ByMaram Ali AlbalawiSarah alatwiShog Ali HomadiEbtihaj Thamer
AleniziEzdehar ShakerHanan HamidJawaher FrejRanay khalf
Systemic lupus erythematosus
Contents:Definition.4 -History of the disease...4 -- Clinical
manifestations.7- Etiology.8- Pathophysiology...11 -
Diagnosis.15-Assessment and Diagnostic Findings 18- Prevention .18-
Treatments..19- Approach considerations. 21- Complications..23-
Prognosis .23- Epidemiology24- medical management.24- nursing
management..25- Patient education25- references .27
Introduction:inflammatory autoimmune collagen disease resulting
from disturbed immune regulation that causes an exaggerated
production of autoantibodies Definition :often abbreviated as SLE
or lupus, is a systemic autoimmune disease (or autoimmune
connective tissue disease) in which the bodys immune system
mistakenly attacks healthy tissue. When the immune system is
functioning normally, it makes proteins called antibodies that
protect against pathogens such as viruses and bacteria. Lupus is
characterized by the presence of antibodies against a persons own
proteins; these are most commonly anti-nuclear antibodies, which
are found in nearly all cases. These antibodies leading to
inflammation. Although the underlying cause of autoimmune diseases
is unknown, most believe that lupus results from both genetic and
environmental stimuli.There are many kinds of lupus. The most
common type is systemic lupus erythematosus (SLE), which affects
many internal organs in the body. SLE most often harms the heart,
joints, skin, lungs, blood vessels, liver, kidneys, and nervous
system. The course of the disease is unpredictable, with periods of
illness (called flares) alternating with remissions. The disease
occurs nine times more often in women than in men, especially in
women in child-bearing years ages 15 to 35, and is also more common
in those of non-European descent.While there is no cure for SLE, it
is treated with immunosuppression, mainly with cyclophosphamide,
corticosteroids and other immunosuppressants. The goal of these
treatments is to keep symptoms under control. SLE can be fatal. The
leading cause of death is from cardiovascular disease due to
accelerated atherosclerosis. Survival for people with SLE in the
United States, Canada, and Europe has risen to approximately 95% at
five years, 90% at 10 years, and 78% at 20 years,and now approaches
that of matched controls without lupus.Childhood systemic lupus
erythematosus generally presents between the ages of 3 and 15, with
girls outnumbering boys 4:1, and typical skin manifestations being
butterfly eruption on the face and photosensitivity. Lupus is Latin
for wolf. In the 18th century, when lupus was just starting to be
recognized as a disease, it was thought that it was caused by a
wolf's bite.This may have been because of the distinctive rash
characteristic of lupus. (Once full-blown, the round, disk-shaped
rashes heal from the inside out, leaving a bite-like
imprint.History of the diseas :The history of SLE can be divided
into three periods: classical, neoclassical, and modern. In each
period, research and documentation advanced the understanding and
diagnosis of SLE, leading to its classification as an autoimmune
disease in 1851, and to the various diagnostic options and
treatments now available to SLE patients. The advances made by
medical science in the diagnosis and treatment of SLE has
dramatically improved the life expectancy of a person diagnosed
with SLE._ EtymologyThere are several explanations ventured for the
term lupus erythematosus. Lupus is Latin for wolf,and "erythro" is
derived from , Greek for "red." All explanations originate with the
reddish, butterfly-shaped malar rash that the disease classically
exhibits across the nose and cheeks.In various accounts, some
doctors thought the rash resembled the pattern of fur on a wolf's
face. More likely is that it is derived from the similarity in
distribution to lupus vulgaris or chronic facial tuberculosis where
the lesions are ragged and punched out and are said to resemble the
bite of a wolf.Another account claims that the term "lupus" did not
come from Latin directly, but from the term for a French style of
mask that women reportedly wore to conceal the rash on their faces.
