Systemic Diseases Flashcards
Autoimmune disease which organs and cells undergo damage initially mediated by tissue binding autoAbs and immune complexes
SLE
SLE most common in
women of childbearing years
SLE arthritis
migratory arthralgia, early morning stiff, tenosyno, small joint syno
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)
renal involvement in SLE
proliferative glomerulonephritis (tells a lot about prognosis)
CV involvement in SLE
pericarditis, hypercoaguability associated with antiphospholipid antibodies
Pulm involvement in SLE
pleurisy or pleural effusion, increased risk of TE
Neuro involvement in SLE
fatigue, headache, poor concentration, hallucinations, chorea, psychosis
Heme features of SLE
variety or neutro/lympho/thrombo/cytopenias and anemia
GI feature of SLE
mouth ulcers, mesenteric vasculitis
SLE treatment
NO CURE; educate patients, reduce inflammation, suppress immune system (glucocorticoids and immunosupressive agents)
Protective measures for SLE patients
suncreen, warm clothing, low dose aspirin, non-live immunizations, psychological support
main anti-inflamm agent for SLE and its toxicities
glucocorticoids - obesity, DM, atherosclerosis, osteoporosis, AVN, cataracts, infections
medication for SLE associated with fatigue, mild arthritis and mucocutaneous manifestations
antimalarial medications (safe for preggo but retinal toxicity)
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)
internal organ involvement in SLE is treated with
mycophenolate mofetil (MMF) - inhibitor of purine synthesis of lymphocytes (good for nephritis tx)
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)
systemic sclerosis is
disorder of connective tissue affecting skin, organs, vasculature with sclerodactyly and Raynaud's 40s and 50s Females LCSS (CREST) DCSS
components of SSc
fibrosis with overproduction of collagen and other connective tissue matrix proteins, vascular injury and obliteration, immune system activation
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
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
Pulm involvement of SSc
major cause of morbidity and mortality; Pulm HTN (LCSS > DCSS), progressive dyspnea, RHF, angina
Renal involvement of SSc
hypertensive renal crisis, malignant HTN and renal failure (DCSS > LCSS)
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
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
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
Eye symptoms in Sjogren’s
keratoconjunctivitis sicca (lack of lubricating tears), conjunctivitis, blepharitis
Oral involvement in Sjogren’s
dry mouth, water needed to swallow food, dental caries
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
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
