A 22-year-old college student presents with symptoms including a malar rash, photosensitivity, arthritis, fatigue, and hair loss. Laboratory tests show a positive ANA, positive anti-Smith antibody, and low white blood cell and platelet counts. This constellation of clinical features and laboratory results makes systemic lupus erythematosus the most likely diagnosis for the patient.
SLE still an enigma where both patient and health care professionals are blind and do more harm than saving the patient. Hope in future anything can be done to save the patient from the grip of lupus,
SLE still an enigma where both patient and health care professionals are blind and do more harm than saving the patient. Hope in future anything can be done to save the patient from the grip of lupus,
Pathogenesis systemic lupus erythematosus by dr bashir ahmed dar associate pr...Prof Dr Bashir Ahmed Dar
Systemic lupus erythematosus is a chronic, multisystem, inflammatory disorder of autoimmune etiology, occurring predominantly in young women. Common manifestations may include arthralgias and arthritis; malar and other skin rashes; pleuritis or pericarditis; renal or CNS involvement; and hematologic cytopenias.
Vasculitis syndrome an approach -and-basic principles of treatmentSachin Verma
Vasculitides are a hetrogenous group of conditions characterized by inflammation and necrosis of blood vessels.
A broad group of syndromes may result from this process,since any type,size, and location of vessel may be involved.
Pathogenesis systemic lupus erythematosus by dr bashir ahmed dar associate pr...Prof Dr Bashir Ahmed Dar
Systemic lupus erythematosus is a chronic, multisystem, inflammatory disorder of autoimmune etiology, occurring predominantly in young women. Common manifestations may include arthralgias and arthritis; malar and other skin rashes; pleuritis or pericarditis; renal or CNS involvement; and hematologic cytopenias.
Vasculitis syndrome an approach -and-basic principles of treatmentSachin Verma
Vasculitides are a hetrogenous group of conditions characterized by inflammation and necrosis of blood vessels.
A broad group of syndromes may result from this process,since any type,size, and location of vessel may be involved.
Systemic Lupus erythematous , is world wide health problem
Here we talk about criteria for diagnosis investigation , Management and complication
With some scenarios to about disease and complication
Taking History and Physical Examination in Hematology.pptxAskin Kaplan
Medical history taking
Assessment of symptoms
Reviewing previous records
Understanding the onset or progression of illness
Personal medical history for other disorders that can cause blood count abnormalities
Hematologic manifestations secondary to other diseases occur more frequently than primary hematologic diseases
Person’s ethnicity or race
Exposures; drugs, chemicals, toxins, radiation, alcohol
Family history
Title: Sense of Smell
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the primary categories of smells and the concept of odor blindness.
Explain the structure and location of the olfactory membrane and mucosa, including the types and roles of cells involved in olfaction.
Describe the pathway and mechanisms of olfactory signal transmission from the olfactory receptors to the brain.
Illustrate the biochemical cascade triggered by odorant binding to olfactory receptors, including the role of G-proteins and second messengers in generating an action potential.
Identify different types of olfactory disorders such as anosmia, hyposmia, hyperosmia, and dysosmia, including their potential causes.
Key Topics:
Olfactory Genes:
3% of the human genome accounts for olfactory genes.
400 genes for odorant receptors.
Olfactory Membrane:
Located in the superior part of the nasal cavity.
Medially: Folds downward along the superior septum.
Laterally: Folds over the superior turbinate and upper surface of the middle turbinate.
Total surface area: 5-10 square centimeters.
Olfactory Mucosa:
Olfactory Cells: Bipolar nerve cells derived from the CNS (100 million), with 4-25 olfactory cilia per cell.
Sustentacular Cells: Produce mucus and maintain ionic and molecular environment.
Basal Cells: Replace worn-out olfactory cells with an average lifespan of 1-2 months.
Bowman’s Gland: Secretes mucus.
Stimulation of Olfactory Cells:
Odorant dissolves in mucus and attaches to receptors on olfactory cilia.
Involves a cascade effect through G-proteins and second messengers, leading to depolarization and action potential generation in the olfactory nerve.
Quality of a Good Odorant:
Small (3-20 Carbon atoms), volatile, water-soluble, and lipid-soluble.
Facilitated by odorant-binding proteins in mucus.
Membrane Potential and Action Potential:
Resting membrane potential: -55mV.
Action potential frequency in the olfactory nerve increases with odorant strength.
Adaptation Towards the Sense of Smell:
Rapid adaptation within the first second, with further slow adaptation.
Psychological adaptation greater than receptor adaptation, involving feedback inhibition from the central nervous system.
