What Is Lupus?

lupus is a hereditary disorder belonging to a category of diseases called “autoimmune” disorders, in which the immune system misperceives various parts as foreign, builds an immune response, and mounts an attack. Musicians Seal, Toni Braxton, and even Michael Jackson have reportedly been diagnosed with lupus. What is lupus? How is it diagnosed?

British musician Seal, singer Toni Braxton, and entertainer Nick Cannon have all been diagnosed with lupus. Singer Lady Gaga even reports that she has "borderline" lupus—what is that?

In my previous episode, we read about a rather mysterious case of a facial rash after sun exposure. It initially appeared as sunburn, by far the most common cause for the rash on the face. However, given the lack of healing through time with typical sunburn, it became clear that it was not the answer. Then later in the evaluation and follow up process, symptoms consistent with a lupus-like condition eventually became the prominent culprit. Furthermore, it turned out that the lupus symptoms were reversibly induced by a medication the patient was taking for acne, of all things.

Therefore, it leaves us with the question: When is it really lupus? How is lupus diagnosed? What are its criteria?

What Is lupus?
lupus is a chronic inflammatory disease with the potential to affect a number of organs in the body. It tends to affect more women than men, and is more common among African Americans.

lupus is short for systemic lupus Erythematosis (SLE), a hereditary disorder belonging to a category of diseases called “autoimmune” disorders. In autoimmune disorders (such as rheumatoid arthritis, Sjogren’s, Hashimoto’s Thyroiditis, etc) the body misperceives various parts as foreign then builds an immune response against these misunderstood anatomic structures and mounts an attack. These attacks wreak havoc on these tissues in the body, causing distressful symptoms that often bring these patients to the doctor’s office.

Like other autoimmune disorders, the disease tends to flare up for a time period and then clear or improve during times of remission.

Symptoms of lupus
lupus is a terrible disease because it can attack almost any organ in the body, producing numerous symptoms, and a picture that differs from one patient to the next. This makes it a challenge to diagnose. The severity of the disease also varies from person to person, even further contributing to a varied constellation of presentations.

Diagnosis of lupus
The word “lupus” may have crossed your path at some point. Perhaps when you rather suddenly experienced a noticeable hair thinning (termed "alopecia"), when you were diagnosed with pleurisy as taking any deep breaths seemed to trigger a painful type of chest pain you had never experienced before, or when your joints seemed to catch on fire after a hard day’s work. All are very common ailments. Perhaps your doctor may have even “tested” your blood for lupus.

But unlike the diagnosis of diabetes, anemia, and high cholesterol, there is no one simple blood test to diagnose lupus. Diagnosis depends on more than just a blood test—it requires a thorough history inquiring about possible symptoms of lupus, a thorough physical exam consisting of physical manifestations of lupus, and then lab work. The combination of these three elements is necessary in order to determine if a patient truly meets criteria for diagnosis.

The take home point: Just because a patient has an abnormal blood test for lupus does not mean they actually have the disease—this is a vital point to remember when seeking your doctor’s advice.

The American Rheumatism Association (ARA) devised lupus criteria in 1997, but was then later revised by the systemic lupus International Collaborating Clinics (SLICC) n 2012, requiring the following criteria for a definite diagnosis:

A. Biopsy proven “nephritis” (inflammatory changes of the kidneys) due to SLE + positive ANA or double-stranded DNA antibodies (see below for more info)

B. 4 of 17 criteria (with at least 1 of the 11 clinical criteria + 1 of 6 immunologic criteria below):


§ Clinical Criteria:

1. Acute lupus skin changes: as in a malar rash (which we discussed in detail in our previous episode) – a red rash on the face shaped like a butterfly

2. Chronic lupus skin changes: as in the classic “discoid” rash of lupus

3. Alopecia (hair loss)

4. Oral or nasal ulcers

5. Joint disease: joint swelling or pain in at least 2 joints AND stiffness in those joints for at least 30 minutes in the morning

6. Pleurisy (inflammation of the lining of the lung, often painful with inspiration), pleural effusion (fluid in the bottom of the lungs, often found on chest xray), or pericarditis (inflammation of the lining of the heart)

7. Kidney disease: significant protein loss in the urine, or red blood cell “casts” (a finding on a “urinanalysis” test)

8. Neurologic symptoms: seizures, psychosis (delusions and/or hallucinations), neuropathy

9. Hemolytic anemia: a specific type of anemia in which red blood cells are destroyed by the disease

10. Low white blood cell count (found on blood test called “CBC”)

11. Low platelets (also discovered on a CBC)

§ Immunologic Criteria: positive levels of either—

1. Antinuclear antibody (ANA): this is often the first blood test that is run to determine if lupus is a possibility, but it is not specific only to lupus and can be abnormal for numerous other reasons. It is only a screener.

2. Anti-Double Stranded DNA antibody

3. Anti-Smooth antibody

4. Antiphospholipid antibody

5. Low “Complement” levels (components of the blood clotting process): low C3 or C4

6. Direct Coombs’ test: this is a test to detect the presence of antibodies that attack and destroy red blood cells.

So as you can see, lupus can attack any number of organs: skin, hair, joint, lungs, kidney, neurologic system, and blood. And diagnosis is not always so cut and dry. In fact, while meeting the criteria above illustrates a definitive lupus diagnosis, it is sometimes also diagnosed rather loosely in those who don’t meet full criteria.

My advice? Even if you meet several but not all criteria above, it may be worthwhile to obtain a thorough evaluation by a rheumatologist, the specialist that often treats lupus once it is diagnosed.



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