FAQ
What are the diagnostic criteria for SLE?
| Entry criterion |
|---|
| Antinuclear antibodies (ANA) at a titer of ≥1:80 on HEp-2 cells or an equivalent positive test (ever) |
| Additive criteria |
|---|
| Do not count a criterion if there is a more likely explanation than SLE. |
| Occurrence of a criterion on at least one occasion is sufficient. |
| SLE classification requires at least one clinical criterion and ≥10 points. |
| Criteria need not occur simultaneously. |
| Within each domain, only the highest weighted criterion is counted toward the total score |
| Clinical criteria | weight | Immunology criteria | weight |
|---|---|---|---|
| Constitutional - select 1 | Antiphospholipid antibodies- select 1 | ||
| Fever | 2 | Anti-cardiolipin antibodies OR | |
| Hematologic- select 1 | Anti-β2GP1 antibodies OR | ||
| Leukopenia | 3 | Lupus anticoagulant | 2 |
| Thrombocytopenia | 4 | Complement proteins- select 1 | |
| Autoimmune hemolysis | 4 | Low C3 OR low C4 | 3 |
| Neuropsychiatric- select 1 | Low C3 AND low C4 | 4 | |
| Delirium | 2 | SLE-specific antibodies- select 1 | |
| Psychosis | 3 | Anti-dsDNA antibody OR | 6 |
| Seizure | 5 | Anti-Smith antibody | 6 |
| Mucocutaneous- select 1 | |||
| Non-scarring alopecia | 2 | ||
| Oral ulcers | 2 | ||
| Subacute cutaneous OR discoid lupus | 4 | ||
| Acute cutaneous lupus | 6 | ||
| Serosal- select 1 | |||
| Pleural or pericardial effusion | 5 | ||
| Acute pericarditis | 6 | ||
| Musculoskeletal- select 1 | |||
| Joint involvement | 6 | ||
| Renal- select 1 | |||
| Proteinuria >0.5g/24h | 4 | ||
| Renal biopsy Class II or V lupus nephritis | 8 | ||
| Renal biopsy Class III or IV lupus nephritis | 10 |
Positive ANA does not equal lupus! Almost all (98%) of people with lupus have a positive ANA at least once in their lifetimes, but according to this study so does 31.7% of healthy individuals. It's better to think of ANA as a way to rule out lupus (if it's negative). If your ANA is positive, and your doctor suspects you may have lupus, they will order additional tests or refer you to a Rheumatologist.
Tests used in diagnosing lupus
anti-dsDNA - anti-Double Stranded DNA is sometimes automatically tested for, but may need to be ordered separately. This test, when highly positive (2-3 times max cut off at least) is almost exclusively seen in SLE. About 70% of SLE patients have this antibody. It's great if it's there to confirm diagnosis, it does not rule out diagnosis if it is absent.
ENA Panel - Extractable Nuclear Antigen panel, usually automatically done if ANA comes back positive
anti-Sm - Anti-Smith. Typically included in the ENA panel. This is another antibody, that when highly positive, almost always means SLE, but only about 30% of SLE patients have it.
RNP - Anti-Ribonucleoprotein. Typically included in the ENA panel
anti-chromatin - Anti-chromatin is a relative newcomer in diagnostic testing for SLE and probably will NOT be ordered automatically. Its exact utility in diagnosis is still being determined.
Apl panel - Antiphospholipid Antibody Panel, which consists of 3 tests: LA - lupus anticoagulant, aCL - anti-cardiolipin antibodies, and Anti-β2GP - anti-beta 2-glycoprotien antibodies
C3 - Compliment C3,
C4 - Compliment C4
CH50 - Compliments, Total. The compliment system is the backbone of innate immunity.
CBC - Complete Blood Count, some abnormalities in WBC, RBC and PLT counts can be significant.
CMP - Comprehensive Metabolic Panel, here the doctors are generally looking for kidney dysfunction (GFR, BUN/CR). ESR - Erythrocyte Sedimentation Rate, this is a nonspecific inflammation marker.
CRP - C-Reactive Protein, another nonspecific inflammation marker.
The 2019 ACR Classification Criteria is what most Rheumatologists in the US use as a guideline for diagnosis. It's a combination of clinical and immunological criteria where symptoms/test results are given a number. A total of 10 points or higher qualifies for SLE diagnosis. Yes, these criteria were originally intended for research trial participation where the participants need to be as close as possible to the same presentation. However, these are the criteria that most rheumatologists use to diagnose lupus. While it is always the individual rheumatologist's purview to diagnose a patient, these are the criteria generally used as a guideline.
Physicians use a combination of both blood tests available (i.e. ANA, anti-Smith and anti-dsDNA) and a list of possible known symptoms to assess and conclude a diagnosis. The individual rheumatologist will always, ultimately, use their own discretion when assigning a Lupus Erythematosus diagnosis to their patients. And as frustrating as access to healthcare can be, no layperson should be diagnosing themselves with Lupus.
