Systemic Diseases

Question 1

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

Answer 1

SLE

Question 2

SLE most common in

Answer 2

women of childbearing years

Question 3

SLE arthritis

Answer 3

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

Question 4

SLE Rashes

Answer 4

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)

Question 5

renal involvement in SLE

Answer 5

proliferative glomerulonephritis (tells a lot about prognosis)

Question 6

CV involvement in SLE

Answer 6

pericarditis, hypercoaguability associated with antiphospholipid antibodies

Question 7

Pulm involvement in SLE

Answer 7

pleurisy or pleural effusion, increased risk of TE

Question 8

Neuro involvement in SLE

Answer 8

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

Question 9

Heme features of SLE

Answer 9

variety or neutro/lympho/thrombo/cytopenias and anemia

Question 10

GI feature of SLE

Answer 10

mouth ulcers, mesenteric vasculitis

Question 11

SLE treatment

Answer 11

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

Question 12

Protective measures for SLE patients

Answer 12

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

Question 13

main anti-inflamm agent for SLE and its toxicities

Answer 13

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

Question 14

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

Answer 14

antimalarial medications (safe for preggo but retinal toxicity)

Question 15

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

Answer 15

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

Question 16

internal organ involvement in SLE is treated with

Answer 16

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

Question 17

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

Answer 17

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)

Question 18

systemic sclerosis is

Answer 18

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

Question 19

components of SSc

Answer 19

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

Question 20

Skin of SSc

Answer 20

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

Question 21

GI involvement of SSc

Answer 21

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

Question 22

Pulm involvement of SSc

Answer 22

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

Question 23

Renal involvement of SSc

Answer 23

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

Question 24

Labs of scleroderma

Answer 24

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

Question 25

Tx of scleroderma

Answer 25

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

Question 26

Sjogren’s syndrome is

Answer 26

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

Question 27

Eye symptoms in Sjogren’s

Answer 27

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

Question 28

Oral involvement in Sjogren’s

Answer 28

dry mouth, water needed to swallow food, dental caries

Question 29

How to diagnose Sjogren’s?

Answer 29

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

Question 30

Treatment for Sjogren’s is based on

Answer 30

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