Systemic Lupus Erythematosus Initial Assessment

Clinical Presentation

The clinical presentation varies in different patients and the disease activity varies over time in a single patient with flares resulting in damage from inflammation. Most patients have arthralgia of the hand. 

Cardiac manifestations of systemic lupus erythematosus include endocarditis, myocarditis, and pericarditis. Constitutional symptoms include fatigue, fever, and weight loss. Dermatological manifestations include alopecia, butterfly rash, livedo reticularis, mucous membrane lesions, photosensitivity, purpura, Raynaud’s phenomenon, urticaria, and vasculitis. Gastrointestinal symptoms include abdominal pain, nausea and vomiting, and oral ulcers. Hematologic signs are anemia, leukopenia, and thrombocytopenia. Musculoskeletal manifestations include arthralgia, arthritis, and myositis. Neuropsychiatric manifestations include cognitive dysfunction, cranial neuropathies, peripheral neuropathies, psychosis, seizures, strokes, and transverse myelitis. Monitor systemic lupus erythematosus patients for neurological and/or psychiatric manifestations as in non-NPSLE patients. It usually appears within 1 year from the time of diagnosis but may also appear before or at the time of diagnosis. Ophthalmological manifestations include dry eyes, optic neuritis, and retinal vasculitis. Pulmonary manifestations include interstitial lung disease, pleurisy, pulmonary hypertension, pneumonitis, and serositis. Renal manifestations include casts, hematuria, nephrotic syndrome, and proteinuria. Reticuloendothelial manifestations include hepatomegaly, lymphadenopathy, and splenomegaly. 

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Systemic Lupus Erythematosus_Background

Diagnosis or Diagnostic Criteria

The diagnosis of systemic lupus erythematosus is based on clinical symptoms and laboratory findings. 
 
2019 European League Against Rheumatism (EULAR) or American College of Rheumatology (ACR) Classification Criteria for Systemic Lupus Erythematosus

The 2019 EULAR or ACR Classification Criteria for systemic lupus erythematosus follows these statements:

• A criterion is not included if there is an explanation other than systemic lupus erythematosus
• It is sufficient for a criterion to occur on at least one occasion
• Criteria need not be present all at the same time
• Only the criterion with the highest weight within each domain will be counted
• Classify as systemic lupus erythematosus if the total score is ≥10 with at least one clinical criterion and the entry criterion is met and if the total score is <10, the inclusion of ACR-97 photosensitivity or a combination of clinical and immunological features can be used to diagnose systemic lupus erythematosus 

Clinical Domains and Criteria

• Constitutional
  • Fever (temperature >38.3°C) is given a score of 2 points
• Hematologic
  • Leukopenia (white blood cell count <4000/mm³) is given a score of 3 points
  • Autoimmune hemolysis (presence of elevated indirect bilirubin, elevated lactate dehydrogenase [LDH], low haptoglobin, reticulocytosis, and positive Coombs’ [direct antiglobulin] test) is given a score of 4 points Thrombocytopenia (platelet count <100,000/mm³) is given a score of 4 points
• Mucocutaneous
  • Non-scarring alopecia (observed by a clinician) is given a score of 2 points
  • Oral ulcers (observed by a clinician) is given a score of 2 points
  • Subacute cutaneous or discoid lupus (observed by a clinician) is given a score of 4 points
  • Acute cutaneous lupus (malar or generalized maculopapular rash observed by a clinician) is given a score of 6 points
• Musculoskeletal
  • Joint involvement (either synovitis involving ≥2 joints or tenderness in ≥2 joints and at least 30 minutes of morning stiffness) is given a score of 6 points
• Neuropsychiatric
  • Delirium (change in consciousness with decreased ability to focus; symptoms developing over hours to <2 days or fluctuating throughout the day; either acute or subacute change in cognition or change in behavior, mood, or affect) is given a score of 2 points
  • Psychosis (hallucinations and/or delusions without insight and no delirium) is given a score of 3 points
  • Seizure (primary generalized seizure, or partial or focal seizure) is given a score of 5 points
• Renal
  • Proteinuria (>0.5 g/24 hour by 24-hour urine or equivalent spot urine protein-to-creatinine ratio) is given a score of 4 points
  • Class II or V lupus nephritis on renal biopsy is given a score of 8 points
  • Class III or IV lupus nephritis on renal biopsy is given a score of 10 points
• Serosal
  • Pleural or pericardial effusion (with imaging evidence) is given a score of 5 points
  • Acute pericarditis (≥2 of the following: Pericardial chest pain, pericardial rub, electrocardiography with new widespread ST elevation or PR depression, or new or worsened pericardial effusion on imaging) is given a score of 6 points

Immunologic Domains and Criteria

• Antiphospholipid antibodies
  • Anti-cardiolipin antibodies (IgA, IgG, or IgM) at medium or high titer OR         
  • Anti-beta-2 glycoprotein 1 antibodies (IgA, IgG, or IgM) OR
  • Lupus anticoagulant positive is given a score of 2 points
• Complement proteins
  • Low C3 OR low C4 is given a score of 3 points
  • Low C3 AND low C4 is given a score of 4 points

   

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• SLE-specific antibodies
  • Anti-dsDNA antibody in an immunoassay with demonstrated ≥90% specificity for systemic lupus erythematosus versus relevant disease controls OR
  • Anti-Smith antibody is given a score of 6 points

   

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Disease Activity Indices

Several reliable and validated measuring tools are available for assessing the disease activity in systemic lupus erythematosus. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and British Isles Lupus Assessment Group (BILAG) are the indices more commonly used and most completely validated

Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)

SLEDAI is a global index for assessing disease activity in the preceding 10 days. It is used for both clinical and research purposes. It consists of twenty-four items which include specific manifestations from nine organ systems with a total score of 105. Other versions of this index include SELENA-SLEDAI, SLEDAI 2000, and Mex-SLEDAI.

British Isles Lupus Assessment Group (BILAG)

BILAG assesses nine organ systems and evaluates relapse occurrences. BILAG 2004 consists of several questions assessed on a 0-4 scale in which 0 is given for not present, 1 is given for improving, 2 is given for same, 3 is given for worse, and 4 is given for new event. Responses are then combined into five states of disease activity which are as follows:

• A: Very active disease needing immunosuppressants, medium- or high-dose corticosteroids or high-dose anticoagulation
• B: Moderate disease activity needing a lower dose of corticosteroid, antimalarials, or NSAIDs
• C: Little disease activity needing only symptomatic treatment
• D: No disease activity at the time but previously present
• E: No current or previous disease activity

Physician Global Assessment (PGA)

PGA is a visual analog scale (VAS) reflecting the physician's judgment of the overall disease activity of systemic lupus erythematosus. The most common visual analog scale tool ranges from 0-3 where 0 is given for none, 1 is given for mild, 2 is given for moderate, and 3 is given for severe. A flare is indicated by an increase in the score of ≥1 since the last patient visit. Reliability is affected by the scale used thus the need for standardization of scoring.   

European Consensus Lupus Activity Measurement (ECLAM)  

ECLAM measures disease activity in the previous month. It evaluates ten organ systems and two laboratory values (eg erythrocyte sedimentation rate [ESR] and complement levels). It consists of 33 items evaluated from 0.5 to 2 based on the type of involvement and a combined global score that ranges from 0 to 17.5.