Lupus – Diagnosis, Symptoms, and Treatment

What is Lupus?

Systemic Lupus Erythematosus – otherwise known as lupus – is an inflammatory autoimmune condition and typically affects women of reproductive age and causes joint pain. Other manifestations of the disease include fatigue, rash, dry eyes/mouth, inflammation in other parts of the body, and blood cell abnormalities.  One of the most problematic lupus complications is kidney disease, which is a common cause of dialysis dependence in young women. Lupus is also associated with an increased risk of heart attack and stroke.

Since this condition may have devastating complications and can affect the quality of life, it is crucial to become educated about its symptoms, diagnosis, and treatment.

What Causes Lupus?

The origin of lupus is not entirely clear, but researchers believe the disease is likely related to genetic (family history) and environmental factors. Lupus is an autoimmune disease – meaning that the body recognizes specific organs and tissues as being foreign and attacks itself.  This commonly manifests with inflammation in the joints, skin, mouth, eyes, and other organs.

How Common is Lupus?

Lupus is a relatively common condition as it affects approximately 1.5 million Americans.  There are about 16,000 new cases of lupus diagnosed per year.

The disease is most frequently seen in women of childbearing age (usually aged between 15-44 years) but can also affect men and children.

The disease is more common in non-Caucasian persons.

Signs and Symptoms

Lupus can cause various symptoms that affect multiple organ systems.  Some of the most common symptoms of lupus include:

• Joint inflammation – warmth, redness, and swelling in the hands and knees
• Photosensitive rash – especially over the face (butterfly rash)
• Hair loss (alopecia)
• Fatigue & depression
• Dry eyes & mouth
• Mouth or nasal ulcers
• Eye inflammation
• Raynaud phenomenon – cold-induced pain and color change in the fingers/toes
• Serositis – inflammation of the lining of organs (e.g., heart, abdominal cavity)
• Blood cell abnormalities – anemia, low white blood cell count, low platelets

Joint inflammation often involves the hands, wrists, and knees – although any joint can become inflamed.  Tendons and bursa may also become inflamed, causing pain with joint movement.  The joint pain in lupus is usually worse in the morning and improves throughout the day.

Inflammation of the heart’s lining (pericarditis) or abdominal cavity (peritonitis) often results in chest pain and abdominal pain, respectively.  Patients with kidney involvement (lupus nephritis) may develop frothy urine, swelling in the legs, and high blood pressure.  Severe disease can lead to kidney failure.

Patients with lupus are also at increased risk for heart attack, stroke, and blood clots in the legs and lungs.

Diagnosis

Lupus is diagnosed based on the patient’s symptoms, physical examination findings, and laboratory tests.  Your doctor will check a comprehensive metabolic panel to evaluate your kidney and liver function.  In addition, they will also review a complete blood count, ESR (erythrocyte sedimentation rate), CRP (C-reactive protein), and complement levels.

Urine analysis is commonly ordered to evaluate for protein and other urine abnormalities.  Women of childbearing age are also tested with a urine pregnancy screen.

Specific antibodies in the blood are highly suggestive of lupus.  These typically include:

• ANA (antinuclear antibody)
• Anti-double stranded DNA
• Anti-Smith antibody

X-rays may be obtained from joints that are painful and swollen.

Lupus Medication and Treatment

The treatment of lupus depends on the severity of the disease and whether specific organs are involved.

Physicians can treat mild cases of joint inflammation with minimal involvement of other organs with non-steroidal anti-inflammatory drugs, including:

• Advil (ibuprofen)
• Naprosyn (naproxen)
• Celebrex (celecoxib)
• Mobic (meloxicam)

When the joint inflammation is moderate to severe and other lupus findings are present – such as rash, fatigue, and organ inflammation – your doctor may prescribe oral corticosteroids such as prednisone.  Corticosteroids work by reducing inflammation in the joints and organs, thereby decreasing pain and tissue swelling. 

Corticosteroids are generally not used long term due to their potential side effects such as weight gain, diabetes, immune suppression, and osteoporosis.  They should also be used cautiously in patients with a history of stomach ulcers and gastrointestinal bleeding.  In these cases, your doctor may recommend the simultaneous use of proton-pump inhibitors (e.g., Prilosec (omeprazole), Protonix (pantoprazole)) to reduce stomach acid.

Many patients with lupus are treated long term with a medication called Plaquenil (hydroxychloroquine) due to its more tolerable side effects and the fact that it may reduce lupus flares.  They may also be started on Trexall (methotrexate).  Rheumatologists typically prescribe these medications.

Patients with severe kidney disease may require Cytoxan (cyclophosphamide) or Cellcept (mycophenolate mofetil).  Patients with end-stage kidney disease requiring dialysis may be candidates for a kidney transplant.

References:

1. Tsokos GC. Systemic lupus erythematosus. N Engl J Med. 2011 Dec 1;365(22):2110-21. - https://www.nejm.org/doi/full/10.1056/NEJMra1100359
2. Lam NC, Ghetu MV, Bieniek ML. Systemic Lupus Erythematosus: Primary Care Approach to Diagnosis and Management. Am Fam Physician. 2016 Aug 15;94(4):284-294. - https://www.ncbi.nlm.nih.gov/pubmed/27548593