MKSAP 7: SLE

Question 1

Provide a basic definition of SLE

Answer 1

characterized by multiorgan involvement and presence of autoantibodies including those directed at intranuclear antigens; loss of tolerance to self-antigens and active autoimmunity

Question 2

What is the basic pathophysiology of SLE?

Answer 2

Abnormalities in how dying cells are handled by immune system. Nuclear material inadequately cleared, upregulating of autoreactive T and B cells and autoantibodies directed against nuclear and other antigens.

Question 3

About ___% of SLE patients are women

Which ethnicities are more impacted

Answer 3

90%

African American, Chinese and Hispanic

Question 4

Name the 3 types of mucocutaneous involvement in SLE

Answer 4

• acute cutaneous SLE
• subacute cutaneous SLE
• chronic SLE

Question 5

Describe findings in acute cutaneous SLE

Answer 5

malar or butterfly rash, 40-50% of patients.
Erythema/edema over the cheeks and bridge ofn ose and potentially forehead and chin, characteristically spares the nasolabial folds.
Generalized form can involve the dorsum of the arms and hands including areas between the fingers but sparing the knuckle pads

Question 6

Describe subacute cutaneous SLE

Answer 6

Photosensitive rash over the arms, neck and face. Erythematous, annular, or polycyclic lesions often with fine scale. May leave postinflammatory changes but does not cause atrophy

Question 7

Which autoantibodies are present in 70% of patients with SCLE?

Answer 7

Anti-Ro/SSA

Question 8

What is the most common chronic cutaneous manifestation of LE?

Answer 8

Discoid LE; most do not go on to develop SLE; usually affects the scalp, face and presents as hypo - or hyperpigmented erythematous, patches or thin plaques. Can cause scarring, atrophy and permanent alopecia

Question 9

What cutenous finding is a common feature of active SLE?

Answer 9

Nonscarring alopecia with hair regrowth a sign of disease control

Question 10

Arterial vasospasm of the digits that happens in 60% of SLE patients

Answer 10

Raynaud phenomenon

Question 11

Joint involvement occurs in ___% of patients with SLE with inflammatory polyarthralgia the most common presentation. Frank arthritis occurs in 40% of patients with SLE.

Answer 11

90%

Question 12

Persistent periarticular inflammation can damage joints supporting soft tissue structures resulting in reducible subluxation of the digits, swan neck deformities and ulnar deviation, called ____

Answer 12

Jaccoud arthropathy

Question 13

Pain or limitation of motion of large joints such as hips should raise concern for ___

Answer 13

osteonecrosis

Up to 37% of patients with SLE develop it by serial MRI but less than 10% become symptomatic

Question 14

What are risk factors for osteonecrosis in SLE patients?

Answer 14

glucocorticoid use, Raynaud’s, lupus vasculitis

Prednisone > 20mg and cushinoid features

Question 15

Typical feature of the pain in patients with SLE and osteonecrosis?

Answer 15

Night pain and use pain meds

Question 16

Lupus nephritis occurs in up to ___% of patients with SLE and presence of ___ abs is a marker for risk

Answer 16

70%

Anti-dsDNA

Question 17

How should all patients with SLE be evaluated for possible nephritis?

Answer 17

Baseline Scr, urine pro-cr ratio, urinalysis with microscopic evaluation

Question 18

Signs and symptoms defining more severe lupus nephritis include:

Answer 18

HTN, LE edema, active urine sediment (proteinuria, hematuria, cellular casts) and elevated Scr

Question 19

Indications for kidney biopsy are:

Answer 19

increased Scr without explanation, proteinuria ? 1000mg/24h, proteinuria >500mg/24h with hematuria, and proteinuria >500mg/24h with cellular casts

Question 20

SLE patients with hypercoagulable states such as antiphospholipid antibody syndrome or nephrotic syndrome may be at risk for ___

Answer 20

renal artery or vein thrombosis