MSK2-Table 1 Flashcards
What is systemic lupus erythematosus (SLE)?
A multisystem autoimmune connective tissue disease characterized by the presence of numerous autoantigens, autoantibodies, and circulating immune complexes with widespread immunologically determined tissue damage
What pt populations does SLE more frequently affect?
Non-white, AA more susceptible with severe disease, 90% female of child bearing age
When is SLE most frequently diagnosed?
Between 15-45 years old
What causes early death in SLE?
Active lupus and infection
What causes late deaths in SLE?
Sequelae of chronic lupus, chronic medications, CVD, infections
What is the most likely pathogenesis of SLE?
Likely genetic component in the setting of immune dysregulation, environment, and hormonal triggers
What environmental triggers are implied with SLE?
UV lights, microorganisms, tobacco, others
What are the 2 ways SLE autoantibodies cause tissue injury?
1) Formation of immune complexes and depostition of Ig at dermal- epidermal jxn and in renal tissue
2) damage or destruction of specific cells causing inflammation, activated PMNs, and release of oxygen radicals and lysozymes that damage and destroy tissues
What drugs can induce SLE?
Procainamide, hydralazine, isoniazid, methyldopa, quinidine, chlorpromazine, phenothiazines, OCPs, anticonvulsants
What are the clinical features of SLE?
Arthritis, arthralgia, and fever
Specifically polyarthralgia
What is the most common constitutional symptom in SLE?
Fatigue, occurs in >90%
What is the onset of symptoms like?
Gradual onset with flares and remission
What can happen with pregnancy and SLE?
May onset during pregnancy and may be present in women with hx of spontaneous abortion
Babies SGA
What vascular condition is common in SLE?
Raynauds
2/3 of pts with SLE have what?
Skin signs- lupus dermatitis
How might the skin conditions present?
vasculitis, purpura or periungual erythema. Classic malar butterfly rash and discoid lesions
What CNS manifestations present in SE?
Epilepsy, depression, dementia, psychosis, hemiplegia, chorea, ataxia, pripheral neuropathy
What are abdominal manifestations in SLE?
Ab pain, peritonitis, splenomegaly, pancreatitis, GI ulcers
What are ocular manifestations in SLE?
Keratoconjunctivitis, episcleritis, retinal vasculitis & retinal exudates
What is diagnostic criteria for SLE?
4 or more of the following during the course of the disease:
-—Malar Rash —Oral Ulcers
—Arthritis
—Serositis (heart/lungs)
—Renal Disease (proteinuria, cellular casts)
—Hematologic Disorders (hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia)
—Immune Disorders (including false + syphilis)
—Neurological Disorders (sz., psychosis)
—Discoid Rash
—Photosensitivity
—+ANA
what immunologic criteria is most common in lupus?
Positive ANA
How should SLE be managed?
REFER
- NSAIDS, exercise, sun protection, smocking cessation, antimalarials, corticosteroids
What is the mainstay tx for SLE and what needs to be monitored?
Antimalarials - Watch for retinal toxicity
- baseline and annual eye exams with visual field testing
When are corticosteroids mainstay?
If antimalarials are insufficient
What corticosteroid combo is superior for preventing renal failure in SLE?
Prednisone + cyclophosphamide
What should be used to tx SLE in pregnancy?
hydroxychloroquine and prednisone/prednisolone
What comorbid conditions are associated with SLE that need to be assessed for and tx?
- -Accelerated atherosclerosis
- -Pulmonary hypertension
- -Antiphospholipid syndrome
- -Osteopenia or osteoporosis
- -Non-Hodgkin lymphoma
What preventative measures can be taken with SLE?
—Sun protection (clothing and sunscreen)
—Vaccination (influenza and pneumococcal)
—Caution with live-attenuated vaccines and immunosuppression
—Suppress recurrent infections (UTIs)
—Prevent osteoporosis (steroids)
—Prevent and control CVD and obesity