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Systemic Lupus Erythematosus- How is it diagnosed?

How is lupus diagnosis made?

Jigsaw puzzle photo

Sometimes, diagnosis of lupus is fairly straightforward when someone has multiple manifestations of the disease (see “Systemic Lupus Erythematosus: What is it?“). Too often, the lupus diagnosis is difficult as symptoms may be vague and nonspecific, and tests may be inconclusive. A rheumatologist is an expert in the field of autoimmune diseases which can make the diagnosis by putting together all the data from the history, physical exam, labs, or imaging studies. It is much like trying to solve a Jigsaw puzzle: you’d like to have as many pieces as possible, but often many pieces are still missing. It takes a trained eye to discover the full picture.


The history

Lupus is a disease that can affect about every organ. Therefore, when evaluating someone for lupus, I ask questions that cover these symptoms- Are there any rashes (skin), ulcers in the mouth or nose (mucous membrane), chest pain or shortness of breath (heart, lungs), etc. Past history is also important as symptoms in the past may also provide some clues. Does anyone in the family has any autoimmune diseases? Genetic can play a role. What medications are you on? Some medications can actually cause lupus. What is your occupation? Are there any environmental risk factors? Is there a history of miscarriages? The list goes on!


They physical exam

The next step in evaluation for lupus is a very thorough physical examination, looking for the signs of the disease- like hair thinning, rashes, ulcers in the mouth, joint inflammation, etc.


Laboratory tests

Several labs can be abnormal in lupus, including:

  • Positive anti-nuclear antibody (ANA), andi-double stranded DNA antibody (dsDNA), anti-smith antibody, anti-RNP antibody, anti-phospholipid antibodies
  • Low blood cell counts: low white cells (leukopenia), red cells (anemia), or platelets (thrombocytopenia)
  • Low complement levels C3, C4. These are parts of the immune system that often depress during a lupus flare
  • Elevated inflammatory markers (ESR, CRP)
  • Abnormal kidney function
  • Presence of blood or protein in the urine


Imaging studies

Imaging studies are not always needed, but when the diagnosis is unclear, they may provide a clue

  • Xrays of the tender or swollen joints
  • Echocardiogram (ultrasound of the heart) to look for fluid around the heart, assess valve function, etc.
  • Chest xray to evaluate the lungs
  • Ultrasound of the kidneys
  • Cat scan (CT) of the lungs or abdomen
  • MRI of the brain
  • Angiogram to rule out vasculitis



Biopsy of the affected organ (like skin, lungs, kidneys, etc) can be used to confirm the diagnosis and rule out other diseases



  • Lumbar puncture, to get a sample of the spinal fluid, can be used to evaluate for presence of lupus in the brain.


Lupus diagnostic criteria

Since lupus can affect any organ in the body, and since many of the symptoms are nonspecific, most rheumatologists use the 1997 diagnostic criteria from the American College of Rheumatology (ACR). This criteria include 11 signs and symptoms thought to be fairly specific for lupus. Presence of 4 criteria is required to meet the classification.

  1.  Malar rash- red “butterfly rash” over the cheeks and nose
  2. Discoid rash- raised patches of skin that can scar
  3. Photosensitivity rash- rash that occurs with exposure to sunlight
  4. Ulcers- in the mouth or nose
  5. Arthritis- tenderness and swelling in joints
  6. Serositis- fluid in the lining around the lungs (pleuritis) or the heart (pericarditis)
  7. Kidney disease- presence of too much protein or blood in the urine
  8. Blood disorder- low white cells (leukopenia), red cells (anemia), or platelets (thrombocytopenia)
  9. Positive ANA
  10. Other antibodies- anti-double stranded DNA, anti-Smith antibodies, or antiphospholipid antibodies


How good is the diagnostic criteria?

It’s pretty good, but it’s not perfect. Some people with lupus may not fulfill the criteria- for example, lupus can be diagnosed on kidney biopsy even if the other criteria are not present.  Also, less frequently, people who fulfill the criteria may not have lupus. Each of the criteria can be seen with other diseases. For example, mouth ulcers can also happen in Crohn’s disease; Rosacea can look just like malar rash, cancer of the bone marrow can cause low white cells, red cells, platelets, etc.  A real detective- ie a rheumatologist- may be needed to figure out the diagnosis!


Also read:

Systemic Lupus Erythematosus- What is it?

Systemic Lupus Erythematosus- Treatment


Systemic Lupus Erythematosus- What is it?


What is lupus?

