Alya Putri Khairani | 130110110220 |

Systemic lupus erythematosus

Diagnosis, Laboratory test, Prognosis and Patient Outcome
DIAGNOSIS
The American College of Rheumatology classification criteria were developed to operationalize the definition of
Systemic Lupus Erythematosus (SLE) to allow comparison of clinical research from different centers, but also serve
to facilitate education and to guide clinical practice. The classification criteria have been critical to research, but
should be viewed as a temporary step until improved understanding of the pathogenesis of SLE emerges

Criterion

Definition

SLE can be diagnosed if any 4 or more of the following 11 criteria are present, serially or
simultaneously, during any interval of observation
Malar Rash
Fixed, flat or raised erythema over the malar eminences, tending to spare the nasolabial
folds
Discoid Rash
Erythematous raised patches with adherent keratotic scaling and follicular plugging (older
lesions may demonstrate atrophic scarring)
Photosensitivity Skin rash as a result of unusual reaction to sunlight, by patient history or physician
observation
Oral Ulcers
Oral or nasopharyngeal ulceration, usually painless, observed by a physician
Arthritis
Nonerosive arthritis involving = 2 peripheral joints, characterized by tenderness, swelling,
or effusion
Serositis
(A) Pleuritis: Convincing history of pleuritic pain or rub heard by a physician or evidence of
pleural effusion
OR
(B) Pericarditis: Documented by ECG or rub or evidence of pericardial effusion
Renal Disorders (A) Persistent proteinuria >0.5 g/day or >3+ if quantitation not performed
OR
(B) Cellular casts: May be red blood cell, hemoglobin, granular, tubular, or mixed
Neurologic
(A) Seizures: In the absence of offending drugs or known metabolic derangements (eg,
Disorders
uremia, ketoacidosis, electrolyte imbalance)
OR
(B) Psychosis: In the absence of offending drugs or known metabolic derangements (eg,
uremia, ketoacidosis, electrolyte imbalance)
Hematologic
(A) Hemolytic anemia: With Reticulocytosis
Disorders
OR
(B) Leukopenia: < 4000/mm3 total on = 2 occasions
OR
(C) Lymphopenia: < 1500/mm3 on =2 occasions
OR
(D) Thrombocytopenia: < 100,000/mm3 in the absence of offending drugs
Immunologic
(A) Anti-DNA: Antibody to native DNA in abnormal titer
Disorders
OR
(B) Anti-Sm: Presence of antibody to Smith (Sm) nuclear antigen
OR
(C) Positive finding of Antiphospholipid antibodies based on:
1. An abnormal serum level of IgG or IgM anticardiolipin antibodies
2. A positive test result for lupus anticoagulant using a standard method, or
3. A false-positive serologic test for syphilis known to be positive for =6 months and
confirmed by Treponema pallidum immobilization or fluorescent treponemal
antibody absorption tests
Antinuclear
An abnormal titer of antinuclear antibody by Immunofluorescence or an equivalent assay at
Antibody (ANA)
any point in time and in the absence of drugs known to be associated with drug-induced
lupus syndrome

Anamnesis & Physical Examination

In patients with suggestive clinical findings, a family history of autoimmune disease should raise further
suspicion of SLE
Constitutional:
Fatigue, fever, arthralgia, and weight changes are the most common symptoms in new cases or recurrent
active SLE flares. SLE-specific fatigue or fever generally occurs in concert with other clinical markers. Fever
may reflect active SLE, infection, and reactions to medications (ie, drug fever)
Musculoskeletal:
Joint pain is one of the most common reasons for the initial clinical presentation of patients with SLE.
Arthralgia, Myalgia, and Frank Arthritis may involve the small joints of the hands, wrists, and knees (usually