Primary Sensations of Smell:
Camphoraceous, Musky, Floral, Pepperminty, Ethereal, Pungent, Putrid.
Odor Detection Threshold:
Examples: Hydrogen sulfide (0.0005 ppm), Methyl-mercaptan (0.002 ppm).
Some toxic substances are odorless at lethal concentrations.
Characteristics of Smell:
Odor blindness for single substances due to lack of appropriate receptor protein.
Behavioral and emotional influences of smell.
Transmission of Olfactory Signals:
From olfactory cells to glomeruli in the olfactory bulb, involving lateral inhibition.
Primitive, less old, and new olfactory systems with different path
HOT NEW PRODUCT! BIG SALES FAST SHIPPING NOW FROM CHINA!! EU KU DB BK substit...GL Anaacs
Contact us if you are interested:
Email / Skype : kefaya1771@gmail.com
Threema: PXHY5PDH
New BATCH Ku !!! MUCH IN DEMAND FAST SALE EVERY BATCH HAPPY GOOD EFFECT BIG BATCH !
Contact me on Threema or skype to start big business!!
Hot-sale products:
NEW HOT EUTYLONE WHITE CRYSTAL!!
5cl-adba precursor (semi finished )
5cl-adba raw materials
ADBB precursor (semi finished )
ADBB raw materials
APVP powder
5fadb/4f-adb
Jwh018 / Jwh210
Eutylone crystal
Protonitazene (hydrochloride) CAS: 119276-01-6
Flubrotizolam CAS: 57801-95-3
Metonitazene CAS: 14680-51-4
Payment terms: Western Union,MoneyGram,Bitcoin or USDT.
Deliver Time: Usually 7-15days
Shipping method: FedEx, TNT, DHL,UPS etc.Our deliveries are 100% safe, fast, reliable and discreet.
Samples will be sent for your evaluation!If you are interested in, please contact me, let's talk details.
We specializes in exporting high quality Research chemical, medical intermediate, Pharmaceutical chemicals and so on. Products are exported to USA, Canada, France, Korea, Japan,Russia, Southeast Asia and other countries.
ARTIFICIAL INTELLIGENCE IN HEALTHCARE.pdfAnujkumaranit
Artificial intelligence (AI) refers to the simulation of human intelligence processes by machines, especially computer systems. It encompasses tasks such as learning, reasoning, problem-solving, perception, and language understanding. AI technologies are revolutionizing various fields, from healthcare to finance, by enabling machines to perform tasks that typically require human intelligence.
Title: Sense of Taste
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the structure and function of taste buds.
Describe the relationship between the taste threshold and taste index of common substances.
Explain the chemical basis and signal transduction of taste perception for each type of primary taste sensation.
Recognize different abnormalities of taste perception and their causes.
Key Topics:
Significance of Taste Sensation:
Differentiation between pleasant and harmful food
Influence on behavior
Selection of food based on metabolic needs
Receptors of Taste:
Taste buds on the tongue
Influence of sense of smell, texture of food, and pain stimulation (e.g., by pepper)
Primary and Secondary Taste Sensations:
Primary taste sensations: Sweet, Sour, Salty, Bitter, Umami
Chemical basis and signal transduction mechanisms for each taste
Taste Threshold and Index:
Taste threshold values for Sweet (sucrose), Salty (NaCl), Sour (HCl), and Bitter (Quinine)
Taste index relationship: Inversely proportional to taste threshold
Taste Blindness:
Inability to taste certain substances, particularly thiourea compounds
Example: Phenylthiocarbamide
Structure and Function of Taste Buds:
Composition: Epithelial cells, Sustentacular/Supporting cells, Taste cells, Basal cells
Features: Taste pores, Taste hairs/microvilli, and Taste nerve fibers
Location of Taste Buds:
Found in papillae of the tongue (Fungiform, Circumvallate, Foliate)
Also present on the palate, tonsillar pillars, epiglottis, and proximal esophagus
Mechanism of Taste Stimulation:
Interaction of taste substances with receptors on microvilli
Signal transduction pathways for Umami, Sweet, Bitter, Sour, and Salty tastes
Taste Sensitivity and Adaptation:
Decrease in sensitivity with age
Rapid adaptation of taste sensation
Role of Saliva in Taste:
Dissolution of tastants to reach receptors
Washing away the stimulus
Taste Preferences and Aversions:
Mechanisms behind taste preference and aversion
Influence of receptors and neural pathways
Impact of Sensory Nerve Damage:
Degeneration of taste buds if the sensory nerve fiber is cut
Abnormalities of Taste Detection:
Conditions: Ageusia, Hypogeusia, Dysgeusia (parageusia)
Causes: Nerve damage, neurological disorders, infections, poor oral hygiene, adverse drug effects, deficiencies, aging, tobacco use, altered neurotransmitter levels
Neurotransmitters and Taste Threshold:
Effects of serotonin (5-HT) and norepinephrine (NE) on taste sensitivity
Supertasters:
25% of the population with heightened sensitivity to taste, especially bitterness
Increased number of fungiform papillae
Couples presenting to the infertility clinic- Do they really have infertility...Sujoy Dasgupta
Dr Sujoy Dasgupta presented the study on "Couples presenting to the infertility clinic- Do they really have infertility? – The unexplored stories of non-consummation" in the 13th Congress of the Asia Pacific Initiative on Reproduction (ASPIRE 2024) at Manila on 24 May, 2024.
