Exam 3 Part 1 Flashcards
More commonly referred to as just “Lupus”
Systemic Lupus Erythematosus (SLE)
Classic systemic autoimmune condition that notoriously affects multiple organ systems
Lupus (SLE)
What makes Lupus so hard to diagnose?
Wide-variety of organs involved
What condition is known as the “Great Imitator”
Lupus (SLE)
A condition that involves high amounts of autoantibody production
Lupus (SLE)
Lupus primarily involves injury to the host’s tissues via Type ___ and Type ___ Hypersensitivities
II
III
Antigens that form in response to nuclear components
Anti-Nuclear Antibodies (ANA’s)
_______ test is characteristically associated with autoimmune conditions and is a common lab test that assists in the diagnosis of Lupus
Anti-Nuclear Antibodies (ANA’s)
Like many autoimmune conditions, Lupus is considered
Idiopathic
A minimum of ________ diagnostic criteria must be met to meet the threshold of diagnosing Lupus.
Four
Malar Rash, Discoid Skin Rash, Photosensitivity and Painless Oral Ulcers would be indicative of
Lupus (SLE)
Blood cell disorders, Immunological Antibodies, Serositis, and Renal Disorders would be indicative of
Lupus (SLE)
Lupus is most likely to affect ___________- age ____ ____.
Reproductive
Females
What population is at the highest risk for developing Lupus?
African-American Females
There is a _______ concordance rate of Lupus in monozygotic twins, but a 3% concordance rate in ______________ twins.
25%
Dizygotic
Individuals with HLA-DR2 or HLA-DR3 are at around a ____-fold increased risk for developing risk
Three
A unique environmental exposure that exacerbates the features of lupus is ______________ _______.
Ultraviolet (UV) Light
The increased reactivity of the skin following exposure to sunlight or UV light.
Photosensitivity
Tissues that are commonly affected by lupus are the ________, joints and serosal membranes.
Skin
Tissues that are commonly affected by lupus are the skin, ________ and serosal membranes.
Joints
Tissues that are commonly affected by lupus are the skin, joints and ___________ ______________.
Serosal Membranes
If red blood cells are affected by Lupus the presentation would be
Hemolytic Anemia (Fatigue)
If Hemolytic Anemia (Fatigue) is present with Lupus, what tissue is affected?
Red Blood Cells
If white blood cells are affected by Lupus the presentation would be
Recurrent or Persistent Infections
If Recurrent or Persistent Infections are present with Lupus, what tissues would be affected?
White Blood Cells
If Platelets are affected by Lupus the presentation would be
Bleeding Tendencies
If Bleeding Tendencies are present with Lupus, what tissue would be affected?
Platelets
If the Kidney’s tissues are affected by Lupus the presentation would be
Glomerulonephritis
Proteinuria
Red Blood Cell “Casts”
Renal Failure
If Glomerulonephritis, Proteinuria, RBC “Casts” or Renal Failure are present with Lupus, what tissue would be affected?
Kidney Tissue
If the skin is affected by Lupus the presentation would be
Inflammation (Malar Rash or Discoid Rashes)
If Inflammation (Malar Rash or Discoid Rashes) is present with Lupus, what tissue is affected?
Skin
If the CNS tissue is affected by Lupus the presentation would be
Seizures
Psychosis
Neurological Dysfunction
Stroke
If Seizures, Psychosis, Neuorological Dysfunction and Stroke is present with Lupus, what tissue would be affected?
CNS TIssue
If joint tissues are affected by Lupus the presentation would be
Nonerosive Arthritis (Jaccoud’s Arthropathy)
If Nonerosive Arthritis (Jaccoud’s Arthropathy) is present with Lupus, what tissue is affected?
Joint Tissue
If the pericardium is affected by Lupus, the presentation would be
Pericarditis (Chest Pain)
If Pericarditis (Chest Pain) is present with Lupus, what tissue is affected?
Pericardium
If the Pleura is affected by Lupus, the presentation would be
Pleuritis (Pleurisy)
If Pleuritis (Pleurisy) is present with Lupus, what tissue is affected?