The mask is called a "loup," French for "wolf."_ Classical
periodThe classical period began when the disease was first
recognized in the Middle Ages. The term lupus is attributed to
12th-century Italian physician Rogerius Frugard, who used it to
describe ulcerating sores on the legs of patients. No formal
treatment for the disease existed and the resources available to
physicians to relieve the suffering of their patients was limited._
Neoclassical periodThe neoclassical period began in 1851 when the
skin disease now known as discoid lupus was documented by French
physician, Pierre Cazenave. Cazenave termed the illness lupus and
added the word erythematosus to distinguish this disease from other
illnesses that affected the skin except they were infectious.
Cazenave observed the disease in several patients and made very
detailed notes to assist others in its diagnosis. He was one of the
first to document that lupus affected adults from adolescence into
the early thirties and that the facial rash is its most
distinguishing feature.Research and documentation of the disease
continued in the neoclassical period with the work of Ferdinand von
Hebra and his son-in-law, Moritz Kaposi. They documented the
physical effects of lupus as well as some insights into the
possibility that the disease caused internal trauma. von Hebra
observed that lupus symptoms could last many years and that the
disease could go "dormant" after years of aggressive activity and
then re-appear with symptoms following the same general pattern.
These observations led Hebra to term lupus a chronic disease in
1872.Kaposi observed that lupus assumed two forms: the skin lesions
(now known as discoid lupus) and a more aggravated form that
affected not only the skin but also caused fever, arthritis, and
other systemic disorders in patients. The latter also presented a
rash confined to the face, appearing on the cheeks and across the
bridge of the nose; he called this the "butterfly rash". Kaposi
also observed those patients who developed the "butterfly rash" (or
malar rash) often were afflicted with another disease such as
tuberculosis, anemia, or chlorisis which often caused death.Kaposi
was one of the first persons to recognize what is now termed
systemic lupus erythematosus in his documentation of the remitting
and relapsing nature of the disease and the relationship of skin
and systemic manifestations during disease activity.The 19th
century's research into lupus continued with the work of Sir
William Osler who, in 1895, published the first of his three papers
about the internal complications of erythema exudativum multiforme.
Not all the patient cases in his paper suffered from SLE but
Osler's work expanded the knowledge of systemic diseases and
documented extensive and critical visceral complications for
several diseases including lupus. Noting that many patients with
lupus had a disease that not only affected the skin but many other
organs in the body as well, Osler added the word "systemic" to the
term lupus erythematosus to distinguish this type of disease from
discoid lupus erythematosus. Osler's second paper noted that
reoccurrence is a special feature of the disease and that attacks
can be sustained for months or even years. Further study of the
disease led to a third paper, published in 1903, documenting
afflictions such as arthritis, pneumonia, the inability to form
coherent ideas, delirium, and central nervous system damage as all
affecting patients diagnosed with SLE._ Modern period
The modern period, beginning in 1920, saw major developments in
research into the cause and treatment of discoid and systemic
lupus. Research conducted in the 1920s and 1930s led to the first
detailed pathologic descriptions of lupus and demonstrated how the
disease affected the kidney, heart, and lung tissue. A major
breakthrough was made in 1948 with the discovery of the LE cell
(the lupus erythematosus cella misnomer, as it occurs with other
diseases as well). Discovered by a team of researchers at the Mayo
Clinic, they discovered that the white blood cells contained the
nucleus of another cell that was pushing against the white's cell
proper nucleus. Noting that the invading nucleus was coated with
anti-body that allowed it to be ingested by a phagocytic or
scavenger cell, they named the antibody that causes one cell to
ingest another the LE factor and the two-nuclei cell result the LE
cell.The LE cell, it was determined, was a part of an anti-nuclear
antibody (ANA) reaction; the body produces antibodies against its
own tissue. This discovery led to one of the first definitive tests
for lupus since LE cells are found in approximately 60% of all
