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SYSTEMIC LUPUS ERYTHROMATOSUS Sex:

Definition -90% cases are in women.


-An autoimmune disease involving multiple organ systems - estrogen-containing oral contraceptives or hormone
that is defined clinically and associated with antibodies replacement have an increased risk of developing SLE.
directed against cell nuclei. -The sex distribution is more equal in those who develop
-Its multisystem manifestations and attendant complications SLE during childhood or when older than 50 years.
from use of immunosuppressive agents make the diagnosis Age:
and management of this entity challenging. Most (80%) cases have been reported to occur in women in
Pathophysiology: their childbearing years.
-Auto antibodies, circulating immune complexes and T Clinical presentation
lymphocytes all contribute to the expression of disease. History:
-Organ systems affected include dermatologic, renal, central -The mean length of time between onset of symptoms and
nervous system (CNS), hematologic, musculoskeletal, diagnosis is 5 years.
cardiovascular, pulmonary, the vascular endothelium, and -The disease is characterized by exacerbations and
gastrointestinal. remissions.
The American College of Rheumatology (ACR) criteria -Many women relate flares of their lupus to the postovulatory
for SLE - “SOAP BRAIN MD” acronym: phase of the menstrual cycle, with resolution of symptoms at
Must include 4 of the following at any time during a patient's the time of menses.
history (specificity 95% and sensitivity 75%): a)-Systemic symptoms include a low-grade fever, fatigue,
Serositis - Pleurisy, pericarditis malaise, anorexia, nausea, and weight loss.
Oral ulcers - Oral or nasopharyngeal, usually painless; Initial presentation may involve one or more organ systems.
palate is most specific b) Arthralgias (53-95%)
Arthritis - Nonerosive Jaccoud type - are the initial complaint in many patients. Often, the pain is
Photosensitivity - Unusual skin reaction to light exposure out of proportion to physical findings.
Blood disorders - Leukopenia, lymphopenia, c) Malar, butterfly rash over the cheeks and bridge of the
thrombocytopenia, Coombs test–positive anemia nose (55-90%)
Renal involvement - Proteinuria (>0.5 g/d or positive on - with photosensitivity to ultraviolet (UV) light has been
dipstick testing; cellular casts) reported (mostly in whites). It also often involves the chin
Antinuclear antibodies (ANAs) - Higher titers generally and ears.
more specific (>1:160) d)Painful or painless ulcers in the nose and mouth are
Immunologic phenomena - Lupus erythematosus (LE) frequent complaints.
cells; anti–double-stranded DNA (dsDNA); anti-Smith (Sm) e)CNS symptoms
antibodies; antiphospholipid antibodies (anticardiolipin -may range from mild cognitive dysfunction to a history of
immunoglobulin G [IgG] or immunoglobulin M [IgM] or seizures (12-59%).
lupus anticoagulant); biologic false-positive serologic test - Any region of the brain, meninges, spinal cord, and cranial
results for syphilis and peripheral nerves can be involved.
Neurologic disorder - Seizures or psychosis -CNS events often occur when SLE is active in other organ
Malar rash - Fixed erythema over the cheeks and nasal systems.
bridge - Intractable headaches and difficulties with memory and
Discoid rash - Raised rimmed lesions with keratotic scaling reasoning are the most common features of neurologic
and follicular plugging disease in patients with lupus.
-Psychiatric symptoms (high-dose steroids also can cause
Incidence psychosis [5-37%]) - If the psychosis gets worse after
Internationally: stopping the steroid, it is most likely related to the disease
Incidence varies worldwide. In Northern Europe, it has been process.
reported to be 40 cases per 100,000. f) Serositis
Mortality/Morbidity: -Pleuritic pain (31-57%), dyspnea, cough, fever, and chest
-Early deaths usually are caused by active disease. pain are important cardiopulmonary complaints.
-Atherosclerosis is a leading cause in late deaths. g) GIT
- Infection and nephritis are major causes of mortality in all Patients may present with abdominal pain, diarrhea, and
stages of SLE. vomiting.
-After dialysis or transplantation, a reduction in disease - Intestinal perforation and vasculitis are important diagnoses
activity and flares has been reported. to exclude.
-Thrombosis, often secondary to antiphospholipid syndrome, A number of other symptoms can be elicited by history
carditis, pneumonitis, pulmonary hypertension, stroke, which can help identify other pathology, including the
myocardial infarction, and cerebritis cause severe morbidity following:
and mortality. Stroke, Pulmonary embolus, Deep venous thrombosis
Race: (DVT),Acute ischemia, Retinal vasculitis
SLE is more common in blacks (1:250) than in whites
(1:1000). However, all ethnic groups are susceptible.

Physical: k)Pulmonary findings


a)-Fever is a challenging problem in SLE. -Tachypnea, cough, and fever are common manifestations of
-It can be a manifestation of active lupus or a representation lupus pneumonitis.
of infection, malignancy, or a drug reaction. -Hemoptysis may signify pulmonary hemorrhage. However,
b)-Malar rash is a fixed erythema sparing the nasolabial infection is the most common cause of infiltrates seen on
folds. It is a butterfly rash that can be flat or raised over the radiographs
cheeks and bridge of the nose. It also often involves the chin Causes:
and ears. -Many of the clinical manifestations of SLE are caused by
c)Discoid rash occurs in 20% of patients with SLE and can the effects of circulating immune complexes on various
be disfiguring secondary to scarring. tissues or to the direct effects of antibodies to cell surface
-It presents as erythematous patches with keratotic scaling components.
over sun-exposed areas of the skin and may occur in the -A genetic predisposition to the development of SLE exists.
absence of any systemic manifestations. The concordance rate in monozygotic twins is 25-70%. Each
d)All patients experience painless or painful oral or patient manifests his or her disease differently