Lec 04: Autoimmune Diseases: Systemic Flashcards
It refers to the inability of the immune system to discriminate between self and non-self.
Autoimmune diseases
Most autoantibodies possess which type of hypersensitivity
Type III
Familiarize the factors that can contribute to the formation of autoimmune diseases
- Genetic factors
- Presence of HLA
- Age
- Exogenous factors
- Unknown causation
Familiarize findings of an autoimmune disease
Elevated serum levels of gamma globulin
Autoantibodies
Decreased levels of complement proteins
Immune complexes
Lesions in tissues
- Localized lesion
- Non-localized lesion
A. Organ specific
B. Midspectrum
C. Systemic
- A and B
- C
Classifications of Autoimmune Diseases
Organ specific, midspectrum, and systemic
Refers to:
● Localized lesion; Antibody specific to the organ
● Cytotoxic (Type II Hypersensitivity)
● There is a formation of autoantibodies which is deposited to the specific organ which triggers immune response.
Organ-specific
Refers to:
● Non-localized lesion; Antibody non-specific to the organ
● Immune complex (Type III Hypersensitivity)
● Unlike organ specific, there is a formation of immune complex which will be deposited to the different parts of the body leading to systemic autoimmune diseases.
● Examples include SLE, RA, Ankylosing Spondylitis, and Miscellaneous
Systemic
The disorders classified under the organ specific and midspectrum
- Cardiovascular disorders
- Endocrine gland disorders
- Pancreatic disorders
- Gastrointestinal disorders
- Autoimmune hematologic disorders
- Neuromuscular disorders
- Renal disorders
Examples of autoimmune diseases classified uner systemic
- Systemic Lupus Erythematosus
- Rheumatoid Arthritis
- Ankylosing spondylitis
- Miscellaneous systemic disorders such as
A. Progressive Systemic Sclerosis
B. Sjogren’s Syndrome
Refers to non-localized; lesion is not speciific to the organ
Its mechanism is the production of immune complex which will be distributed to the different parts of the body, triggering immune response and inflammatory reactions
Systemic
Refers to:
● Disease of the connective tissues; expresses as vasculitis
● The whole body is affected
Systemic Lupus Erythematosus
Most frequent manifestation of SLE
Arthritis and Polyarthralgia
Environmental factors of SLE
● UV light/sunlight
● Drugs (Procainamide, Hydralazine, Isoniazid)
● Infectious Agents
● Hormonal changes
Genes associated with SLE susceptivility
HLA-A1, HLA-B8, HLA-DR3
Refers to the name of the rash that manifests in SLE upon exposure to UV lights
Erythematous rash / Butterfly rash / Malar rash
What causes the end-stage renal disease in SLE?
Glomerulonephritis
Aside from infection, what is the most common death of SLE patients?
Cardiac disease
In SLE, immune complexes may be possibly deposited to the heart leading to cardiac involvement, ________, ________, ________
Pericarditis, tachycardia, ventricular enlargement
Refers to the collective name for seizure, mild cognitive dysfunction, psychoses, depression
Neuropsychiatric manifestations
Formation of immune complexes is part of which type of hypersensitivity?
3
Refers to polymorphonuclear neutrophils with ingested body
LE cells
Refers to the antibody-coated nucleus of another neutrophil
LE body
Le factor reacts with what?
DNP and complement
Formed when the anti-nuclear antibodies bind to the nucleus of degraded neutrophils
LE body
LE cells are often in _____ formation
Rosette
T or F: Patients with SLE has Hypocomplemetemia and has C1q, C2, and C3 deficiency
False (C1, C2, and C4)
T or F: There is hypogammaglobulinemia in SLE because there is a depletion of suppressor T cells hence no activation of B cells to plasma cells
False (hypergammaglobulinemia) if nag-true ka uto-uto ka same kayo ng mga boses ko sa utak
Hallmark of SLE but not diagnostic
Anti-nuclear Antibodies
Refers to the antibodies to nucleus and nuclear components
ANA
Match
- Most specific marker for SLE
- Measurement using Crithidia luciliae as
substrate is confirmatory for SLE - Reactive with denatured DNA that reacts with
purine and pyrimidine bases of DNA. - Associated with SLE, systemic sclerosis,
localized scleroderma, and other connective
tissues disease - Associated with SLE in the active stage,
lupus nephritis - SLE may have glomerulonephritis leading to
end stage renal disease.
A. Anti-dsDNA
B. Anti-ssDNA
- A
- A
- B
- B
- A
- A
Refers to:
● Not specific for SLE and may also be found in patients with RA, Felty’s syndrome, Sjogren’s syndrome, systemic sclerosis, and primary biliary cirrhosis but in lower levels.
