Lupus and Scleroderma Lecture Powerpoint

Question 1

Systemic lupus erythmatosis definition

Answer 1

Chronic relapsing inflammatory multisystem disorder of connective tissue characterized principally by skin, joints, kidneys involvements, thought to represent failure of the regulatory mechs of the immune system leaading to expression of autoantibodies

Question 2

Pathogenesis of SLE

Answer 2

• Autoimmune B lymphocyte hyperactivity
• production of autoantibodies
• formation of immune commplexes tissue deposition and activation of inflammatory cells
• tissue injury

Question 3

SLE classification criteria for SLE

Answer 3

4 criteria that must satisfy at least 4 criteria including one clinical criterion and one immunologic criterion or lupus nephritis proven by biopsy in presence of ANA or dsDNA antibodies

Question 4

ANA is positive in ___% of patients with SLE, so negative argues ___ of diagnosis

Answer 4

99%, against

Question 5

Anti-DNA antibody

Answer 5

Very specific for SLE with few false positive results but not very sensitive as only detects 50% of SLE, increases with activity level of disease

Question 6

Nervous system involvement in lupus (3)

Answer 6

• diffuse manifestations such as headache or psychosis
• seizures
• localized to cranial neuropathies or movement disorders

Question 7

Cardiopulmonary involvement in lupus (6)

Answer 7

• Pericarditis
• abnormal EKG
• myocarditiis
• endocarditis
• acute pneumonitis
• interstitial lung dz

Question 8

Hematologic manifestations of lupus (3)

Answer 8

• anemia
• leukopenia
• antiphospholipid antibodies or anti factor antibodies

Question 9

Management of SLE (6)

Answer 9

• treatment based on organ system involvement
• skin protection for sun exposure
• hydroxychloroquine useful for dermatological and musculoskeletal manifestations
• topical or systemic corticosteroids
• NSAIDS/COX2 agents for musculoskeletal manifestations
• immunosuppressants

Question 10

Scleroderma (systemic sclerosis) definition

Answer 10

Rare rheumatic disease charaacterized by deposition of fibrous connective tissue in the skin and other tissues accompanied by vascular lesions, especially in the skin, lungs, and kidneys. May be systemic or localized with the systemic form being either diffuse or limited

Question 11

90% of patients with scleroderma have antecedent ____

Answer 11

raynaud’s phenomenon

Question 12

Diffuse vs limited scleroderma

Answer 12

Diffuse can involve any part of body, limited is typically found on the forearms down (CREST syndrome)

Question 13

Organs frequently involved in scleroderma (4)

Answer 13

• skin
• GI involvement
• pulmonary
• renal

Question 14

Morphea

Answer 14

Localzed scleroderma without systemic involvement, just one patch of skin

Question 15

GI manifestations of scleroderma (3)

Answer 15

• esophageal hypomotility, reflux
• stomach hypomotlity
• colon wide mouth diverticulum

Question 16

Pulmonary mainfestations of scleroderma (3)

Answer 16

• Leading cause of mortality in scleroderma
• interstitial lung disease 80%
• pulmonary hypertension

Question 17

Musculoskeletal manifestations of scleroderma (3)

Answer 17

• mild symmetric inflammatory polyarthritis
• flexion contractures related to fibrosis
• tendon friction rubs/crepitus

Question 18

Renal manifestations of scleroderma

Answer 18

• mild proteinuria
• scleroderma renal crisis (rapidly progressive malignant hypertension, proteinuria, renal insufficiency, microangiopathic hemolysis)

Question 19

Cardiac manifestations of scleroderma (2)

Answer 19

• Cardiac dz

- pericarditis

Question 20

Another term for systemic sclerosis with limited scleroderma is….

Answer 20

….CREST syndrome

Question 21

Management of scleroderma

Answer 21

-symptomatic treatment (raynauds ca2+ channel blocker, GI PPI, renal improvement with ACE inhibitor)

Question 22

Most common inflammatory myopathy

Answer 22

Polymyositis

Question 23

Clinical presentation of inflammatory myositis (4)

Answer 23

• muscle weakness (loss of physical strength opposed to pain)
• elevated muscle enzymes (CK)
• heliotrope rash (dermatomyositis)
• gottren’s papules

Question 24

Inflammatory myositis treatment options (3)

Answer 24

• corticosteroids
• methotrexate and azathioprine
• monitor muscle strength and enzymes