Muscu - Connective Tissue - Autoimmune Flashcards
1
Q
System Lupus Erythematosus
A
- Systemic Lupus Erythematosus is a chronic multisystem disease of autoimmune origin. The damage to body tissues is mediated through numerous autoantibodies and immune complexes.
- SLE, Lupus
2
Q
System Lupus Erythematosus - Epidemiology
A
- Becasue of variable reporting, the overall prevalence ranges from 15-50 per 100,000 in the US
- Incidence varies from 1.8-7.6/100,000 per year
- Female to male ratio is 9:1
- Can be as high as 1 in 700 women of child bearing age; and 1 in 245 for african-american women
3
Q
System Lupus Erythematosus - Etiology
A
- Failure of a mechanism of self-tolerance in the immune system
- Principal organs attacked include:
- skin
- joints
- kidney
- serosal membranes
- Many other organs are affected
- Numerous antibodies detectable in SLE patients
- antinuclear antibodies
- Anti-dsDNA
- Anti-Sm
- Anti- RNP
- Anti - Ro (SS-A)
- Anti - La (SS-B)
- Antihistones
- ANtiphospholipids
- Antierythrocyte
- Antiplatlet
- Antineuronal
- Antiribosomal
- Numerous HLA DR/DQ genes increase susceptibility to SLE
- Infectious agents could be triggers for the hyperactivity in the immune system; SLE patients tend to have elevated titers to Epstein-Barr Virus
- Lupus can also be drug-induced, such drugs as:
- hydralazine
- Procainamide
- Isoniazid
- D-penicillamine
4
Q
System Lupus Erythematosus - Presentation
A
- Acute or insidious in onset; chronic, remitting and relapsing in temporal profile
- Often presents as a butterfly rash in young women accompanied by fever, joint pain, pleuritis pain, and photosensitivity
- Other clinical problems as protean but neurologic, renal and cardiac are common
5
Q
System Lupus Erythematosus - DIagnostic Testing
A
- Immunofluorescence testing:
- ANA antibodies
- Double stranded DNA antibodies
- Smith antibodies Sm, SS-A, and SS-B
- ACR Criteria for diagnosis (4 or more needed)
- Malar rash
- Discoid rash
- Photosensitivity
- Oral ulcers
- Arthritis (intermittent polyarthritis)
- Serosisis (pleuritis, pericarditis)
- Renal disorders (proteinuria)
- Neurologic Disorders (seizures or psychosis)
- Hematologic disorder (hemolytic anemia, leukopenia)
- Immunologic disorder (Anti-ds DNA, snit-Sm, antiphospholipid_
- Antinuclear antibody
6
Q
System Lupus Erythematosus - Treatment
A
- Non-life threatening presentations
- suppression of symptoms using analgesics and antimalarials
- Life-threatening presentations
- systemic glucocorticoids
- Cytotoxic drugs
7
Q
System Lupus Erythematosus - Management with Complications
A
- Rare acute cases can progress to death within weeks
- Typically the temporal profile of SLE is one of repeated flare-ups and remissions
- Most common causes of death are renal failure, and intercurrent infections
- Pregnancy and Lupus
- increase in fetal loss in lupus patients
- glucocorticoid use recommended for suppression of disease activity during pregnancy
8
Q
System Lupus Erythematosus - Prevention and Health Promotion
A
- Appropriate vaccines
- Strategies to control osteoporosis in patients on long term glucocorticoids
- Strategies to control hypertension and athrosclerosis
9
Q
Erythema Nodosum
A
- Panniculitis is an inflammatory reaction in the subcutaneous fat and fascia. Erythema nodosum (EN), the most common form of panniculitis, is an acute onset of a tender, nodular, erythematous eruption that usually is limited to the extensor aspects of the lower legs
10
Q
Erythema Nodosum - History
A
- Very rare, incidence is 0.02 - 0.03 cases /1000 patients/year.
- Associated with infections such as:
- Beta-hemolytic streptococcal infections
- Tuberculosis infections
- Associated with drug use such as:
- Sulfonamides
- Oral contraceptives
- Also associated with:
- Sarcoidosis
- Inflammatory bowel disease
- Lesions are erythematous and edematous with nuetrophilic infiltration
11
Q
Erythema Nodosum - Presentation
A
- Presents as poorly defined but very tender erythematous plaques and nodules, typically in the lower extremity
12
Q
Erythema Nodosum - Diagnostic Testing
A
- Biopsy
13
Q
Systemic Sclerosis
A
- Systemic sclerosis is a rare, multiisystem, chronic disorder of unknown cause (most likely autoimmune in nature), characterized by excessive tissue fibrosis, small blood vessel vasculopathy and autoimmunity. Tissue thickening is especially prominent in the dermis resulting in thickening skin.
AKA - Scleroderma, Progressive systemic sclerosis
14
Q
Systemic Sclerosis - Epidemiology
A
- Systemic sclerosis is a rare disease
- Systemic sclerosis is diagnosed in approx. 67 males and 265 females per 100,000 people each year.
- Female predominance is 3:1
- Peak incidence 50-60 years old
15
Q
Systemic Sclerosis - Etiology
A
- Unknown triggering agent
- Abnormal immune response from CD+4 T-cells and TH2
- Abnormal secretion of growth factor cytokines
- T cell mediated fibrogenesis and vascular injury
- Abnormal humoral response as well, ANAs are detectable in most patients
- Skin is most commonly effected but other organs system commonly involved include: GI, renal, heart, skeletal, muscle and lungs
- Microvascular disease is present early on in the disease process
- initial event in the disease
- involves endothelial cell damage and the release of platlet factors that can stimulate periadventital fibrosis.
- Leads to a state favoring vasoconstriction and ischemia in the tissue
- Genetic component involving the HLA class II genes
- Systemic sclerosis can also be chemically induced