* Induced by drugs such as procainamide and hydralazine
Antihistone
Familiarize the antihistones
● Anti-H1
● Anti-H2A
● Anti-H2B
● Anti-H3
● Anti-H4
Which drugs can induce antihistones?
Procainamide and hydralazine
Match
- Highly specific for patients with
SLE - May also be seen in patients with
Mixed Connective Tissue Disease (MCTD), Rheumatoid Arthritis (RA), Scleroderma,
Sjogren syndrome - LE factor: an antibody that reacts with DNP
- Seen in patients with serositis, and again, not specific for SLE
- Seen in px with SLE dermatitis (Cutaneous) and Neonatal SLE
- Seen in px with SLE and Sjogren’s
A. Anti-Smith
B. Anti-DNP
C. Anti-RNP
D. Anti-SS-A/RO
E. Anti-SS-BLA
- A
- C
- B
- B
- D
- E
Diagnostic of SLE
High ANA and high anti-dsDNA
Refers to
- Antibodies to nucleolar RNA (Anti-RNA)
- Associated with SLE, Scleroderma, Sjogren’s, and Raynaud’s Phenomenon
Anti-nucleolar
What is highly sensitive for CREST variant of scleroderma (CREST syndrome)?
Calcinosis cutis
Raynaud’s Phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia
Refers to:
● This antibody is a secondary to systemic lupus erythematosus
● Not specific for SLE
● It is classified as primary and secondary. SLE is secondary.
Anti-Phospholipid Antibody
When there is anti-phospholipid antibody in SLE, which is classified as primary and secondary?
Primary - antiphospholipid antibody syndrome
Secondary - SLE
The problem here is that inside the body if there is a presence of antiphospholipid antibody, it can activate platelets adhesion and activation leading to ______. This will also prolong the _____ result of the patient.
Thrombosis, APTT
Antibody found in SLE that refers to:
- Formation of immune complex
- This can lead to hemolytic diseases, lysing the red cells.
Antibody to RBCs
Refers to:
● It is also present in SLE.
● It is not specific for rheumatoid arthritis. It is also present in other autoimmune diseases like SLE.
Rheumatoid factor
Refers to:
- Chronic systemic disease:
○ Synovial membranes and articular structures of multiple joints.
○ Multiple organs such as heart and lungs
Rheumatoid arthritis
T or F: The cause of Rheumatoid Arthritis is unknown but it is believed to be genetically inherited
True
Match
- Elevated level of uric acid
- An autoimmune disease
- As the uric acid increases, it can be
deposited in the joints, - There is an immune complex formation
that gets deposited to the different parts of the body
A. Rheumatoid Arthritis
B. Gouty arthritis
- B
- A
- B
- A
Genes associated with RA susceptibility
HLA-D4, HLA-DRB1
Familiarize the environmental and lifestyle factors of RA
Hormonal changes; Smoking and Obesity
Familiarize the infectious agents that can possibly trigger antigens for RA
○ Proteus mirabilis
○ Mycoplasma organisms
○ Epstein-Barr Virus (EBV)
○ Rubella Virus
○ Porphyromonas gingivalis
In SLE, pannus tissue adheres to articular cartilage, and the cells within the pannus produce _______ that can destroy cartilage
Proteinases
Refer to:
- Anti-Antibody
- Immunoglobulins that interact specifically with the Fc portion of human or animal IgG
- IgG, IgA, but predominantly IgM
Rheumatoid Factor
T or F: RF is an antibody
True (its an antibody against antibody)
Refers to the specific marker for RA
Anti-cyclic citrullinated proteins
This can be detected in the sera from individuals 14 years before the first onset of symptoms of RA appear
Anti-CCP
Refers to:
- Form of chronic inflammation of the spine and sacroiliac joints
- Autoimmune disease with the highest criteria for HL disease relationship
Ankylosing spondylitis
Gene associated with Ankylosing Spondylitis
HLA-B27
Two examples of miscellaneous systemic Disorders
a. Progressive Systemic Sclerosis (Scleroderma)
b, Sjogren’s Syndromee
Refers to Chronic Connective Tissue Disorder characterized by diffuse fibrosis
Progressive Systemic Sclerosis (Scleroderma)
Antibodies present in Scleroderma
Anti-RNA and Anti-RNP
Refers to:
- Affects the lacrimal, salivary, and other excretory glands
- Results in keratoconjunctivitis sicca and xerostomia
Sjogren’s Syndrome
T or F: In Sjogren’s Syndrome, all ANA are present
False (with exceptions of Anto-centromere and Anti-smith)
Pattern in immunofluorescence assay of Sjogren’s Syndrome
Speckled pattern