Lupus erythematosus
Is
chronic auto immune disease
that
can be fatal, though with recent medical advances, fatalities are becoming
increasingly rare
the
attacks the body’s cells and tissue, resulting in inflammation and tissue
damage. SLE can affect any part of the body, but most often harms the, , , the
skin ,joints ,lung .kidney ,blood vesseles and central nervous system.
The
course of the disease is unpredictable, with periods of illness (called flares)
alternating with remission.
Lupus
can occur at any age, and is most common in women, particularly black young
people
Lupus
is treatable, mainly with stroid and immunosupresive and, though there is currently no cure.
However, many people with Lupus lead long and substantial lives
Classification:
systemic
lupus erythematosus
drug
induce lupus
Discoid
lupus erythematosus
. Subacute
cutaneous lupus erythematosus
. Neonatal
lupus
Signs
and symptoms SLE is one of several diseases known as "the great imitators.
because its symptoms vary so widely it often mimics or is mistaken for other
illnesses, and because the symptoms come and go unpredictably. Diagnosis can be
elusive, with patients sometimes suffering unexplained symptoms and untreated
SLE for years. Common initial and chronic complaints fever malaise
,arthrlagia
,skin rash,fatigue ,mylagia .
.
Dermatological
manifestations
As
many as 30% of patients present with some dermatological symptoms (and 65%
suffer such symptoms at some point), with 30% to 50% suffering from the classic
malar rash Patients may present with discoid lupus (thick, red scaly patches on
the skin.alopacia,mouth,nasal and vaginal ulcer.
Musculoskeletal
manifestations: Patients most often seek medical attention forjoint pain 90% of them during the course of the
illness with or with out muscle pain unlike RA lupus arthritis less disable
with out distraction of the joint few than 10% have joint deformites of hand
and feet.
Hematological manifestations
Anemia
and iron deficiency may develop in as many as half of patients and low
platelets and WBC may be due to the disease or a side-effect of pharmacological
treatment. patients may have antiphosphlipid syndrome with thrombosis where
autoantibodies to phospholipids are present in the patient s serum.
Abnormalities associated with antiphospholipid antibody syndrome include a
paradoxical prolonged PTT (which usually occurs in hemorrhagic disorders) and a
positive test for antiphospholipid antibodies with anticardiolipin may be
positive and false positive VDRL.
Cardiac manifestations
Patients
may present with inflammation of various parts of the OF THE HEART like
myocarditis, endocarditis and pericarditis , endocarditis of SLE is
characteristically non-infective[libman-sacks]mostly involve mitral and
tricaspid.atherosclrosis occur more in SLE patients.
Pulmonary manifestations
Like
plurtic involvement and hemmorgic lung, intersial fibrosis and infection
.pulmonary infraction and hypertension.
Renal
invlovment: one of the major that involved in SEL and cause morbidity and
mortality after infection. and lead to hypertension ,it manifested by edema and
oliguria and diagnosed by GUE where found RBC cast ,granular cast, and
proteinuria with increased creatinine and blood urea, Because of early
recognition and management of SLE, end stage renal failure occurs in less than
5% of patients.
There
are six stages of renal involvement.
Neurological
manifestations
10%
have CNS affection manifested by seizer or psychological abnormities or
headache and septic meningitis.
Other:
Systemic
vasculitis , GIT involvement, lupus cystitis, and pancreatis.
Causes
Still
the cause unknown. its chronic inflammatory disease ,type three
hypersensitivity,with potential type 2 involvement. characterised by the body s
production of antibodies against the nuclear components of its own cells. There
are three mechanisms by which lupus is thought to develop: genetic
predisposition, environmental triggers and drug reaction (drug-induced lupus).
Genetics
Lupus
run in families, but no single "lupus gene" has yet been identified.
Instead, multiple genes appear to influence a person s chance of lupus
developing when triggered by environmental factors. The most important genes
are located at 6 chromosome. where mutations may occur randomly .
Environmental triggers
The
second mechanism may be due to environmental factors. These factors may not
only exacerbate existing lupus conditions, but also trigger the initial onset.
