CASE HISTORY
( SLE )
MADE BY / NADA MOHAMED AZAZY
HAGER NADY RAMADAN
GROUP : 92 B
VNMU
Internal Medicine department
Curator name : Nada Mohamed Azazy / Hager Nady
Ramadan
Group / 92 b
Year of study : 5th course
Passport Data
Name : Manal
Age : 23 y.o
Gender : female
Occupation : teacher
Anamnesis
Fever for 2 weeks duration ( low grade intermittent fever
which associated with chills but no rigors and responded well
to antipyretics )
Loss of appetite and loss of weight about 2-3 kg over the
preceding weeks
She complained of lethargy and tiredness
No sore throat , ear ache or discharge
No history of any contact with mud or stranger water
She denied headache , constipation , diarrhea and abdominal
pain
Fever was not associated with joint pain or early morning
stiffness
No alopecia
No hemoptysis , night sweat , past or contact history of TB
No past history history of valvular heart disease or childhood
rheumatic fever
No history of consumption of raw milk , meat or contact with
animals or birds
No sexual promiscuity
No blood transfusions , tattoos or any high risk behavior
No history of recurrent infections or bleeding
She has not been on any long term drugs
She has regular menstrual period
No history of miscarriages or abortions
No significant surgical history
Not known allergies for drugs , food or plasters
No significant family history
No similar complain in the past
No history of malaria , typhoid
Photosensitivity
Respiratory system :
Tachypnea
Pleural effusion
No cough , sputum
Pleurisy
Renal system :
Nephrotic syndrome
Proteinuria
Hematuria
Glomerulonephritis
Cardiovascular system :
Pericarditis
Ischemic coronary artery disease
Myocarditis
Thromboembolism
Dermatological :
Butterfly rash
Raynaud's phenomenon
Vasculitis
Urticaria
Neuropsychiatric :
Acute confusional state
Psychosis
Seizures
Anxiety disorder
Mononeuropathy
Gastrointestinal :
Nausea
Dyspepsia
Abdominal pain
Hematological :
Leukopenia
Lymphopenia
Anemia
Thrombocytopenia
Musculoskeletal :
Arthralgia
Arthropathy
Myalgia
Arthritis
Preliminary diagnosis
Systemic lupus erythematosus
Investigations
CBC : -low rbc (anemia) ,- leukopenia ,-thrombocytopenia
C reactive protein : increase
Sedimentation rate : increase
Antinuclear antibody : positive
Ani-double stranded DNA antibody : positive
Widal test : negative
Urinalysis : proteinurea
Creatine kinase assay : increase
Skin biopsy : malar rash
Anti-smith antibody : increase
Serum albumin : decrease
Rheumatoid factor : positive
Malaria parasite : negative
Mantoux test : negative
Liver function test : increase
Kidney function test : increase
Bone marrow aspiration : no abnormal cells in bone marrow
histology
HIV : negative
Lymph node biopsy : - lymph node architecture
is effaced . There are areas of necrosis
surrounded by histiocytes . - multinucleated
giant cells and well formed granulomas are not
present . - there is no evidence of apoptotic
debris suppuration or lymph node malignancy .
Complement levels : C3 = vary between normal to slightly
reduced . C4 = reduced . CH50 = reduced
Urine protein to creatinine ratio : increased
Anti ribosomal P protein antibodies : increased
Direct Coombs test : positive
Sonography findings
Gastrointestinal :
ascites . hepatomegaly . splenomegaly . gallbladder wall
thickening (
gallbladder distension
)
Pulmonary : pleural
effusion . -echo-free
space between the
visceral and parietal
pleura
Joints : synovial effusion
Renal : generally hyperechoic kidneys
Raynaud's syndrome : in this case show flow volume and
vessel size irregulations
Echocardiography :
decreased ejection fraction . pericardial effusion .
Electrocardiogram
Myocarditis : ECG show ;
sinus tachycardia , diffuse T
wave inversions , ST
segment elevation without
reciprocal depression , low
voltage of QRS complexes ,
arrhythmias
Pericarditis : ST segment
depression (always in aVR ) . reduced voltage with quasi
specific ST-T waves due to
increase in scar tissue , fluid
and fibrin .