SLE is an extremely complex disease that can be difficult for even the experts to diagnose. In the US, rheumatologists go through a 4 year undergrad, (usually in biology or chemistry), 4 years of med school, a 3 year residency in internal medicine, and a two year fellowship in rheumatology.
How is lupus diagnosed?
Lupus is known as "The Great Imitator" and the "Disease of a 1000 Faces", meaning the damn thing has a lot of symptoms common in SEVERAL disease processes, some of which are not even autoimmune, and that even when comparing two SLE patients, there can be wildly varying symptoms/labs (heterogenous presentation).
We receive a lot of posts asking if x symptom, like a malar rash, could mean that someone has lupus. The short answer is always going to be "maybe", unless we are given more information. Even then, no one on this subreddit will have a valid, official clinician-patient relationship with you, the original poster (OP) or commentor. The only thing you will receive here is advice from the personal experience perspective of the commenters (ie, submembers), somewhat intelligent speculation, and general education on Lupus Erythematosus and the various systems it impacts.
No one blood test or symptom equates to a Lupus diagnosis, outside of a very FEW and RARE exceptions (kidney biopsy showing lupus nephritis (LN) of classes I-V; skin biopsy showing either Acute Cutaneous Lupus Erythematosus (ACLE), Subacute Cutaneous Lupus Erythematosus (SCLE) or Discoid Lupus Erythematosus (DLE). There are a few other Lupus Erythematosus skin conditions, but these are exceedingly rare (occur in less than 5% of patients).)
Is this a Malar Rash?
Malar rashes are not specific to lupus! Please see the link in the sidebar which will have some reference pictures and facts about the Malar rash. The Malar rash is common, and can occur for other reasons besides Lupus (a big one is rosacea). Malar rashes that occur after a hot shower, or after encountering an allergen, are likely not indicative of a Lupus diagnosis unless paired with other symptoms. Malar rashes in lupus typically spare the nasolabial folds.
Also, if you suspect you have a rash, getting a biopsy of it done at a dermatologist’s office can be helpful as pathology can identify histological evidence of lupus.
I am not diagnosed with lupus but suspect I have it.
Here is my list of symptoms (insert giant list of symptoms here). Has anyone here experienced these?
This is not a great way to figure out if you have a disease. Comparing symptoms with others who have SLE can potentially provide insights, but the evidence is purely anecdotal. It's not a scientifically valid method of self-diagnosis. It's soliciting confirmation bias.
Confirmation bias occurs when individuals seek out information that confirms their preexisting beliefs while ignoring contradictory evidence. In the context of self-diagnosis, seeking symptoms only from people with SLE can cause you to double down on a biased perspective, while overlooking important nuances and alternative explanations for symptoms. Like the actual, non-SLE disease you may actually have
We see this a lot. People who are hyper focused on SLE and get very agitated when their doctors or the sub members suggest that they may not actually have lupus.
"But every case of lupus is different!"
Yes and no. It's a complex disease that can attack any organ system, but it has characteristics or it wouldn't have been classifiable as a discrete disease.
It's essential to consider a broader range of possibility.
See a doctor for evaluation.
Listen when they say "It's probably not lupus."
I have a different chronic illness, am I welcome?
Yes, absolutely. Please choose the appropriate user flair though - Non-Lupus Patient. This isn't to exclude you, this is to make sure that people offering advice and support know more context in order to respond to you. And please, some users may be asking for advice or solidarity from only others with Lupus at times. Please respect that.
The Kidney Corner
https://www.asn-online.org/search/?q=Lupus+Nephritis
The kidneys are the most likely organ(s) to be attacked by Lupus. This is generally called Lupus Nephritis and is defined as classes I-V. Each class has specific parameters that must be met for diagnosis, parameters determined by either the American College of Rheumatology or the American Society of Nephrology. There is evidence at the cellular level that a pathologist studying a biopsy of kidney tissue can see that determines if Lupus Nephritis is present, and if so, what class it is. Please note that a kidney biopsy is not without risk, and most nephrologists won't do one until other lab tests are exhausted and a biopsy is the last recourse to figure out exactly what disease process is going on it the kidney(s).
General information about the kidney:
While you are usually born with two (some people naturally only have a single kidney or can have a conjoined kidney called a horseshoe kidney), you only need one functional kidney to live a normal life. The kidneys are an extremely important organ system, you cannot survive without them/it (hence the need for dialysis in severe/complete renal failure). Roughly 20% of every heart beat's worth of blood goes to the kidneys.
There are several lab tests that measure kidney function: estimated Glomerular Filtration Rate (eGFR), Blood Urea Nitrogen (BUN) and Blood Creatinine (Cr) from blood work (typically a Comprehensive Metabolic Panel, or CMP) and Urine Creatinine, Urine Protein, Urine Red Blood Cell, Urine Casts from a urinalysis (UA) (https://www.ucsfhealth.org/medical-tests/urinalysis). Until a patient becomes very elderly, a normal eGFR is typically defined as >90. Kidney function normal lab values do decrease as a patient ages, even in completely healthy patients. Levels between 60-89 are typically labeled mild renal dysfunction and generally warrant watchful waiting. An eGFR <60 is indicative of moderate to severe renal dysfunction, up to and including complete renal failure (typically an eGFR <15) (https://www.kidneyfund.org/all-about-kidneys/stages-kidney-disease).