people diagnosed with lupus. (Note: The LE cell test is rarely
performed as a definitive lupus test today as LE cells do not
always occur in lupus patients and can occur in individuals with
other autoimmune diseases. Their presence can be helpful in
establishing a diagnosis but no longer indicates a definitive SLE
diagnosis.The discovery of the LE cell led to further research and
this resulted in more definitive tests for lupus. Building on the
knowledge that those with SLE had auto-antibodies that would attach
themselves to the nuclei of normal cells, causing the immune system
to send white blood cells to fight off these "invaders", a test was
developed to look for the anti-nuclear antibody (ANA) rather than
the LE cell specifically. This ANA test was easier to perform and
led not only to a definitive diagnosis for lupus but also many
other related diseases. This discovery led to the development of
what are now known as autoimmune diseases.To ensure that the
patient has lupus and not another autoimmune disease, the American
College of Rheumatology (ACR) established a list of clinical and
immunologic criteria that, in any combination, point to SLE. The
criteria include symptoms that the patient can identify (e.g. pain)
and things that a physician can detect in a physical examination
and through laboratory test results. The list was originally
compiled in 1971, initially revised in 1982, and further revised
and improved in 2009.Medical historians have theorized that people
with porphyria (a disease that shares many symptoms with SLE)
generated folklore stories of vampires and werewolves, due to the
photosensitivity, scarring, hair growth, and porphyrin brownish-red
stained teeth in severe recessive forms of porphyria (or
combinations of the disorder, known as dual, homozygous, or
compound heterozygous porphyrias).Useful medication for the disease
was first found in 1894, when quinine was first reported as an
effective therapy. Four years later, the use of salicylates in
conjunction with quinine was noted to be of still greater benefit.
This was the best available treatment until the middle of the
twentieth century, when Hench discovered the efficacy of
corticosteroids in the treatment of SLE.clinical manifestations
:Onset is insidious or acute. SLE can go undiagnosed for many years
. The clinical course is one of exacerbation and remissions ._
Classic symptoms : fever , fatigue , weight loss , and possibly
arthritis , pleurisy _ Musculoskeletal system : arthralgias and
arthritis ( synovitis ) are common presenting features .Joint
swelling , tenderness , and pain on movement are common ,
accompanied by morning stiffness ._ Integumentary system : several
different types are seen ( eg , subacute cutaneous lupus
erythematosus lupus erythematosus [SCLE] discoid lupus
erythematosus [ DLE ]) .A butterfly rash across the bridge of the
nose and cheeks occurs in more than half of patient and may be a
precursor to systemic involvement .Lesions worsen during
exacerbations (flares) and may be provoked by sunlight or
artificial ultraviolet tight .Oral ulcers and involve buccal mucosa
or nard palate ._Cardiovascular system : pericarditis is the most
common clinical cardiac manifestation. Women who have SLE are also
at risk for early atherosclerosis papular , erythematous , and
purpuric lesions may occur on fingers , elbows , toes , and
extensor surfaces of forearms or lateral sides of hands and may
progress to necrosis . Varied and frequent neuropsychiatric
presentation , generally demonstrated by subtle changes in behavior
or cognitive ability .Etiology :The specific causes of systemic
lupus erythematosus remain undefined. Research suggests that many
factors, including genetics, hormones, and the environment (eg,
sunlight, drugs), contribute to the immune dysregulation observed
in lupus. (See the diagram below.)
Within the healthy population, a subset of individuals has small
amounts of low-titer antinuclear antibody (ANA) or other
autoantibody such as anti-Ro(SSA), anti-La(SSB), or antithyroid
antibodies. In lupus, increased production of specific
autoantibodies (anti-dsDNA, anti-RNP, and anti-Smith antibodies)
leads to immune complex formation and tissue damage from direct
binding in tissues, immune complex deposition in tissues, or
both.Evidence suggests that people with systemic lupus
erythematosus (SLE) have antigen-specific antibody responses to
DNA, other nuclear antigens, ribosomes, platelets, erythrocytes,
leukocytes, and tissue-specific antigens. The resulting immune
complexes cause widespread tissue damage. Cell-mediated autoimmune
responses also play a pathophysiologic role.