Prix Galien International 2024 Forum ProgramLevi Shapiro
June 20, 2024, Prix Galien International and Jerusalem Ethics Forum in ROME. Detailed agenda including panels:
- ADVANCES IN CARDIOLOGY: A NEW PARADIGM IS COMING
- WOMEN’S HEALTH: FERTILITY PRESERVATION
- WHAT’S NEW IN THE TREATMENT OF INFECTIOUS,
ONCOLOGICAL AND INFLAMMATORY SKIN DISEASES?
- ARTIFICIAL INTELLIGENCE AND ETHICS
- GENE THERAPY
- BEYOND BORDERS: GLOBAL INITIATIVES FOR DEMOCRATIZING LIFE SCIENCE TECHNOLOGIES AND PROMOTING ACCESS TO HEALTHCARE
- ETHICAL CHALLENGES IN LIFE SCIENCES
- Prix Galien International Awards Ceremony
Knee anatomy and clinical tests 2024.pdfvimalpl1234
This includes all relevant anatomy and clinical tests compiled from standard textbooks, Campbell,netter etc..It is comprehensive and best suited for orthopaedicians and orthopaedic residents.
263778731218 Abortion Clinic /Pills In Harare ,sisternakatoto
263778731218 Abortion Clinic /Pills In Harare ,ABORTION WOMEN’S CLINIC +27730423979 IN women clinic we believe that every woman should be able to make choices in her pregnancy. Our job is to provide compassionate care, safety,affordable and confidential services. That’s why we have won the trust from all generations of women all over the world. we use non surgical method(Abortion pills) to terminate…Dr.LISA +27730423979women Clinic is committed to providing the highest quality of obstetrical and gynecological care to women of all ages. Our dedicated staff aim to treat each patient and her health concerns with compassion and respect.Our dedicated group ABORTION WOMEN’S CLINIC +27730423979 IN women clinic we believe that every woman should be able to make choices in her pregnancy. Our job is to provide compassionate care, safety,affordable and confidential services. That’s why we have won the trust from all generations of women all over the world. we use non surgical method(Abortion pills) to terminate…Dr.LISA +27730423979women Clinic is committed to providing the highest quality of obstetrical and gynecological care to women of all ages. Our dedicated staff aim to treat each patient and her health concerns with compassion and respect.Our dedicated group of receptionists, nurses, and physicians have worked together as a teamof receptionists, nurses, and physicians have worked together as a team wwww.lisywomensclinic.co.za/
Report Back from SGO 2024: What’s the Latest in Cervical Cancer?bkling
Are you curious about what’s new in cervical cancer research or unsure what the findings mean? Join Dr. Emily Ko, a gynecologic oncologist at Penn Medicine, to learn about the latest updates from the Society of Gynecologic Oncology (SGO) 2024 Annual Meeting on Women’s Cancer. Dr. Ko will discuss what the research presented at the conference means for you and answer your questions about the new developments.
These lecture slides, by Dr Sidra Arshad, offer a quick overview of physiological basis of a normal electrocardiogram.
Learning objectives:
1. Define an electrocardiogram (ECG) and electrocardiography
2. Describe how dipoles generated by the heart produce the waveforms of the ECG
3. Describe the components of a normal electrocardiogram of a typical bipolar leads (limb II)
4. Differentiate between intervals and segments
5. Enlist some common indications for obtaining an ECG
Study Resources:
1. Chapter 11, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 9, Human Physiology - From Cells to Systems, Lauralee Sherwood, 9th edition
3. Chapter 29, Ganong’s Review of Medical Physiology, 26th edition
4. Electrocardiogram, StatPearls - https://www.ncbi.nlm.nih.gov/books/NBK549803/
5. ECG in Medical Practice by ABM Abdullah, 4th edition
6. ECG Basics, http://www.nataliescasebook.com/tag/e-c-g-basics
New Directions in Targeted Therapeutic Approaches for Older Adults With Mantl...i3 Health
i3 Health is pleased to make the speaker slides from this activity available for use as a non-accredited self-study or teaching resource.