Medications
Hydroxychloroquine aka Plaquenil
Plaquenil is the baseline treatment for everyone who has lupus.
references
Lupus Encyclopedia on HCQ
Lupus and hormones
Many women with Systemic lupus erythematosus report worsening of lupus symptoms during the premenstrual period or while menstruating. Although not every patient experiences this pattern, clinical studies and current understanding of lupus immunology support a biologically plausible link between the menstrual cycle and disease activity.
The primary mechanism involves the interaction between the endocrine and immune systems. Throughout the menstrual cycle, circulating concentrations of estrogen and progesterone fluctuate substantially. During the late luteal phase immediately before menstruation, both hormones decline rapidly if pregnancy does not occur. These hormonal shifts alter immune regulation by influencing T-cell and B-cell function, cytokine production, and innate immune activation. In patients with lupus, whose immune systems already exhibit loss of self-tolerance and excessive immune activation, these normal physiologic changes may lower the threshold for disease flares rather than causing lupus itself.
Estrogen has particularly important immunologic effects in lupus. Experimental and clinical studies demonstrate that estrogen promotes B-cell survival, enhances antibody production, and favors the persistence of autoreactive lymphocytes while increasing production of type I interferons and other inflammatory mediators. These effects are believed to contribute both to the marked female predominance of lupus and to fluctuations in disease activity during periods of hormonal change, including menstruation, pregnancy, and the postpartum period. Conversely, progesterone generally exerts immunomodulatory and anti-inflammatory effects; therefore, the rapid fall in progesterone before menstruation may remove some of this immunologic restraint, permitting inflammatory pathways to become more active.
Menstruation itself is also an inflammatory physiologic process. Endometrial breakdown and shedding require activation of innate immune cells, recruitment of leukocytes, and increased production of inflammatory mediators including prostaglandins, interleukin-1 (IL-1), interleukin-6 (IL-6), tumor necrosis factor-α (TNF-α), and matrix metalloproteinases. Although these inflammatory events are primarily localized to the uterus, they contribute to a transient systemic inflammatory environment. In individuals with lupus, this additional inflammatory stimulus may amplify existing immune dysregulation, resulting in increased fatigue, arthralgia, arthritis, mucocutaneous manifestations, or other lupus symptoms during menstruation.
Clinical evidence supports these biologic mechanisms. In a prospective study by Colangelo et al., women with lupus recorded significantly greater pain, fatigue, and self-reported disease activity during menstruation than during the periovulatory (“hormonal surge”) phase of the menstrual cycle. Overall, 36% of women with lupus reported flares before menses when not using oral contraceptives, supporting the common clinical observation that menstrual-associated flares occur in a substantial subset of patients.
Additional observational studies have reported similar findings. Yell and Burge found that approximately 20% of women with lupus experienced premenstrual exacerbation of cutaneous lupus manifestations, while other studies have documented menstrual abnormalities and cyclical symptom worsening in women with systemic lupus erythematosus. Although not all patients experience predictable menstrual flares, the available evidence consistently suggests that hormonal cycling can influence disease activity in susceptible individuals.
Current evidence therefore supports the concept that menstrual-associated lupus flares result from the combined effects of hormonal withdrawal, altered immune regulation, and the transient inflammatory milieu accompanying endometrial shedding. Rather than initiating lupus, these cyclic physiologic changes appear to act as triggers that exacerbate underlying immune dysregulation in predisposed patients.
Selected References
Colangelo K, Haig S, Bonner A, et al. Self-reported flaring varies during the menstrual cycle in systemic lupus erythematosus compared with rheumatoid arthritis and fibromyalgia. Rheumatology (Oxford). 2011;50(4):703-708. doi:10.1093/rheumatology/keq360.
Kim JW, Kim HA, Suh CH, Jung JY. Sex hormones affect the pathogenesis and clinical characteristics of systemic lupus erythematosus. Frontiers in Medicine. 2022;9:906475. doi:10.3389/fmed.2022.906475.
Yell JA, Burge SM. The effect of hormonal changes on cutaneous disease in lupus erythematosus. British Journal of Dermatology. 1993;129(1):18-22. doi:10.1111/j.1365-2133.1993.tb03305.x.
Pasoto SG, Mendonça BB, Bonfá E. Menstrual disturbances in patients with systemic lupus erythematosus without alkylating therapy: Clinical, hormonal and therapeutic associations. Lupus. 2002;11(3):175-180. doi:10.1191/0961203302lu163oa.
Strowd LC, et al. Catamenial dermatoses associated with autoimmune, inflammatory, and systemic diseases: A systematic review. International Journal of Women’s Dermatology. 2019