceptible to infection due to the immune response. Infection can bring on flares, and this can increase the risk of infection even more. They often get urinary tract infections, respiratory infections, colds, yeast infections, salmonella, herpes, shingles. Pneumonia accounts for 1/3 of lupas deaths.

7. Cancer: Lupas increases the risk of developing cancer, especially non-Hodgkin's lymphoma and lung cancer, although they are less likely to die of cancer compared to those who do not have lupas unless they get the two forementioned cancers.

8. Bone tissue death (avascular necrosis): 10% of those with lupas develop weak bones that break easy. This results from less blood supply to bone tissue. Hip bones are often broken. This can be caused by lupas or meds used to treat it (corticosteroids).

9. Pregnancy: increases risk of preeclampsia and lupas often flares during pregnancy. Premature birth is a risk. Doctors advise only having children when lupas is controlled or in remission for at least 2 years.