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SYSTEMIC LUPUS
ERYTHEMATOSUS
Presented by:Saakshi Deokar
Roll no: 303
Subject: Pharmacotherapeutics
SYSTEMIC LUPUS ERYTHEMATOSUS
o Systemic lupus erythematosus (SLE), is the most common type of lupus.
SLE is an autoimmune disease in which the immune system attacks its
own tissues, causing widespread inflammation and tissue damage in the
affected organs. It can affect the joints, skin, brain, lungs, kidneys, and
blood vessels.
o The hallmark of this disease is production of antibodies against cell
nuclear components that leads to chronic inflammatory autoimmune
disease.
ETIOLOGY
◦ Genetic susceptibility
1.Immunoglobulin receptor
◦ Chemicals
1.Hydrazine
2.Aromatic amines
◦ Environmental factors
1.Sunlight
2.Infections ( Epstein –Barr virus )
◦ Estrogen levels
◦ Elevated levels of prolactin
PATHOPHYSIOLOGY
◦ Summary of the pathogenesis of systemic lupus erythematosus (SLE) and
resveratrol mechanisms of action. The immune dysregulation caused by
environmental triggers and genetic predisposition leads to increased apoptosis.
Decreased clearance causes recognition of self-antigens by the immune system,
activation of B and T cells, and the production of self-antibodies. Those
antibodies, mainly IgG, cause the formation of immune complexes that can lead
to renal impairment in kidneys and vasculopathy and atheromatous plaque
formation in blood vessels. Atheroma plaques are caused by the action of anti-
oxidized low-density lipoprotein (LDL), which is very common in SLE, which leads
to the decrease of ABCA1 and ABCG1 by a yet unknown mechanism.
ANTIBODIES Which are formed during
this process
◦ Certain ab are formed against the following parameters
1.ds DNA
2.ss DNA
3.Histone
4.Smith Ag
5.Small nuclear ribo cleoprotein
6.Ro Ag
7.La Ag
8.Prothrombin activator complex
9.cardiolipin
Clinical manifestations
◦ Non specific Manifestations are present .
1. Fatigue
2. Fever
3. Anorexia
4. Weight loss
◦ Musculoskeletal
1. Arthralgia
2. Myalgia
3. Arthritis
Some Diagnostic Symptoms:
 Butterfly (malar) rash on cheeks
 Rash on face, arms, neck, torso (discoid rash)
 Skin rashes that result from exposure to sunlight or ultraviolet light (photosensitivity)
 Mouth or nasal sores (ulcers), usually painless
 Joint swelling, stiffness, pain involving two or more joints (arthritis)
 Inflammation of the membranes surrounding the lungs (pleuritis) or heart (pericarditis)
 Abnormalities in urine, such as increased protein or clumps of red blood cells or kidney
cells, called cell casts
 Nervous system problems, such as seizures or psychosis, without known cause
 Problems with the blood, such as reduced numbers of red blood cells (anemia), platelets,
or white blood cells
 Laboratory tests showing increased autoimmune activity (antibodies against normal
tissue)
 Positive antinuclear antibody (ANA) test