X-Ray
Pleural effusion : visible small
amounts of fluids layering
against the dependent parietal
pleura
Osteoporosis : linear
sclerosis and cortical
thickening more frequent
in metaphysical and
epiphyseal fracture
Differentiating systemic lupus erythematous
from other diseases
Systemic lupus erythematosus (SLE) must be
differentiated from other diseases that cause skin rash,
arthritis, positive autoimmune serology, weight loss,
fevers and chronic pain, such as rheumatoid arthritis
(RA), mixed connective tissue disease (MCTD),
systemic sclerosis (SSc), dermatomyositis (DM),
polymyositis (PM), and other autoimmune diseases.
Diagnosis
Systemic lupus erythematosus
Treatment
Treatment goals in systemic lupus erythematosus (SLE) include:
Ensure long-term survival
Achieve the lowest possible disease activity
Prevent organ damage
Minimize drug toxicity
Improve quality of life
Preferred regimen : Hydroxychloroquine PO 200 to 400 mg
daily as a single daily dose or in 2 divided doses AND
methylprednisolone as intravenous "pulse"; 0.5 to 1 g/day for
three days in acutely ill patients, or 1 to 2 mg/kg/day in more
stable patients
Alternative regimen(1): Hydroxychloroquine PO 200 to 400
mg daily as a single daily dose or in 2 divided doses AND
prednisone oral; 40-60 mg/day
Alternative regimen (2): Mycophenolate , - For induction: 1 g
twice daily for 6 months in combination with a glucocorticoid ,
- For maintenance: 0.5-3 g daily or 1 g twice daily . Initial
period of intensive immunosuppressive therapy (induction
therapy) to control the disease and halt tissue injury
Alternative regimen (3): Cyclophosphamide IV 500 mg once
every 2 weeks for 6 doses or 500 to 1,000 mg/m2 once every
month for 6 doses or 500 to 1,000 mg/m2 every month for 6
months, then every 3 months for a total of at least 2.5 years
Alternative regimen (4): Rituximab IV: 375 mg/m2 once
weekly for 4 doses or 1,000 mg (flat dose) on days 0 and 15
or 500 to 1,000 mg on days 1 and 15
Fever management : Preferred regimen: Celecoxib PO 100
to 200 mg twice daily , For fever management even in SLE
patients with “sulfa” allergy
Alternative regimen: Acetaminophen 1000 mg every 6
hours; maximum daily dose: 3000 mg daily
Raynaud's phenomenon treatment
Preferred regimen (1): Calcium channel blocker (nifedipine) 10 to 30 mg 3 times daily
Preferred regimen (2): Antiplatelet therapy with low-dose aspirin (75 or 81 mg/day) in all
patients with secondary Raynaud phenomenon
Alternative regimen (1): Phosphodiesterase (PDE) inhibitor (sildenafil) 20 mg once or
twice daily
Inadequate response to a CCB
Alternative regimen (2): Addition of topical nitroglycerin (NTG)
Inadequate response to a CCB
A PDE inhibitor is not available, effective, or well-tolerated
Alternative regimen (3): Intravenous (IV) infusions of a prostaglandin (PG) especially
prostacyclin (PGI2) analogue for extremely severe patients with raynaud's phenomenon
Lupus nephritis treatment :
Preferred regimen: Immunosuppressive therapy with
glucocorticoids plus either intravenous or oral
mycophenolate mofetil: 0.5 g of mycophenolate mofetil
twice daily for the first week, then 1 g twice daily for the
second week, and thereafter increase the dose to 1.5 g
twice daily
Prevention
Primary Prevention :
There is no established method for prevention of systemic lupus
erythematosus. However, preventing exposure to the risk factors
listed below, especially flare-up-related risk factors, can help to
decrease flare-up episodes or may even be helpful in SLE
prevention:
Preventing exposure to ultraviolet (UV) light
Cigarette smoking cessation
Prevent working in crystalline silica work environment (e.g., cleaning
powders, soil, pottery materials, cement, etc.)
Prevent caring for a pet (especially a dog)
Secondary Prevention :
Aims of secondary prevention measures for SLE include:[1][2]
Preventing glucocorticoid usage and using the minimum dosage to prevent
osteonecrosis and osteoprosis side effects
Decreasing atherosclerotic events in patients
Low doses of aspirin
ACE inhibitors
Statins
For prevention and/or early diagnosis of malignancies: Regular age-related
specific cancer screening recommended for the general population