They include certain medications like antidepressant and antibiotics , extreme
stress, exposure to sunlight, hormones, and infections. Some researchers have
sought to find a connection between certain infectious agents like bacteria or
virus but no pathogen is linked. UV radiation has been shown to trigger the
photosensitive lupus rash, but some evidence also suggests that UV light is
capable of altering the structure of the DNA, leading to the creation of auto
antibodies.
Drug
reactions
is a reversible condition
that usually occurs in patients being treated for a long-term illness.
Drug-induced lupus mimics systemic lupus. However, symptoms of drug-induced
lupus generally disappear once a patient is taken off the medication which
triggered the episode. There are about 400 medications currently in use that
can cause this condition, though the most common drugs,procaimaide,hydralzine
and quindine.
Non-SLE forms of lupus
Discoid
(cutaneous) lupus is limited to skin symptoms and is diagnosed via biopsy of
skin rash on the face, neck or scalp and ANF USUALLY negative ,10% change to
SLE.
Path physiology
a
disturbance of the normal functioning of the body. One manifestation of lupus
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.
Diagnosis
Some
physicians make a diagnosis on the basis of the ACR classification criteria but
other used it for researches only . and patients may have lupus but never meet
the full criteria.
patients must meet the following three
criteria to be classified as having SLE: (i) patient must present with four of the below eleven
symptoms (ii) either simultaneously or serially (iii) during a given period of
observation.
1- malar rash
2-..Discoid lupus
3-Discoid
lupus
4-Oral ulcers: include
oral or nasopharyngeal ulcers
5-Arthritis: nonerosive
arthritis of two or more peripheral joints, with tenderness, swelling or
effusion
6-Renal
disorder: More than 0.5 g
per day protein in urine, or cellular cast seen in urine under a microscope.
7- Neurologic disorder , Seizuresor psychosis
8-
Serositis: Pleuritis,pericarditis
9-Hematologic
disorder, Hemolytic anemia, leukopenia, white blood cell count<4000/ul), lymphopenia,
( <1500/ul ), thrombocytopenia, (<100000/uL) in the
absence of offending drug.
Hypocomplementemia
is also seen, due to either consumption of C3 and C4 by immune complex-induced
inflammation, or to congenitally complement deficiency, which may predispose to
SLE
10-Anti-nuclear antibody, test positive, sensitivity99%,specificity45%.
11-Immunologic
disorder: Positive, anti-Sm,antidsDNA, anti-phospholipid antibody, false
positiveVDRL test.
Some
patients, especially those with antiphospholipid syndrome, may have SLE
without four criteria and SLE.
Common
misdiagnoses; Porphyria
Common
dual diagnoses; RA,SCURVY, FIFROMYLGIA.
Drug therapy:
Due
to the variety of symptoms and organ system involvement with Lupus patients,
the severity of the SLE in a particular patient must be assessed in order to
successfully treat SLE. Mild or remittent disease can sometimes be safely left
untreated. If required, non-steroidal anti-inflammatory drug and anti-malarials
may be used.
Disease-modifying
antirheumatic drugs (DMARDs) are used preventively to reduce incidence of
flares, the process of the disease, and lower the need for steroid use; when flares occur,
they are treated with corticosteroids. DMARDs commonly in use are
anti-malarials and immunosuppressants (e.g. methotrexate and azathioprine).
Hydroxychloroquine (trade name Plaquenil) is an FDA approved anti-malarial used
for constitutional, cutaneous, and articular manifestations, while
Cyclophosphamide (trade names Cytoxan and Neosar) is used for severe
glomerulonephritis or other organ-damaging complications, and in 2005,
mycophenolic acidCellCept became accepted for treatment of lupus nephritis.
In
more severe cases, medications that modulate the immune system (primarily
corticosteroids and Immunosuppresive drug immunosuppressants) are used to
control the disease and prevent re-occurrence of symptoms (known as flares).