OTHER LUPIC SYMPTOMS
Chest pain
Hair loss
Mouth sores
Photosensitivity
Anemia
Repeated miscarriages
Headache
Dizziness
Depression
Seizures
Memory disturbances
clinical investigations
o FLUROSCENT ANTINUCLEAR ANTIBODY(ANA)
◦ ECG
◦ DOPPLER
◦ BLOOD CULTURE
◦ ANTI-DNA
◦ ANTI-RNP
◦ ANTI-RO
◦ ANTI-LA
OTHER LABORATORY TESTS LIKE :
 CBC { RBC, WBC,PLATELETS }
 URINANALYSIS
 SEDIMENTATION RATE
 RHEUMATOID FACTOR
 SKIN BIOPSAY
 KIDNEY BIOPSAY
TREATMENT
◦ NON PHARMACOLOGICAL
1. Enough rest
2. Minimal exertion
3. Avoid smoking
4. Limit exposure to sunlight
TREATMENT
◦ PHARMACOLOGICAL
DRUGS
1.NSAID’S
Aspirin,Ibuprofen,Indomet
hacin
2.CORTICOSTEROIDS
Prednisolone
Prednisone
Methylprednisolone
Hydrocortisone
3.ANTIMALARIAL
HYDROCHLOROQUINE/CH
LOROQUINES
4. Immune suppressants
Cyclophosphamide
AZATHIOPRINE
DOSE AND ROUTE
440mg OD/220mg every 8
to 12 hours
1-2MG/KG/DAY
200-400MG PO OD
1-3MG/KG PO OD
1.5-2.5 mg/kg PO OD
INDICATION
FEVER
ARTHRITIS
SKIN RASH
SEVERE
inflammation
CONDITION
MILD CONDTION
OF ARTHIRITIS
LUPUS
NEPHRITIS
To treat
autoimmune
CASE PRESENTATION
◦ DEMOGRAPHIC DETAILS
Name-XYZ
Age-15years
Gender-female
Weight-38kg
Height-5.3ft
D.O.A-29/11/19
D.O.D-28/12/19
CHIEF COMPLAINTS
• Chest pain in left side
• Body ache
• Fever
PAST MEDICAL HISTORY
k/c/o SLE
PAST MEDICATION HISTORY
Bone marrow aspiration { Breast bone}
SOCIAL HISTORY
No social history
Marital status :Unmarried
LABORATORY FINDINGS
NO.
1
2
3
4
5
6
7
8
TEST NAME
RBC
ESR
WBC
LYMPHOCYTES
MID CELLS
NEUTROPHILS
EOSINOPHILS
HAEMOGLOBIN
MCV
MCH
MCHC
PLATELET
NORMAL VALUES
4-5m/cmm
0-20mm/hr
5000-10000/cmm
20-45%
1-10%
40-75%
1-6%
12-15g/dl
75-95fL
25-35pg
30-35g/dl
1500000-
4500000/cmm
TEST VALUE
4.12m/cmm
64mm/hr
1000/cmm
20.8%
5.8%
72.9%
0.4%
10.7g/dl
83.5fL
26.0pg
31.1g/dl
7930/cmm
NO.
9
10
11
12
13
14
15
16
17
18
TESTS
HEART RATE
RESPIRATORY RATE
CPK
Na
K
Cl
Ca
SERUM CREATININE
NORMAL VALUE
60-100/min
12-20/min
55-170IU/L
136-145mmol/L
3.5-5.1mmol/L
98-107mmol/L
4.3-5.3mmol/L
0.5-1.11mg%
TEST VALUE
140/min
30/min
30IU/L
127mmol/L
4.2mmol/L
94mmol/L
1.5mmol/L
0.46mg%
Other investigations
1.Doppler(Lower limb)
Normal arterial and venous colour
2.ECG
Tachycardia
3.Blood culture
No growth
Provisional diagnosis
Pancytopenia
Final diagnosis
SLE
THROMBOCYTOPENIA
TREATMENT
1.TAB Romiplastinm 250mcg once in every 2weeks
2.TAB dexamethasone 4mg OD
3. TAB hydroquinine 200mg OD
4.TAB calcimax forte 250mg BD
5.TAB Folvite 5mg OD
Clinical notes
Day1
Right shoulder pain with dry cough.
Investigations- hypocalcemia , hypophosphatamia
Advice-CBC
Results-thrombocytopenia
Day2
Severe abdominal pain.
Advice-USG abdomen
Results-mild ascites , splenomegaly , bilateral pleural effusion
Day3
Abdominal pain
Advice-started with methylprednisolone and hydroxychlorethine
Day4
Increased drowsiness , headache , nausea , bradycardia
Day5
Patient shifted to ICU after bradycardia and hypotension.
