Rheumatology
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| SLE prevalence | 90% of all pts = women; occur in all sexes, age / ethnic; US: 15-50/100,000 (highest: AA females)
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| SLE epi | urban > rural; tripled in 40 yrs; M: less photosensitivity, more serositis; Elderly: milder disease
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| SLE age of onset | 20% < 16; 65% btw 16-55; 15% >55
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| SLE pathophysiology | >100 genes; many environ factors; UV light (triggers photosensitivity rashes); drugs (sulfonamides); SMK; infxn (EBV, ?parvo B-19); hormones
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| 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
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| SLE genl clinical findings | systemic, cardiopulmonary; GI, Neuropsychiatric; derm
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| SLE: Cardiopulmonary findings: | pleuritis/pl effusion / serositis; pericarditis / effusion; interstitial pneumonia; pulmo HTN
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| SLE: GI findings: | hepatitis; HSM; esophageal dysmotility; pancreatitis
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| SLE: Neuropsychiatric findings: | cognitive impairment; HA
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| SLE: Derm findings: | photosensitivity; malar rash; discoid lesions; nonspecific maculopapular lesions; ulcers of oral, genital, nasal mucosa; alopecia
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| most serious manifestation of SLE = | renal disease; may be asx early; leading cause of mortality
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| 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
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| 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
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| most common SLE clin findings | systemic; arthralgias/myalgias; photosensitivity; malar rash; cognitive impairment
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| 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
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| 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
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| 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
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| acute cutaneous lupus = | localized or generalized; usu do have systemic dz (3 forms: classic malar rash; genl erythema; bullous dz)
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| subacute cutaneous lupus = | annular or papulosquamous (AKA psoriaform: sl raised)
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| chronic cutaneous lupus = | discoid lupus; host of nonspecific LE lesions
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| Prevalence of systemic lupus in cutaneous lupus pts | Acute: 100% have systemic lupus; Subacute: 50%; Chronic cutaneous: 25%
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| Discoid lesions: incidence | develop in 25% of SLE pts; also may occur in absence of any other clinical manifestations
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| DLE pt: risk of developing SLE | DLE pt 5-10% risk of developing SLE which tends to be mild
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| Patients with DLE: number of discoid lesions: | Increased number of lesions correlates with increased risk (of developing SLE)
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| DLE common sites | face, neck, scalp
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| SLE tx: | Systemic glucocorticoids (mainstay of life-threatening manifestations); major organ involvement guides tx options
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| SLE: tx: mild-moderate MS sx | NSAIDs
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| SLE: tx: cutaneous & mild-moderate MS sx | Anti-malarials (mostly hydroxychloroquinine)
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| SLE: tx: mod-severe dz (major organ involvemt) or severe/ refractory dz w/o organ involvemt | Azathioprine, methotrexate, cyclophosphamide, (CellCept)
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| Fibromyalgia & SLE | 50% of patients with lupus have fibromyalgia
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| Livedo reticularis seen in: | SLE; APS
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| MCTD Sx: | severe Raynaud; swollen, puffy hands, arthritis; nail capillary changes; may have any/ many sx of SLE & scleroderma
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| MCTD Testing: | pos anti-RNP Ab
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| MCTD Tx | like SLE & scleroderma, depending on sx
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| UCTD | Lupus-Light; a few sx like autoimmune, but not enough to fulfill the dx; usu pts won’t progress to lupus
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| 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
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| Drugs that precipitate SLE flare | procainamide, hydralazine, INH, methyldopa, quinidine, chlorpromazine, cimetidine, HCTZ; penicillin; sulfonamides; terbinafine
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| SLE pathophysiology | >100 genes; many environ factors; UV light; sulfonamides; SMK; EBV, ?parvo B19); hormones
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| SLE: Cardiopulmonary findings: | pleuritis/pl effusion / serositis; pericarditis / effusion; interstitial pneumonia; pulmo HTN
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| SLE: GI findings: | hepatitis; HSM; esophageal dysmotility; pancreatitis
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| SLE: Derm findings: | photosensitivity; malar rash; discoid lesions; nonspecific maculopapular lesions; ulcers of oral, genital, nasal mucosa; alopecia; occur in almost all SLE pts
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| Mixed Connective Tissue Dz defn | overlap syndrome of lupus & scleroderma
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| Mixed Connective Tissue Dz Sx: | severe Raynaud; swollen, puffy hands, arthritis; nail capillary changes; may have any/ many sx of SLE & scleroderma
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| Collagen vascular dz includes: | RA, SLE, Scleroderma, Dermatomyositis, Polyarteritis nodosa
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| Female with arthralgias, malar rash, +ANA, + anti-double stranded DNA Abs, +anti-phospholipid Abs = | SLE
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| Dusky red, well localized single or multiple papules or plaques usually of face | Discoid lupus
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| SLE tx: | Exercise & sun protection; Systemic glucocorticoids (mainstay of life-threatening sxs); major organ involvement guides tx options
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| SLE: tx: mild-moderate MS sx | NSAIDs
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| SLE: tx: cutaneous & mild-moderate MS sx | Anti-malarials (mostly hydroxychloroquinine), c'steroids
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| SLE: tx: mod-severe dz (major organ involvemt) or severe/ refractory dz w/o organ involvemt | Azathioprine, methotrexate, cyclophosphamide, mycophenolate (CellCept)
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