This slide deck presented by Dr. Kami Maddocks, Professor-Clinical in the Division of Hematology and
Associate Division Director for Ambulatory Operations
The Ohio State University Comprehensive Cancer Center, will provide insight into new directions in targeted therapeutic approaches for older adults with mantle cell lymphoma.
STATEMENT OF NEED
Mantle cell lymphoma (MCL) is a rare, aggressive B-cell non-Hodgkin lymphoma (NHL) accounting for 5% to 7% of all lymphomas. Its prognosis ranges from indolent disease that does not require treatment for years to very aggressive disease, which is associated with poor survival (Silkenstedt et al, 2021). Typically, MCL is diagnosed at advanced stage and in older patients who cannot tolerate intensive therapy (NCCN, 2022). Although recent advances have slightly increased remission rates, recurrence and relapse remain very common, leading to a median overall survival between 3 and 6 years (LLS, 2021). Though there are several effective options, progress is still needed towards establishing an accepted frontline approach for MCL (Castellino et al, 2022). Treatment selection and management of MCL are complicated by the heterogeneity of prognosis, advanced age and comorbidities of patients, and lack of an established standard approach for treatment, making it vital that clinicians be familiar with the latest research and advances in this area. In this activity chaired by Michael Wang, MD, Professor in the Department of Lymphoma & Myeloma at MD Anderson Cancer Center, expert faculty will discuss prognostic factors informing treatment, the promising results of recent trials in new therapeutic approaches, and the implications of treatment resistance in therapeutic selection for MCL.
Target Audience
Hematology/oncology fellows, attending faculty, and other health care professionals involved in the treatment of patients with mantle cell lymphoma (MCL).
Learning Objectives
1.) Identify clinical and biological prognostic factors that can guide treatment decision making for older adults with MCL
2.) Evaluate emerging data on targeted therapeutic approaches for treatment-naive and relapsed/refractory MCL and their applicability to older adults
3.) Assess mechanisms of resistance to targeted therapies for MCL and their implications for treatment selection
2. Case Study
• 22 year old college student
• New onset of red cheeks, hives in the
sun, fatigue, Raynaud’s, weight loss,
hair loss and stiff hands in the morning
• Her cousin has JRA
4. Lupus
• Represent a range of disease
processes characterized by the
development of autoantibodies with
associated manifestations and organ
damage
• Some forms limited to skin , or occur
after exposure to a drug
5. SLE
• Systemic lupus erythematosus:
• Prototypical form of lupus
• Multiorgan autoimmune disease that often
presents insidiously with significant
heterogeneity of expression in individuals
• Severity from mild to life threatening
depending on the affected organ
• Medications used for treatment increase
morbidity - organ damage
6. Chronic Cutaneous Lupus
(CCL)
• Limited to the skin
• No systemic manifestations
• Includes:
– Discoid lupus
– Subacute cutaneous lupus erythematosus
– Lupus paniculitis
• Only 5% of CCL develop SLE
7. Drug induced Lupus (DILE)
• Triggered by certain medications
• Resolution after DC of the drug
• Anti-Histone ab test positive
• Absence of anti-DsDNA
10. SLE
• More common in Female than male
• Female : Male ratio 9:1
• Incidence of SLE in US is :
• Women 9.4 per 100,000
• Men 1.54 per 100,000
• More common in African-American and
Hispanic population
11. SLE
• Disease onset more common in the 20s,
and 30s
• May occur at any age
• Symptoms/disease activity “waxes and
wanes”
• “Flares” sometimes evident by clinical
symptoms, other times only by laboratory
results changes
12. SLE
• Pathogenesis :
• Exact cause is unk.