Impression-CNS depression due to steroids overdose
Advice-stop steroid Tramadol
Day6
Patient is drowsy
MRI shows lepto meningia enhancement
To start- maletol IV (200mg)
Day7
Severe pain in lower limbs , more in ankles
Advice-Doppler , CBC , creatinine
Results-normal arterial and venous colour.
To start- rhomboplastin IV in every 2weeks
Day8
No headache , no fever , no lower limb , better clinically.
To continue rhomboplastin IV in every 2weeks.
Discharge medication
◦ As the patient was in a good condition no other medications than
rhomboplastin was suggested.
◦ Patient was advised To continue rhomboplastin IV in every 2weeks.
PATIENT COUNCILING
◦ Patients should be provided with information, counseling and support
◦ Involve all the family members in counseling regarding the disease
◦ Family must have a thorough understanding of the disease, its potential severity
and complications of the disease and treatment
◦ Patient must learn to cope and monitor the disease
◦ He or she should be able to distinguish signs and symptoms that may precede a
disease flare and consult with the physician immediately
◦ Educate all patients regarding possible complications from unplanned
pregnancy, poor compliance , recreational drug use and infection
◦ For married patients, contraception is important during the active phase of the
disease
◦ Advise patients to join other SLE patients in support groups
◦ Patients must be advised to wear clothing with long sleeves, use umbrella or
sunscreen lotion and refrain from sunbathing to prevent exacerbation of
dermatologic symptoms
◦ Use sunscreen with at least a sun-protection factor (SPF) of 15-30 to prevent
dermal or systemic disease flares upon exposure to ultraviolet light
◦ Patients must have appropriate diet to prevent obesity, osteoporosis and dyslipidemia
◦ Patients on corticosteroids should have a no-added-salt, low-fat and calcium-sufficient diet
◦ Advise patients to seek medical help when they have fever
◦ Exposure to immunosuppressive drugs used in SLE may predispose patients to infection
◦ Encourage patients to maintain a normal lifestyle
◦ Regular exercise to maintain appropriate weight and bone density
◦ Advise patients that fatigue and stress are associated with disease flare
◦ Smoking cessation is recommended
◦ Patient’s blood pressure and glucose and lipid levels should be assessed and managed
appropriately
◦ Immunization should be updated for all patients
◦ Patients should have appropriate immunizations (ie influenza, pneumococcal)
◦ Patients with SLE are often complement deficient and are at risk for encapsulated organisms
◦ Antibiotic prophylaxis for all dental, genitourinary and other invasive procedures for patients at
high risk of infections (e.g. patients with valvular abnormalities, on immunosuppressive therapy)
◦ Patients should routinely undergo gynecologic assessments, dental care and ophthalmologic
exam especially for patients taking corticosteroids and Hydroxychloroquine
◦ Psychological support may also be necessary
THERAPEUTIC
INTERPRETENTION
◦ Advances in diagnostics, optimized treatment (also of comorbidities), and regular
monitoring of disease activity and damage have clearly improved the prognosis
of SLE. The goal of treatment is remission or at least minimization of disease
activity and prevention of flares. Nowadays, antimalarials are the basic treatment
for every patient with SLE, whereas glucocorticoids should only be used when
acutely indicated. If reduction or tapering of glucocorticoids proves impossible,
extended immunosuppression with azathioprine, methotrexate, or
mycophenolate mofetil is recommended. Should the patient fail to respond,
belimumab can be administered.
◦ Although mycophenolate mofetil is not officially approved for use in SLE, there is
now international consensus that its good efficacy with low toxicity make this
agent an effective alternative to short-term administration of low-dose
intravenous cyclophosphamide in the induction therapy for proliferative lupus
nephritis.