Alya Putri Khairani | 130110110220 |

symmetrical, polyarticular). Arthritis of the proximal Interphalangeal (PIP) and Metacarpophalangeal (MCP)
joints of the hands, as well as the wrists, is the most common musculoskeletal finding in SLE. Tenderness,
edema, and effusions accompany a polyarthritis tDerhat is symmetric, nonerosive, and usually nondeforming.
Jaccoud arthropathy is the term used to describe the nonerosive hand deformities due to chronic arthritis and
tendonitis that develop in 10% of patients with SLE. In contrast to rheumatoid arthritis, SLE arthritis or
arthralgia may be asymmetrical, with pain that is disproportionate to swelling
Dermatologic:
Malar rash is a fixed erythema that typically spares the nasolabial folds. It is a butterfly-shaped rash that
can be flat or raised over the cheeks and bridge of the nose
Photosensitive rash is often macular or diffusely erythematous in sun-exposed areas of the face, arms,
or hands and generally persists for more than 1 day
Discoid rash occurs in 20% of patients with SLE and can result in disfiguring scars. The discoid rash can
present as erythematous patches with keratotic scaling over sun-exposed areas of the skin. Follicular
plugging may create scarring that may be well demonstrated in the ears
Renal:
Edema of periorbital or peripheral regions, anasarca, and morning presacral edema upon arising from bed are
common physical findings related to nephrotic syndrome or volume overload with renal failure. Specific signs
and symptoms of renal disease may not be apparent until advanced nephrotic syndrome or renal failure is
present; therefore, it is important to obtain a urine analysis, protein estimate, serum BUN, and creatinine level
on a regular basis.
Neuropsychiatric:
Headache is the most commonly seen CNS finding in SLE, but it is nonspecific. Altered mental status in SLE
may be secondary to aseptic meningitis, seizures, psychosis, or organic brain syndrome
Cardiopulmonary:
Pleuropericardial friction rubs and signs of effusions may be found. Tachypnea, cough, and fever are common
manifestations of lupus pneumonitis. Hemoptysis may signify pulmonary hemorrhage secondary to the
disease. Systolic murmurs are reported in up to 70% of cases. Pulmonary hypertension, vasculitis with digital
infarcts, and splinter hemorrhages may be observed
Gastrointestinal
Abdominal tenderness and pain may be linked to peritonitis, pancreatitis, mesenteric vasculitis, or nonlupusrelated processes
Opthalmologic:
Retinal vasculitis can lead to blindness and is demonstrated by sheathed narrow retinal arterioles with white
exudates adjacent to the vessels. SLE-associated optic neuritis is uncommon, but it should be considered in
patients with vision loss

LABORATORY TEST

Antinuclear Antibody (ANA)

ANA is present in nearly everybody with active lupus. Doctors often use the ANA test as a screening tool. Plus,
looking at patterns of the antibodies can sometimes help doctors determine the specific disease a person has.
That, in turn, helps determine which treatment would be most appropriate
Antiphospholipid Antibodies (APL)
APLs are present in about one out of every two people with lupus. Their presence can help confirm a diagnosis.
A positive test is also used to help identify women with lupus that have certain risks that require preventive
treatment and monitoring. Those risks include blood clots,miscarriage, or preterm birth
Anti-Sm
Anti-Sm is an antibody directed against Sm, a specific protein found in the cell nucleus.The protein is found in
about 30% of people with lupus. It's rarely found in people without lupus. So a positive test can help confirm a
lupus diagnosis
Anti-dsDNA
Anti-dsDNA is a protein directed against double-stranded DNA. DNA is the material that makes up the body's
genetic code. Approximately one out of every two people with lupus has a positive anti-dsDNA test. Also, the
test is very specific for lupus. Therefore, a positive test can be useful in confirming a diagnosis. For many
people, the titer, or level, of the antibodies rises as the disease becomes more active. So doctors can also use
it to help measure disease activity. Also, the presence of anti-dsDNA indicates a greater risk of lupus nephritis,
a kidney inflammation that occurs with lupus. So a positive test can alert doctors to the need to monitor the
kidneys

Suggested diagnostic protocol for investigation of suspected SLE

Alya Putri Khairani | 130110110220 |

PROGNOSIS AND PATIENT OUTCOME

Survival in patients with SLE in the United States, Canada, Europe, and China is approximately 95% at 5 years,
90% at 10 years, and 78% at 20 years. In the United States, African Americans and Hispanic Americans with a
mestizo heritage have a worse prognosis sis than Caucasians, whereas Africans in Africa and Hispanic Americans
with a Puerto Rican origin do not Poor prognosis (~50% mortality in 10 years) in most series is associated with (at
the time of diagnosis) high serum creatinine levels [>124 mol/L (>1.4 mg/dL)], hypertension, nephrotic syndrome
(24-h urine protein excretion >2.6 g), anemia [hemoglobin <124 g/L (<12.4 g/dL)], hypoalbuminemia,
hypocomplementemia, aPL, male sex, and ethnicity (African American, Hispanic, and mestizo heritage). Data
regarding outcomes in SLE patients with renal transplants show mixed results: some series have a twofold increase
in graft rejection compared to patients with other causes of ESRD, whereas others show no differences. Overall
patient survival is comparable (85% at 2 years). Lupus nephritis occurs in approximately 10% of transplanted
kidneys. Disability in patients with SLE is common due primarily to chronic fatigue, arthritis, and pain, as well as
renal disease. As many as 25% of patients may experience remissions, sometimes for a few years, but these are
rarely permanent. The leading causes of death in the first decade of disease are systemic disease activity,
renal failure, and infections; subsequently, thromboembolic events become increasingly frequent
causes of mortality