• Genetic factors -10% of SLE patients
have a family member with lupus
• Environmental factors - UV light (most
important)
• Possible Also infections, smoking, and
toxin exposure
13. SLE
• Autoantibodies bind to proteins and
tissues
• Deposition of immune - complexes
leads to an inflammatory cascade
• With activation of complement system
• And production of inflammatory
cytokines
14. SLE Clinical Presentation
• Most commonly affected organ
systems:
– Musculoskeletal
– Dermatological
– Renal
– Hematological
• Any organ can be affected
15. SLE Clinical Presentation
• Musculoskeletal lupus
– Isolated arthralgias ( more often stiffness
rather than pain)
– Synovitis.arthritis similar to RA
– Morning stiffness
– Gel phenomenon after immobility
– Small joints of the hands( MTPs,PIP’s)
– Wrists
16. SLE Clinical Presentation
• Knees are less affected
• Jaccoud’s arthropathy:
– Progressive ulnar deviation
– Swan-necking deformaties
– Reversible or correctible
• Typically non erosive bony changes
18. SLE Dermatological
• Several skin manifestations
• Malar Rash – Butterfly Rash
– Erythematous
– Photosensitive
– Flat or raised - maculopapular
– On the cheeks and Bridge of the nose
– Spares naso-labial folds
21. SLE Dermatological
• Maculopapular rash also common on:
– V-area of the neck, and extremities
– Areas associated to sun exposure
• Biopsy of the skin – demonstrate
immunoglobulin and complement
deposition at the dermal-epidermal
junction: “lupus band sign”
23. SLE Dermatological
• Discoid lupus(chronic cutaneous)
– Involve deeper Dermis, raised patches,
keratotic scaling, follicular plugging
– May lead to permanent loss of hair follicle
– Disfiguring hyper- or hypopigmented scars
may occur after resolution
– Typically on face ( inside ears, above eye
brows, upper palate )
– Neck, scalp and forearms
28. SLE Renal Involvement
• Proteinuria alone
• Proteinuria and Hematuria
• Cellular casts in urine : RBCs
• Glomerular involvement
• Elevated serum creatinine level
• Possible Renal Failure
• May result in Nephrotic Syndrome:
– Low serum albumin, and elevated cholesterol
29. Renal Involvement
• International society of Nephrology
Classification for lupus nephritis
– I minimal mesangial
– II Mesangial proliferative
– III focal proliferative
– IV diffuse proliferative
– V membranous
– VI irreversible renal sclerosis
Done by Kidney Biopsy
Class IV most dangerous – can rapidly progress to
Renal Failure
31. SLE Serositis
• Pleurisy
• Occurs most commonly as pleurisy:
– Pain on deep inspiration
– Sometimes associated with pleural effusion
– Listen for inspiratory / expiratory rub
– Exudative effusion if tapped
• Differentiate from pulmonary emboli and
infection pleuritic chest pain
32. Serositis
• Pericarditis:
– Positional pain
– Worse with recumbency
– Better leaning forward
• Ascites:
– Possible due to serositis
– r/o infarcted bowel, infection, or Budd-Chiari
syndrome (oclussion hepatic or IVC)
33. Hematological abnormalities
• Most common
• Leukopenia and Lymphopenia
• Medication induced cytopenias
– Corticosteroids associated lymphopenia
• Thrombocytopenia
– Antiphospholipid antibodies
– Immunosupressive drugs
– Heparin administration
35. Neurologic manifestations
• Most likely as a result of immunecomplex
deposition in small blood vessels
• Rarely attributable to vasculitis
• Most common neurologic complaint in SLE:
– Cognitive impairment – 80%of SLE patients by 10
years after Dx.
– Represents accumulated damage and not ongoing
CNS SLE
– Formal cognitive function testing to establish
baseline
36. Neurologic manifestations
• Mild to severe
– Seizures: also from thrombosis,uremia,toxic
– Psychosis: think about steroids psychosis
– Encephalopathy
– Coma
38. Organ Damage
• In >50% of SLE patients over time
• Significant % from corticosteroids therapy
– Obesity/ Diabetes
– HTN/ Hyperlipedimia
– Cataracs/Glaucoma
– Osteoporosis/Osteonecrosis
– Infections
– Depression/Psychosis
39. Organ Damage
• Accelerated Atherosclerosis – the major
cause of death in SLE
• Risk of MI – increased 50 fold
• Counsel about modifiable
cardiovascular risks
40. Organ Damage
• Renal damage occurs in at least 25% of
patients with lupus nephritis in spite of
maximal therapy
• Recommendation for renal biopsy:
– In patients with 500mg/day proteinuria
– Active urinary sediment
– Rising serum creatinine
41. Malignancy risk
• Lymphoma and Solid tumors risk is
increased independent to therapy
• Patients with secondary Sjogren’s
syndrome may have special risk of non-
Hodgkin’s Lymphoma
42. Diagnosis
• Often difficult – multiple manifestations
which evolve over time / more in the early
stage
• Clinical diagnosis supported by Hx,
Physical Exam and Laboratory tests
• Make take months to years for the typical
picture to unfold
• ACR classification criteria for SLE is
helpful but not needed for Dx
45. Laboratory tests/Ab testing for
SLE
• Tests for diagnosis
• Tests for prognosis
• Tests to determine appropriate
treatment (What organ involvement is
occurring?)