Autoantibody production, by relatively few B lymphocytes, may be
a byproduct of polyclonal B-cell activation in which many more B
lymphocytes are activated, perhaps not in response to specific
antigenic stimuli. Data on 3 adolescents with lupus demonstrated a
high percentage of mature naive B cells (25-50% vs 5-20% in healthy
adolescents) producing self-reactive antibodies even before they
participated in an immune response, suggesting defective
checkpoints in B-cell development.[3]The discovery of viral like
particles in lymphocytes in patients with lupus led to the theory
that viral infection causes polyclonal activation in lupus.
However, these particles may simply be breakdown products of
intracellular materials. This assumption was supported by evidence
in which specific viruses, such as Epstein-Barr virus and
cytomegalovirus, in lupus white blood cells (WBCs), were not
isolated in polymerase chain reaction (PCR) assay. Thus, positive
titers to infectious agents in patients with lupus may be another
manifestation of nonspecific polyclonal activation of B cells, an
important point during initial evaluation and diagnosis. However,
viral stimulation of the innate immune system (dendritic cells),
coupled with genetic defects in the innate and adaptive immune
responses, could lead to loss of tolerance and increasingly
specific autoantibody formation.The presence of measurable
autoantibodies implies a loss of tolerance to self-antigens and may
include T-lymphocyte abnormalities. Early studies suggested a loss
of T-suppressor function; however, subsequent investigations have
centered on defects of programmed cell death, or apoptosis. This
process of programmed cell death may be dysregulated in lupus,
resulting in cells with the potential for self-reactivity
persisting instead of undergoing the normal process of apoptosis.T
cells from patients with lupus have been found with increased
levels of Bcl-2, a protein that delays apoptosis. Patients have
also been found to have lymphocytes that underwent increased
apoptosis. One explanation may be that in lupus, lymphocytes that
make self-reactive antibodies survive in the host but undergo
increased cell turnover after an inciting trigger, such as a viral
infection, begins the process that manifests as lupus.Over the past
15 years, studies of lupus have implicated the importance of innate
immunity. Plasmacytoid dendritic cells are decreased in the blood
of lupus patients but are found in high concentration at sites of
inflammation such as the kidney and skin, secreting
alpha-interferons.The presence of high concentrations of interferon
in the sera of lupus patients was originally described by Lars
Ronnblom and was a seminal observation of lupus pathophysiologic
mechanisms.Plasmacytoid dendritic cells endocytose immune complexes
and nucleic debris through the Fc gamma-receptor IIa, activating
toll-like receptors 7 and 9 and triggering production of
interferon-alpha and other proinflammatory cytokines. The excess
necrotic and apoptotic materials are due to ultraviolet damage,
viral infection, and genetic differences, some of which are listed
below and which include impaired clearance of these materials.
Necrotic materials are also due to neutrophil responses to
infection. Neutrophils can extrude their nuclear materials to form
neutrophil extracellular traps or NETosis, immobilizing bacteria
and fungi. NETosis triggers an interferon signal and plasmacytoid
dendritic cell activation that can induce lupus.Other immunologic
mechanisms may also be important, including defects in macrophage
phagocytic activity or handling of immune complexes. In addition,
deficiencies of complement components, including C4, C2, and C1q,
have been associated with lupus, likely due to defective clearance
of immune complexes.Complement receptors may be abnormal in some
patients, leading to problems with clearance of immune complexes
and subsequent deposition into tissues. This may, in association
with dyslipoproteinemia, lead to significant vascular
complications.The predominance of lupus in females suggests sex
hormones may play a role in autoimmune diseases. Research found
that patients with lupus did not have different serum levels of
estrogen and prolactin than did controls; however, free androgen
was lower, whereas follicle-stimulating hormone (FSH) and
luteinizing hormone (LH) levels were higher in postpubertal boys