Reference
1.Fauci,Kasper,Hauser;”Harrison’s principles of internal
medicine;17th edition; page no.2066 to 2078
2.Joseph dipiro,Robert talbert;”Pharmacotherapy A
pathophysiologic approach;7th edition;page no.1435 to 1445
SLE.ppt.pptx

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SLE.ppt.pptx

  • 1. SYSTEMIC LUPUS ERYTHEMATOSUS Presented by:Saakshi Deokar Roll no: 303 Subject: Pharmacotherapeutics
  • 2. SYSTEMIC LUPUS ERYTHEMATOSUS o Systemic lupus erythematosus (SLE), is the most common type of lupus. SLE is an autoimmune disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs. It can affect the joints, skin, brain, lungs, kidneys, and blood vessels. o The hallmark of this disease is production of antibodies against cell nuclear components that leads to chronic inflammatory autoimmune disease.
  • 3. ETIOLOGY ◦ Genetic susceptibility 1.Immunoglobulin receptor ◦ Chemicals 1.Hydrazine 2.Aromatic amines ◦ Environmental factors 1.Sunlight 2.Infections ( Epstein –Barr virus ) ◦ Estrogen levels ◦ Elevated levels of prolactin
  • 5. ◦ Summary of the pathogenesis of systemic lupus erythematosus (SLE) and resveratrol mechanisms of action. The immune dysregulation caused by environmental triggers and genetic predisposition leads to increased apoptosis. Decreased clearance causes recognition of self-antigens by the immune system, activation of B and T cells, and the production of self-antibodies. Those antibodies, mainly IgG, cause the formation of immune complexes that can lead to renal impairment in kidneys and vasculopathy and atheromatous plaque formation in blood vessels. Atheroma plaques are caused by the action of anti- oxidized low-density lipoprotein (LDL), which is very common in SLE, which leads to the decrease of ABCA1 and ABCG1 by a yet unknown mechanism.
  • 6. ANTIBODIES Which are formed during this process ◦ Certain ab are formed against the following parameters 1.ds DNA 2.ss DNA 3.Histone 4.Smith Ag 5.Small nuclear ribo cleoprotein 6.Ro Ag 7.La Ag 8.Prothrombin activator complex 9.cardiolipin
  • 7. Clinical manifestations ◦ Non specific Manifestations are present . 1. Fatigue 2. Fever 3. Anorexia 4. Weight loss ◦ Musculoskeletal 1. Arthralgia 2. Myalgia 3. Arthritis
  • 8. Some Diagnostic Symptoms:  Butterfly (malar) rash on cheeks  Rash on face, arms, neck, torso (discoid rash)  Skin rashes that result from exposure to sunlight or ultraviolet light (photosensitivity)  Mouth or nasal sores (ulcers), usually painless  Joint swelling, stiffness, pain involving two or more joints (arthritis)  Inflammation of the membranes surrounding the lungs (pleuritis) or heart (pericarditis)  Abnormalities in urine, such as increased protein or clumps of red blood cells or kidney cells, called cell casts  Nervous system problems, such as seizures or psychosis, without known cause  Problems with the blood, such as reduced numbers of red blood cells (anemia), platelets, or white blood cells  Laboratory tests showing increased autoimmune activity (antibodies against normal tissue)  Positive antinuclear antibody (ANA) test
  • 9. OTHER LUPIC SYMPTOMS Chest pain Hair loss Mouth sores Photosensitivity Anemia Repeated miscarriages Headache Dizziness Depression Seizures Memory disturbances
