Nelson IHO Week 5

Question 1

tend to involve blood vessels and connective tissue and are often called collagen vascular disease

Answer 1

systemic autoimmune diseases

Question 2

combination of susceptibility genes coupled with environmental factors that lead to increased burden of nuclear antigens-what ds?

Answer 2

SLE

Question 3

SOAP BRAIN MD in Lupus

Answer 3

serositis, oral ulcers, arthritis, photosensitivity/pulmonary fibrosis, blood cells, renal, Raynauds, ANA, immunologic (anti-Sm, anti-dsDNA), neuropsych, malar rash, discoid rash

Question 4

‘wire loop’ lesions respresenting extensive subendothelial deposits of immune complexes

Answer 4

Lupus Nephritis

Question 5

SLE limited to the skin, chronic photosensitive dermatosis with atrophy and scarring; no systemic manifestations and negative dsDNA

Answer 5

chronic discoid lupus erythematosis

Question 6

Drug Induced Lupus Erythematosus

Answer 6

procainamide and hydralazine: bind histones to become immunogenic

Question 7

How to dx SLE?

Answer 7

ANA then anti dsDNA then anti-Sm (Smith)

Question 8

primarily attacks the joints via production of a nonsuppurative proliferative inflammatory synovitis that often progresses to destruction of the articular cartilage and ankylosis (B and T cell response)

Answer 8

Rheumatoid Arthritis

Question 9

Genes related to RA

Answer 9

HLA-DRB1 and PTPN22

Question 10

Post translational conversion of arginine to citrulline to make citrullinated peptides are produced during inflammation (triggered by smoking and infx)-name the ds?

Answer 10

RA

Question 11

Pannus Formation

Answer 11

Mass of inflamed synovium

Question 12

central fibrinoid necrosis surrounded by palisade of macrophages and scattered chronic inflammatory cells

Answer 12

RA

Question 13

dry eye (keratoconjunctivitis sicca) and dry mouth (xerostomia) from autoimmune, immunologically mediated destruction of lacrimals glands and salivary glands-name the ds

Answer 13

Sjogren Syndrome

Question 14

Pathology: lymphocytic inflammation involving lacrimal and salivary glands that is followed by fibrosis and gland atrophy as the disease develops; can see parotid gland enlargement dt inflmmation

Answer 14

Sjogren Syndrome

Question 15

Antibodies to ribonucleoproteins SS-A and SS-B

Answer 15

Sjogren Syndrome

Question 16

Increased risk of lymphoma (marginal zone)

Answer 16

5% of Sjogren

Question 17

focal lymphoid infiltration in the minor salivary gland; lip biopsy

Answer 17

Sjogren

Question 18

chronic inflammation, widespread dammage to small blood vessels and progressive interstitial and perivascular fibrosis of the skin and multiple organs

Answer 18

Systemic Sclerosis (scleroderma)

Question 19

Diffuse scleroderma

Answer 19

widespread skin involvement at onset with rapid progression and early visceral involvement

Question 20

Limited scleroderma

Answer 20

skin involvement is confined to the fingers, forearms, and face with late visceral involvement (some CREST syndrome)

Question 21

CREST syndrome

Answer 21

calcinosis, Raynaud’s, esophageal dysmotility, sclerodactyly and telagiectasia

Question 22

Pathology: secondary to ischemic damage and fibrosis in the affected organs; antibodies to Scl-70 (DNA topoisomerase 1) and patients with CREST syndrome may have anti-centromere antibodies

Answer 22

Systemic Sclerosis (Scleroderma)

Question 23

Clinical Findings in Scleroderma

Answer 23

Raynaud’s, skin (sclerotic atrophy), GI (esophageal fibrosis, Lungs (interstitial fibrosis, musculoskeletal (non-destructive arthritis), Kidneys (vascular thickening to HTN)

Question 24

Extensive deposition of dense collagen in the dermis

Answer 24

Scleroderma

Question 25

autoimmune ds with immunologic injury and damage to small blood capillaries in the skeletal muscle, along with skin involvement and characteristic skin rash

Answer 25

Dermatomyositis (inflammatory myopathies)

Question 26

classic rash is a violaceious discoloration of upper eyelids associated with periorbital edema; accompanied by a scaling erythematous eruption or dusky red patches over the knuckles, elbows, and knees

Answer 26

Dermatomyositis (inflammatory myopathies)

Question 27

muscle weakness typically affects proximal muscles first and is symmetric (often accompanied by myaligias-muscle pain)

Answer 27

Dermatomyositis (inflammatory myopathies)

Question 28

interstitial lung disease, dysphagia secondary to involvement of oropharyngeal and esophageal muscles and myocarditis; need to test for malignancy

Answer 28

Dermatomyositis (inflammatory myopathies)

Question 29

elevated creatinine kinase

Answer 29

Dermatomyositis (inflammatory myopathies)

Question 30

same as Dermatomyositis (inflammatory myopathies), but without skin involvement

Answer 30

Polymyositis

Question 31

Anti-Jo1 directed against histidyl t-RNA synethase

Answer 31

Polymyositis

Question 32

immunologic injury to muscle by activated CD8+ ctyotoxic T cells; muscle biopsy shows lymphocytic inflammation surrounding and invading muscle fibers; necrotic and regenerating muscle fibers are found throughout the fascicle ; no vascular injury is seen

Answer 32

Polymyositis

Question 33

heliotrope rash (violet colored)

Answer 33

Dermatomyositis

Question 34

6 Mixed Connective Tissue Disease

Answer 34

systemic lupus erythematosis, rheumatoid arthritis, systemic slcerosis (scleroderma), polymyositis, dermatoyositis, secondary sjogren syndrome (Sjogren associated with one of the above)

Question 35

the overlap features and the presence of the distinctive anti-U1-RNP antibody

Answer 35

Mixed Connective Tissue Disease