47. Ab testing for SLE
• ANA(anti-nuclear antibodies)
• Positive in 95-99% of SLE
• Occurs in 20% of healthy people,
elderly, with drugs, thyroid disease,
RA,SS, pulmonary fibrosis.
• If positive + clinical symptoms continue
workup
48. What is ANA?
• Antinuclear antibody is an autoantibody
against part of the cell nucleus
• It is a screening test for SLE: so if
negative, it makes SLE highly unlikely
49. Frequent ANA patterns
• Speckled
• Homogeneous / Diffuse
• Nucleolar
• Rim / Peripheral
• Centromere
50.
51. Positive test
• Titers: stronger positive, the dilution is
larger (higher denominator)
– Ex. 1/1280 is a strong positive
• Pattern can change depending on the
dilution
– Ex. 1/80 speckled and homogenous and
1/640 homogenous
52. Is there utility in following the
titer?
• No
• In general repeating the test is a waste
of money
• A positive test in past or at any time can
count in the diagnostic criteria for SLE
53. When do I order anti DNA?
• It is an auto-antibody directed against
the DNA in the cells nuclei
• Only order anti DNA in the presence of
a positive ANA, when you are clinically
suspecting SLE
• It is very specific for SLE, but very
insensitive
54. What does anti DNA correlate
with?
• It is highly specific for SLE
• It correlates with renal SLE (but not
100%)
• Thus, it can be a bad prognosticator
and it is part of the diagnostic criteria
55. What is an ENA
• ENA is extractable nuclear antigens
• The lab will do a screen to see if it is
positive or negative
• If positive, more assays are done to
determine which antibody is positive
56. ENA examples
• Anti Ro, (anti SSA)
• Anti La, (anti SSB)
• Anti Sm (Smith – fairly sensitive for SLE)
• Anti RNP (goes with MCTD and SLE)
• Anti Scl 70 (Topoisomerase 1) – goes with
diffuse scleroderma esp with interstitial lung
disease
• Other: anti Jo1(myositis)
57. Anti-Ro(anti-SSA) and
La(anti-SSB)
• + Anti Ro:
– is associated with cutaneous SLE features
including rash and photosensitivity
– is often + in ANA negative SLE
– can go with anti La in Sjogren’s S.
– can increase the risk of congenital heart
block in babies whose moms are +
58. The results of the college
student
• WBC 2.8, Hbg 11.1, Plt 43
• Creatinine 1.0, urine neg for protein and
blood
• ANA 1/320 speckled
• ENA: + for anti Smith (Sm)
• antiDNA negative
59. Does she have SLE?
• + ANA
• Low WBC and plt
• + anti Sm
• Malar rash
• Photosensitivity
• Possible inflammatory arthritis
• She has at least 5 criteria
61. Antiphospholipid antibody
syndrome (APS)
• Half are associated with SLE
• Occurs in 10-20% of SLE patients
• Syndrome of arterial and or venous
clotting (CVA, DVT, PE), recurrent
abortions and often livedo reticularis,
low platelets
62. Antiphospholipid antibody
syndrome (APS)
• Positive tests may include
– Lupus anticoagulant (false prolongation of PTT)
– Anticardiolipin antibody (aCL) or other
antiphospholipid antibodies
– False positive VDRL
– Abnormal RVV time (Russel venon viper time)
63. APS
• Treatment varies on symptoms and
signs
• ASA or LMW heparin in pregnancy
• Warfarin if DVT
• ASA and possibly warfarin if CVA
(Cerebro-vascular-accident)
64. SLE Management - treatment
• No cure – chronic condition
• TTo aimed at:
– Reducing inflammation
– Suppressing immune system
– Closely monitoring patients to ID and to treat
disease features as early as possible
– Minimize therapy adverse effects
65. Management - treatment
• Patient Education and prophylactic measures
to avoid flares :
• Sunscreens SPF >30 and protective clothing
– Photosensitivity, Raynaud’s Phen.