Patients who require steroids frequently may develop obesity, diabetes mellitus
diabetes and osteoporosis. Depending on the dosage, corticosteroids can cause
other side effects such as a puffy face, an unusually large appetite and
difficulty sleeping. Those side effects can subside if and when the large
initial dosage is reduced, but long term use of even low doses can cause
elevated blood pressure and cataracts. Due to these side effects, steroids are
avoided if possible.
Since
a large percentage of Lupus patients suffer from varying amounts of chronic
pain, stronger prescription analgesics may be used if over-the-counter drugs,
mainly non-steroidal anti-inflammatory drug do not provide effective relief.
Moderate pain in Lupus patients if typically treated with mild prescription
opiates such as Dextropropoxyphene (trade name Darvocet), and Co-codamol (trade
name Tylenol #3). Moderate to severe chronic pain is treated with stronger
opioids such as Hydrocodone (trade names Lorcet, Lortab, Norco, Vicodin,
Vicoprofen) or longer-acting continuous release opioidssuch as Oxycodone (trade names OxyContin), MS Contin, or Methadone. The
Fentanyl Duragesic Transdermal patch is also a widely-used treatment option for
chronic pain due to Lupus complications because of its long-acting timed
release and easy usage. When opioids are used for prolonged periods drug
tolerance, chemical dependency and (rarely) addiction may occur. Opiate
addiction is not typically a concern for Lupus patients, since the condition is
not likely to ever completely disappear. Thus, lifelong treatment with opioids
is fairly common in Lupus patients that exhibit chronic pain symptoms;
accompanied by periodic titration that is typical of any long-term opioid regimen.
Lifestyle changes:
Other
measures such as avoiding sunlight or covering up with sun protective clothing
can also be effective in preventing problems due to photosensitivity. Weight
loss is also recommended in overweight and obese patients to alleviate some of
the effects of the disease, especially where joint involvement is significant.
Treatment Research
Other
immunosuppressants(A drug that lowers the body s normal immune response) and
bone marrow transplant autologous stem cell transplants are under investigation
as a possible cure. Recently, treatments that are more specific in modifying
the particular subset of the immune cells (e.g. B- or T- cells) or cytokine
Prevention:
While
most infants born to mothers with lupus are healthy, pregnant mothers with SLE
should remain under a doctor s care until delivery. Neonatal lupus is rare, but
identification of mothers at highest risk for complications allows for prompt
treatment before or after birth. In addition, SLE can flare during pregnancy
and proper treatment can maintain the health of the mother for longer. Women
pregnant and known to have the antibodies for anti-Ro (SSA) or anti-La (SSB)
should have echocardiograms during the 16th and 30th weeks of pregnancy to
monitor the health of the heart and surrounding vasculature
Prognosis:
In
the 1950s, most patients diagnosed with SLE lived fewer than five years.
Advances in diagnosis and treatment have improved survival to the point where
over 90% of patients now survive for more than ten years and many can live
relatively asymptomatically. The most common cause of death is infection due to immunosuppression, as a result of medications used to manage the disease. Prognosis is normally
worse for men and children than for women. Fortunately, if symptoms are present
after age 60, the disease tends to run a more benign course. The ANA is the
most sensitive screening test and ANTI Sm [antismith] is the most specific and
the anti Ds-DNA antibody is also fairly specific and often fluctuates with
disease activity. The ds-DNA titer is therefore sometimes useful to diagnose or
monitor acute flares or response to treatment.
Epidemiology:
In
the United States
alone, it is estimated that between 270,000 and 1.5 million people have lupus,
making it more common than cystic
fibrosis or cerebral palsy. The disease affects both
females and males, though young women are diagnosed nine times more often than
men. SLE occurs with much greater severity among African-American women, who
suffer more severe symptoms as well as a higher mortality rate, Worldwide, a
conservative estimate states that over 5 million people have lupus.
Although
SLE can occur in anyone at any age, it is most common in women of childbearing
age. It affects 1 in
4000 people in the United
States, with women becoming afflicted far
more often than men. The disease appears to be more prevalent in women of
African, Asian, Hispanic and Native American origin but this may be due to
socioeconomic factors.