Connective Tissue Disorders Flashcards
Chronic inflammatory disorder characterized by diminished lacrimal and salivary gland function
Sjogren’s Syndrome
a combination of dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia)
Sicca complex
What is the suggested pathogenesis of Sjogren’s?
Lymphocytic infiltration into lacrimal and salivary glands that impairs function
What are complications of keratoconjunctivitis sicca [KCS]?
corneal ulceration and infection of the eyelids
Complications of xerostomia
dental caries, oral candidiasis, chronic esophagitis, and laryngotracheal reflux
result of decreased salivary flow, absence of the normal gastric acid buffer, and reflux of gastric acid into the esophagus and trachea.
laryngotracheal reflux
Presence of what antibodies suggests a diagnosis of Sjogren’s?
anti-Ro/SSA and/or anti-La/SSB
If presence of antibodies is negative, what is needed for a diagnosis of primary SS?
positive salivary gland biopsy
Tests performed by opthalmologists that are recommended when SS is suspected
Schirmer and Rose Bengal tests
Treatment options for xerostomia
dried fruit slices, sugar-free candies/gum, or artificial saliva
Why should you recommend sugar free candy/gum over sugarless?
Sugarless candies contain fructose which is potentially carcinogenic and should be avoided
Used in the treatment of keratoconjunctivitis that replaces the aqueous layer
hypromellose 0.3% or methylcellulose 0.3% drops
Used in the treatment of keratoconjunctivitis that help the aqueous layer spread over the hydrophobic corneal and conjunctival epithelium
Spreading agents (polyvinyl alcohol, polyethylene glycol, dextran)
Used in the treatment of keratoconjunctivitis in patients without relief from artificial tears and lubricants
Topical Cyclosporine (Restasis)
may be a clinical feature affecting only the skin and adjacent tissues OR it may be associated with systemic involvement
scleroderma
Name for limited cutaneous sclerosis
CREST syndrome
Earliest areas of the body involved with thickening and hardening of scleroderma
fingers, hands, and face
Most common initial manifestation of scleroderma. Also associated with SLE
Raynaud’s
Pathophysiology of Raynaud’s
abnormal vasoconstriction of digital arteries and cutaneous arterioles in response to cold, stress, temp. changes
Pharmacological treatment for Raynaud’s
calcium channel blockers (nifedipine or amlodipine) and low dose ASA
What are the pulmonary manifestations of systemic sclerosis?
pulmonary fibrosis and pulmonary vascular disease (pulmonary HTN)
What needs to be ordered to assess lung function in patient’s with pulmonary manifestations of systemic sclerosis?
PFTs with diffusion
What is the treatment for the renal manifestations of systemic sclerosis?
ACE inhibitors. Avoid diuretics
acute onset of oliguric renal failure. seen early in the disease. has been associated with use of moderate-high steroid therapy
scleroderma renal crisis
What are the components of CREST syndrome?
calcinosis, Raynaud’s, esophageal dysmotility, sclerodactly, telangiectasia
Idiopathic inflammatory myopathies characterized by shared features of proximal skeletal muscle weakness and evidence of muscle inflammation
Polymyositis/Dermatomyositis
What is the main difference between Polymyositis and Dermatomyositis?
DM has cutaneous eruption and PM doesn’t have cutaneous eruption
Name for the rash that can be associated with Polymyositis and Dermatomyositis that occurs over PIP and MCP joints
Gottron’s sign
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)
erythmatous poikiloderma
Describe the muscle weakness associated with Polymyositis and Dermatomyositis
typically symmetric and proximal
What happens to the following lab results in a patient with polymyositis/dermatomyositis: CK, LDH, AST, ALT, and ANA?
they’re all elevated
Cellular infiltrate focused around blood vessels. Abnormal muscle fibers grouped in one portion of the fascicle suggesting microinfarction
pathogenesis of dermatomyositis
Cellular infiltrate within the fascicle and abnormal muscle cells scattered throughout the fascicle
pathogenesis of polymyositis
Which idiopathic inflammatory myopathy is associated with cancer 90% of the time?
dermatomyositis (DM)
