Flashcards in Connective Tissue Disorders Deck (56):
What is lupus?
An auto-immune disorder with connective tissue disease picture
When is the onset of SLE?
20-30 years old, think childbearing age
What population is more likely to have lupus?
What drugs have been known to induce lupus?
What is thought to cause SLE?
Chronic inflammation multi-system disease characterized by a generation of antinuclear antibody production that either directly damage tissue or form an immune complex which deposits resulting in damage
What test is the most sensitive indicator for SLE?
Immunofluorescence test for ANA
What causes the inflammatory response throughout the body?
Immune complexes causes inflammation and tissue injury as they are deposited throughout the body
How is diagnosis confirmed that SLE is present?
Three out of four typical manifestation: ANA, rash, thrombocytopenia, serositis and nephritis
What confirms diagnosis of SLE according to SLICC classifications criteria?
At least four criteria including one clinical and one lab criteria OR biopsy proven lupus nephritis with positive ANA or anti DNA
Why is Lupus an independent risk factor for heart disease?
Increased LDL and valvular disease
What is the most common pulmonary manifestation of SLE?
Why do providers face such difficulty when ventilating patients with Lupus?
Like ventilating restrictive disease
Why should the provider be concerned if a patient with SLE has had a recent episode of pneumonia?
Patients with Lupus do not recover well from pneumonia, if recent illness and elective surgery cancel procedure
What is the most common renal complication from lupus?
Glomerulonephritis with proteinuria and casts
How are the joints affected in SLE?
Mimic arthritis however non erosive disease process
Why are patients with SLE prone to osteoporosis?
Long term steroid use contributes to bone loss
What is the primary treatment for SLE?
Steroids are considered the mainstay of treatment
What are other pharmacologic agents used in treatment of SLE?
What stress does of steroids should be given prior to surgery in patients with SLE?
Minor surgery: 1.5-2x steroid dose morning of surgery
Moderate & Moderate surgery: 2x steroid dose morning of surgery
What factors determine anesthetic management in patients with SLE?
Drug used in treatment
Degree of organ dysfunction
CNS involvement (seizures/stroke)
What airway considerations should be taken into account on induction of patients with SLE?
What anesthetic agents should be avoided in patients with SLE?
Agents that have an active metabolite (renal or liver impairment)
What is Rheumatoid arthritis?
A chronic inflammatory disease characterized by symmetric poly-arthropathy and systemic involvement
When is RA typically diagnosed?
Peak age of occurrence is 20-50y/o
What is believed to cause RA?
Antigen or microbe that leads to an immunological response in a genetically predisposed individual
Where is the target damage in RA?
What can help a provider differentiate RA from osteoarthritis?
Osteoarthritis is usually unilateral compared to symmetric involvement in RA
What causes the destruction to the articular cartilage?
Progressive proliferative synocitis
What are the characteristics of chronic synovitis?
Infiltrated by CD4 and t cells
Increased osteoclast activity leading to bone erosion
Pannus replaced by fibrosis and calcification and permanent ankylosis of joints
What portion of axial insolvent is present in RA?
Upper cervical spine
What lab value may indicate RA and why?
90% of patients will have circulating autoantibody levels of Rheumatoid Factor (RF)
What lab value could also indicate RA but isn't as sensitive?
Anti CCP antibody will be present in 1/3 of patient
What structure acts as a pivot point for the rotation of C1??
What joint allows flexion-extension, side bending and rotational movements of the head?
Zygapophyseal (facet) joints
What organ system is affected by RA that isn't in SLE?
Eye, scleritis and corneal ulceration
What are the treatment goals for RA?
Relieve pain, preserve joint function, prevent deformity and limit systemic complications
What class of drugs are the primary treatment for RA?
Drugs that alter immune response
What additional treatments can be used for RA?
Drugs that treat pain and inflammation (ASA, NSAIDs)
Biological agents (Humira, Embril)
What cardiac pathology us most often seen in patients with RA?
Dysrhythmias resulting from nodules in the cardiac conduction system
What is the most common pulmonary complication from RA?
Pleural effusion and restrictive lung disease due to rheumatoid nodules in the lung tissue
Why are patients with RA a potential for a difficult airway?
Erosion of ligaments by rheumatoid involvement of the bursar around the odontoid process of C2 causes atlanto-axial subluxation
What can occur if care is not taken to avoid neck flexion with DVL?
May push odontoid into foramen magnum which could potentially reduce vertebral artery blood flow
At what level is atlanto-axial stability expected and at what level should FOI be done due to chance of paralysis with flexion?
Greater than 3mm suspect subluxation
Instability greater than 5mm requires FOI with inline stabilization or awake
When should the provider suspect cricoarytenoid arthritis in patients with RA?
If hoarseness, inspiratory stridor, dyspnea or tenderness over the larynx is present
How should the provider process if the patient has cricoarythnoid arthritis?
Potential difficult airway and use smaller ETT
What is Ehler-Danlos syndrome?
Inherited connective tissue disorders caused by abnormal production of procollagen and collagen
What are the three main manifestations of Ehler Danlos syndrome?
Joint hyper mobility and instability
Skin texture anomalies
Vascular and internal organ fragility
How many subtype of Ehler Danlos syndrome are there?
What are the three most common subtypes of Ehler Danlos syndrome?
How are mature collagen cells formed?
Procollagen precursor for hydroxylation and glycosylation to yield mature collagen molecules
What are the major required to diagnosis classic Ehler Danlos syndrome?
• Skin hyper extensibility (excessive skin over eye)
• Widening atrophic scars
• Joint hyper mobility (muscle hypotonia)
What are some of the minor criteria to diagnosis Classic Ehler Danlos syndrome?
• Smooth velvety skin
• Easy bruising
• Muscle hypotonia
What are the major required to diagnosis vascular Ehler Danlos syndrome?
• Thin translucent skin
• Arterial, intestinal, uterine rupture
• Extensive bruising
• Facial features (prominent eyes, thin nose, small lips)
What are some of the minor criteria to diagnosis Vascular Ehler Danlos syndrome?
• Tendon/muscle rupture
• Early varicose veins
• Gingival recession
Which subtype of Ehler Danlos has the worst prognosis?
Vascular due to propensity to rupture arteries