• Avoid estrogen containing oral contraceptives
– Lupus flares, hyperthrombotic states
• Avoid medications such as:
– Sulfonamides, Echinacea, and Melatonin
66. Management – treatment
• Low dose ASA for patients with
+Antiphospholipid Abs
– Potentially avoid thrombosis
• Psychological support
– Depression and anxiety
• Routine immunizations
– Influenza (yearly) and pneumococcus vaccine
(every 5-10 years)
- Live virus vaccines not recommended
67. Management – treatment
• Enforce regularly scheduled:
– Colonoscopy
– Pap smears
– Mammograms
– Increased risk of cancer
68. Management – treatment
• Baseline and periodic bone
densitometry
• Biphosphonates – not given to patients
with renal insufficiency or potential to
have pregnancies
• Osteopenic patients: Biphosphonates,
CaCO3 and Vit D
• Management of HTN and Lipid levels
69. Management – treatment
• Pregnancy
– High risk
– 90 % successful
– Flares can occur
– High disease activity with increased
DsDNA level and decreased complement
levels
– Increased pre-eclampsia, preterm births,
and fewer live births
70. Management – treatment
• Risk of Neonatal Lupus in + Ro(anti SSA) AB
• Cross placenta
• 2-5 % risk of congenital heart block in the
baby, hemolytic anemia, and rashes
• Women with medium to high titer
anticardiolipin /anti-B2 glycoprotein, hx of
pregnancy loss or severe preeclampsia –
ASA and Heparin during pregnancy and 6
months after.
71. Management – treatment
• Cutaneous Lupus:
• Hydroxychloroquine (Plaquenil)
– 200 mg PO BID
– Risk for retinopathy (<1 of 5000 exposed)
– Eye exam once a year
• Alternate: Quinacrine 100mg PO QD
– Higher ocular toxicity
• If no response: Cellcept, Methotrexate,or
Imuran
72. Management – treatment
• Musculoskeletal symptoms
– NSAIDS – mild arthralgias
– COX 2 inhibitors
• Do not use for long periods of time
• Proton pump inhibitors- PRN
• Hydroxychloroquine – 200mg PO BID
• Low – Dose Prednisone <7.5mg/day
74. Management – treatment
• Serositis
• Mild serositis: may respond to NSAIDS
• Moderate S: Triamcinolone 100mg IMx1
• Severe : Methylprednisolone IV pulse
(1000mg over 90minutes x 3days ) followed
by oral Prednisone tapering dose
• Maintenance immunosupressive regimen if
persistent / recurrent serositis
75. Management – treatment
• Lupus Nephritis
• Mycophenolate Mofetil (Cellcept)
induction and maintenance therapy
• Recent studies show potential
superiority of Cellcept as induction and
safety profile when compared to
Cytoxan
76. Neprhitis (cont)
• Cyclophosphamide (Cytoxan)
induction therapy- IV pulse
• Induction IV 500-750 mg/m2 body
surface, monthly, for 6 months
• Maintenance IV quaterly for 2 years
• Hemorrhagic Cystitis
• Long term risk for Urinary Bladder
malignancy
77. Management – treatment
• CNS Lupus
• IV Methylprednisolone pulse
• IV monthly Cyclophosphamide
• Additional antiepileptic medication in the
case of seizures/ Neuro-consult
• Psychosis-antipsychotic drugs and
mood stabilizers
78. Management – treatment
• Hematological Lupus
• Plt count < 30,000 bleeding may occur
• Severe hemolytic anemia /
thrombocytopenia
• High dose steroids, if no improvement
intravenous immunoglobulin
• Rituximab
79. Case study
Patient Name: Melisa T.
Age: 19
Sex: Female
Description: Melisa, a young Latina student, is taking
mycophenolate mofetil (MMF) for lupus nephritis
(LN) has come in for a routine follow-up visit. How
would you monitor progress, and, based on the lab
results, are there any changes you would make to
her regimen?
80. • 19-year-old Latina patient with systemic
lupus erythematosus (SLE) is seen today
for a routine follow-up visit. She was
referred 1 year ago with polyarthritis,
thought by her primary care provider to
be due to rheumatoid arthritis (RA). After
confirmation of SLE, she was found to
have 3+ proteinuria, 10 RBCs/HPF, and
class IV diffuse proliferative nephritis
seen on renal biopsy. Treatment with
high-dose prednisone plus
mycophenolate mofetil (MMF; first 2
g/day, then 3 g/day) stabilized her lupus
nephritis (LN): urine protein decreased to
0.3 g/day and revealed no RBCs.
81. • Eventually, prednisone was tapered down to
10 mg.Today she denies significant joint pain
or swelling, rashes, chest pains, skin or
mucous membrane abnormalities, or
neurological symptoms. Past history and
family history are unremarkable. She has
never been pregnant, but is determined to
have children with her fiance in the future. At
present she uses barrier methods (estimated
90% of the time). She is willing to use other
forms of contraception for a period of time,
but despite her present monogamy she
rejects use of an IUD due to prior pelvic
inflammatory disease. Review of systems is
otherwise normal. Physical exam reveals
obesity without evidence of malar or other
rash.