Pleura
If the vessel wall is affected by Lupus, the presentation would be
Vasculitis
If Vasculitis is present with Lupus, what tissue is affected?
Vessel Wall
If the heart tissue is affected by Lupus, the presentation would be
Carditis and Possible Arrhythmia
If Carditis and Possible Arrhythmia are present with Lupus, what tissue is affected?
Heart Tissue
Approximately half of all Lupus patients have clinically significant __________ damage
Renal
Renal damage is the result of __________ ______________ deposition within the glomeruli
Immune Complex
When deposition of immune complex occurs within the glomeruli, an inflammatory reaction is initiated. This inflammatory reaction is known as
Lupus Nephritis (Glomerulonephritis)
Lupus Nephritis (Glomerulonephritis) is a Type ______ Hypersensitivity reaction
III
_________ Failure is the most common cause of death in someone with Lupus.
Renal
Characteristic pattern of cardiac valve damage that is specific to Lupus patients.
Libman-Sacks Endocarditis
Patients with coronary artery involvement commonly present with _________ prior to suffering a heart attack
Angina
Patients with coronary artery involvement commonly present with angina prior to suffering a ________ ________
Heart Attack
An autoimmune condition that is most well-known for producing dry eyes and dry mouth
Sjörgen Syndrome
Dry mouth and dry eyes with Sjörgen Syndrome is the result of CD4+ T Cells infiltrating the ________ and __________ glands.
Lacrimal
Salivary
Dry eyes associated with Sjörgen Syndrome are referred to as
Keratoconjunctivitis Sica
Dry mouth associated with Sjörgen Syndrome are referred to as
Xerostomia
Enlargement of the __________ Gland is common amount Sjörgen Syndrome patients.
Parotid
Up to _______ of Sjörgen Syndrome patients will test positive for Anti-Nuclear Antibodies (ANA’s)
80%
Up to 80% of Sjörgen Syndrome patients will test positive for ______-_______ __________________.
Anti-Nuclear Antibodies (ANA’s)
Sjörgen Syndrome is most likely to affect _________ who are between the ages of 30 and 60 years of age.
Women
Sjörgen Syndrome that occurs as an isolated disorder is known as
Sicca Syndrome
If Sjörgen Syndrome developes after the onset of another autoimmune condition(s), it is known as
Secondary Sjörgen Syndrome
Secondary Sjörgen Syndrome is most likely to develop in patients diagnosed with
Rheumatoid Arthritis
T/F. Sicca Syndrome is more common than Secondary Sjörgen Syndrome
False (Secondary Sjörgen Syndrome is)
Often referred to as “Scleroderma” because of its frequent involvement with the skin.
Systemic Sclerosis
Autoimmune condition that causes chronic inflammation in multiple soft tissues, resulting in fibrosis.
Systemic Sclerosis
Hardening of the involved tissues in Systemic Sclerosis is due to excessive __________ deposition.
Collagen
Abnormal hardening of a body tissue
Sclerosis
Widespread hardening of tissues
Systemic Sclerosis
Once the inflammatory reaction of Systemic Sclerosis begins, ____________ recruit fibroblasts to the site, depositing scar tissue
Cytokines
Patients with Systemic Sclerosis may have _________________ fibroblasts, which contribute to the excessive fibrosis.
Hyperactive
Systemic Sclerosis has a female-to-male ratio of ___-to-____
3 to 1
Systemic Sclerosis occurs in adults who are around age ____-___ years of age
40-60
Women tend to develop Systemic Sclerosis at a[n] ____________ age.
Younger
Men tend to develop Systemic Sclerosis at a[n] ______________ age.
Elderly
Systemic Sclerosis often involves soft tissue fibrosis that damages the body’s __________________.
Vasculature
Most cases (95%) of Systemic Sclerosis are found on the
Skin (Cutaneous Tissues)
Second most common cases (90%) of Systemic Sclerosis are found at the
Alimentary Tract
Defined by its aggressive and severe nature
Diffuse Systemic Sclerosis
Widespread, involving many visceral tissues.
