SLE

Question 1

Lab diagnostic testing

Answer 1

Anti-nuclear antibody (ANA)- Pos in 95% of patients. Anti-dsDNA, Anti- Sm antibodies, low C3 and C4

Question 2

TX for SLE

Answer 2

nsaids for pain relief, antimalarials- skin and MS issues, glucocorticoids for significant organ involvement, immunosuppressives last line

Question 3

FDA approved drug for lupus as additive therapy

Answer 3

belimumab

Question 4

what patient population has to be monitored closely in lupus?

Answer 4

PREGNANT patients- avoid during active disease.

Question 5

pregnant patients with lupus

Answer 5

glucocorticoids

Question 6

lupus can affect all organ systems, but characteristic finding in most patients-

Answer 6

fatigue, fever, arthritis, butterfly rash lesion

Question 7

scleroderma disorders

Answer 7

autoimmune CT disorder causing skin THICKENING d/t overproduction and accummulation of collagen in skin

Question 8

patient population more common in lupus and scleroderma disorders

Answer 8

women. scleroderma- 40’s

Question 9

localized scleroderma includes with PE findings

Answer 9

morphea and linear scleroderma

Question 10

patient with discolored oval shaped thickened patches of skin. Patches remit spontaneously

Answer 10

MORPHEA- scleroderma manifestation

Question 11

child with dermatomal, unilateral bands or streaks of hardened skin.

Answer 11

linear scleroderma

Question 12

en coup de sabre

Answer 12

frontal linear scleroderma- presents on frontal SCALP- distortion of skull

Question 13

what classification of scleroderma has skin sclerosis restricted to hands, face, and neck and a/w CREST syndrome

Answer 13

limited cutaneous systemic sclerosis

Question 14

vascular manifestations in scleroderma

Answer 14

telangectasias, raynaud’s, “watermelon stomache”- gastric antral vascular ectasia

Question 15

GERD and SKIN changes (thickening)- think

Answer 15

scleroderma

Question 16

dx of scleroderma disorders

Answer 16

skin biopsy if doubt, cliniccal evaluation for extracutaneous disease, lab screening to confirm diagnosis- positive ANA, sed rate elevated, RF may be elevated

Question 17

what are most freq causes of mortality from scleroderma

Answer 17

pulmonary HTN, interstitial lung dz, and scleroderma renal crisis

Question 18

what scleroderma types a/w premature death

Answer 18

diffuse and limited cutaneous systemic sclerosis

Question 19

tx in scleroderma

Answer 19

ORGAN-BASED. derm- CCB for calcinosis, adequate lubrication, minimize contact with water, renal- ACE-I, empiric use of PPI for GI, glucocorticoids for MS and lung issues

Question 20

scloeroderma patients have increased risk of what cancer

Answer 20

lung

Question 21

survival avg of scleroderma patients

Answer 21

12 years from diagnosis