82. • Current Conditions
• Lupus nephritis
• Obesity
• Systemic lupus erythematosus
• Current Medications
• 10 mg prednisone daily
• 1500 mg mycophenolate mofetil bid
• 600 mg-200 units calcium-vitamin D bid
• Daily multivitamin
83. Original Lab results
• Anticardiolipin antibodies: medium
positive titer
• Lupus Anticoagulant: not detected
• Beta2 Glycoprotein I: negative
• Urine hCG Negative
• Urine protein/creatinine ratio =
0.3, which correlates with 300 mg
proteinuria/day.
84. Renal Biopsy
• 24 Apr 07 - Renal biopsy from 13 months ago was consistent with lupus nephritis
Class IV (no crescents noted).
QuickTime™ and a
decompressor
are needed to see this picture.
88. Current lab tests
• A CBC should be ordered several
times a year in patients with SLE
(every 3 months if taking MMF) to
screen for leukopenia,
thrombocytopenia, and/or anemia.
90. • A chemistry panel that checks
fasting glucose, creatinine, lipids,
and hepatic enzymes should be
ordered several times a year
(every 3 months if taking MMF) in
patients with SLE.
92. In anticipation of obtaining a renal
biopsy, coagulation studies are
indicated.
• Prothrombin Time 12 seconds
• 11 - 15
• Partial Thromboplastin Time 29
seconds
• 25 - 40
• International Normalized Ratio 1.0
• 0.8 - 1.2
93. Dyslipidemia is a potential consequence of
proteinuria; LDL-C levels should be checked
regularly. Abnormal lipid levels deserve vigorous
treatment.
• Triglycerides 173 mg/dL
• 0 - 150
• Total Cholesterol 225 mg/dL
• 0 - 200
• LDL Cholesterol 110 mg/dL
• 62 - 130
• HDL Cholesterol 79 mg/dL
• 50 - 60
94. Renal biopsy:
Class IV lupus nephritis with a high level of activity
(crescents)
QuickTime™ and a
decompressor
are needed to see this picture.
96. • A urine protein/creatinine ratio is an efficient
and reasonably accurate method to quantitate
proteinuria. Values > 0.5 (correlating to > 0.5
g (500 mg)/24 h) suggest the need for a renal
biopsy to stage possible lupus nephritis (3659
).
• Urine protein/creatinine ratio = 2.0
97. • Antiphospholipid Antibodies: With her
repeatedly positive anticardiolipin
.
antibody determinations and intrinsic
high risk for thrombotic complications,
continued monitoring is prudent.
• Anticardiolipin antibodies: 80 GPL U/mL
(high positive)
• Lupus anticoagulant: not present
• Beta2 Glycoprotein I: negative
98. Possible options
• Due to the failure of MMF for decreasing
proteinuria to below 500-1,000 mg/day in this
patient, discontinuation of its use and
substitution of rituximab, or the combination
of cyclophosphamide with leuprolide
premedication, or perhaps azathioprine is a
reasonable next step.
99. • Patients with lupus nephritis and this degree
of ongoing pathologic activity require use of
an immunosuppressive agent, such as
cyclophosphamide lyophilized, azathioprine,
tacrolimus, or an experimental therapy such
as rituximab
• Although cyclophosphamide remains the
standard for use in rapidly-progressive
crescentic lupus nephritis, its use is highly
associated with infertility noted in about 50%
of patients using this drug. The incidence of
infertility can be decreased to approximately
15%, however, with leuprolide injections 2
weeks prior to the monthly cyclophosphamide
100. • Some success has been reported in
open-label series using rituximab ,which
peripherally depletes B-lymphocytes
and is approved for use in rheumatoid
arthritis.
• While not FDA-approved for use in
lupus nephritis, the anti-rejection agent
tacrolimus has been associated with
some degree of success in a small
number of patients
101. • Azathioprine has been shown to have
equivalent efficacy to MMF in
maintenance trials, but patients are
unlikely to have a better response to
azathioprine than they had to MMF.
102. • Standard Dosing Ranges
• 1,000-1,500 mg of mycophenolate mofetil 500
mg oral tablet BID maximum, 2,000-3,000 mg
daily maximum
• 1-80 mg of predniSONE 10 mg oral tablet
QID maximum, 1-80 mg daily maximum
• 1-4 ea of calcium-vitamin D 600 mg-200 units
oral tablet QID maximum, 1-4 ea daily
maximum
• 1 ea of Multiple Vitamins oral capsule once a
day maximum