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Flashcards in Systemic Diseases Deck (30):
1

Autoimmune disease which organs and cells undergo damage initially mediated by tissue binding autoAbs and immune complexes

SLE

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SLE most common in

women of childbearing years

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SLE arthritis

migratory arthralgia, early morning stiff, tenosyno, small joint syno

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SLE Rashes

Rash from exposure to UV light that is butterfly (raised/painful/itchy and on cheeks), subacute cutaneous lupus erythematous (migratory, annular), or discoid lupid lesions (hyperkeratosis, follicular plugging --> alopecia)

5

renal involvement in SLE

proliferative glomerulonephritis (tells a lot about prognosis)

6

CV involvement in SLE

pericarditis, hypercoaguability associated with antiphospholipid antibodies

7

Pulm involvement in SLE

pleurisy or pleural effusion, increased risk of TE

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Neuro involvement in SLE

fatigue, headache, poor concentration, hallucinations, chorea, psychosis

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Heme features of SLE

variety or neutro/lympho/thrombo/cytopenias and anemia

10

GI feature of SLE

mouth ulcers, mesenteric vasculitis

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SLE treatment

NO CURE; educate patients, reduce inflammation, suppress immune system (glucocorticoids and immunosupressive agents)

12

Protective measures for SLE patients

suncreen, warm clothing, low dose aspirin, non-live immunizations, psychological support

13

main anti-inflamm agent for SLE and its toxicities

glucocorticoids - obesity, DM, atherosclerosis, osteoporosis, AVN, cataracts, infections

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medication for SLE associated with fatigue, mild arthritis and mucocutaneous manifestations

antimalarial medications (safe for preggo but retinal toxicity)

15

if glucocorticoids aren't effective then can use these two drugs in SLE

azthioprine (side effects: infections, heme malig) and MTX (side effects: infection, liver abnormalities, alopecia, pneumonitis, toxic for pregs)

16

internal organ involvement in SLE is treated with

mycophenolate mofetil (MMF) - inhibitor of purine synthesis of lymphocytes (good for nephritis tx)

17

cyclophosphamide is used to treat SLE with these signs and has these toxicities

SLE with neuro, nephritis, vasculitis, internal organ damge --> reserved for most SEVERE diseases because pancytopenia, alopecia, mucositis, hemorrhagic cystitis (acute) and transitional cell CA, heme maligancy, sterility, premature menopause, infections (chronic)

18

systemic sclerosis is

disorder of connective tissue affecting skin, organs, vasculature with sclerodactyly and Raynaud's
40s and 50s
Females
LCSS (CREST)
DCSS

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components of SSc

fibrosis with overproduction of collagen and other connective tissue matrix proteins, vascular injury and obliteration, immune system activation

20

Skin of SSc

non-pitting edema of fingers, shiny, taut, erythema and tortuous dilatation of capillary loops in nail fold bed, thin lips
restricted to sites distal to elbow/knee LCSS
restricted to sites proximal to elbow/knee DCSS

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GI involvement of SSc

smooth muscle atrophy and fibrosis in the lower 2/3 of the esophagus lead to reflux with erosive esophagitis --> dysphagia and odynophagia, stomach - early satiety/outlet obstruction, GI bleeding, bacterial overgrowth, pseudo obstruction

22

Pulm involvement of SSc

major cause of morbidity and mortality; Pulm HTN (LCSS > DCSS), progressive dyspnea, RHF, angina

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Renal involvement of SSc

hypertensive renal crisis, malignant HTN and renal failure (DCSS > LCSS)

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Labs of scleroderma

ESR+
Raise level of IgG
ANA+ in 70%
30% of DCSS have Ab to topoisomerase 1 Scl-70
60% of LCSS have anticentromere Ab

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Tx of scleroderma

ameliorate effects of diseases on target organs, avoid cold, Ca antags, angiotensin II blockers, epoprostenol for digital ischemia, antibiotics higher dose/longer course for infection, PPI for reflux, ACE-I for HTN, endothelin 1 antag for pulm HTN

26

Sjogren's syndrome is

autoimmune disorder of unknown cause characterized by lymphocytic infiltration of salivary and lachrymal glands --> leading to glandular fibrosis and exocrine failure
females

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Eye symptoms in Sjogren's

keratoconjunctivitis sicca (lack of lubricating tears), conjunctivitis, blepharitis

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Oral involvement in Sjogren's

dry mouth, water needed to swallow food, dental caries

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How to diagnose Sjogren's?

Schimer tear test (tear flow over 5 minutes) or staining with rose Bengal to find epithelial abnormalities. lid biopsy for focal lymphocytic infiltrative of the minor salivary glands, Ab for Anti-Ro and anti-La

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Treatment for Sjogren's is based on

Symptoms
Lachrymal substitues: hypromellose and lubricating ointment at night
Soft contact lenses
Artificial saliva and oral gels for xerostomia
Sugar free chewing gum, lozenges
Tx of oral candidiasis, vaginal dryness