and girls with SLE.Drugs, such as anticonvulsants and
antiarrhythmic agents, can also play a role in the pathogenesis of
lupus. These drugs can cause a lupuslike syndrome, which resolves
when the drug is discontinued or can be implicated as the trigger
in systemic lupus. Sun exposure leading to inflammation and
apoptosis of skin cells can also trigger systemic lupus._Genetic
susceptibility :The use of microarray technology to detect
candidate susceptibility genes has led to the identification of
several potential gene-risk candidates, including the P-selectin
gene (SELP), the interleukin-1 receptor-associated kinase 1 gene
(IRAK1), PTPN22, and the interleukin-16, protein tyrosine
phosphatase receptor type T, toll-like receptor (TLR) 8, and CASP
10 genes.pathophysiology :This disturbance is brought about by some
combination of genetic , hormonal ( as evidenced by the usual onset
during the childbearing years ) , and environmental factors (
sunlight , thermal burns). Certain medication , such as hydralazine
(Apresoline ) , procainamide ( Pronestyl ) , isoniazid or INH (
Nydrazid ) , chlorpromazine ( thorazine ) , and some antiseizure
medications , have been implicated in chemical or drug induced SLE
. Specifically , B cells and T cells both contribute to the immune
response in SLE . B cells are instrumental in promoting the onset
and flares of the disease . One manifestation of SLE is
abnormalities in apoptosis, a type of programmed cell death in
which aging or damaged cells are neatly disposed of as a part of
normal growth or functioning.In SLE, the body's immune system
produces antibodies against itself, particularly against proteins
in the cell nucleus. SLE is triggered by environmental factors that
are unknown.The immune system must balance between being sensitive
enough to protect against infection, and becoming sensitized to
attack the body's own proteins (autoimmunity). During an immune
reaction to a foreign stimulus, such as bacteria, virus, or
allergen, immune cells that would normally be deactivated due to
their affinity for self tissues can be abnormally activated by
signaling sequences of antigen-presenting cells. Thus triggers may
include viruses, bacteria, allergens (IgE and other
hypersensitivity), and can be aggravated by environmental
stimulants such as ultraviolet light and certain drug reactions.
These stimuli begin a reaction that leads to destruction of other
cells in the body and exposure of their DNA, histones, and other
proteins, particularly parts of the cell nucleus. The body's
sensitized B-lymphocyte cells will now produce antibodies against
these nuclear-related proteins. These antibodies clump into
antibody-protein complexes which stick to surfaces and damage blood
vessels in critical areas of the body, such as the glomeruli of the
kidney; these antibody attacks are the cause of SLE. Researchers
are now identifying the individual genes, the proteins they
produce, and their role in the immune system. Each protein is a
link on the autoimmune chain, and researchers are trying to find
drugs to break each of those links.
SLE is a chronic inflammatory disease believed to be a type III
hypersensitivity response with potential type II involvement.
Reticulate and stellate acral pigmentation should be considered a
possible manifestation of SLE and high titers of anti-cardiolipin
antibodies, or a consequence of therapy._Abnormalities in cell
death signaling :Apoptosis is increased in monocytes and
keratinocytesExpression of Fas by B cells and T cells is
increasedThere are correlations between the apoptotic rates of
lymphocytes and disease activity.Necrosis is increased in T
lymphocytes.Tingible body macrophages (TBMs) large phagocytic cells
in the germinal centers of secondary lymph nodes express CD68
protein. These cells normally engulf B cells that have undergone
apoptosis after somatic hypermutation. In some people with SLE,
significantly fewer TBMs can be found, and these cells rarely
contain material from apoptotic B cells. Also, uningested apoptotic
nuclei can be found outside of TBMs. This material may present a
threat to the tolerization of B cells and T cells. Dendritic cells
in the germinal center may endocytose such antigenic material and
present it to T cells, activating them. Also, apoptotic chromatin
and nuclei may attach to the surfaces of follicular dendritic cells
