CT Diseases McGowan DSA

Question 1

Systemic Lupus Erythematosus- essentials of dx

Answer 1

• mainly occurs in young women
• rash over areas exposed to sunlight
• joint sx’s in 90% of pts- mult system involvement
• anemia, leukopenia, thrombocytopenia
• glomerulonephritis, CNS dz, complications of antiphospholipid ab’s- major sources of dz morbidity
• serologic findings- antinuclear ab’s (100%), anti-ds DNA ab’s (2/3), low serum complement levels (during dz flares)

Question 2

Criteria for the classification of SLE

Answer 2

(when has 4 or more)

• malar rash
• discoid rash
• photosensitivity
• oral ulcers
• arthritis
• serositis
• kidney- >0.5 g/day proteinuria OR >3+ dipstick proteinuria
• neurologic dz- seizures, psychosis
• hematologic- hemolytic anemia, leukopenia (<4000), lymphopenia (<1500), or thrombocytopenia (<100,000)
• immunologic- ab to DNA, Sm ab, ab’s to antiphospholipid ab’s
• positive ANA

Question 3

SLE- signs and sx’s

Answer 3

• butterfly (malar) rash (50%)
• joint sx (90%)- arthritis
• conjunctivitis, photophobia, blurring of vision
• pleurisy, pleural effusion, bronchopneumonia, pneumonitis
• pericardium involvement

Question 4

SLE- course and prognosis

Answer 4

• 10 yr survival- >85%
• relapsing, remitting course
• prednisone- severe flares
• can have a virulent course- impairment of lungs, heart, brain, kidneys
• mortality- bimodal pattern- infections leading cause of death in early yrs after dx; atherosclerosis major cause of death in later yrs- avoid smoking, reduce risk factors for atherosclerosis

Question 5

SLE- ab’s

Answer 5

• ds-DNA- specific for sLE!
• U1-RNP
• smith ag- specific for SLE!!
• RO (SS-A)/ LA (SS-B) nucleoproteins
• anti-PL (phospholipid-protein complexes)
• mult nuclear ag’s (generic ANAs)

Question 6

Systemic sclerosis- ab’s

Answer 6

• DNA topoisomerase I
• centromeric proteins (CENPs) A, B, C
• RNA polymerase III

Question 7

Sjogren syndrome- ab’s

Answer 7

• Ro/SS-A

- La/SS-B

Question 8

Autoimmune myositis- ab’’s

Answer 8

• histidyl aminoacyl-tRNA synthetase, Jo1
• Mi-2 nuclear ag
• MDA5
• TIFYy nuclear protein

Question 9

RA- ab’s

Answer 9

• CCP- specific

- RF

Question 10

Causes of secondary Raynaud phenomenon

Answer 10

• Rheumatic dz’s- scleroderma, SLE, dermatomyositis, Sjogren syndrome, vasculitis
• Neurovascular compression and occupational
• medications
• hematologic disorders
• endocrine disorders
• miscellaneous

Question 11

Systemic Vasculitides- classification scheme

Answer 11

(size of predominant BVs involved)

• Large-vessel vasculitides- takayasu arteritis, giant cell arteritis, Behcet dz
• Medium-vessel vasculitides- polyarteritis nodosa, Buerger dz, primary angiitis of CNS
• Small-vessel vasculitides- immune complex mediated, cutaneous leukocytoclastic angiitis, HSP, essential cryoglobulinemia, ANCA-assc disorders (granulomatosis w polyangiitis, microscopic polyangiitis, eosinophilic granulomatous w polyangiitis)

Question 12

typical clinical manifestations of large, medium, and small-vessel involvement by vasculitis

Answer 12

constitutional sx’s- fever, wt loss, malaise, arthralgia/arthritis

• Large- limb claudication, asymmetric BPs, absence of pulses, bruits, aortic dilation
• Medium- cutaneous nodules, ulcers, livedo reticularis, digital gangrene, mononeuritis multiplex, microaneurysms
• Small- purpura, vesiculobullous lesions, urticaria, glomerulonephritis, alveolar hemorrhage, cutaneous extravascular necrotizing granulomas, splinter hemorrhages, uveitis, episcleritis, scleritis

Question 13

Granulomatosis with Polyangiitis (Wegener Granulomatosis) - essentials of dx

Answer 13

• classic triad- upper and lower resp tract dz, glomerulonephritis
• suspect if mild resp sx’s (nasal congestion, sinusitis) are refractory to usual tx
• pathology- triad of small-vessel vasculitis, granulomatous infl, and necrosis
• ANCAs (90%)- directed against proteinase-3 (less commonly against myeloperoxidase- severe, active dz)
• kidney dz is rapidly progressive w/o tx

Question 14

Behcet syndrome- essentials of dx

Answer 14

• Asian, Turkish, or Middle Eastern background most commonly
• recurrent, painful aphthous ulcers of mouth and genitals
• erythema nodosum-like lesions; follicular rash; pathergy phenomenon (formation of a sterile pustule at the site of a needle stick)
• ant or posterior uveitis (post uveitis may be asx until significant damage to retina has occurred)
• variety of neurologic lesions that can mimic MS, particularly thru involvement of white matter of the brainstem