  • 10. clinical investigations o FLUROSCENT ANTINUCLEAR ANTIBODY(ANA) ◦ ECG ◦ DOPPLER ◦ BLOOD CULTURE ◦ ANTI-DNA ◦ ANTI-RNP ◦ ANTI-RO ◦ ANTI-LA
  • 11. OTHER LABORATORY TESTS LIKE :  CBC { RBC, WBC,PLATELETS }  URINANALYSIS  SEDIMENTATION RATE  RHEUMATOID FACTOR  SKIN BIOPSAY  KIDNEY BIOPSAY
  • 12. TREATMENT ◦ NON PHARMACOLOGICAL 1. Enough rest 2. Minimal exertion 3. Avoid smoking 4. Limit exposure to sunlight
  • 13. TREATMENT ◦ PHARMACOLOGICAL DRUGS 1.NSAID’S Aspirin,Ibuprofen,Indomet hacin 2.CORTICOSTEROIDS Prednisolone Prednisone Methylprednisolone Hydrocortisone 3.ANTIMALARIAL HYDROCHLOROQUINE/CH LOROQUINES 4. Immune suppressants Cyclophosphamide AZATHIOPRINE DOSE AND ROUTE 440mg OD/220mg every 8 to 12 hours 1-2MG/KG/DAY 200-400MG PO OD 1-3MG/KG PO OD 1.5-2.5 mg/kg PO OD INDICATION FEVER ARTHRITIS SKIN RASH SEVERE inflammation CONDITION MILD CONDTION OF ARTHIRITIS LUPUS NEPHRITIS To treat autoimmune
  • 14. CASE PRESENTATION ◦ DEMOGRAPHIC DETAILS Name-XYZ Age-15years Gender-female Weight-38kg Height-5.3ft D.O.A-29/11/19 D.O.D-28/12/19
  • 15. CHIEF COMPLAINTS • Chest pain in left side • Body ache • Fever PAST MEDICAL HISTORY k/c/o SLE PAST MEDICATION HISTORY Bone marrow aspiration { Breast bone} SOCIAL HISTORY No social history Marital status :Unmarried
  • 16. LABORATORY FINDINGS NO. 1 2 3 4 5 6 7 8 TEST NAME RBC ESR WBC LYMPHOCYTES MID CELLS NEUTROPHILS EOSINOPHILS HAEMOGLOBIN MCV MCH MCHC PLATELET NORMAL VALUES 4-5m/cmm 0-20mm/hr 5000-10000/cmm 20-45% 1-10% 40-75% 1-6% 12-15g/dl 75-95fL 25-35pg 30-35g/dl 1500000- 4500000/cmm TEST VALUE 4.12m/cmm 64mm/hr 1000/cmm 20.8% 5.8% 72.9% 0.4% 10.7g/dl 83.5fL 26.0pg 31.1g/dl 7930/cmm
  • 17. NO. 9 10 11 12 13 14 15 16 17 18 TESTS HEART RATE RESPIRATORY RATE CPK Na K Cl Ca SERUM CREATININE NORMAL VALUE 60-100/min 12-20/min 55-170IU/L 136-145mmol/L 3.5-5.1mmol/L 98-107mmol/L 4.3-5.3mmol/L 0.5-1.11mg% TEST VALUE 140/min 30/min 30IU/L 127mmol/L 4.2mmol/L 94mmol/L 1.5mmol/L 0.46mg%
  • 18. Other investigations 1.Doppler(Lower limb) Normal arterial and venous colour 2.ECG Tachycardia 3.Blood culture No growth
  • 20. TREATMENT 1.TAB Romiplastinm 250mcg once in every 2weeks 2.TAB dexamethasone 4mg OD 3. TAB hydroquinine 200mg OD 4.TAB calcimax forte 250mg BD 5.TAB Folvite 5mg OD
  • 21. Clinical notes Day1 Right shoulder pain with dry cough. Investigations- hypocalcemia , hypophosphatamia Advice-CBC Results-thrombocytopenia Day2 Severe abdominal pain. Advice-USG abdomen Results-mild ascites , splenomegaly , bilateral pleural effusion Day3 Abdominal pain Advice-started with methylprednisolone and hydroxychlorethine Day4 Increased drowsiness , headache , nausea , bradycardia
  • 22. Day5 Patient shifted to ICU after bradycardia and hypotension. Impression-CNS depression due to steroids overdose Advice-stop steroid Tramadol Day6 Patient is drowsy MRI shows lepto meningia enhancement To start- maletol IV (200mg) Day7 Severe pain in lower limbs , more in ankles Advice-Doppler , CBC , creatinine Results-normal arterial and venous colour. To start- rhomboplastin IV in every 2weeks Day8 No headache , no fever , no lower limb , better clinically. To continue rhomboplastin IV in every 2weeks.