and make this material available for activating other B cells that
may have randomly acquired self-specificity through somatic
hypermutation . Necrosis, a pro-inflammatory form of cell death, is
increased in T lymphocytes, due to mitochondrial dysfunction,
oxidative stress, and depletion of ATP._Clearance deficiency:
Impaired clearance of dying cells is a potential pathway for the
development of this systemic autoimmune disease. This includes
deficient phagocytic activity and scant serum components in
addition to increased apoptosis.Monocytes isolated from whole blood
of SLE sufferers show reduced expression of CD44 surface molecules
involved in the uptake of apoptotic cells. Most of the monocytes
and tingible body macrophages (TBMs), which are found in the
germinal centres of lymph nodes, even show a definitely different
morphology; they are smaller or scarce and die earlier. Serum
components like complement factors, CRP, and some glycoproteins
are, furthermore, decisively important for an efficiently operating
phagocytosis. With SLE, these components are often missing,
diminished, or inefficient.Recent research has found an association
between certain lupus patients (especially those with lupus
nephritis) and an impairment in degrading neutrophil extracellular
traps (NETs). These were due to DNAse1 inhibiting factors, or NET
protecting factors in patient serum, rather than abnormalities in
the DNAse1 itself.[49] DNAse1 mutations in lupus have so far only
been found in some Japanese cohorts.The clearance of early
apoptotic cells is an important function in multicellular
organisms. It leads to a progression of the apoptosis process and
finally to secondary necrosis of the cells if this ability is
disturbed. Necrotic cells release nuclear fragments as potential
autoantigens, as well as internal danger signals, inducing
maturation of dendritic cells (DCs), since they have lost their
membranes' integrity. Increased appearance of apoptotic cells also
simulates inefficient clearance. That leads to maturation of DCs
and also to the presentation of intracellular antigens of late
apoptotic or secondary necrotic cells, via MHC molecules.
Autoimmunity possibly results by the extended exposure to nuclear
and intracellular autoantigens derived from late apoptotic and
secondary necrotic cells. B and T cell tolerance for apoptotic
cells is abrogated, and the lymphocytes get activated by these
autoantigens; inflammation and the production of autoantibodies by
plasma cells is initiated. A clearance deficiency in the skin for
apoptotic cells has also been observed in people with cutaneous
lupus erythematosus (CLE).
_Accumulation in germinal centers :In healthy conditions,
apoptotic lymphocytes are removed in germinal centres (GC) by
specialized phagocytes, the tingible body macrophages (TBM), which
is why no free apoptotic and potential autoantigenic material can
be seen. In some people with SLE, accumulation of apoptotic debris
can be observed in GC because of an ineffective clearance of
apoptotic cells. In close proximity to TBM, follicular dendritic
cells (FDC) are localised in GC, which attach antigen material to
their surface and, in contrast to bone marrow-derived DC, neither
take it up nor present it via MHC molecules.Autoreactive B cells
can accidentally emerge during somatic hypermutation and migrate
into the germinal center light zone. Autoreactive B cells,
maturation coincidentally, normally do not receive survival signals
by antigen planted on follicular dendritic cells, and perish by
apoptosis. In the case of clearance deficiency, apoptotic nuclear
debris accumulates in the light zone of GC and gets attached to
FDC. This serves as a germinal centre survival signal for
autoreactive B-cells. After migration into the mantle zone,
autoreactive B cells require further survival signals from
autoreactive helper T cells, which promote the maturation of
autoantibody-producing plasma cells and B memory cells. In the
presence of autoreactive T cells, a chronic autoimmune disease may
be the consequence.
_Anti-nRNP autoimmunity:
Autoantibodies to nRNP A and nRNP C initially targeted
restricted, proline-rich motifs. Antibody binding subsequently
spread to other epitopes. The similarity and cross-reactivity
between the initial targets of nRNP and Sm autoantibodies
identifies a likely commonality in cause and a focal point for
intermolecular epitope spreading.