Our immune system is designed to fight foreign “things”- like bacteria and viruses. To do this, the immune system produces antibodies that attack these foreign things. When our immune system mistakenly produces antibodies that attack and fight our own tissues, it causes an autoimmune disease. Systemic lupus erythematosus (commonly referred to as lupus, for short) is an autoimmune disease where the immune system produces antibodies that can attack many different organs in our body. This can cause pain, swelling, fatigue, and damage to organs like kidneys, heart, brain, etc.


Who is at risk for lupus?

The exact cause of lupus is not known. Some component of the disease may be genetic. Environmental factors like infection, exposure to sunlight, stress and hormones can also play a role. Women are 9 times more likely to get lupus than men. Lupus is a chronic condition, and can have periods of flares (disease exacerbation) and remission (when the disease is quiet). There is no cure for lupus, but very effective treatments can be tailored to your type of lupus.


What are the signs and symptoms of lupus?

Signs and symptoms of lupus can vary greatly in each individual. While some people with lupus may have just a rash or joint pain, others may have involvement of more crucial organs like the brain or the kidneys. The following are the most common signs and symptoms of lupus.

  • Fatigue

Fatigue is the most common symptom of lupus. It can be caused by ongoing inflammation from the disease, or from anemia, depression, sleeping disturbances, or a side effect of medications. In addition, some people with lupus may also have thyroid disease, which can cause fatigue.


  • Fever

Fever is thought to be the result of the inflammation caused by the immune system.


  • Weight changes

Weight loss can be caused from decreased appetite. Weight gain may be seen when the kidneys are involved, or in people who are treated with long term steroids.


  • Hair lossDiscoid_lupus_erythematosus34

There may be hair thinning or hair loss around the hairline, or patches of hair loss anywhere on the scalp.


  • Rashesmalar 1

Many types of rash can be seen in lupus:

Malar rash (ie. acute cutaneous rash)- this is a butterfly-shaped rash over the cheeks and nose. It usually occurs or worsens after sun exposure.

Discoid rash– is usually round, raised, and scaly patches of skin that most commonly occurs on the face but can be seen anywhere. These have a tendency to scar.SCLE

Photosensitivity rash (subacute cutaneous rash, tumid lupus)- areas of redness in sites of sun exposure like arms, neck, chest.

Bullous rash– blister-like rash that can happen all over the body

Panniculitis (ie. Lupus profundus)- painful nodules under the skin


  • Mouth sores (oral ulcers)

Painful ulcers in the mouth or nose can accompany flares of lupus.


  • Joint pain

Pain and stiffness in the joints is a common symptom of lupus. There also may be inflammation of the joints. Typical joints include fingers, wrists, and knees.


  • Lung disease

Can lead to shortness of breath or lung failure. Some of the lung manifestations of lupus include:

Pleurisy/Pleuritis– refers to inflammation in the lining of the lungs. It can cause shortness of breath and a sharp pain when taking a deep breath.

Interstitial lung disease- is inflammation and scarring in the lung tissue that can significantly affect breathing.

Pleural effusion– is the fluid that can collect in the lining of the lungs.


  • Heart disease

Pericarditis– is inflammation of the lining of the heart. It can cause a sharp pain when taking a deep breath.

Myocarditis– refers to inflammation of the heart muscle.

Endocarditis– is inflammation of the heart valves.

Coronary artery disease and myocardial infarction (ie. Heart attack).


  • Kidney disease

Unlike normal kidneys, kidneys affected by lupus can leak protein and/or blood into the urine (termed proteinuria or hematuria). In more advance stages, there may be severe kidney failure requiring dialysis.

  • Gastrointestinal problems

Lupus can impact the pancreas (pancreatitis) which can cause abdominal pain, nausea, and vomiting. It can cause inflammation of the colon (colitis) leading to abdominal pain and cramping, and blood in the stools.

  • Neurologic problems

A variety of neurologic problems can be seen in people with lupus. Symptoms like headaches, trouble with memory and concentration (commonly called “lupus fog”) are more common. Weakness and numbness in the hands and feet (peripheral neuropathy) is less common. In severe cases, lupus can cause seizures or strokes.

  • Vasculitis

Refers to inflammation of the blood vessels. This can lead to ulcers in the skin or dysfunction of the organ supplied by the blood vessel.

  • Blood clots

About 10% of people with lupus also have certain antibodies (anti-phospholipid antibodies) that can cause blood clots. Some of the manifestations of this include clots in the legs (deep vein thrombosis or DVT), clots in the lungs (pulmonary embolism or PE), clots in the brain causing strokes or seizures, or clots in the uterus causing miscarriages.

Next section:


Systemic Lupus Erythematosus- How is it diagnosed?


Systemic Lupus Erythematosus- Treatment