  • 23. Discharge medication ◦ As the patient was in a good condition no other medications than rhomboplastin was suggested. ◦ Patient was advised To continue rhomboplastin IV in every 2weeks.
  • 24. PATIENT COUNCILING ◦ Patients should be provided with information, counseling and support ◦ Involve all the family members in counseling regarding the disease ◦ Family must have a thorough understanding of the disease, its potential severity and complications of the disease and treatment ◦ Patient must learn to cope and monitor the disease ◦ He or she should be able to distinguish signs and symptoms that may precede a disease flare and consult with the physician immediately ◦ Educate all patients regarding possible complications from unplanned pregnancy, poor compliance , recreational drug use and infection ◦ For married patients, contraception is important during the active phase of the disease ◦ Advise patients to join other SLE patients in support groups ◦ Patients must be advised to wear clothing with long sleeves, use umbrella or sunscreen lotion and refrain from sunbathing to prevent exacerbation of dermatologic symptoms ◦ Use sunscreen with at least a sun-protection factor (SPF) of 15-30 to prevent dermal or systemic disease flares upon exposure to ultraviolet light
  • 25. ◦ Patients must have appropriate diet to prevent obesity, osteoporosis and dyslipidemia ◦ Patients on corticosteroids should have a no-added-salt, low-fat and calcium-sufficient diet ◦ Advise patients to seek medical help when they have fever ◦ Exposure to immunosuppressive drugs used in SLE may predispose patients to infection ◦ Encourage patients to maintain a normal lifestyle ◦ Regular exercise to maintain appropriate weight and bone density ◦ Advise patients that fatigue and stress are associated with disease flare ◦ Smoking cessation is recommended ◦ Patient’s blood pressure and glucose and lipid levels should be assessed and managed appropriately ◦ Immunization should be updated for all patients ◦ Patients should have appropriate immunizations (ie influenza, pneumococcal) ◦ Patients with SLE are often complement deficient and are at risk for encapsulated organisms ◦ Antibiotic prophylaxis for all dental, genitourinary and other invasive procedures for patients at high risk of infections (e.g. patients with valvular abnormalities, on immunosuppressive therapy) ◦ Patients should routinely undergo gynecologic assessments, dental care and ophthalmologic exam especially for patients taking corticosteroids and Hydroxychloroquine ◦ Psychological support may also be necessary
  • 26. THERAPEUTIC INTERPRETENTION ◦ Advances in diagnostics, optimized treatment (also of comorbidities), and regular monitoring of disease activity and damage have clearly improved the prognosis of SLE. The goal of treatment is remission or at least minimization of disease activity and prevention of flares. Nowadays, antimalarials are the basic treatment for every patient with SLE, whereas glucocorticoids should only be used when acutely indicated. If reduction or tapering of glucocorticoids proves impossible, extended immunosuppression with azathioprine, methotrexate, or mycophenolate mofetil is recommended. Should the patient fail to respond, belimumab can be administered. ◦ Although mycophenolate mofetil is not officially approved for use in SLE, there is now international consensus that its good efficacy with low toxicity make this agent an effective alternative to short-term administration of low-dose intravenous cyclophosphamide in the induction therapy for proliferative lupus nephritis.
  • 27. Reference 1.Fauci,Kasper,Hauser;”Harrison’s principles of internal medicine;17th edition; page no.2066 to 2078 2.Joseph dipiro,Robert talbert;”Pharmacotherapy A pathophysiologic approach;7th edition;page no.1435 to 1445