SLE

Question 1

Types of Hypersensitivity Reactions

Answer 1

I: Immediate (anaphylactic)
II: cytotoxic; target cell receptors- platelet-thrombocytopenia; target fixed tissue antigen- goodpastures, M.G.
III: immune complex; vasculitis, SLE
IV: cell mediated- tuberculin reaction, allograft rejection

Question 2

Describe SLE

Answer 2

Antigen-antibody complexes along vascular & tissue basement membranes

Question 3

Factors Influencing SLE

Answer 3

Genetic
UV light (rash)
Bacterial & viral infections
Drugs
Abnormal stimulation of immune system
Sex hormones

Question 4

Pathogenesis of SLE

Answer 4

B-lymphocyte hyperactivity with exaggerated antibody production
Antigen-antibody complexes deposited along basement membranes of vasculature & tissue
Localized inflammatory responses: complement, neutrophil migration, cell-mediated tissue injury

Question 5

Course of SLE

Answer 5

Onset insidious or acute (rare but devastating)

Characterized by exacerbations & remission

Question 6

Constitutional Manifestations of Systemic Lupus Erythematosus

Answer 6

Fatigue
Fever
Weight loss
Malaise
Anorexia

Question 7

Skin Manifestations of SLE

Answer 7

Butterfly rash (malar rash)

Question 8

Systemic Manifestations of SLE

Answer 8

Polyarthritis
Arthralgias
Joint pain
Alopecia
Fingertip lesions
Periungual erythema
Nail fold infarcts
Splinter hemorrhages
Raynaud's phenomenon

Question 9

Renal Features of SLE

Answer 9

Glomerulonephritis
Nephrotic syndrome: proteinuria, hyaline casts
HTN

Question 10

Ocular Features of SLE

Answer 10

Conjunctivitis
Photophobia
Transient or permanent monocular blindness
Blurred vision
Cotton wool spots on retina

Question 11

Musculoskeletal Features of SLE

Answer 11

Transient polyarthritis with symmetric involvement
Osteonecrosis of hips
Fibromyalgia often present

Question 12

Pulmonary Features of SLE

Answer 12

Transient basilar pneumonic infiltrates
Pleural effusions
Pleuritis
Bronchopneumonia
Restrictive lung disease
Alveolar hemorrhage with massive hemoptysis (rare)
Death (rare)

Question 13

Cardiac Features of SLE

Answer 13

Pericarditis
Myocarditis: tachycardia, ventricular arrhythmia, conduction problems, CHF, cardiomyopathy
Increased muscle enzymes: MB or CPK
Libman-Sacks Endocarditis: vegetation on valves, break off may allow colonization

Question 14

Serositis of SLE

Answer 14

Pleuritis
Pericarditis
Peritonitis

Question 15

Vascular Features of SLE

Answer 15

Raynaud’s phenomenon
Arterial or venous thrombosis
Lupus vasculitis
Livedo reticularis

Question 16

GI Tract Features of SLE

Answer 16

Transient, non-specific abdominal pain
Increased incidenc of primary biliary cirrhosis
Vasculitis of mesentery can cause infarction or perforation of the bowel

Question 17

CNS Features of SLE

Answer 17

COnfusion
Memory deficits
Disorientation
Hypomania
Delirium
Schizophrenia
Grand mal seizures
Temporal lobe seizures
Severe headaches
Stroke: anti-phospholipid antibodies

Question 18

Labs in SLE

Answer 18

CBC
Elevated ESR & CRP
UA
ANA
Anti dsDNA
Anti ssDNA
AntiENA: anti SM & anti RNP
Elevated nonspecific IgG, IgM levels
Reduced complement levels
Decreased IgA levels
False positive for syphilis
Anti-histone

Question 19

CBC Findings in SLE

Answer 19

Normocytic, normochromic, hemolytic anemia
Lymphocytopenia
Thrombocytopenia
Prolonged PTT

Question 20

UA Findings in SLE

Answer 20

Proteinuria
RBCs/WBCs
Cellular or hyaline casts

Question 21

Differential Diagnosis of SLE

Answer 21

Antiphospholipid syndrome
Fibromyalgia
Hepatitis C
Lyme disease
Lymphoma, B-cell
MCTD
Polyarteritis Nodosa
Rheumatic fever
Rheumatoid arthritis
Scleroderma
Serum sickness
TTP

Question 22

Diagnosis of SLE

Answer 22

Clinical

Criteria for classification of SLE

Question 23

Criteria for Classification of SLE

Answer 23

Characteristic rash across cheek
Discoid lesion rash
Photosensitivity
Oral ulcers
Arthritis
Inflammation of membranes in the lungs, heart, or abdomen
Evidence of kidney disease
Evidence of severe neurologic diseaseBlood disorders
Immunologic abnormalities
Positive ANA

Question 24

Physical Findings of SLE

Answer 24

Butterfly rash
HTN
Alopecia
Discoid lesions

Question 25

Lab Findings of SLE

Answer 25

``` ANA dsDNA Anti Sm ribonuclear protein Hypocomplementemia UA: WBC, RBC, proteinuria, hyaline casts ```

Question 26

Goals of Treatment of SLE

Answer 26

Incurable Reducing inflammation Suppressing the immune system Closely following patients clinically to identify disease features early

Question 27

Patient Education & Prevention of Disease Flares

Answer 27

``` Sunscreen & protective clothing Protective, warm clothing Avoidance of vasoconstrictive drugs Psychological support Routine immunization ```

Question 28

Pharmacologic Treatment of SLE

Answer 28

``` Glucocorticoids Antimalarials Immunosuppresants IVIG Plasmapheresis Belimumab: fully human monoclonal antibody ```

Question 29

Drug Induced Lupus Syndrome

Answer 29

Musculoskeletal, pulmonary, & polyserositic symptoms Renal & neurologic disease uncommon No hypocomplementemia ANA present but return to normal with removal of drug Reversal with discontinuation of drug

Question 30

Most Common Culprits of Drug Induced Lupus Syndrome

Answer 30

``` Procainamide Hydralazine Methyldopa Quinidine INH ```

Question 31

Define Discoid Lupus

Answer 31

Chronic cutaneous lupus involving plaque-like lesions of the head, scalp,& neck

Question 32

Differential Diagnosis of Drug Induced Lupus

Answer 32

``` Actinic keratosis Dermatomyositis Keratoacanthomas Granuloma annulare Lichen planus Subacute cutaneous lupus Psoriasis rosacea Sarcoidosis SCC Syphilis Warts ```

Question 33

Goals of Drug Induced Lupus Treatment

Answer 33

Improve patients appearance Control existing lesions & limit scarring Prevent development of further lesions

Question 34

Treatment of Drug Induced Lupus

Answer 34

Sunscreen Topical corticosteroids Antimalarial agents