Diffuse Systemic Sclerosis
Patients with _______________ Systemic Sclerosis have a worse prognosis and do not live as long as individuals with ________________ Systemic Sclerosis
Diffuse
Limited
Less severe type of Sclerosis and involves small areas of the skin (often fingers, forearms and face).
Limited Systemic Sclerosis
Small areas of the skin, often confined to the fingers, affected by Limited Systemic Sclerosis is known as
Sclerodactyly
Condition describing a characteristic pattern of tissue involvement in those with limited systemic sclerosis.
CREST Syndrome
The ‘C’ in CREST Syndrome stands for
Calcinosis of the subcutaneous tissues, especially in the hands
The ‘R’ in CREST Syndrome stands for
Reaynaud’s Phenomenon
The ‘E’ in CREST Syndrome stands for
Esophageal Dysmotility (Aperistalsis)
The ‘S’ in CREST Syndrome stands for
Sclerodactyly
The ‘T’ in CREST Syndrome stands for
Telangiectasia (Spider Veins)
Genetically determined immunodeficiency disorder
Primary Immunodeficiency Disorder
Immunodeficiency disorder that is acquired as a consequence of infections, cancer, severe malnutrition, or as a side-effect of chemotherapy, radiation therapy, or immunosuppressive medication therapy.
Secondary Immunodeficiency Disorder
_________ results in a predisposition to developing infections and disease such as cancer, in more severe situations.
Immunodeficiency
Individuals with many forms of immunodeficiency have an increased risk of developing ___________ conditions
Autoimmune
Inherited disorders of the immune system that are frequently detected between 6 months and 2 years of age, due to susceptibility to recurrent infections
Primary Immunodeficiency
X-linked Agammaglobulinemia or XLA
Bruton’s Disease
The mutation for this condition is on the x-chromosome (x-linked), only males are affected.
Bruton’s Disease
Bruton’s Disease becomes apparent after maternal antibodies are depleted around _________ after birth.
6 Months
6 months after birth recurrent __________ and ________ infections become noticibly more frequent and more intense.
Bacterial
Viral
__________ infections frequently involve the respiratory tract and cause sinusitis, bronchitis, pharyngitis, bronchitis, or pneumonia.
Bacterial
Bacterial or viral infections of the middle ear may cause _____ _____
Otitis Media
_________ infections of the gastrointestinal tract (G.I. Tract) may cause abdominal pain, nausea, vomiting or diarrhea
Viral
In patients with Bruton’s disease, the body’s immune deficiency stems from a mutation in the ______ _______ ______ gene
Bruton’s Tyrosine Kinase (BTK)
Bruton’s Tyrosine Kinase (BTK) halts the ___________ of pre-B cells into mature and fully functional B Cells.
Differentiation
When the immune system needs to produce antibodies, __________ ___ cells transition into plasma cells, which produce antibodies.
Functional B
Since patients with Bruton’s disease are unable to produce fully functional mature B cells they are unable to produce antibodies, which results in
Agammaglobulinemia
Absence of gamma globulins (antibodies) in the blood.
Agammaglobulinemia
Since the BTK gene is located on the x chromosome, this condition always affects
Males
The additional x chromosome that _______ possess protection against this and other x-linked conditions
Females
Females may not be affected by Burton’s Disease, however they may still be ________ and pass the mutation to their offspring.
Carriers
Group of different disorders that are grouped together because they manifest in similar ways.
Severe Combined Immunodeficiency (SCID)
What do all patients with SCID have in common?
Impaired development of T cells and or B Cells
A lack of appropriate lymphocyte production is present in all cases of
SCID
______ and ______ of the lymphatic tissues occurs in most forms of SCID
Atrophy
Hypoplasia
SCID is most common among
Native Americans
Within the first year of life, patients with SCID will experience
Intense Oral Thrush (Oral Candadiasis) Severe Diaper Rash Cytomegaloviral Infection of the Upper Respiratory Tract Fungal Infections of the lungs Varicella Zoster Viral Infection
Fungal infection that occurs from any form of the Candida Fungi (yeast)
Candadiasis