_Others: Elevated expression of HMGB1 was found in the sera of
patients and mice with systemic lupus erythematosus, high mobility
group box 1 (HMGB1) is a nuclear protein participating in chromatin
architecture and transcriptional regulation. Recently, there is
increasing evidence HMGB1 contributes to the pathogenesis of
chronic inflammatory and autoimmune diseases due to its
proinflammatory and immunostimulatory properties.Diagnosis_
Laboratory tests : Antinuclear antibody (ANA) testing and
anti-extractable nuclear antigen (anti-ENA) form the mainstay of
serologic testing for SLE. Several techniques are used to detect
ANAs. Clinically the most widely used method is indirect
immunofluorescence (IF). The pattern of fluorescence suggests the
type of antibody present in the patient's serum. Direct
immunofluorescence can detect deposits of immunoglobulins and
complement proteins in the patient's skin. When skin not exposed to
the sun is tested, a positive direct IF (the so-called lupus band
test) is an evidence of systemic lupus erythematosus.ANA screening
yields positive results in many connective tissue disorders and
other autoimmune diseases, and may occur in normal individuals.
Subtypes of antinuclear antibodies include anti-Smith and
anti-double stranded DNA (dsDNA) antibodies (which are linked to
SLE) and anti-histone antibodies (which are linked to drug-induced
lupus). Anti-dsDNA antibodies are highly specific for SLE; they are
present in 70% of cases, whereas they appear in only 0.5% of people
without SLE.The anti-dsDNA antibody titers also tend to reflect
disease activity, although not in all cases.Other ANA that may
occur in people with SLE are anti-U1 RNP (which also appears in
systemic sclerosis and mixed connective tissue disease), SS-A (or
anti-Ro) and SS-B (or anti-La; both of which are more common in
Sjgren's syndrome). SS-A and SS-B confer a specific risk for heart
conduction block in neonatal lupus.Other tests routinely performed
in suspected SLE are complement system levels (low levels suggest
consumption by the immune system), electrolytes and kidney function
(disturbed if the kidney is involved), liver enzymes, and complete
blood count.
The lupus erythematosus (LE) cell test was commonly used for
diagnosis, but it is no longer used because the LE cells are only
found in 5075% of SLE cases, and they are also found in some people
with rheumatoid arthritis, scleroderma, and drug sensitivities.
Because of this, the LE cell test is now performed only rarely and
is mostly of historical significance._ Diagnostic criteria : Some
physicians make a diagnosis on the basis of the American College of
Rheumatology (ACR) classification criteria. The criteria, however,
were established mainly for use in scientific research including
use in randomized controlled trials which require higher confidence
levels, so many people with SLE may not pass the full criteria._
Criteria : The American College of Rheumatology (ACR) established
eleven criteria in 1982,which were revised in 1997[58] as a
classificatory instrument to operationalise the definition of SLE
in clinical trials. They were not intended to be used to diagnose
individuals and do not do well in that capacity. For the purpose of
identifying patients for clinical studies, a person has SLE if any
4 out of 11 symptoms are present simultaneously or serially on two
separate occasions. Useful mnemonic for remembering the diagnostic
findings or symptoms of SLE is SOAP BRAIN MD (S=serositis, O=oral
ulcers, A=arthritis, P=photosensitivity, pulmonary fibrosis,
B=blood cells, R=renal, Raynauds, A=ANA, I=immunologic (anti-Sm,
anti-dsDNA), N=neuropsych, M=malar rash, D=discoid rash), however,
not in order of diagnostic importance.1- Malar rash (rash on
cheeks); sensitivity = 57%; specificity = 96%.2- Discoid rash (red,
scaly patches on skin that cause scarring); sensitivity = 18%;
specificity = 99%.3- Serositis: Pleurisy (inflammation of the
membrane around the lungs) or pericarditis (inflammation of the
membrane around the heart); sensitivity = 56%; specificity = 86%
(pleural is more sensitive; cardiac is more specific).4- Oral
ulcers (includes oral or nasopharyngeal ulcers); sensitivity = 27%;
specificity = 96%.5- Arthritis: nonerosive arthritis of two or more
peripheral joints, with tenderness, swelling, or effusion;
sensitivity = 86%; specificity = 37%.6- Photosensitivity (exposure
to ultraviolet light causes rash, or other symptoms of SLE
flareups); sensitivity = 43%; specificity = 96%.7- Bloodhematologic
disorderhemolytic anemia (low red blood cell count) or leukopenia
(white blood cell count
