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Lupus is a rare disease with diverse, often non-specific symptoms. Years can pass until a correct diagnosis is made.
Joint pain or inflammation is the initial symptom in 60% of affected individuals and prompts a doctor’s visit.
It can be difficult to diagnose Systemic Lupus Erythematosus (SLE), as not all classification criteria are always met at the outset.
Skin changes are the first symptom in approximately 20% of affected individuals.
Diverse symptoms complicate diagnosis
It is important that Systemic Lupus Erythematosus is diagnosed as quickly as possible to ensure optimal and timely treatment for those affected.
General symptoms such as severe☺ fatigue and fever are often present concurrently and are the predominant initial symptom in 5–10% of affected individuals.
In 10–15% of affected individuals, SLE begins with the involvement of internal organs such as the kidneys or the nervous system.
Symptoms and Laboratory Values
During a doctor’s consultation, it is important for affected individuals to provide precise information about all their symptoms.
The diagnosis of SLE typically involves: A clinical examination by the physician, which follows the SLE diagnostic criteria. Laboratory tests, which specifically determine autoantibodies, among other things.
The search for specific antibodies in the blood – antinuclear antibodies (ANA), antibodies against dsDNA, antibodies against the Smith antigen, and against cardiolipin – can aid in diagnosis.
Other laboratory values also contribute to the diagnosis of SLE: inflammation parameters, decreased complement factors (C3, C4), decrease in leukocytes, lymphocytes, or platelets, etc.
Examination of kidney function parameters, including urinalysis, can also provide indications of an SLE condition.
SLE Diagnostic Criteria
The diagnosis of SLE is based on the ACR/EULAR classification criteria.
The new ACR and EULAR classification criteria from 2019 for lupus include positive ANA antibodies as an entry criterion for lupus diagnosis.
Clinical Criteria
Constitutional Symptoms | Points |
|---|---|
Fever | 2 |
Skin | Points |
|---|---|
Non-scarring alopecia | 2 |
Oral ulcers | 2 |
Subacute cutaneous or discoid LE | 4 |
Acute cutaneous LE | 6 |
Arthritis | Points |
|---|---|
Synovitis in ≥ 2 joints or tenderness in ≥ joints with morning stiffness ≥ 30 min | 6 |
Neurology | Points |
|---|---|
Delirium | 2 |
Psychosis | 3 |
Seizures | 5 |
Serositis | Points |
|---|---|
Pleural or pericardial effusion | 5 |
Acute pericarditis | 6 |
Hematology | Points |
|---|---|
Leukopenia | 3 |
Thrombocytopenia | 4 |
Autoimmune hemolysis | 4 |
Kidneys | Points |
|---|---|
Proteinuria ≥ 0.5g/24h | 4 |
Lupus nephritis (histol.) Type II, V | 8 |
Lupus nephritis (histol.) Type III, IV | 10 |
Immunological Criteria
Antiphospholipid-AK | Points |
|---|---|
Anticardiolipin IgG ≥ 40 GPL | 2 |
Anti-β2GP1-lgG ≥ 40 GPL | 2 |
Lupus anticoagulant | 2 |
Complement | Points |
|---|---|
C3 or C4 decreased | 3 |
C3 and C4 decreased | 4 |
Auto-AK | Points |
|---|---|
Anti-dsDNA-AK | 6 |
Anti-Smith-AK | 6 |
Classification as SLE ANA ≥ 1:80 and ≥ 10 points
ACR/EULAR criteria 2019 for the classification of systemic lupus erythematosus by: Aringer M.: EULAR/ACR classification criteria for SLE. Semin Arthritis Rheum 2019; 49, S14-S17
Therapy and therapy goals
In therapy, the doctor and those affected by lupus each have their own responsibilities.
There are areas for which the doctor is responsible: laboratory tests, medical assessments, keeping an eye on the latest research. Then there are areas for which people affected by lupus are responsible themselves: lifestyle, adherence to therapy (compliance), taking medication. However, there are also areas that doctors and people affected by lupus can only address together.
In consultation with their treating doctor, those affected should find out which therapy is most suitable for them and offers the greatest possible benefit. Many factors play a role, including personal ones (family situation, autonomy, mobility, motivation, values, etc.).
We recommend a comprehensive assessment by a lupus specialist.
People affected by lupus and their doctor should define a therapy goal. The therapy goal is re-analysed at every consultation; if necessary, the therapy and the therapy goal are adapted to the course of lupus. It is important that lupus patients are included in the considerations and decisions.
In principle, the goal of therapy is to ensure a stable state of lupus.