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SLE

Rheumatology

QuestionAnswer
SLE prevalence 90% of all pts = women; occur in all sexes, age / ethnic; US: 15-50/100,000 (highest: AA females)
SLE epi urban > rural; tripled in 40 yrs; M: less photosensitivity, more serositis; Elderly: milder disease
SLE age of onset 20% < 16; 65% btw 16-55; 15% >55
SLE pathophysiology >100 genes; many environ factors; UV light (triggers photosensitivity rashes); drugs (sulfonamides); SMK; infxn (EBV, ?parvo B-19); hormones
SLE pathogenesis: 5 stages 1: Susceptibility 2: innate/adaptive immune responses 3: AutoAbs, immune complexes (complexes trigger complement) in visceral tissue capillaries 4: inflammation 5: tissue damage
SLE genl clinical findings systemic, cardiopulmonary; GI, Neuropsychiatric; derm
SLE: Cardiopulmonary findings: pleuritis/pl effusion / serositis; pericarditis / effusion; interstitial pneumonia; pulmo HTN
SLE: GI findings: hepatitis; HSM; esophageal dysmotility; pancreatitis
SLE: Neuropsychiatric findings: cognitive impairment; HA
SLE: Derm findings: photosensitivity; malar rash; discoid lesions; nonspecific maculopapular lesions; ulcers of oral, genital, nasal mucosa; alopecia
most serious manifestation of SLE = renal disease; may be asx early; leading cause of mortality
SLE classic renal manifestation = nephritis (histo classed as I-VI); 50% pts also develop nephrotic syn; untx’d nephritis can lead to ESRD in 2 years
SLE classic MS presentation polyarthritis similar in distribution to RA; non-erosive, non-deforming; pt sx may be disproportionate to physical findings; also: ischemic necrosis; myalgias
most common SLE clin findings systemic; arthralgias/myalgias; photosensitivity; malar rash; cognitive impairment
Most common Ab’s assoc with SLE ANA (best screening test); dsDNA (hi titer diagnostic); anti-Smith (specific not sensitive); anti-RNP (not spec for SLE); Ro, La. False pos VDRL
Drug-induced lupus = Syndrome of pos ANA assoc w/ constellation of sx (malaise, fever, arthritis +/-myalgias, rash, serositis); autoimmune-mediated response; W > AA; renal/ CNS sx rare; MANY causative drugs
DILE: dx criteria 1 or more SLE clinical sx; pos ANA; no hx SLE before suspected drug; drug taken 3 wks-2 yrs prior to sx; Drug withdrawal led to sx improvement
acute cutaneous lupus = localized or generalized; usu do have systemic dz (3 forms: classic malar rash; genl erythema; bullous dz)
subacute cutaneous lupus = annular or papulosquamous (AKA psoriaform: sl raised)
chronic cutaneous lupus = discoid lupus; host of nonspecific LE lesions
Prevalence of systemic lupus in cutaneous lupus pts Acute: 100% have systemic lupus; Subacute: 50%; Chronic cutaneous: 25%
Discoid lesions: incidence develop in 25% of SLE pts; also may occur in absence of any other clinical manifestations
DLE pt: risk of developing SLE DLE pt 5-10% risk of developing SLE which tends to be mild
Patients with DLE: number of discoid lesions: Increased number of lesions correlates with increased risk (of developing SLE)
DLE common sites face, neck, scalp
SLE tx: Systemic glucocorticoids (mainstay of life-threatening manifestations); major organ involvement guides tx options
SLE: tx: mild-moderate MS sx NSAIDs
SLE: tx: cutaneous & mild-moderate MS sx Anti-malarials (mostly hydroxychloroquinine)
SLE: tx: mod-severe dz (major organ involvemt) or severe/ refractory dz w/o organ involvemt Azathioprine, methotrexate, cyclophosphamide, (CellCept)
Fibromyalgia & SLE 50% of patients with lupus have fibromyalgia
Livedo reticularis seen in: SLE; APS
MCTD Sx: severe Raynaud; swollen, puffy hands, arthritis; nail capillary changes; may have any/ many sx of SLE & scleroderma
MCTD Testing: pos anti-RNP Ab
MCTD Tx like SLE & scleroderma, depending on sx
UCTD Lupus-Light; a few sx like autoimmune, but not enough to fulfill the dx; usu pts won’t progress to lupus
SLE Dx criteria must meet 4 of 11 criteria: malar, discoid, photosensitivity, oral ulcers, arthritis, serositis (ht,lung, peritoneal), renal, ANA, hemo, imm dz (anti-DNA, anti-Sm), neuro
Drugs that precipitate SLE flare procainamide, hydralazine, INH, methyldopa, quinidine, chlorpromazine, cimetidine, HCTZ; penicillin; sulfonamides; terbinafine
SLE pathophysiology >100 genes; many environ factors; UV light; sulfonamides; SMK; EBV, ?parvo B19); hormones
SLE: Cardiopulmonary findings: pleuritis/pl effusion / serositis; pericarditis / effusion; interstitial pneumonia; pulmo HTN
SLE: GI findings: hepatitis; HSM; esophageal dysmotility; pancreatitis
SLE: Derm findings: photosensitivity; malar rash; discoid lesions; nonspecific maculopapular lesions; ulcers of oral, genital, nasal mucosa; alopecia; occur in almost all SLE pts
Mixed Connective Tissue Dz defn overlap syndrome of lupus & scleroderma
Mixed Connective Tissue Dz Sx: severe Raynaud; swollen, puffy hands, arthritis; nail capillary changes; may have any/ many sx of SLE & scleroderma
Collagen vascular dz includes: RA, SLE, Scleroderma, Dermatomyositis, Polyarteritis nodosa
Female with arthralgias, malar rash, +ANA, + anti-double stranded DNA Abs, +anti-phospholipid Abs = SLE
Dusky red, well localized single or multiple papules or plaques usually of face Discoid lupus
SLE tx: Exercise & sun protection; Systemic glucocorticoids (mainstay of life-threatening sxs); major organ involvement guides tx options
SLE: tx: mild-moderate MS sx NSAIDs
SLE: tx: cutaneous & mild-moderate MS sx Anti-malarials (mostly hydroxychloroquinine), c'steroids
SLE: tx: mod-severe dz (major organ involvemt) or severe/ refractory dz w/o organ involvemt Azathioprine, methotrexate, cyclophosphamide, mycophenolate (CellCept)
Created by: Abarnard