Connective Tissue Disorders

Question 1

Chronic inflammatory disorder characterized by diminished lacrimal and salivary gland function

Answer 1

Sjogren’s Syndrome

Question 2

a combination of dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia)

Answer 2

Sicca complex

Question 3

What is the suggested pathogenesis of Sjogren’s?

Answer 3

Lymphocytic infiltration into lacrimal and salivary glands that impairs function

Question 4

What are complications of keratoconjunctivitis sicca [KCS]?

Answer 4

corneal ulceration and infection of the eyelids

Question 5

Complications of xerostomia

Answer 5

dental caries, oral candidiasis, chronic esophagitis, and laryngotracheal reflux

Question 6

result of decreased salivary flow, absence of the normal gastric acid buffer, and reflux of gastric acid into the esophagus and trachea.

Answer 6

laryngotracheal reflux

Question 7

Presence of what antibodies suggests a diagnosis of Sjogren’s?

Answer 7

anti-Ro/SSA and/or anti-La/SSB

Question 8

If presence of antibodies is negative, what is needed for a diagnosis of primary SS?

Answer 8

positive salivary gland biopsy

Question 9

Tests performed by opthalmologists that are recommended when SS is suspected

Answer 9

Schirmer and Rose Bengal tests

Question 10

Treatment options for xerostomia

Answer 10

dried fruit slices, sugar-free candies/gum, or artificial saliva

Question 11

Why should you recommend sugar free candy/gum over sugarless?

Answer 11

Sugarless candies contain fructose which is potentially carcinogenic and should be avoided

Question 12

Used in the treatment of keratoconjunctivitis that replaces the aqueous layer

Answer 12

hypromellose 0.3% or methylcellulose 0.3% drops

Question 13

Used in the treatment of keratoconjunctivitis that help the aqueous layer spread over the hydrophobic corneal and conjunctival epithelium

Answer 13

Spreading agents (polyvinyl alcohol, polyethylene glycol, dextran)

Question 14

Used in the treatment of keratoconjunctivitis in patients without relief from artificial tears and lubricants

Answer 14

Topical Cyclosporine (Restasis)

Question 15

may be a clinical feature affecting only the skin and adjacent tissues OR it may be associated with systemic involvement

Answer 15

scleroderma

Question 16

Name for limited cutaneous sclerosis

Answer 16

CREST syndrome

Question 17

Earliest areas of the body involved with thickening and hardening of scleroderma

Answer 17

fingers, hands, and face

Question 18

Most common initial manifestation of scleroderma. Also associated with SLE

Answer 18

Raynaud’s

Question 19

Pathophysiology of Raynaud’s

Answer 19

abnormal vasoconstriction of digital arteries and cutaneous arterioles in response to cold, stress, temp. changes

Question 20

Pharmacological treatment for Raynaud’s

Answer 20

calcium channel blockers (nifedipine or amlodipine) and low dose ASA

Question 21

What are the pulmonary manifestations of systemic sclerosis?

Answer 21

pulmonary fibrosis and pulmonary vascular disease (pulmonary HTN)

Question 22

What needs to be ordered to assess lung function in patient’s with pulmonary manifestations of systemic sclerosis?

Answer 22

PFTs with diffusion

Question 23

What is the treatment for the renal manifestations of systemic sclerosis?

Answer 23

ACE inhibitors. Avoid diuretics

Question 24

acute onset of oliguric renal failure. seen early in the disease. has been associated with use of moderate-high steroid therapy

Answer 24

scleroderma renal crisis

Question 25

What are the components of CREST syndrome?

Answer 25

calcinosis, Raynaud's, esophageal dysmotility, sclerodactly, telangiectasia

Question 26

Idiopathic inflammatory myopathies characterized by shared features of proximal skeletal muscle weakness and evidence of muscle inflammation

Answer 26

Polymyositis/Dermatomyositis

Question 27

What is the main difference between Polymyositis and Dermatomyositis?

Answer 27

DM has cutaneous eruption and PM doesn't have cutaneous eruption

Question 28

Name for the rash that can be associated with Polymyositis and Dermatomyositis that occurs over PIP and MCP joints

Answer 28

Gottron's sign

Question 29

Name for the rash that can be associated with Polymyositis and Dermatomyositis that occurs over upper anterior chest and around over the shoulders (shawl sign)

Answer 29

erythmatous poikiloderma

Question 30

Describe the muscle weakness associated with Polymyositis and Dermatomyositis

Answer 30

typically symmetric and proximal

Question 31

What happens to the following lab results in a patient with polymyositis/dermatomyositis: CK, LDH, AST, ALT, and ANA?

Answer 31

they're all elevated

Question 32

Cellular infiltrate focused around blood vessels. Abnormal muscle fibers grouped in one portion of the fascicle suggesting microinfarction

Answer 32

pathogenesis of dermatomyositis

Question 33

Cellular infiltrate within the fascicle and abnormal muscle cells scattered throughout the fascicle

Answer 33

pathogenesis of polymyositis

Question 34

Which idiopathic inflammatory myopathy is associated with cancer 90% of the time?

Answer 34

dermatomyositis (DM)